Topic: Congenital

Abstract

Improved Outcomes of Heart Transplantation in Adults With Congenital Heart Disease Receiving Regionalized Care.

Nguyen VP, Dolgner SJ, Dardas TF, Verrier ED, McMullan DM, Krieger EV
Background
The number of adult congenital heart disease (CHD) patients undergoing heart transplantation is increasing rapidly. CHD patients have higher surgical risk at transplantation. High-volume adult CHD transplant centers may have better transplant outcomes.
Objectives
This study aimed to evaluate the effect of center CHD transplant volume and expertise on transplant outcomes in CHD patients.
Methods
The authors studied heart transplantations in CHD patients age ≥18 years using the United Network of Organ Sharing (UNOS) database for the primary outcomes of waitlist mortality and post-transplant outcomes at 30 days and 1 year. Transplant centers were assessed by status as the highest CHD transplant volume center in a UNOS region versus all others, presence of Adult Congenital Heart Association accreditation, and adult versus pediatric hospital designation.
Results
Between January of 2000 and June of 2018, 1,746 adult CHD patients were listed for transplant; 1,006 (57.6%) of these underwent heart transplantation. After adjusting for age, sex, listing status, and inotrope requirement, waitlist mortality risk was lower at Adult Congenital Heart Association accredited centers (hazard ratio: 0.730; p = 0.020). Post-transplant 30-day mortality was lower at the highest volume CHD transplant center in each UNOS region (hazard ratio: 0.706; p = 0.014).
Conclusions
Designated expertise in CHD care is associated with improved waitlist outcomes for CHD patients listed for transplantation. Post-transplant survival was improved at the highest volume regional center. These findings suggest a possible advantage of regionalization of CHD transplantation.

Copyright © 2019 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

J Am Coll Cardiol: 09 Dec 2019; 74:2908-2918
Nguyen VP, Dolgner SJ, Dardas TF, Verrier ED, McMullan DM, Krieger EV
J Am Coll Cardiol: 09 Dec 2019; 74:2908-2918 | PMID: 31806135
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Abstract

Stem Cell-Derived Cardiomyocytes and Beta-Adrenergic Receptor Blockade in Duchenne Muscular Dystrophy Cardiomyopathy.

Kamdar F, Das S, Gong W, Klaassen Kamdar A, ... Zhang J, Garry DJ
Background
Although cardiomyopathy has emerged as a leading cause of death in Duchenne muscular dystrophy (DMD), limited studies and therapies have emerged for dystrophic heart failure.
Objectives
The purpose of this study was to model DMD cardiomyopathy using DMD patient-specific human induced pluripotent stem cell (hiPSC)-derived cardiomyocytes and to identify physiological changes and future drug therapies.
Methods
To explore and define therapies for DMD cardiomyopathy, the authors used DMD patient-specific hiPSC-derived cardiomyocytes to examine the physiological response to adrenergic agonists and β-blocker treatment. The authors further examined these agents in vivo using wild-type and mdx mouse models.
Results
At baseline and following adrenergic stimulation, DMD hiPSC-derived cardiomyocytes had a significant increase in arrhythmic calcium traces compared to isogenic controls. Furthermore, these arrhythmias were significantly decreased with propranolol treatment. Using telemetry monitoring, the authors observed that mdx mice, which lack dystrophin, had an arrhythmic death when stimulated with isoproterenol; the lethal arrhythmias were rescued, in part, by propranolol pre-treatment. Using single-cell and bulk RNA sequencing (RNA-seq), the authors compared DMD and control hiPSC-derived cardiomyocytes, mdx mice, and control mice (in the presence or absence of propranolol and isoproterenol) and defined pathways that were perturbed under baseline conditions and pathways that were normalized after propranolol treatment in the mdx model. The authors also undertook transcriptome analysis of human DMD left ventricle samples and found that DMD hiPSC-derived cardiomyocytes have dysregulated pathways similar to the human DMD heart. The authors further determined that relatively few patients with DMD see a cardiovascular specialist or receive β-blocker therapy.
Conclusions
The results highlight mechanisms and therapeutic interventions from human to animal and back to human in the dystrophic heart. These results may serve as a prelude for an adequately powered clinical study that examines the impact of β-blocker therapy in patients with dystrophinopathies.

Copyright © 2020 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

J Am Coll Cardiol: 16 Mar 2020; 75:1159-1174
Kamdar F, Das S, Gong W, Klaassen Kamdar A, ... Zhang J, Garry DJ
J Am Coll Cardiol: 16 Mar 2020; 75:1159-1174 | PMID: 32164890
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Abstract

Tricuspid Intervention Following Pulmonary Valve Replacement in Adults With Congenital Heart Disease.

Deshaies C, Trottier H, Khairy P, Al-Aklabi M, ... Poirier NC,
Background
Tricuspid regurgitation (TR) is common among adults with corrected tetralogy of Fallot (TOF) or pulmonary stenosis (PS) referred for pulmonary valve replacement (PVR). Yet, combined valve surgery remains controversial.
Objectives
This study sought to evaluate the impact of concomitant tricuspid valve intervention (TVI) on post-operative TR, length of hospital stay, and on a composite endpoint consisting of 7 early adverse events (death, reintervention, cardiac electronic device implantation, infection, thromboembolic event, hemodialysis, and readmission).
Methods
The national Canadian cohort enrolled 542 patients with TOF or PS and mild to severe TR who underwent isolated PVR (66.8%) or PVR+TVI (33.2%). Outcomes were abstracted from charts and compared between groups using multivariable logistic and negative binomial regression.
Results
Median age at reintervention was 35.3 years. Regardless of surgery type, TR decreased by at least 1 echocardiographic grade in 35.4%, 66.9%, and 92.8% of patients with pre-operative mild, moderate, and severe insufficiency. In multivariable analyses, PVR+TVI was associated with an additional 2.3-fold reduction in TR grade (odds ratio [OR]: 0.44; 95% confidence interval [CI]: 0.25 to 0.77) without an increase in early adverse events (OR: 0.85; 95% CI: 0.46 to 1.57) or hospitalization time (incidence rate ratio: 1.17; 95% CI: 0.93 to 1.46). Pre-operative TR severity and presence of transvalvular leads independently predicted post-operative TR. In contrast, early adverse events were strongly associated with atrial tachyarrhythmia, extracardiac arteriopathy, and a high body mass index.
Conclusions
In patients with TOF or PS and significant TR, concomitant TVI is safe and results in better early tricuspid valve competence than isolated PVR.

Copyright © 2020 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

J Am Coll Cardiol: 09 Mar 2020; 75:1033-1043
Deshaies C, Trottier H, Khairy P, Al-Aklabi M, ... Poirier NC,
J Am Coll Cardiol: 09 Mar 2020; 75:1033-1043 | PMID: 32138963
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Abstract

Inherited Thoracic Aortic Disease: New Insights and Translational Targets.

Fletcher AJ, Syed MBJ, Aitman TJ, Newby DE, Walker NL

Inherited thoracic aortopathies denote a group of congenital conditions that predispose to disease of the thoracic aorta. Aortic wall weakness and abnormal aortic hemodynamic profiles predispose these patients to dilatation of the thoracic aorta, which is generally silent but can precipitate aortic dissection or rupture with devastating and often fatal consequences. Current strategies to assess the future risk of aortic dissection or rupture are based primarily on monitoring aortic diameter. However, diameter alone is a poor predictor of risk, with many patients experiencing dissection or rupture below current intervention thresholds. Developing tools that improve the risk assessment of those with aortopathy is internationally regarded as a research priority. A robust understanding of the molecular pathways that lead to aortic wall weakness is required to identify biomarkers and therapeutic targets that could improve patient management. Here, we summarize the current understanding of the genetically determined mechanisms underlying inherited aortopathies and critically appraise the available blood biomarkers, imaging techniques, and therapeutic targets that have shown promise for improving the management of patients with these important and potentially fatal conditions.



Circulation: 11 May 2020; 141:1570-1587
Fletcher AJ, Syed MBJ, Aitman TJ, Newby DE, Walker NL
Circulation: 11 May 2020; 141:1570-1587 | PMID: 32392100
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Abstract

Oral fluconazole use in the first trimester and risk of congenital malformations: population based cohort study.

Zhu Y, Bateman BT, Gray KJ, Hernandez-Diaz S, ... Straub L, Huybrechts KF
Objective
To examine the risk of congenital malformations associated with exposure to oral fluconazole at commonly used doses in the first trimester of pregnancy for the treatment of vulvovaginal candidiasis.
Design
Population based cohort study.
Setting
A cohort of pregnancies publicly insured in the United States, with data from the nationwide Medicaid Analytic eXtract 2000-14.
Participants
Pregnancies of women enrolled in Medicaid from three or more months before the last menstrual period to one month after delivery, and infants enrolled for three or more months after birth.
Interventions
Use of fluconazole and topical azoles was established by requiring one or more prescriptions during the first trimester of pregnancy.
Main outcome measures
Risk of musculoskeletal malformations, conotruncal malformations, and oral clefts (primary outcomes), associated with exposure to oral fluconazole, diagnosed during the first 90 days after delivery, were examined.
Results
The study cohort of 1 969 954 pregnancies included 37 650 (1.9%) pregnancies exposed to oral fluconazole and 82 090 (4.2%) pregnancies exposed to topical azoles during the first trimester. The risk of musculoskeletal malformations was 52.1 (95% confidence interval 44.8 to 59.3) per 10 000 pregnancies exposed to fluconazole versus 37.3 (33.1 to 41.4) per 10 000 pregnancies exposed to topical azoles. The risks of conotruncal malformations were 9.6 (6.4 to 12.7) versus 8.3 (6.3 to 10.3) per 10 000 pregnancies exposed to fluconazole and topical azoles, respectively; risks of oral clefts were 9.3 (6.2 to 12.4) versus 10.6 (8.4 to 12.8) per 10 000 pregnancies, respectively. The adjusted relative risk after fine stratification of the propensity score was 1.30 (1.09 to 1.56) for musculoskeletal malformations, 1.04 (0.70 to 1.55) for conotruncal malformations, and 0.91 (0.61 to 1.35) for oral clefts overall. Based on cumulative doses of fluconazole, the adjusted relative risks for musculoskeletal malformations, conotruncal malformations, and oral clefts overall were 1.29 (1.05 to 1.58), 1.12 (0.71 to 1.77), and 0.88 (0.55 to 1.40) for 150 mg of fluconazole; 1.24 (0.93 to 1.66), 0.61 (0.26 to 1.39), and 1.08 (0.58 to 2.04) for more than 150 mg up to 450 mg of fluconazole; and 1.98 (1.23 to 3.17), 2.30 (0.93 to 5.65), and 0.94 (0.23 to 3.82) for more than 450 mg of fluconazole, respectively.
Conclusions
Oral fluconazole use in the first trimester was not associated with oral clefts or conotruncal malformations, but an association with musculoskeletal malformations was found, corresponding to a small adjusted risk difference of about 12 incidents per 10 000 exposed pregnancies overall.

© Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.

BMJ: 19 May 2020; 369:m1494
Zhu Y, Bateman BT, Gray KJ, Hernandez-Diaz S, ... Straub L, Huybrechts KF
BMJ: 19 May 2020; 369:m1494 | PMID: 32434758
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Abstract

Epigenetic Modulation in the Initiation and Progression of Pulmonary Hypertension.

Cheng X, Wang Y, Du L

Pulmonary hypertension (PH) is a severe disease with multiple etiologies. In addition to genetics, recent studies have revealed the epigenetic modulation in the initiation and progression of PH. In this review, we summarize the epigenetic mechanisms in the pathogenesis of PH, specifically, DNA methylation, histone modifications, and microRNAs. We further emphasize the diagnostic and therapeutic potential of these epigenetic hallmarks in PH. Finally, we highlight the developmental reprogramming in adult-onset PH because of adverse perinatal exposures such as intrauterine growth restriction and extrauterine growth restriction. Therefore, epigenetic modifications provide promise for the therapy and prevention of PH.



Hypertension: 29 Sep 2019; 74:733-739
Cheng X, Wang Y, Du L
Hypertension: 29 Sep 2019; 74:733-739 | PMID: 31476913
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Abstract

Beyond the One Gene-One Disease Paradigm: Complex Genetics and Pleiotropy in Inheritable Cardiac Disorders

Cerrone M, Remme CA, Tadros R, Bezzina CR, Delmar M

Inheritable cardiac disorders, which may be associated with cardiomyopathic changes, are often associated with increased risk of sudden death in the young. Early linkage analysis studies in Mendelian forms of these diseases, such as hypertrophic cardiomyopathy and long-QT syndrome, uncovered large-effect genetic variants that contribute to the phenotype. In more recent years, through genotype-phenotype studies and methodological advances in genetics, it has become evident that most inheritable cardiac disorders are not monogenic but, rather, have a complex genetic basis wherein multiple genetic variants contribute (oligogenic or polygenic inheritance). Conversely, studies on genes underlying these disorders uncovered pleiotropic effects, with a single gene affecting multiple and apparently unrelated phenotypes. In this review, we explore these 2 phenomena: on the one hand, the evidence that variants in multiple genes converge to generate one clinical phenotype, and, on the other, the evidence that variants in one gene can lead to apparently unrelated phenotypes. Although multiple conditions are addressed to illustrate these concepts, the experience obtained in the study of long-QT syndrome, Brugada syndrome, and arrhythmogenic cardiomyopathy, and in the study of functions related to SCN5A (the gene coding for the α-subunit of the most abundant sodium channel in the heart) and PKP2 (the gene coding for the desmosomal protein plakophilin-2), as well, is discussed in more detail.



Circulation: 12 Jan 2019; 140:595-610
Cerrone M, Remme CA, Tadros R, Bezzina CR, Delmar M
Circulation: 12 Jan 2019; 140:595-610 | PMID: 31403841
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Abstract

Massive Cardiomegaly (>1000 g Heart) and Obesity.

Roberts WC, Khan OS

Described herein are certain clinical and morphologic findings in 9 patients who at necropsy had hearts weighing >1000 g, a weight approximately 3 times normal. With the exception of 2 patients with hypertrophic cardiomyopathy, the common finding in the remaining 7 patients was obesity. None had valvular heart disease, the previously described major cause of massive cardiomegaly. Thus, obesity needs to be added to the causes of massive cardiomegaly, a cause not previously recognized. Electrocardiograms in 4 patients disclosed high total 12-lead QRS voltage on the electrocardiogram in only one despite the massive cardiomegaly.

Copyright © 2019 Elsevier Inc. All rights reserved.

Am J Cardiol: 14 Jan 2020; 125:277-281
Roberts WC, Khan OS
Am J Cardiol: 14 Jan 2020; 125:277-281 | PMID: 31847959
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Abstract

Paradigm of Sudden Death Prevention in Hypertrophic Cardiomyopathy.

Maron BJ, Rowin EJ, Maron MS

Hypertrophic cardiomyopathy (HCM) is a worldwide genetic heart disease and a common cause of sudden death in the young. Penetration of the implantable cardioverter-defibrillator (ICD) into this patient population over the past 20 years has made accurate selection of patients for primary prevention ICDs a priority. Consequently, a new paradigm has emerged in the management of this complex disease with ICD therapy responsible for a substantial decrease in overall HCM-related mortality (to 0.5%/y) and independent of patient age. Selection of candidates for ICDs has matured substantially with the formulation of an enhanced risk stratification algorithm. One or more contemporary risk markers judged major within a given patient\'s clinical profile, in association with physician judgment and shared decision-making, is sufficient to consider a primary prevention ICD implant. An enhanced American College of Cardiology/American Heart Association risk factor model (including new contrast-magnetic resonance-based markers, such as left ventricular apical aneurysm) used prospectively to make ICD decisions proved to be 95% sensitive for identifying patients who would experience ≥1 appropriate device therapies terminating ventricular tachycardia/fibrillation. The number of HCM patients required to treat with ICDs to save 1 patient with abolition of lethal ventricular tachyarrhythmias was 6:1, similar to randomized defibrillator trials in other cardiomyopathies. In contrast to patients with ischemic heart disease, after ICD shock HCM patients rarely experience transformation to heart failure deterioration or sudden arrhythmic death. The mathematically derived risk score model proposed by the European Society of Cardiology was inferior for identifying high-risk patients susceptible to arrhythmic sudden death with a sensitivity of only 33%, leaving many patients exposed to the possibility of sudden death without ICDs. In conclusion, introduction of the ICD associated with a matured risk stratification algorithm has altered management strategy and clinical course of many HCM patients, making the likelihood of sudden death prevention a reality and fulfilling the aspiration of preservation of life and reduced mortality for this vulnerable patient population.



Circ Res: 01 Jan 2019; 125:370-378
Maron BJ, Rowin EJ, Maron MS
Circ Res: 01 Jan 2019; 125:370-378 | PMID: 31518168
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Abstract

Electrocardiogram Standards for Children and Young Adults Using Z-scores.

Bratincsák A, Kimata C, Limm-Chan BN, Vincent K, Williams M, Perry JC

- Normative ECG values for children are based on relatively few subjects and are not standardized, resulting in interpersonal variability of interpretation. Recent advances in digital technology allow a more quantitative, reproducible assessment of ECG variables. Our objective was to create the foundation of normative ECG standards in the young utilizing Z-scores.- One-hundred-and-two ECG variables were collected from a retrospective cohort of 27085 study subjects with no known heart condition, ages 0-39 years. The cohort was divided into 16 age groups by gender. Median, interquartile range and range were calculated for each variable adjusted to body surface area.- Normative standards were developed for all 102 ECG variables including heart rate; P, R, and T axis; R-T axis deviation; PR interval, QRS duration, QT and QTc interval; P, Q, R, S, and T amplitudes in 12 leads; as well as QRS and T wave integrals. Incremental Z-score values between -2.5 and 2.5 were calculated to establish upper and lower limits of normal. Historical ECG interpretative concepts were reassessed and new concepts observed.- Electronically acquired ECG values based on the largest pediatric and young adult cohort ever compiled provide the first detailed, standardized, quantitative foundation of traditional and novel ECG variables. Expression of ECG variables by Z-scores lends an objective and reproducible evaluation without interpreter bias that can lead to more confident establishment of ECG-disease correlations and improved automated ECG readings in high volume cardiac screening efforts in the young.



Circ Arrhythm Electrophysiol: 06 Jul 2020; epub ahead of print
Bratincsák A, Kimata C, Limm-Chan BN, Vincent K, Williams M, Perry JC
Circ Arrhythm Electrophysiol: 06 Jul 2020; epub ahead of print | PMID: 32634327
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Abstract

Atrial Septal Defect and the Risk of Ischemic Stroke in the Perioperative Period of Noncardiac Surgery.

Smilowitz NR, Subashchandran V, Berger JS

Stroke is a serious complication of noncardiac surgery. Congenital defects of the interatrial septum may be a potent risk factor for perioperative stroke. The aim of the present study was to determine the association between atrial septal defect (ASD) or patent foramen ovale (PFO) and in-hospital perioperative ischemic stroke after non-cardiac surgery in a large nationwide cohort of patients hospitalized in the United States. Patients undergoing noncardiac surgery between 2004 and 2014 were identified using the Healthcare Cost and Utilization Project\'s National Inpatient Sample. Patients without an in-hospital echocardiogram were excluded. The presence of an ostium secundum-type ASD or PFO was identified by ICD-9 diagnosis code 745.5. The primary study outcome was perioperative acute ischemic stroke. Between 2004 and 2014, there were 639,985 admissions for noncardiac surgery with an in-hospital echocardiogram. An ASD or PFO was documented in 9,041 (1.4%) hospitalizations. Perioperative ischemic stroke occurred more frequently in patients with an ASD or PFO compared with those without an ASD or PFO (35.1% vs 6.0%, p <0.001). The association between ASD or PFO and ischemic stroke persisted after adjustment for demographics and clinical covariates (adjusted odds ratio 6.30, 95% confidence interval, 5.59 to 7.10) and in all non-cardiac surgery subtypes. In conclusion, in a large, nationwide analysis of patients undergoing noncardiac surgery, a diagnosis of ASD or PFO was associated with an increased risk of acute ischemic stroke overall and in all surgical subtypes. Additional measures are necessary to mitigate stroke risk in patients with septal defects who are planned for non-cardiac surgery.

Published by Elsevier Inc.

Am J Cardiol: 30 Sep 2019; 124:1120-1124
Smilowitz NR, Subashchandran V, Berger JS
Am J Cardiol: 30 Sep 2019; 124:1120-1124 | PMID: 31375244
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Abstract

Usefulness of Neuromuscular Co-morbidity, Left Bundle Branch Block, and Atrial Fibrillation to Predict the Long-Term Prognosis of Left Ventricular Hypertrabeculation/Noncompaction.

Stöllberger C, Hasun M, Winkler-Dworak M, Finsterer J

The prognosis of patients with left ventricular hypertrabeculation/noncompaction (LVHT) is assessed controversially. LVHT is associated with other cardiac abnormalities and with neuromuscular disorders (NMD). Aim of the study was to assess cardiac and neurological findings as predictors of mortality rate in adult LVHT-patients. Included were patients with LVHT diagnosed between 1995 and 2019 in 1 echocardiographic laboratory. Patients underwent a baseline cardiologic examination and were invited for a neurological investigation. In January 2020, their survival status was assessed. End points were death or heart transplantation. LVHT was diagnosed by echocardiography in 310 patients (93 female, aged 53 ± 18 years) with a prevalence of 0.4%/year. A neurologic investigation was performed in 205 patients (67%). A specific NMD was found in 33 (16%), NMD of unknown etiology in 123 (60%) and the neurological investigation was normal in 49 (24%) patients. During follow-up of 84 ± 71 months, 59 patients received electronic devices, 105 patients died, and 6 underwent heart transplantation. The mortality was 4.7%/year, the rate of heart transplantation/death 5%/year. By multivariate analysis, the following parameters were identified to elevate the risk of mortality/heart transplantation: increased age (p = 0.005), inpatient (p = 0.001), presence of a specific NMD (p = 0.0312) or NMD of unknown etiology (p = 0.0365), atrial fibrillation (p = 0.0000), ventricular premature complexes (p = 0.0053), exertional dyspnea (p = 0.0023), left bundle branch block (p = 0.0201), and LVHT of the posterior wall (p = 0.0158). In conclusion, LVHT patients should be systematically investigated neurologically since neurological co-morbidity has a prognostic impact.

Copyright © 2020 Elsevier Inc. All rights reserved.

Am J Cardiol: 31 Jul 2020; 128:168-173
Stöllberger C, Hasun M, Winkler-Dworak M, Finsterer J
Am J Cardiol: 31 Jul 2020; 128:168-173 | PMID: 32650915
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Abstract

Initial experiences with a novel biodegradable device for percutaneous closure of atrial septal defects: From preclinical study to first-in-human experience.

Li YF, Xie YM, Chen J, Li BN, ... Wang SS, Zhang ZW
Objective
To evaluate the feasibility, safety, and effectiveness of a novel, absorbable atrial septal defect (ASD) closure device made of poly-l-lactic acid (PLLA) in a swine model of ASD and for the first time in humans.
Methods
A preclinical safety study was conducted using a swine model of ASD. In a clinical setting, five pediatric patients underwent ASD closure with the PLLA device with fluoroscopic and transthoracic echocardiography guidance. The procedural results and clinical outcomes at 1 day, 30 days, 3 months, and 6 months after closure were analyzed.
Results
The 24- and 36-month follow-up results of the preclinical study demonstrated that the PLLA device exhibited good endothelialization and degradability in the swine model. In the clinical study, successful device implantation was achieved in all five patients (median age, 3.6 years; range, 3.1-6.5 years). The mean defect size was (13.6 ± 2.7) mm. Follow-up at 30 days, 3 months, and 6 months was completed in all five cases. The complete defect closure rates with no residual shunt at 30 days, 3 months, and 6 months follow-up were 60% (3/5), 80% (4/5), and 80% (4/5), respectively. No device dislodgement, significant aortic valve or mitral valve regurgitation, new onset cardiac arrhythmia, or other adverse events were reported.
Conclusion
The study results demonstrated that it is feasible to implant the PLLA device for closure of small to medium sized ASDs without significant residual shunts or severe adverse events in humans. The PLLA device exhibited good endothelialization and degradability in the swine model at 24 and 36 months. Further studies to evaluate long-term safety and effectiveness with the device in a large cohort of patients are warranted.

© 2019 The Authors. Catheterization and Cardiovascular Interventions published by Wiley Periodicals, Inc.

Catheter Cardiovasc Interv: 11 Nov 2019; epub ahead of print
Li YF, Xie YM, Chen J, Li BN, ... Wang SS, Zhang ZW
Catheter Cardiovasc Interv: 11 Nov 2019; epub ahead of print | PMID: 31714687
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Abstract

Contraceptive methods of privately insured US women with congenital heart defects.

Anderson KN, Tepper NK, Downing K, Ailes EC, Abarbanell G, Farr SL
Background
The American Heart Association recommends women with congenital heart defects (CHD) receive contraceptive counseling early in their reproductive years, but little is known about contraceptive method use among women with CHD. We describe recent female sterilization and reversible prescription contraceptive method use by presence of CHD and CHD severity in 2014.
Methods
Using IBM MarketScan Commercial Databases, we included women aged 15 to 44 years with prescription drug coverage in 2014 who were enrolled ≥11 months annually in employer-sponsored health plans between 2011 and 2014. CHD, CHD severity, contraceptive methods, and obstetrics-gynecology and cardiology provider encounters were identified using billing codes. We used log-binomial regression to calculate adjusted prevalence ratios (aPRs) and 95% confidence intervals (CIs) to compare contraceptive method use overall and by effectiveness tier by CHD presence and, for women with CHD, severity.
Results
Recent sterilization or current reversible prescription contraceptive method use varied slightly among women with (39.2%) and without (37.3%) CHD, aPR = 1.04, 95% CI [1.01-1.07]. Women with CHD were more likely to use any Tier I method (12.9%) than women without CHD (9.3%), aPR = 1.41, 95% CI [1.33-1.50]. Women with severe, compared to non-severe, CHD were less likely to use any method, aPR = 0.85, 95% CI [0.78-0.92], or Tier I method, aPR = 0.84, 95% CI [0.70-0.99]. Approximately 60% of women with obstetrics-gynecology and <40% with cardiology encounters used any included method.
Conclusions
There may be missed opportunities for providers to improve uptake of safe, effective contraceptive methods for women with CHD who wish to avoid pregnancy.

Published by Elsevier Inc.

Am Heart J: 20 Jan 2020; 222:38-45
Anderson KN, Tepper NK, Downing K, Ailes EC, Abarbanell G, Farr SL
Am Heart J: 20 Jan 2020; 222:38-45 | PMID: 32014720
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Abstract

A 3-Year-Old Girl With a Mediastinal Mass.

Wentink M, Peeters D, van der Burg M, Vermont C, Duijts L, Driessen GJ
A 3-year-old girl was referred to a pediatric pulmonologist for dyspnea and recurrent upper respiratory tract infections (RTIs). The patient was born full term to unrelated Dutch parents after an uneventful pregnancy and birth. The year before presentation, she had suffered from pneumonia and > 10 upper RTIs. Apart from the recurrent RTIs, which started in infancy, her medical history was not significant and did not include allergies or eczema. An adenotonsillectomy was performed at the age of 2 years, and she was treated with multiple antibiotic regimens and inhalation therapy with salbutamol and corticosteroids, with no relief of symptoms.

Chest: 30 Dec 2018; 155:e13-e16
Wentink M, Peeters D, van der Burg M, Vermont C, Duijts L, Driessen GJ
Chest: 30 Dec 2018; 155:e13-e16 | PMID: 30616742
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Abstract

Long-Term Survival of Patients With Coarctation Repaired During Infancy (from the Pediatric Cardiac Care Consortium).

Oster ME, McCracken C, Kiener A, Aylward B, ... Hunting J, Kochilas LK

Patients who undergo coarctation repair during infancy have excellent early survival but long-term survival is unknown. We aimed to describe the long-term survival of patients with coarctation repaired during infancy and determine predictors of mortality. We performed a retrospective cohort study using data from the Pediatric Cardiac Care Consortium for patients with coarctation who underwent surgical repair before 12 months of age between 1982 and 2003. Long-term transplant-free survival was obtained by linkage with the National Death Index and the Organ Sharing Procurement Network. Kaplan Meier survival plots were constructed, and univariate and multivariable analyses were performed to determine predictors of mortality. We identified 2,424 coarctation patients who met inclusion criteria. At 20 years postoperatively, 94.5% of all patients and 95.8% of those discharged after initial operation remained alive, respectively. Significant multivariable predictors of mortality included surgical weight <2.5 kg (hazard ratio [HR] 3.70, 95% confidence interval [CI] 2.19 to 6.24), presence of a genetic syndrome (HR 2.40, 95% CI 1.13 to 5.10), and repair before 1990 (HR 1.91, 95% CI 1.09 to 3.34). None of the other factors examined including age at repair, gender, coarctation type, or surgical approach were found to be statistically significant. Over half of the deaths were due to the underlying congenital heart disease or other cardiovascular etiology. Overall long-term survival of patients who undergo coarctation repair during infancy is excellent. However, patients do experience small continued survival attrition throughout early adulthood. Ongoing monitoring of this cohort is necessary to assess late mortality risk.

Copyright © 2019 Elsevier Inc. All rights reserved.

Am J Cardiol: 31 Aug 2019; 124:795-802
Oster ME, McCracken C, Kiener A, Aylward B, ... Hunting J, Kochilas LK
Am J Cardiol: 31 Aug 2019; 124:795-802 | PMID: 31272703
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Abstract

Impact of Right Ventricular Geometry and Left Ventricular Hypertrophy on Right Ventricular Mechanics and Clinical Outcomes in Hypoplastic Left Heart Syndrome.

Rösner A, Bharucha T, James A, Mertens L, Friedberg MK
Background
Right ventricular (RV) function is a major determinant of survival in hypoplastic left heart syndrome (HLHS). However, the relation of RV geometry to myocardial mechanics and their relation to transplant-free survival are incompletely characterized.
Methods
We retrospectively studied 48 HLHS patients from the Hospital for Sick Children, Toronto, (median age, 2.2; interquartile range, 3.62 years) at different surgical stages. Patients were grouped by the presence (n = 23) or absence (n = 25) of RV \"apical bulging\" defined as a sigmoid-shaped septum with the RV leftward apical segment contiguous with the left ventricular (LV) lateral wall. Regional and global RV strain were measured using speckle-tracking echocardiography, and regional strains were analyzed for patterns and peak values. These were compared between HLHS anatomical subtypes and between patients with versus without apical bulging. We further investigated the association between RV geometry and dysfunction with the outcomes of heart failure, death, or transplant.
Results
RV global (-7.3% ± 2.8% vs -11.2% ± 4.4%; P = .001), basal septal (-3.8% ± 3.2% vs -11.4% ± 5.8%; P = .0001) and apicolateral (-5.1% ± 3.5% vs -8.0% ± 5.8%, P = .001) longitudinal strain were lower in patients with versus without apical bulging, respectively. Apical bulging was equally prevalent in all HLHS anatomical variants. Twenty of 22 (91%) patients with apical bulging displayed hypertrophy of the LV apical and lateral segments. Death or transplantation were approximately equal in both groups but related to reduced RV global strain in patients with (seven of seven) and not in those without apical bulging (two of eight; P = .022).
Conclusions
These results suggest that the finding of apical bulging is related to the presence of a hypertrophied hypoplastic LV, with a negative impact on regional and global RV function. Therefore, analysis of RV and LV geometry and mechanics may aid in the assessment and prognostication of this high-risk population.

Copyright © 2019 American Society of Echocardiography. All rights reserved.

J Am Soc Echocardiogr: 29 Sep 2019; 32:1350-1358
Rösner A, Bharucha T, James A, Mertens L, Friedberg MK
J Am Soc Echocardiogr: 29 Sep 2019; 32:1350-1358 | PMID: 31351794
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Abstract

Clinical Utility of Echocardiography in Former Preterm Infants with Bronchopulmonary Dysplasia.

Nawaytou H, Steurer MA, Zhao Y, Guslits E, ... Fineman JR, Keller RL
Background
The clinical utility of echocardiography for the diagnosis of pulmonary vascular disease (PVD) in former preterm infants with bronchopulmonary dysplasia (BPD) is not established. Elevated pulmonary vascular resistance (PVR) rather than pulmonary artery pressure (PAP) is the hallmark of PVD. We evaluated the utility of echocardiography in infants with BPD in diagnosing pulmonary hypertension and PVD (PVR >3 Wood units × m) assessed by cardiac catheterization.
Methods
A retrospective single center study of 29 infants born ≤29 weeks of gestational age with BPD who underwent cardiac catheterization and echocardiography was performed. PVD was considered present by echocardiography if the tricuspid valve regurgitation jet peak velocity was >2.9 m/sec, post-tricuspid valve shunt systolic flow velocity estimated a right ventricular systolic pressure >35 mm Hg, or systolic septal flattening was present. The utility (accuracy, sensitivity, and positive predictive value [PPV]) of echocardiography in the diagnosis of PVD was tested. Subgroup analysis in patients without post-tricuspid valve shunts was performed. Echocardiographic estimations of right ventricular pressure, dimensions, function, and pulmonary flow measurements were evaluated for correlation with PVR.
Results
The duration between echocardiography and cardiac catheterization was a median of 1 day (interquartile range, 1-4 days). Accuracy, sensitivity, and PPV of echocardiography in diagnosing PVD were 72%, 90.5%, and 76%, respectively. Accuracy, sensitivity, and PPV increased to 93%, 91.7%, and 100%, respectively, when infants with post-tricuspid valve shunts were excluded. Echocardiography had poor accuracy in estimating the degree of PAP elevation by cardiac catheterization. In infants without post-tricuspid valve shunts, there was moderate to good correlation between indexed PVR and right ventricular myocardial performance index (rho = 0.89, P = .005), systolic to diastolic time index (0.84, P < .001), right to left ventricular diameter ratio at end systole (0.66, P = .003), and pulmonary artery acceleration time (0.48, P = .05).
Conclusions
Echocardiography performs well in screening for PVD in infants with BPD and may be diagnostic in the absence of a post-tricuspid valve shunt. However, cardiac catheterization is needed to assess the degree of PAP elevation and PVR. The diagnostic utility of echocardiographic measurements that correlate with PVR should be evaluated prospectively in this patient population.

Copyright © 2019 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

J Am Soc Echocardiogr: 12 Jan 2020; epub ahead of print
Nawaytou H, Steurer MA, Zhao Y, Guslits E, ... Fineman JR, Keller RL
J Am Soc Echocardiogr: 12 Jan 2020; epub ahead of print | PMID: 31948712
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Abstract

Spontaneous Closure Rates of Ventricular Septal Defects (6,750 Consecutive Neonates).

Zhao QM, Niu C, Liu F, Wu L, Ma XJ, Huang GY

Although ventricular septal defect (VSD) is a common and simple congenital heart disease in newborns, its true incidence and spontaneous closure (SC) rate remains topics of controversy. This study aims to provide data on the true incidence and SC rate of VSD in the Chinese neonatal population. We conducted a prospective study at 3 hospitals, all newborns underwent echocardiography. Those with a diagnosis of isolated VSD were included in the study group and underwent a 7-year follow-up period. In 6,750 newborns, VSDs were detected in 113 cases (incidence rate of 16.7%), accounting for 62.8% of congenital heart disease, of which 35 were perimembranous (5.2%), 72 were muscular (10.7%), and 6 were doubly committed juxta-arterial (0.9‰). During the 7-year follow-up period, 18 cases required surgical or transcatheter closure. The SC rate in those with perimembranous VSD and muscular VSD (mVSD) were 51.4% (18 of 35) and 97.2% (70 of 72), respectively. Excluding doubly committed juxta-arterial, perimembranous site and defects ≥4 mm are risk factors for VSD that do not spontaneously close. Independent predictive factors for perimembranous VSD which do not spontaneously close is defects ≥4 mm. There was no significant difference in the SC rate at different times between the 4 mVSD sites. In conclusion, this study provides the true incidence and SC rate for Chinese newborns with VSD.

Copyright © 2019 Elsevier Inc. All rights reserved.

Am J Cardiol: 14 Aug 2019; 124:613-617
Zhao QM, Niu C, Liu F, Wu L, Ma XJ, Huang GY
Am J Cardiol: 14 Aug 2019; 124:613-617 | PMID: 31208700
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Abstract

Hemodynamics in Adults With the Shone Complex.

Jain CC, Warnes CA, Egbe AC, Cetta F, ... Connolly HM, Miranda WR

Patients with Shone complex (SC) have multiple left-sided obstructive lesions and thus are at risk for left ventricular (LV) remodeling, LV diastolic dysfunction and pulmonary hypertension. Yet, to date, there has been no description of hemodynamics in adults with SC. Retrospective chart review of 25 patients with SC who underwent cardiac catheterization at Mayo Clinic, MN between 2002 and 2019 was performed. SC was defined as multiple left-sided obstructive lesions in the presence of an anatomically abnormal mitral valve. Median age was 32 years (22.5, 42) and 15 patients (60%) were female. The majority of patients (84%) had history of coarctation of the aorta, 10 (40%) had subaortic stenosis, 11 (44%) had prior aortic valve replacement, and 10 (40%) had prior mitral valve replacement. Structural disease at the time of catheterization which warranted intervention within the next year was present in 13 patients (52%). The mean LV end-diastolic pressure was 21.3 ± 9.0 mm Hg (>15 mm Hg in 71%), pulmonary artery peak systolic pressure was 55.4 ± 13.4 mm Hg, and the pulmonary artery mean pressure was 37.0 ± 9.4 mm Hg (>20 mm Hg in 96%). During a mean follow-up of 8.3 ± 4.4 years, there were 7 deaths (28%) and 3 additional patients (12%) underwent cardiac transplantation. In conclusion, adults with SC who underwent catheterization showed significant left-sided heart and pulmonary vascular remodeling. Elevated LV end-diastolic pressure and pulmonary artery pressures were highly prevalent. There were high mortality and cardiac transplant rates in our cohort.

Copyright © 2020 Elsevier Inc. All rights reserved.

Am J Cardiol: 15 Jun 2020; epub ahead of print
Jain CC, Warnes CA, Egbe AC, Cetta F, ... Connolly HM, Miranda WR
Am J Cardiol: 15 Jun 2020; epub ahead of print | PMID: 32703525
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Abstract

Left Atrial Strain Correlates with Elevated Filling Pressures in Pediatric Heart Transplantation Recipients.

Loar RW, Pignatelli RH, Morris SA, Colquitt JL, ... Denfield SW, Tunuguntla HP
Background
Noninvasive assessment of diastolic function in pediatric heart transplantation (PHTx) patients is important for monitoring of rejection, cardiac allograft vasculopathy, and nonspecific graft failure. We hypothesized that left atrial strain (LAS) would correlate with pulmonary capillary wedge pressure (PCWP) and that cutoff values to identify elevated left ventricular (LV) filling pressure could be derived for clinical practice and future testing.
Methods
This was a secondary analysis of a prospectively collected cohort of PHTx patients undergoing same-day cardiac catheterization with biopsy and transthoracic echo. There were 70 patients with 85 clinical encounters. Traditional mitral inflow Doppler, LAS, LV diastolic strain and strain rate, and ratios for mitral E to LV diastolic strain and strain rate were assessed. Correlation with PCWP was performed, and receiver operator characteristic curves were generated for an elevated mean PCWP, acute rejection, and cardiac allograft vasculopathy.
Results
Decreased LAS during the atrial reservoir phase (Ɛres) correlated with higher invasively measured PCWP (r = -0.40, P < .001). An Ɛres cutoff of 14.5% had good discriminatory ability for an elevated PCWP (sensitivity 75%, specificity 82%), and Ɛres > 22.0% had 100% negative predictive value; Ɛres was superior to other measures of diastolic function. Subanalyses for recent acute rejection (n = 9) showed good discriminatory ability for Ɛres of 14.5% (sensitivity 89%, specificity 74%).
Conclusions
LAS correlates with invasively measured PCWP and can identify elevated pressures better than traditional and other advanced diastolic function parameters. Use of LAS in PHTx patients may aid in noninvasive monitoring for rejection and nonspecific graft dysfunction.

Copyright © 2019 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

J Am Soc Echocardiogr: 23 Jan 2020; epub ahead of print
Loar RW, Pignatelli RH, Morris SA, Colquitt JL, ... Denfield SW, Tunuguntla HP
J Am Soc Echocardiogr: 23 Jan 2020; epub ahead of print | PMID: 31987750
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Abstract

Echocardiographic Indices of the Left and Right Heart in a Normal Black African Population.

Nel S, Nihoyannopoulos P, Libhaber E, Essop MR, ... Meel R, Peters F
Background
It is unknown whether ethnic differences occur with regard to right heart echocardiographic parameters. The aim of this study therefore was to establish normative values of left and right heart parameters in a black African population and to evaluate the effect of age and body mass index (BMI) on specific right ventricle (RV) parameters.
Methods
Two hundred fifty-three normal subjects were prospectively studied. A standardized echocardiographic examination was conducted with the RV focused view used to derive RV measurements. All left and right heart measurements were made in accordance with the American Society of Echocardiography 2015 chamber guideline recommendations. Right ventricle free wall strain was assessed using an RV focused apical four-chamber view.
Results
The average age was 36.3 ± 12.2 years, and 59% of patients were female. The mean left ventricular ejection fraction was 62.3% ± 5.7%. The RV linear measurements (RV base, 31.0 ± 4.5 mm; midcavity, 26.3 ± 5.8 mm) were not associated with sex, age, or BMI except for the RV length (64.6 ± 8.9 mm), which was greater in male patients. Tricuspid annular plane systolic excursion (TAPSE) was 21.7 ± 2.8 mm, fractional area change was 42.1% ± 5.5%, tricuspid annular peak systolic velocity RV S\' was 12.1 ± 1.9 m/sec, and RV free wall strain was -31.5% ± 8.6%. Age and BMI were not associated with right atrial (RA) volumetric measurements, RV linear measurements, or any RV functional parameters except TAPSE and RV A\', which increased with BMI.
Conclusions
This study establishes normal left and right heart parameters in a black African population. Aging was not associated with RA or RV parameters except for RV E\' and A\'. BMI does not affect RA/RV measurements but may cause variability in TAPSE and RV A\'.

Copyright © 2019 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

J Am Soc Echocardiogr: 16 Jan 2020; epub ahead of print
Nel S, Nihoyannopoulos P, Libhaber E, Essop MR, ... Meel R, Peters F
J Am Soc Echocardiogr: 16 Jan 2020; epub ahead of print | PMID: 31959528
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Abstract

Comparison of Economic Self-sufficiency and Educational Attainment in Adults with Congenital Heart Disease vs Siblings without Heart Disease and to General Population.

Madsen NL, Marino BS, Woo JG, Olsen M

Among children with congenital heart disease (CHD), there is often neurodevelopmental and behavioral impairment with unclear implications regarding adult socioeconomic achievements. We aimed to compare economic self-sufficiency and educational attainment in CHD adults with sibling and general population controls. Using Danish population-based nationwide registries this cohort study aimed to include all CHD subjects greater than 13 years born between 1963-1993. Comparison cohorts included: 1) sibling cohort, and 2) general population cohort matched 10:1 on birth year and gender. We computed cumulative incidences of time to first full year of economic self-sufficiency, as well as educational attainment. We assessed the relative probability of self-sufficiency in all cohorts before 30 years of age, defined by Statistics Denmark federal standard. In total, we identified 7,019 CHD subjects, 6,257 full siblings and 68,805 general population controls. The cumulative incidence of self-sufficiency by age 20 and 35 years for CHD subjects (49% and 84%, respectively) was lower than sibling (68% and 96%) and general population cohorts\' (67% and 95%). The relative probability of self-sufficiency for CHD subjects compared with siblings was 0.44 (95% CI: 0.39-0.49). By age 30, adults with CHD were less likely than their siblings to attain all levels of education. Among those achieving higher educational milestones, differences in self-sufficiency between cohorts were absent by age 35. In conclusion, CHD is associated with reduced adult economic self-sufficiency, and the relationship between educational level attained and self-sufficiency may suggest that targeted interventions have the potential to improve adult self-sufficiency.

Copyright © 2020. Published by Elsevier Inc.

Am J Cardiol: 27 Aug 2020; epub ahead of print
Madsen NL, Marino BS, Woo JG, Olsen M
Am J Cardiol: 27 Aug 2020; epub ahead of print | PMID: 32866448
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Abstract

Value of Exercise Stress Echocardiography in Children with Hypertrophic Cardiomyopathy.

El Assaad I, Gauvreau K, Rizwan R, Margossian R, Colan S, Chen MH
Background
Exercise stress echocardiography (ESE) is a valuable diagnostic and prognostic tool in adults with hypertrophic cardiomyopathy (HCM). Inducible and resting left ventricular outflow tract gradients are important predictors of heart failure. However, there are minimal data on the utility of this modality in children.
Methods
Retrospective review of all pediatric HCM patients who underwent ESE at Boston Children\'s Hospital (January 2007-June 2018) was carried out. Patients were assigned to one of three categories based on left ventricular outflow tract gradients: group 1: <30 mm Hg at rest and exercise; group 2: <30 mm Hg at rest and ≥30 mm Hg with exercise; and group 3: ≥ 30 mm Hg at rest and exercise. Records were reviewed for earliest occurrence of composite endpoint of any one of the following: cardiac syncope, chest pain, nonsustained and sustained ventricular tachycardia, aborted cardiac arrest, heart failure class ≥ II, or HCM-related death/transplantation.
Results
A total of 91 children (67% males) with median age 12 years (6-24 years) at first ESE and median left ventricle wall thickness of 20 mm formed the cohort. Median follow-up duration was 3 years. During ESE, only one child experienced an event and was resuscitated. Of the 91 children, 25 were classified as group 1, 40 as group 2, and 26 as group 3. Twenty-six patients met the composite endpoint, including two heart transplant, one aborted cardiac arrest, and one sudden cardiac death. Group 3 patients had a 5 times higher risk of developing symptoms and/or serious clinical outcome at any age (hazard ratio = 5.18; 95% CI, [1.39-19.2]; P = .014). During our short follow-up time, group 2 patients had a higher risk of outcome, but this did not achieve statistical significance (hazard ratio = 1.95; 95% CI, [0.5-7.6]; P = .33).
Conclusions
In this large series of pediatric patients with HCM, ESE can be performed safely and served as an effective tool to identify the lowest risk patients for cardiac outcome.

Copyright © 2020 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

J Am Soc Echocardiogr: 08 Apr 2020; epub ahead of print
El Assaad I, Gauvreau K, Rizwan R, Margossian R, Colan S, Chen MH
J Am Soc Echocardiogr: 08 Apr 2020; epub ahead of print | PMID: 32279939
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Abstract

Myocardial Function Following Repair of Anomalous Origin of Left Coronary Artery from the Pulmonary Artery in Children.

Naqvi N, Babu-Narayan SV, Krupickova S, Muthialu N, ... Tsang V, Marek J
Objective
We aimed to assess the change in global and regional myocardial function before and after surgical revascularization and their added value when compared with conventional measures in children with anomalous left coronary artery from the pulmonary artery (ALCAPA).
Methods
Advanced echocardiographic assessment was performed pre- and postoperatively in 22 children with ALCAPA (eight male; median surgery age, 0.4 years; interquartile range, 0.21-1.05) and 22 healthy controls. Measurements included global and segmental longitudinal, radial, and circumferential two-dimensional speckle-tracking strain and postsystolic index.
Results
Global strains were lower in preoperative patients than in controls (longitudinal: -9% vs -21%; P < .001; circumferential: -11% vs -21%; P < .001; radial: 18% vs 60%; P < .001) and improved postoperatively when compared with preoperative findings (longitudinal: -9% pre vs -16% post; P = .002, circumferential:-11% pre vs -17% post; P = .012, radial: 18% pre vs 53% post; P = .001). Preoperatively, patients with normal global systolic function on conventional echocardiography had significantly impaired global longitudinal and radial strain compared with healthy controls. Global mechanical dyssynchrony improved significantly postoperatively (longitudinal postsystolic index 43 pre vs 6 post, P < .001; circumferential 15 pre vs 2 post, P = .001; radial 48 pre vs 5 post, P = .003). Despite overall improvement in most segments, global longitudinal and circumferential and segmental peak strain in some of the segments supplied by the ALCAPA remained postoperatively abnormal.
Conclusions
This study shows that myocardial deformation indices were a more sensitive measure of LV dysfunction in patients before and after ALCAPA repair than conventional echocardiographic measures. We believe, therefore, they should be added to routine preoperative and serial postoperative follow-up assessment.

Copyright © 2020 American Society of Echocardiography. All rights reserved.

J Am Soc Echocardiogr: 27 Feb 2020; epub ahead of print
Naqvi N, Babu-Narayan SV, Krupickova S, Muthialu N, ... Tsang V, Marek J
J Am Soc Echocardiogr: 27 Feb 2020; epub ahead of print | PMID: 32122741
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Abstract

Surveillance of Congenital Heart Defects among Adolescents at Three U.S. Sites.

Lui GK, McGarry C, Bhatt A, Book W, ... Zaidi A, Van Zutphen AR

The prevalence, co-morbidities, and healthcare utilization in adolescents with congenital heart defects (CHDs) is not well understood. Adolescents (11 to 19 years old) with a healthcare encounter between January 1, 2008 (January 1, 2009 for MA) and December 31, 2010 with a CHD diagnosis code were identified from multiple administrative data sources compiled at 3 US sites: Emory University, Atlanta, Georgia (EU); Massachusetts Department of Public Health (MA); and New York State Department of Health (NY). The estimated prevalence for any CHD was 4.77 (EU), 17.29 (MA), and 4.22 (NY) and for severe CHDs was 1.34 (EU), 3.04 (MA), and 0.88 (NY) per 1,000 adolescents. Private or commercial insurance was the most common insurance type for EU and NY, and Medicaid for MA. Inpatient encounters were more frequent in severe CHDs. Cardiac co-morbidities included rhythm and conduction disorders at 20% (EU), 46% (MA), and 9% (NY) as well as heart failure at 3% (EU), 15% (MA), and 2% (NY). Leading noncardiac co-morbidities were respiratory/pulmonary (22% EU, 34% MA, 16% NY), infectious disease (17% EU, 22% MA, 20% NY), non-CHD birth defects (12% EU, 23% MA, 14% NY), gastrointestinal (10% EU, 28% MA, 13% NY), musculoskeletal (10% EU, 32% MA, 11% NY), and mental health (9% EU, 30% MA, 11% NY). In conclusion, this study used a novel approach of uniform CHD definition and variable selection across administrative data sources in 3 sites for the first population-based CHD surveillance of adolescents in the United States. High resource utilization and co-morbidities illustrate ongoing significant burden of disease in this vulnerable population.

Copyright © 2019 The Authors. Published by Elsevier Inc. All rights reserved.

Am J Cardiol: 30 Jun 2019; 124:137-143
Lui GK, McGarry C, Bhatt A, Book W, ... Zaidi A, Van Zutphen AR
Am J Cardiol: 30 Jun 2019; 124:137-143 | PMID: 31030970
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Abstract

Barriers and Facilitators of Palliative Care and Advance Care Planning in Adults with Congenital Heart Disease.

Steiner JM, Dhami A, Brown CE, Stout KK, ... Engelberg RA, Kirkpatrick JN

Palliative care has potential to improve quality of life and goal-concordant care for patients with adult congenital heart disease (ACHD). However, it is rarely employed prior to critical illness because the best methods for implementation are not well-defined. We qualitatively evaluated ACHD patients\' understanding of and opinions regarding palliative care and advance care planning (ACP) to better define the needs of this population. We conducted a thematic analysis of 25 semi-structured interviews with patients with ACHD in which we assessed participants\' perspectives on the need for, and barriers and facilitators to, the use of palliative care and ACP. In a group of participants with ACHD (mean age 38, 48% male) classified as simple (24%), moderate (32%), or complex (44%), we identified 4 major themes: 1) using knowledge to combat future uncertainties; 2) unfamiliarity with and limited exposure to palliative care and ACP; 3) facilitators and barriers to engaging in palliative care and ACP; and 4) importance of timing and presentation of ACP discussions. In conclusion, participants expressed a desire for knowledge about ACHD progression and treatment. They supported routine incorporation of palliative care and ACP and identified related facilitators and barriers to doing so. Importantly, timing and format of these discussions must be individualized using shared decision-making between clinicians, patients, and their families.

Copyright © 2020. Published by Elsevier Inc.

Am J Cardiol: 27 Aug 2020; epub ahead of print
Steiner JM, Dhami A, Brown CE, Stout KK, ... Engelberg RA, Kirkpatrick JN
Am J Cardiol: 27 Aug 2020; epub ahead of print | PMID: 32866444
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Abstract

Comparison of Left Ventricular Mass Calculation Methods via Two-Dimensional Echocardiogram in Children, Adolescents, and Young Adults With Systemic Hypertension.

Lang SM, Ittleman BR, Hahn E, Moore RA, ... Kimball TR, Statile CJ

Left ventricular (LV) mass is a major determining tool for myocardial injury in hypertensive patients. Issues with LV mass calculations exist given that there are multiple methods to assess mass, including from the parasternal long axis (PLA), parasternal short axis (PSA), and 2-dimensional (2D) volumetric methods. The aim of this study was to compare the agreement of LV mass calculations using the PLA, PSA, and 2D volumetric methods. This study retrospectively reviewed 200 consecutive, initial echocardiograms for the indication of hypertension. A single reader calculated the LV mass in each patient via the PLA, PSA, and 2D volumetric methods. Percent differences for each study were calculated. LV mass threshold cutoffs of 51 g/m (cardiac organ injury) and 38.6 g/m (elevated LV mass) were used to compare categorical differences between the different measurement methods. Paired comparisons demonstrated an absolute mean percent difference of 8.46% to 9.41% among the different methods. LV mass calculated by the 2D volumetric method was less compared with PLA and PSA methods (31.64 vs 33.90 vs 35.51 g/m; p < 0.0001). Fewer patients were classified as having cardiac target organ injury or elevated LV mass via 2D volumetric calculation, compared with PLA and PSA methods (p = 0.02 and p = 0.03, respectively). In conclusion, there is a small but important difference in LV mass calculations for patients with hypertension. These results emphasize the need for consistency within echocardiography laboratories as surveillance studies are common in this patient population.

Copyright © 2019 Elsevier Inc. All rights reserved.

Am J Cardiol: 14 Jul 2019; 124:239-244
Lang SM, Ittleman BR, Hahn E, Moore RA, ... Kimball TR, Statile CJ
Am J Cardiol: 14 Jul 2019; 124:239-244 | PMID: 31088660
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Abstract

Early detection of ventricular arrhythmias in adults with congenital heart disease using an insertable cardiac monitor (EDVA-CHD study).

Sakhi R, Kauling RM, Theuns DA, Szili-Torok T, ... Roos-Hesselink JW, Yap SC
Background
Sudden cardiac death (SCD) due to ventricular arrhythmias (VA) is an important mode of death in adults with congenital heart disease (CHD). Risk stratification is difficult in this heterogeneous population. Insertable cardiac monitors (ICM) may be useful for risk stratification. The purpose of the present study was to evaluate the use of ICM for the detection of VA in adults with CHD.
Methods
In this prospective single-center observational study we included consecutive adults with CHD deemed at risk of VA who received an ICM between March 2013 and February 2019. The decision to implant an ICM was made in a Heart Team consisting of a cardiac electrophysiologist and a cardiologist specialized in CHD.
Results
A total of 30 patients (mean age, 38 ± 15 years; 50% male) received an ICM. During a median follow-up of 16 months, 8 patients (27%) had documented nonsustained VA. Of these 8 patients, 3 (10%) received a prophylactic ICD. Furthermore, ICM-detected arrhythmias were present in 22 patients (73%) leading to a change in clinical management in 16 patients (53%). Besides the patients receiving an ICD, 10 patients (33%) had a change in their antiarrhythmic drugs, 6 patients (20%) underwent an electrophysiology study, and 1 patient (3%) received a pacemaker.
Conclusions
The detection of VA by the ICM contributed to the clinical decision to implant a prophylactic ICD. Furthermore, ICM-detected arrhythmias led to important changes in the clinical management. Therefore, long-term arrhythmia monitoring by an ICM seems valuable for risk stratification in adults with CHD.

Copyright © 2020 The Authors. Published by Elsevier B.V. All rights reserved.

Int J Cardiol: 03 Feb 2020; epub ahead of print
Sakhi R, Kauling RM, Theuns DA, Szili-Torok T, ... Roos-Hesselink JW, Yap SC
Int J Cardiol: 03 Feb 2020; epub ahead of print | PMID: 32057477
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Abstract

Staffing, activities, and infrastructure in 96 specialised adult congenital heart disease clinics in Europe.

Thomet C, Moons P, Budts W, De Backer J, ... Schwerzmann M,
Background
Clinical guidelines emphasise the need for specialised adult congenital heart disease (ACHD) programmes. In 2014, the working group on Grown-up Congenital Heart Disease of the European Society of Cardiology (ESC) published recommendations on the organisation of specialised care for ACHD. To appraise the extent to which these recommendations were being implemented throughout Europe, we assessed the number of patients in active follow-up and available staff resources in European ACHD programmes.
Methods
We conducted a descriptive, cross-sectional, paper-based survey of specialised ACHD centres in Europe in late 2017 concerning their centre status in 2016. Data from 96 ACHD centres were analysed. We categorised ACHD programmes into seven different centre types based on their staff resources and composition of interdisciplinary teams.
Results
Only four centres fulfilled all medical and non-medical staffing requirements of the ESC recommendations. Although 60% of the centres offered all forms of medical care, they had incomplete non-medical resources (i.e., specialised nurses, social workers, or psychologists). The participating centres had 226,506 ACHD patients in active follow-up, with a median of 1500 patients per centre (IQR: 800-3400). Six per cent of the patients were followed up in a centre that lacked a CHD surgeon or congenital interventional cardiologist.
Conclusions
A minority of European ACHD centres have the full recommended staff resources available. This suggests that as of 2016 either ACHD care in Europe was still not optimally organised, or that the latest ESC recommendations were not fully implemented in clinical practice.

Copyright © 2019 Elsevier B.V. All rights reserved.

Int J Cardiol: 30 Sep 2019; 292:100-105
Thomet C, Moons P, Budts W, De Backer J, ... Schwerzmann M,
Int J Cardiol: 30 Sep 2019; 292:100-105 | PMID: 31085084
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Abstract

Reduced Exercise Capacity in Diabetes Mellitus Is Not Associated with Impaired Deformation or Twist.

Roberts TJ, Barros-Murphy JF, Burns AT, MacIsaac RJ, ... Prior DL, La Gerche A
Background
Exercise capacity is frequently reduced in people with diabetes mellitus (DM) and may be due to subclinical cardiac dysfunction. Speckle-tracking echocardiography is now widely available; however, the clinical utility and significance of left ventricular (LV) strain and twist parameters remain uncertain. We hypothesized that LV strain and twist would be reduced in DM subjects during exercise.
Methods
Adults with type 1 or type 2 DM and age- and sex-matched controls performed cardiopulmonary exercise testing (VO peak) and supine bicycle exercise echocardiography. Detailed echocardiographic assessment of biventricular function was performed at baseline and repeated during incremental exercise to maximal intensity.
Results
Of the 60 participants completing the study protocol, 51 (34 DM, 17 controls; mean age, 42 ± 13 years; 69% male; DM duration, 16 ± 10 years) had sufficient image quality to assess LV deformation and twist mechanics at rest. Of these, 38 (25 DM, 13 controls) were able to be assessed immediately after exercise. Baseline LV systolic and diastolic function using standard echocardiography measurements were similar between groups. Resting LV global longitudinal strain, twist, twist rate and untwist rate, and the corresponding peak exercise and reserve measures did not differ significantly. As compared with the control subjects, exercise capacity was reduced in the DM cohort (VO peak 33 ± 10 vs 41 ± 12 mL/minute/kg; P = .02); however, no correlation was observed between VO peak and LV twist reserve (R = 0.28, P = .09), LV twist rate reserve (R = 0.14, P = .39), or LV untwist rate reserve (R = 0.24, P = .14).
Conclusions
Despite reduced VO peak, LV twist mechanics at rest and after maximal intensity exercise did not differ significantly in a cohort of asymptomatic DM subjects with normal resting LV systolic and diastolic function compared with age- and sex-matched controls. This would suggest that exercise capacity can be reduced in the absence of subclinical cardiac dysfunction and that noncardiac factors should be considered as alternative explanations.

Copyright © 2019 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

J Am Soc Echocardiogr: 28 Jan 2020; epub ahead of print
Roberts TJ, Barros-Murphy JF, Burns AT, MacIsaac RJ, ... Prior DL, La Gerche A
J Am Soc Echocardiogr: 28 Jan 2020; epub ahead of print | PMID: 32007323
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Impact:
Abstract

Access site related vascular complications with third generation transcatheter heart valve systems.

Ruge H, Burri M, Erlebach M, Lange R
Objectives
This study examines the impact of anatomical and procedural factors on Valve Academic Research Consortium-2-defined vascular complications at the femoral access site in transcatheter aortic valve replacement (TAVR) with third generation transcatheter heart valve (THV)-systems.
Background
Randomized clinical trials reported on vascular complications with current THV-systems. However, clinical presentation and consequences of these events are not well studied.
Methods
All patients who underwent a transfemoral TAVR using an Edwards Sapien3®/Sapien3ultra® or a Medtronic Evolut-R®/Evolut-PRO® have been identified from our institutional database. Only procedures utilizing the PerClose-ProGlide® vascular closure device were included. Risk factors for vascular complications were analyzed with a logistic regression model. Preoperative and procedural data were collected. The postoperative course of patients with and without vascular complications was compared.
Results
A total of 878 patients met the inclusion criteria. Of these, 152 patients (17.3%) had an access-site related vascular complication (87 major complications, 9.9%). Sheath-to-femoral-artery-ratio (SFAR) (OR per 0.1 increase = 1.35, p < .001) and more than 2 vessel entries with large bore sheaths (OR = 1.76, p = .029) were independent risk factors for vascular complications. Female gender (OR = 1.44, p = .07) and two vessel entries with large bore sheaths (OR = 1.2, p = .53) increased the risk, although no statistical significance was shown. Age (OR = 1.07, p = .62), body mass index (OR = 1.1 per 5 points, p = .32) and vessel wall calcification at puncture site (OR = 0.93, p = .7) had no influence on vascular complications. Patients with vascular complications had a higher need for blood transfusion (p < .001) and a higher in-hospital mortality (2.6 vs. 0.4%, p = .019).
Conclusions
Procedural risk assessment should include SFAR calculation and consider the need for large bore sheath exchange. This might reduce the vascular trauma, lower vascular complication rates and improve the clinical outcome after TAVR.

© 2020 The Authors. Catheterization and Cardiovascular Interventions published by Wiley Periodicals LLC.

Catheter Cardiovasc Interv: 25 Jun 2020; epub ahead of print
Ruge H, Burri M, Erlebach M, Lange R
Catheter Cardiovasc Interv: 25 Jun 2020; epub ahead of print | PMID: 32588968
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Impact:
Abstract

Lonuigitudinal Strain and Strain Rate for Estimating Left Ventricular Filling Pressure in Heart Transplant Recipients.

Colak A, Muderrisoglu H, Pirat B, Eroglu S, ... Sezgin A, Sade LE

Traditional parameters have limited value to estimate left ventricular filling pressure (LVFP) in orthotopic heart transplant (OHT) recipients. We hypothesized that global longitudinal strain (GLS), diastolic and systolic strain rate (SR) would be depressed in OHT recipients with elevated LVFP and could overcome the limitations of traditional parameters. We studied consecutively OHT patients at the time of endomyocardial biopsies and retrospectively pre-transplantation studies conforming to the same protocol. Comprehensive echocardiography with strain measurements was performed. Results were compared to pulmonary capillary wedge pressure (PCWP) obtained from right heart catheterization that was performed just after the echocardiography study. In all, 74 studies were performed in 50 OHT recipients. Mean PWCP was 11.8±4.3 mmHg (range: 4-25 mmHg). Several parameters, but not left atrial volume index, mitral inflow velocities, annular velocities, and their ratio (E/e\'), were different between studies with normal (n=47) and elevated PCWP (n=27). Area Under Curve (AUC) for GLS (0.932*), E/e\' (0.849*), and systolic SR (0.848*) (*p<0.0001) were more accurate than traditional parameters for predicting PCWP>12 mmHg. GLS, systolic SR and E/e\' remained accurate regardless of LV ejection fraction and allograft vasculopathy. Meanwhile, E/e\' was accurate to predict PWCP in native failing hearts before transplantation. Changes in GLS and E/e\' tracked accurately changes in PCWP. In conclusion, traditional indices of diastolic function perform poorly in OHT recipients, whereas GLS and E/e\' provide reliable means of LVFP, irrespective of ejection fraction and allograft vasculopathy. These parameters also track reasonably well the changes in LVFP.

Copyright © 2020. Published by Elsevier Inc.

Am J Cardiol: 26 Sep 2020; epub ahead of print
Colak A, Muderrisoglu H, Pirat B, Eroglu S, ... Sezgin A, Sade LE
Am J Cardiol: 26 Sep 2020; epub ahead of print | PMID: 32998008
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Impact:
Abstract

Reshaping bicuspid aortic valve stenosis with an hourglass-shaped balloon for transcatheter aortic valve replacement: A pilot study.

Zhao ZG, Feng Y, Liao YB, Li YJ, ... Chen M, Leon MB
Objectives
We evaluated the safety and usefulness of preparatory anatomical reshaping with a geometric hourglass-shaped balloon to optimize transcatheter aortic valve replacement (TAVR) outcomes in bicuspid aortic valve (BAV) stenosis.
Background
TAVR has been increasingly performed for BAV stenosis; however, technical challenges remain. Procedural results are suboptimal given unfavorable valvular anatomies.
Methods
Eligible patients with BAV stenosis were enrolled to undergo aortic valve predilatation with the hourglass-shaped TAV8 balloon before TAVR using the self-expandable Venus A-Valve. Procedural details and outcomes were compared to a sequential group of patients with BAV who underwent TAVR with the same device following preparatory dilatation using a cylindrical balloon.
Results
A total of 22 patients were enrolled in the TAV8 group and 53 were included in the control group. Valve downsizing was less common in the TAV8 group (36.4 vs. 67.9%; p = .012). Stable valve release and optimal implant depth were consistently achieved in the TAV8 group with no requirement for a second valve (0 vs. 17.0%; p = .039) and with higher device success rates (100.0 vs 77.4%; p = .014). Residual aortic regurgitation graded as ≥mild was less common in the TAV8 group (13.6 vs 45.3%; p = .009). Mortality was similar (0 vs. 3.8%; p = 1); no major/disabling stroke or conversion to open-heart surgery was seen in either group within 30 days.
Conclusions
Compared with standard cylindrical balloon valvuloplasty, preparatory reshaping with the hourglass-shaped balloon before self-expandable TAVR in BAV was associated with significantly better procedural results and may encourage more promising outcomes.

© 2020 Wiley Periodicals, Inc.

Catheter Cardiovasc Interv: 13 Jan 2020; epub ahead of print
Zhao ZG, Feng Y, Liao YB, Li YJ, ... Chen M, Leon MB
Catheter Cardiovasc Interv: 13 Jan 2020; epub ahead of print | PMID: 31943783
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Impact:
Abstract

Low bone mineral density in adults with complex congenital heart disease.

Sandberg C, Johansson K, Christersson C, Hlebowicz J, Thilén U, Johansson B
Aims
The majority of children with complex congenital heart disease (CHD) survive into adulthood due to advances in medical care. Adult patients with CHD have an increased incidence of diagnoses related to ageing such as heart failure, dementia, cancer and sarcopenia, despite a relatively low age. They also have a shorter life expectancy. It is unknown if their bone structures also show signs of premature ageing. We therefore investigated Bone Mineral Content (BMC) and bone mineral density (BMD) in an adult population with complex CHD.
Methods
The total body BMC and BMD was examined using dual energy X-ray absorptiometry (DXA) in 73 adults with complex CHD (mean age 35.8 ± 14.3, women n = 22) and 73 age and sex matched controls.
Results
The adults with complex CHD had lower total body BMC (2.6 ± 0.5 kg vs. 2.9 ± 0.5 kg, p < 0.001) and BMD (1.18 ± 0.12 g/cmvs. 1.26 ± 0.11 g/cm, p < 0.001) compared to controls. BMD was lower for patients with single ventricle physiology and for the other complex diagnoses, and it persisted after correction for most common risk factors for osteoporosis.
Conclusion
Adults with complex CHD have reduced total body BMC and BMD compared to healthy controls. These results are a sign of frailty that conforms with other previously reported signs of premature ageing. The risk of osteoporosis is low in our relatively young population, but it is assumed to increase with ageing. We recommend that clinicians pay close attention to risk factors for osteoporosis, and are generous in administering DXA-measurements in order to prevent future fractures among adults with complex CHD.

Copyright © 2020. Published by Elsevier B.V.

Int J Cardiol: 03 Jul 2020; epub ahead of print
Sandberg C, Johansson K, Christersson C, Hlebowicz J, Thilén U, Johansson B
Int J Cardiol: 03 Jul 2020; epub ahead of print | PMID: 32634489
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Impact:
Abstract

Importance of Nonlongitudinal Motion Components in Right Ventricular Function: Three-Dimensional Echocardiographic Study in Healthy Volunteers.

Lakatos BK, Nabeshima Y, Tokodi M, Nagata Y, ... Kovács A, Takeuchi M
Background
Global right ventricular (RV) function is determined by the interplay of different motion components related to the myofiber architecture, and the relative importance of these components is still not thoroughly characterized. The aims of this study were to quantify the relative contributions of longitudinal, radial, and anteroposterior motion components to global RV function and to examine their determining factors in a large cohort of healthy volunteers using three-dimensional echocardiography.
Methods
Three hundred healthy adults with a balanced age range and an equal sex distribution were investigated at two centers. A three-dimensional mesh model of the right ventricle was generated, and its motion was decomposed along the three anatomically relevant axes. Multiplicative relative contributions were measured by dividing the ejection fraction (EF) values generated by shortening in the longitudinal, radial, and anteroposterior directions by global RV EF (longitudinal EF index [LEFi], radial EF index [REFi], and anteroposterior EF index, respectively). The circumferential contribution was defined as shortening in the radial and anteroposterior directions, omitting only longitudinal shortening.
Results
Circumferential EF index was markedly higher compared with LEFi (79 ± 7% vs 47 ± 9%, P < .001). LEFi (47 ± 9%) and anteroposterior EF index (49 ± 7%) were found to be similar in the pooled population, whereas REFi (44 ± 10%) was lower (P < .001). In younger individuals (20-39 years of age), the relative contribution of longitudinal shortening was significantly higher compared with the radial component; however, in the older age groups, LEFi and REFi were comparable. Age, body surface area, heart rate, and RV end-diastolic volume were independent predictors of LEFi and REFi, but all with opposite effects on the two motion directions.
Conclusions
In contrast to the traditional viewpoint, the contributions of the radial and anteroposterior motion directions may be of comparable significance with that of longitudinal shortening in determining global RV function. Standard parameters referring only to longitudinal shortening of the right ventricle may be inadequate to characterize RV function thoroughly.

Copyright © 2020 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

J Am Soc Echocardiogr: 29 Jun 2020; epub ahead of print
Lakatos BK, Nabeshima Y, Tokodi M, Nagata Y, ... Kovács A, Takeuchi M
J Am Soc Echocardiogr: 29 Jun 2020; epub ahead of print | PMID: 32620323
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Impact:
Abstract

Variation in the outer diameter of vascular sheaths commonly used in infant cardiac catheterization.

Mathis C, Romans R, Divekar A
Objective
To compare the outer diameter (OD) of conventional and radial sheaths from different manufacturers for sizes typically used in infant catheterization.
Background
The smallest sheath size is recommended to minimize risk of arterial injury in infants. However, sheath size refers to its internal diameter (ID), but it is the larger OD that determines risk. The OD varies between manufacturers and is frequently not known to the end user.
Methods
Three authors measured the OD of 3, 3.3, 4, 5, and 6 French (Fr) sheaths from different manufacturers using a Mitutoyo digital micrometer. Continuous variables are reported as mean ± SD. The midshaft and proximal-shaft sheath measurements were compared using an independent samples t test. Agreement between author measurements was tested using intra- and inter-rater reliability analysis. Manufacturer sheath OD measurements were obtained. Statistical significance was set at p < .05.
Results
There was no difference in the measured sheath diameters at the midpoint and proximally (p < .05). The intra-rater and inter-rater reliability were excellent (Intraclass correlation coefficient 1.0). Among conventional sheaths the OD varied between manufacturers, Terumo sheaths had the smallest and Galt sheaths had the largest OD. Radial sheaths had the smallest OD (1 Fr smaller) when compared to similar sized conventional sheaths. For instance, the OD of 4 Fr radial sheath (1.610 ± 0.006 mm) is essentially the same as the OD of the conventional 3 Fr (1.644 ± 0.016 mm) and 3.3 Fr (1.635 ± 0.005 mm) sheaths.
Conclusions
Our study shows variation in the OD of sheaths used in infant catheterization. The radial sheaths offer the smallest OD across sizes.

© 2020 Wiley Periodicals, Inc.

Catheter Cardiovasc Interv: 28 Feb 2020; epub ahead of print
Mathis C, Romans R, Divekar A
Catheter Cardiovasc Interv: 28 Feb 2020; epub ahead of print | PMID: 32112611
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Impact:
Abstract

Left ventricular myocardial deformation as measure of hemodynamic burden in congenital valvular aortic stenosis.

Reddy SC, Zhang J, Jani V, Wolfe SB, ... Kutty S, Pignatelli RH
Background
Changes in 2D echocardiography (2DE) speckle tracking imaging (STI) derived left ventricular (LV) strain (S) and strain rate (SR) precedes diminution of LV ejection fraction (LVEF) in adult valvular aortic stenosis (AS). We prospectively examined whether 2DE-STI derived multidirectional LV S and SR correlate with AS severity in children using LV mass index (MI) as the principal outcome variable.
Methods
52 children (10.4 ± 7.3 years) with isolated congenital AS were included; 13 mild (2.5 m/s < V < 3.0 m/s), 25 moderate (3.0 m/s < V < 4.0 m/s), and 14 severe (V > 4.0 m/s). 2DE including Doppler and STI longitudinal strain (LS), strain rate (LSR), circumferential strain (CS), and strain rate (CSR) were measured. Univariate and multivariable linear regressions identified correlations between LVMI and strain indices.
Results
Three clinical and 2DE variables, and four strain indices were independently associated with LVMI. LVMI correlated positively with systolic blood pressure and aortic regurgitation, and negatively with LVEF. LVMI correlated positively with LSR (four-chamber) and CSR (basal), and negatively with segmental CS in the inferior (basal) and anteroseptal (distal) segments. LVMI showed significant inverse association with GLS (P = .05), GLSR (P < .001), CS (P < .005), CSR (P < .0001), RSR (P < .001), independent of AS severity.
Conclusions
Independent of clinical and 2DE findings including contemporaneous Doppler estimates of AS gradient, both longitudinal and circumferential strain indices correlate with LVMI as a measure of cumulative hemodynamic burden. This association implies subclinical LV dysfunction.

Copyright © 2020. Published by Elsevier B.V.

Int J Cardiol: 13 Jul 2020; epub ahead of print
Reddy SC, Zhang J, Jani V, Wolfe SB, ... Kutty S, Pignatelli RH
Int J Cardiol: 13 Jul 2020; epub ahead of print | PMID: 32679139
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Impact:
Abstract

The ACTN3 577XX null genotype is associated with low left ventricular dilation-free survival rate in patients with Duchenne muscular dystrophy: ACTN3 genotype relates cardiomyopathy in DMD.

Nagai M, Awano H, Yamamoto T, Bo R, Matsuo M, Iijima K
Background
Duchenne muscular dystrophy (DMD) is a fatal progressive muscle-wasting disease caused by mutations in the DMD gene. Dilated cardiomyopathy is the leading cause of death in DMD; therefore, further understanding of this complication is essential to reduce morbidity and mortality.
Methods
A common null variant (R577X) in the ACTN3 gene, which encodes α-actinin-3, has been studied in association with muscle function in healthy individuals, however not yet examined with cardiac phenotype in DMD. Here, we determined the ACTN3 genotype in 163 Patients with DMD and examined the correlation between ACTN3 genotypes and echocardiographic findings in 77 of the 163 patients.
Results
The genotypes 577RR(RR), 577RX(RX), and 577XX(XX) were identified in 13 (17%), 44 (57%), and 20 (26%) of 77 patients, respectively. We estimated cardiac involvement-free survival rate analyses using Kaplan-Meier curves. Remarkably, the left ventricular (LV) dilation (LVDd>55 mm)-free survival rate was significantly lower in XX null genotype patients (P<0.01). XX null genotype showed a higher risk for LV dilation (hazard ratio 9.04).
Conclusions
This study revealed that ACTN3 XX null genotype was associated with a lower LV dilation-free survival rate in DMD. These results suggest that ACTN3 genotype should be determined at the time of diagnosis of DMD to improve patients\' cardiac outcomes.

Copyright © 2020. Published by Elsevier Inc.

J Card Fail: 09 Aug 2020; epub ahead of print
Nagai M, Awano H, Yamamoto T, Bo R, Matsuo M, Iijima K
J Card Fail: 09 Aug 2020; epub ahead of print | PMID: 32791185
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Impact:
Abstract

Heart transplantation at a single tertiary adult congenital heart disease centre: Too little, too late?

Merás P, Riesgo-Gil F, Rybicka J, Barradas-Pires A, ... Simon A, Gatzoulis MA
Background
Heart failure (HF) is the leading cause of death in adults with congenital heart disease (ACHD). Heart transplantation can be an effective therapy for them, albeit unfavourable anatomy, end-organ damage, pulmonary vascular disease, HLA sensitization and lack of robust selection criteria currently limit its application.
Methods
Consecutive CHD patients considered for heart or combined heart and lung transplantation at our tertiary ACHD centre between 2000 and 2018 constitute our study population. Baseline characteristics and outcome, including transplantation and death, were obtained for all patients from designated databases, medical records and the UK Office for National Statistics.
Results
From a total of more than 9000 active ACHD patients under follow-up, 166 (median age 40.4 years) fulfilled inclusion criteria, with a broad spectrum of underlying diagnosis: univentricular heart 22.3%, systemic right ventricle 22.3%, systemic-to-pulmonary shunts and Eisenmenger syndrome 16.3%, left sided valvular lesions 14.5%, tetralogy of Fallot 12.7%, CHD associated with cardiomyopathy 4.8% and other 7.2%. There was a high overall mortality with 39.2% of patients dying over a median follow-up of 2.7 years. A minority of patients (22.9%) were eventually listed and only 13.3% from the 166 patients were actually transplanted. Survival at 1 year after transplantation was 81.8% and remained high long-term (65.5% at 15 years). We describe herewith in detail characteristics and outcome of our cohort and present the transplantation pathway followed.
Conclusion
Of the small number of patients with ACHD considered for heart transplantation at a large tertiary centre, there was high overall mortality, with only a fraction of patients being actually transplanted. Patients who received transplantation, however, had a good outcome. Better patient selection and timing are clearly warranted so that more ACHD patients are considered and potentially benefit from this effective form of therapy.

Copyright © 2020. Published by Elsevier B.V.

Int J Cardiol: 12 Aug 2020; epub ahead of print
Merás P, Riesgo-Gil F, Rybicka J, Barradas-Pires A, ... Simon A, Gatzoulis MA
Int J Cardiol: 12 Aug 2020; epub ahead of print | PMID: 32798622
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Impact:
Abstract

Global Right Heart Assessment with Speckle-Tracking Imaging Improves the Risk Prediction of a Validated Scoring System in Pulmonary Arterial Hypertension.

Stolfo D, Albani S, Biondi F, De Luca A, ... Emdin M, Sinagra G
Background
Right ventricular (RV) function and right atrial (RA) remodeling are major determinants of outcome in pulmonary arterial hypertension (PAH). Strain echocardiography is emerging as a valuable approach for the study of RV and RA function. We sought to assess the incremental prognostic value of serial combined speckle-tracking examination of right chambers in newly diagnosed therapy-naïve PAH patients.
Methods
The study endpoint was a composite of all-cause mortality, hospitalizations due to worsening PAH, and initiation of parenteral prostanoids. Patients were assessed at baseline and at first revaluation after initiation of treatment. Right ventricular free-wall longitudinal strain (FWLS) and RA peak atrial longitudinal strain (PALS) were used as measures of RV and RA function.
Results
Eighty-three patients were included. Mean RV-FWLS and RA-PALS were -13.9% ± 6.1% and 23.1% ± 11.4%. The best performing prognostic score among the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension, French Pulmonary Hypertension Registry, and Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) scores was the REVEAL (area under the curve = 0.79, P < .001). With the identified cutoffs, both RV-FWLS (hazard ratio for RV-FWLS < -13.2% = 0.366; 95% CI, 0.159-0.842; P = .018) and RA-PALS (hazard ratio for RA-PALS > 20% = 0.399; 95% CI, 0.176-0.905; P = .028) were independently associated with the primary outcome after correction for the REVEAL score. The combined assessment of RV-FWLS and RA-PALS in addition to the REVEAL score determined a net improvement in prediction of 0.439 (95% CI, 0.070-0.888, P = .04). At 5 months (interquartile range, 4-8) of follow-up, RV-FWLS and RA-PALS improved significantly only in patients free from the primary outcome (P < .001 and P = .001, respectively).
Conclusions
The combined assessment of RV-FWLS and RA-PALS determined an improvement in outcome prediction of validated prognostic risk scores and should be considered within the multiparametric evaluation of patients with PAH.

Copyright © 2020 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

J Am Soc Echocardiogr: 30 Jul 2020; epub ahead of print
Stolfo D, Albani S, Biondi F, De Luca A, ... Emdin M, Sinagra G
J Am Soc Echocardiogr: 30 Jul 2020; epub ahead of print | PMID: 32747222
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Impact:
Abstract

Echocardiographic Assessment of Cardiac Function in Pediatric Survivors of Anthracycline-Treated Childhood Cancer.

Slieker MG, Fackoury C, Slorach C, Hui W, ... Nathan PC, Mertens L
Background
Anthracycline-induced cardiotoxicity is a major cause of morbidity and mortality in childhood cancer survivors (CCSs). Echocardiographic myocardial strain imaging is recommended in adult patients with cancer, but its role in pediatric CCSs has not been well established. Aims of this study were to determine the prevalence of abnormalities in left ventricular strain in pediatric CCSs, to compare strain with other echocardiographic measurements and blood biomarkers, and to explore risk factors for reduced strain.
Methods
CCSs ≥3 years from their last anthracycline treatment were enrolled in this multicenter study and underwent a standardized functional echocardiogram and biomarker collection. Regression analysis was used to identify factors associated with longitudinal strain (LS).
Results
Five hundred forty-six pediatric CCSs were compared with 134 healthy controls. Abnormal left ventricular ejection fraction (<50%) and mean LS ( score, <-2) was found in 0.8% and 7.7% of the CCSs, respectively. LS was significantly lower in CCSs than in controls, but the absolute difference was small (0.7%). Lower LS in CCSs was associated with older current age and higher body surface area. Sex, cumulative anthracycline dose, radiotherapy, and biomarkers were not independently associated with LS. Circumferential strain, diastolic parameters, and biomarkers were not significantly different in pediatric CCSs.
Conclusions
Global systolic function and LS are only mildly reduced in pediatric CCSs, and most LS values are within normal range. This makes single LS measurements of limited added value in identifying CCSs at risk for cardiac dysfunction. The utility of strain imaging in the long-term follow-up of CCS remains to be demonstrated.



Circ Cardiovasc Imaging: 29 Nov 2019; 12:e008869
Slieker MG, Fackoury C, Slorach C, Hui W, ... Nathan PC, Mertens L
Circ Cardiovasc Imaging: 29 Nov 2019; 12:e008869 | PMID: 31826678
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Impact:
Abstract

Left Ventricular Diastolic Function in Healthy Adult Individuals: Results of the World Alliance Societies of Echocardiography Normal Values Study.

Miyoshi T, Addetia K, Citro R, Daimon M, ... Asch FM,
Background
The World Alliance Societies of Echocardiography (WASE) study was conducted to describe echocardiographic normal values in adults and to compare races and nationalities using a uniform acquisition and measurement protocol. This report focuses on left ventricular (LV) diastolic function.
Methods
WASE is an international, cross-sectional study. Participants were enrolled with equal distribution according to age and gender. Echocardiograms were analyzed in a core laboratory based on the latest American Society of Echocardiography/European Association of Cardiovascular Imaging guidelines. Left ventricular diastolic function was assessed by E, E/A, e\' velocities, E/e\', left atrial volume index (LAVI), and tricuspid regurgitation velocity. Determination of LV diastolic function was made using the algorithm proposed by the guidelines.
Results
A total of 2,008 subjects from 15 countries were enrolled. The majority were of white or Asian race (42.8%, 41.8%, respectively). E and E/e\' were higher in female patients, while LAVI was similar in both genders. Consistent increase in E/e\' and decrease in E/A, E, and e\' were found as age increased. The upper limit of normal for LAVI was higher in WASE compared with the guidelines. The lower limits of normal for e\' were smaller in elder groups than those in the guidelines, while the upper limits of normal for E/e\' were below the guideline values. These findings suggest that the cutoff value for LAVI should be shifted upward and age-specific cutoff values for e\' should be considered. In WASE, <93.6% of patients were classified as normal LV diastolic function using the guidelines\' algorithm, and the proportion increased to 97.4% when applying the revised cutoff values for LAVI obtained in our study.
Conclusions
Guideline-recommended normal values for e\' velocities and LAVI should be reconsidered. The algorithm for the determination of LV diastolic function proposed by the guidelines is useful, but adjustments to LAVI could further improve it.

Copyright © 2020 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

J Am Soc Echocardiogr: 29 Jul 2020; epub ahead of print
Miyoshi T, Addetia K, Citro R, Daimon M, ... Asch FM,
J Am Soc Echocardiogr: 29 Jul 2020; epub ahead of print | PMID: 32741597
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Impact:
Abstract

Use of the Gore Viabahn VBX balloon-expandable endoprosthesis in the congenital heart disease population.

Cole JM, Salavitabar A, Armstrong AK, Berman DP

The Gore Viabahn VBX balloon-expandable endoprosthesis (W. L. Gore & Associates, Flagstaff, AZ) is a flexible covered stent that is FDA-approved for the treatment of iliac artery stenosis, including lesions at the aortic bifurcation. In this case series, we report the first use of the VBX covered stent in congenital heart disease, highlighting several of its unique advantages.

© 2019 Wiley Periodicals, Inc.

Catheter Cardiovasc Interv: 04 Jul 2019; epub ahead of print
Cole JM, Salavitabar A, Armstrong AK, Berman DP
Catheter Cardiovasc Interv: 04 Jul 2019; epub ahead of print | PMID: 31276274
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Impact:
Abstract

Novel shunt modification with an adjustable stent-embedded \"fenestrated\" septal occluder in a patient with pulmonary hypertension.

Yadlapati A, Wax D, Rich S, Ricciardi MJ

A 60-year-old woman with progressive dyspnea and cyanosis, O2-dependent pulmonary hypertension despite optimal medical therapy and remote atrial septostomy presented with worsening cyanosis and right-to-left shunting. The creation of a \"fenestrated\" ASD closure device with the insertion of a peripheral stent through an AMPLATZERâ„¢ ASD closure device was deployed to minimize right to left shunting and allow for enlargement of the shunt if needed. This case demonstrates the benefit of diminishing a right to left shunt with a self-fabricated fenestrated AMPLATZER device to improve symptoms in pulmonary hypertension patients with a pre-existing ASD.

© 2019 Wiley Periodicals, Inc.

Catheter Cardiovasc Interv: 31 May 2019; 93:1382-1384
Yadlapati A, Wax D, Rich S, Ricciardi MJ
Catheter Cardiovasc Interv: 31 May 2019; 93:1382-1384 | PMID: 30838741
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Impact:
Abstract

Diagnostic Accuracy of Fetal Echocardiography in Congenital Heart Disease.

Mozumdar N, Rowland J, Pan S, Rajagopal H, ... Srivastava S, Stern KWD
Background
The accuracy of fetal echocardiography (FE) is not well defined, and reporting of diagnostic discrepancies (DDs) is not standardized. The authors applied a categorization scheme developed by the American College of Cardiology Quality Metric Working Group and applied it to FE.
Methods
A retrospective single-center study was conducted of prenatally diagnosed major structural congenital heart disease, defined as expected need for intervention within the first year of life. DDs between pre- and postnatal findings were identified and categorized. Minor DDs had no clinical impact, moderate DDs had impact without harm, and severe DDs resulted in adverse events. Multivariate regression analysis was used to determine factors associated with discrepancy.
Results
From December 2008 to September 2017, 17,096 fetal echocardiograms were obtained, among which 222 fetuses with a median gestational age at first FE of 24 weeks were included. There were 30 DDs (13.5%), of which the majority were false negatives (56.7%). Most were minor or moderate in severity, with one severe DD. The majority were possibly preventable (90%), with the most common contributing factor being technical limitations (43.3%). The most common anatomic segment involved was the ventricular septum (23%), primarily missed septal defects. Comparing cases with DDs versus those without, those with DDs were more likely to have high anatomic complexity (16.7% vs 3.6%, P = .01), maternal comorbidities (40.0% vs 22.1%, P = .03), and a younger maternal age (median, 27 vs 30 years, P = .02). They were also more likely to have later gestation at initial FE (median, 29.5 vs 24 weeks, P = .003), to have fewer total fetal echocardiograms (median, 2 vs 3, P = .002), and to have a fellow as the initial sonographer (36.7% vs 16.7%, P = .03). There were no significant differences in maternal race/ethnicity, fetal comorbidities, and interpreting physician experience between cases with DDs and those without. On multivariate analysis, variables associated with DD included high anatomic complexity, maternal comorbidities, and fellow as initial imager. A greater number of fetal echocardiograms was associated with reduced DD.
Conclusions
FE had a DD rate of 13.5%, mostly minor and moderate in severity. Factors associated with DD included high anatomic complexity, maternal comorbidities, fellow as the initial sonographer, and fewer fetal echocardiograms. Strategies to reduce DD could include a regular secondary review and repeat FE, particularly when anatomic complexity is high.

Copyright © 2020 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

J Am Soc Echocardiogr: 18 Aug 2020; epub ahead of print
Mozumdar N, Rowland J, Pan S, Rajagopal H, ... Srivastava S, Stern KWD
J Am Soc Echocardiogr: 18 Aug 2020; epub ahead of print | PMID: 32828627
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Abstract

A comparative study of minimal lower-sternal incision device closure, minimal right thoracic incision device closure, and midsternal open repair of isolated perimembranous VSD, a retrospective cohort study.

Dai XF, Chen Q, Zhang GC, Chen LW
Objective
To compare transthoracic device closure via a minimal lower-sternal incision or minimal right thoracic incision and surgical repair via midsternal incision for perimembranous ventricular septal defect (VSD).
Methods
We retrospectively analyzed the clinical data of 147 patients with isolated perimembranous VSD who were treated in our hospital from June 2017 to December 2017. According to the therapeutic approaches, the patients were divided into group A(transthoracic device closure via a minimal lower-sternal incision), group B((transthoracic device closure via a minimal right thoracic incision) and group C(surgical repair via midsternal incision). The clinical data of the three groups were statistically analyzed.
Results
The three groups of patients had satisfactory outcomes for VSD closure. No complications, including third-degree atrioventricular block, large residual shunt requiring re-operation, newly moderate-severe aortic or tricuspid regurgitation, occluder detachment were reported. Compared with group C, the operative time, duration of mechanical ventilation, length of ICU stay, drainage volume, blood transfusion volume, length of the incision, and length of postoperative hospital stay were significantly lower in the device groups (A and B).
Conclusion
Transthoracic device closure via a minimal lower-sternal incision or minimal right thoracic incision and surgical repair via midsternal incision are sufficiently safe procedures for the treatment of isolated perimembranous VSD and can achieve satisfactory early clinical efficacy. Both device approaches have the advantages of a quick recovery and good cosmetic appearance of the incision.

Copyright © 2019 Elsevier B.V. All rights reserved.

Int J Cardiol: 21 Nov 2019; epub ahead of print
Dai XF, Chen Q, Zhang GC, Chen LW
Int J Cardiol: 21 Nov 2019; epub ahead of print | PMID: 31785954
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Abstract

Nonapical Right Ventricular Pacing Is Associated with Less Tricuspid Valve Interference and Long-Term Progress of Tricuspid Regurgitation.

Yu YJ, Chen Y, Lau CP, Liu YX, ... Tse HF, Yiu KH
Background
Tricuspid regurgitation (TR) is a well-known complication after permanent pacemaker implantation. The aim of this study was to compare the degree of TR and the relationship of lead position across the tricuspid valve (TV) between patients with right ventricular apical (RVA) and non-RVA pacing determined by three-dimensional echocardiography.
Methods
Conventional and three-dimensional echocardiography was performed in 284 patients to determine the change in TR severity following permanent pacemaker implantation. Transvenous lead locations were based on fluoroscopic images. This was a retrospective study, and the selected pacing mode was not randomized.
Results
RVA pacing had more frequent severe TR (37.9% vs 25.7%, P = .03) compared with non-RVA pacing. Severe TR occurred in 9.7%, 12.6%, and 58.8% of patients when the lead passed through the middle, between the commissures, and impinging the TV leaflets, respectively. Non-RVA leads were more likely to be positioned in the middle of the TV (30.3% vs 12.1%, P < .01) and had the lowest chance of leaflet impingement (33.6% vs 51.5%, P < .01) compared with RVA leads. RVA pacing was associated with worsening of grade ≥2 TR severity compared with non-RVA pacing (42.4% vs 27.6%, P < .01). A TV lead passage angle of -15° to 15° minimized TR.
Conclusions
Pacing-induced TR is more prevalent with RVA than non-RVA pacing. Preferential lead impingement on the TV leaflet, as determined by TV lead passage angle, can explain the development and progression of pacing-induced TR.

Copyright © 2020 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

J Am Soc Echocardiogr: 18 Aug 2020; epub ahead of print
Yu YJ, Chen Y, Lau CP, Liu YX, ... Tse HF, Yiu KH
J Am Soc Echocardiogr: 18 Aug 2020; epub ahead of print | PMID: 32828623
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Abstract

Percutaneous mitral valve repair in adults with congenital heart disease: Report of the first case-series.

Alshawabkeh L, Mahmud E, Reeves R
Background
Systemic atrioventricular valve regurgitation (AVVR) is frequently encountered in adults with congenital heart disease (CHD). Surgical intervention is the mainstay of therapy, but in a specific high-risk subset, percutaneous valve repair might offer a lower-risk alternative.
Methods
Three patients with complex CHD and severe symptomatic AVVR underwent percutaneous mitral valve repair at a single center. All were deemed to be high-risk for surgery by a multidisciplinary CHD team and provided informed consent for the compassionate use of the MitraClip (Abbott, Santa Clara, CA). Three-dimensional heart models were generated for the procedure, which was performed by an adult CHD cardiologist (who provided imaging support) and an interventional cardiologist with expertise in CHD and percutaneous mitral valve repair.
Results
The first case was a 39 year-old-woman with [S,L,D] dextrocardia, double outlet right ventricle, mild tricuspid hypoplasia, and a secundum atrial septal defect, who was palliated at age 35 with a right bidirectional Glenn and later developed severe, symptomatic mitral regurgitation, and underwent placement of one MitraClip XT device. Two patients with L-loop transposition of the great arteries each successfully underwent placement of two MitraClip XT devices; one patient had a single-leaflet detachment of one of the clips with no change in regurgitation or clip position on follow-up. All patients had significant reduction of AVVR and improvement in NYHA functional class.
Conclusions
Percutaneous atrioventricular valve repair in adults with CHD is feasible with the MitraClip but requires significant preprocedural planning and a multidisciplinary team that combines CHD and interventional therapeutic expertise.

© 2020 Wiley Periodicals LLC.

Catheter Cardiovasc Interv: 07 Sep 2020; epub ahead of print
Alshawabkeh L, Mahmud E, Reeves R
Catheter Cardiovasc Interv: 07 Sep 2020; epub ahead of print | PMID: 32898313
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Abstract

Initial experience with transcatheter pacemaker implantation for adults with congenital heart disease.

Russell MR, Galloti R, Moore JP
Background
Transvenous pacemaker systems have significant advantages over epicardial systems in patients with congenital heart disease (CHD). Frequently though, unique anatomic challenges preclude the use of transvenous leads. Although originally developed for patient with normal anatomy, leadless pacemaker systems have enormous potential in the CHD population.
Objective
To describe an initial experience with leadless pacemaker implantation in adult patients with CHD who were not the candidates for traditional transvenous pacing.
Methods
This was a retrospective review of the experience with Micra Transcatheter Pacing System implantation in adult patients with CHD. Patient demographics, clinical history, pacing indications, procedural details, clinical outcomes, and pacing characteristics at follow-up are reported.
Results
Three patients with intracardiac shunts or tricuspid valve disorders who underwent leadless pacemaker placement are described. Pacing indications included sinus node dysfunction in two and permanent atrial fibrillation with atrioventricular (AV) block in one. There were no procedural or thromboembolic complications over the follow-up period. Pacing characteristics were acceptable and ventricular pacing burden remained low except for the single patient with AV block.
Conclusions
Leadless pacemaker systems are a viable pacing option for appropriately selected adult patients with CHD when transvenous pacing is not a suitable option.

© 2019 Wiley Periodicals, Inc.

J Cardiovasc Electrophysiol: 30 Jul 2019; 30:1362-1366
Russell MR, Galloti R, Moore JP
J Cardiovasc Electrophysiol: 30 Jul 2019; 30:1362-1366 | PMID: 31045294
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