Effect of Losartan or Atenolol on Children and Young Adults With Bicuspid Aortic Valve and Dilated Aorta

doi:10.1016/j.amjcard.2020.12.050

The American Journal of Cardiology

Volume 144, 1 April 2021, Pages 111-117

https://doi.org/10.1016/j.amjcard.2020.12.050 Get rights and content

Highlights

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Bicuspid aortopathy increases aortic aneurysm and dissection risk.
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Medical prophylaxis reduces aortic growth rates in other types of aortopathy.
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Therapy with losartan or atenolol decreases aortic growth rates in young patients.

Bicuspid aortic valve aortopathy is defined by dilation of the aortic root (AoRt) and/or ascending aorta (AsAo), and increases risk for aortic aneurysm and dissection. The effects of medical prophylaxis on aortic growth rates in moderate to severe bicuspid aortopathy have not yet been evaluated. This was a single-center retrospective study of young patients (1 day to 29 years) with bicuspid aortopathy (AoRt or AsAo z-score ≥ 4 SD, or absolute dimension ≥ 4 cm), treated with either losartan or atenolol. Maximal diameters and BSA-adjusted z-scores obtained from serial echocardiograms were utilized in a mixed linear effects regression model. The primary outcome was the annual rate of change in AoRt and AsAo z-scores during treatment, compared with before treatment. The mean ages (years) at treatment initiation were 14.2 ± 5.1 (losartan; n = 27) and 15.2 ± 4.9 (atenolol; n = 18). Median treatment duration (years) was 3.1 (IQR 2.4, 6.0) for losartan, and 3.7 (IQR 1.4, 6.6) for atenolol. Treatment was associated with decreases in AoRt and AsAo z-scores (SD/year), for both losartan and atenolol (pre- vs post-treatment): losartan/AoRt: +0.06 ± 0.02 vs -0.14 ± 0.03, p < 0.001; losartan/AsAo: +0.20 ± 0.03 vs -0.09 ± 0.05, p < 0.001; atenolol/AoRt: +0.07 ± 0.03 vs -0.02 ± 0.04, p = 0.04; atenolol/AsAo: +0.21 ± 0.04 vs -0.06 ± 0.06, p < 0.001. Treatment was also associated with decreases in absolute growth rates (cm/year) for all comparisons (p ≤ 0.02). Medical prophylaxis reduced proximal aortic growth rates in young patients with at least moderate and progressive bicuspid aortopathy.

Section snippets

Methods

We conducted a single-center, retrospective pilot study of patients with BAV and aortic dilation (aged 1 day to 29 years) followed at Boston Children's Hospital (BCH) from 1990 to 2018. Management of BAV aortopathy was organized by a center-specific, quality-improvement Standardized Clinical Assessment and Management Plan (SCAMP).²⁴ The SCAMP specified medical prophylaxis with either losartan or atenolol for patients with severe dilation of the AoRt and/or AsAo. The treating physician

Results

A total of 41 patients who met study criteria were identified. Four patients were included in both treatment groups, having received each drug at different times, resulting in 27 patients in the losartan group and 18 in the atenolol group. Baseline pre-treatment characteristics (Table 1A) were generally similar in the 2 treatment groups, excluding patients from the losartan (n = 5) and atenolol (n = 4) groups without serial pre-treatment echocardiographic data. The mean age at initiation of

Discussion

BAV is a common heart defect associated with aortic aneurysm, dissection and death during adulthood. This pilot study addresses the utility of medical prophylaxis for proximal aortic enlargement in young patients with BAV aortopathy. Our study results suggest that both angiotensin II receptor blockers and beta blockers reduce the rate of growth of the proximal aorta in patients with moderate to severe BAV-associated aortopathy. Before initiation of medical prophylaxis, AoRt and AsAo z-scores

Disclosures

None

Declaration of Competing Interest

The authors declare that they have no known competing financial interests or personal relations that could have appeared to influence the work reported in this study.

Acknowledgments

The research team thanks the New England Congenital Cardiology Association and its membership for encouragement and collaboration. The team is grateful to Kathryn Lemay, Sabryna Therrien, and Marcia Dubuque for administrative support.

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Cited by (4)

Serial Magnetic Resonance Imaging for Aortic Dilation in Tetralogy of Fallot With Pulmonary Stenosis
2023, American Journal of Cardiology
Aortic dilation occurs in patients with repaired tetralogy of Fallot (TOF), but the rate of growth is incompletely characterized. The aim of this study was to assess the rates of growth of the aortic root and ascending aorta in a cohort of pediatric and adult patients with sequential magnetic resonance angiography Magnetic Resonance Imaging (MRI) data. Using serial MRI data from pediatric and adult patients with repaired TOF, we performed a retrospective analysis of the rates of growth and associations with growth of the aortic root and ascending aorta. Patients with pulmonary atresia or absent pulmonary valve were excluded. Between years 2005 to 2021, a total of 99 patients were enrolled. A follow-up MRI was performed an average of 5.9 ± 3.7 years from the initial study. For the cohort aged ≥16 years, the mean rate of change in diameter was 0.2 ± 0.5 mm/year at the ascending aorta and 0.2 ± 0.6 mm/year at the sinus of Valsalva. For the entire cohort, the mean change in cross-sectional area indexed to height at the ascending aorta was 7 ± 12 mm²/m/year and at the sinus of Valsalva was 10 ± 16 mm²/m/year. Younger age was associated with higher rates of growth of the sinus of Valsalva while the use of β blockers or angiotensin-converting enzyme inhibitors was associated with a slower rate of growth. There were no cases of aortic dissection in this cohort. We conclude that serial MRI demonstrates a slow rate of growth of the aorta in the TOF.
New England Congenital Cardiology Association Bicuspid Aortopathy Registry (NECCA BAR): A regional preventive cardiovascular care collaboration
2022, Progress in Pediatric Cardiology
Citation Excerpt :
Our pediatric cardiology clinical practice aim is to decrease aortic growth rates during childhood, and potentially reduce disease-related risk for aortic surgery, aortic dissection, and sudden death during adulthood. We recently published a report showing that medical therapy decreases proximal aortic growth rates in young BAV patients with progressive dilation [17]. We integrated this evidence into a New England preventive care strategy, including regional pediatric bicuspid aortopathy practice guidelines and a patient registry.
Bicuspid aortic valve is the most common congenital heart defect, and its association with proximal aortic dilation (aortopathy) increases risk for aortic aneurysm and dissection in adults. Maximal aortic root and ascending aortic dimensions are predictors of adverse cardiovascular outcomes. Recent single center data demonstrate medical therapy with beta or angiotensin II receptor blockers tempers aortic growth rates. Although randomized clinical trials may not be feasible, larger population studies would better inform bicuspid aortic valve preventive cardiovascular care.
Improve regional pediatric bicuspid aortopathy collaboration with standardized medical management guidelines and patient care registry.
The New England Congenital Cardiology Association (NECCA) developed consensus-based prophylactic treatment guidelines for bicuspid aortopathy and designed a regional clinical registry (NECCA BAR) to study cardiovascular practice variation and short-term outcomes in subjects with moderate to severe bicuspid aortopathy. Individuals 6 months to 25 years of age with bicuspid aortic valve (all variants) and body surface area-adjusted aortic root or ascending aorta z score ≥ 4.0 SD, and/or absolute dimension ≥4.0 cm, are eligible for inclusion. Standardized bicuspid aortopathy medical management guidelines were established among New England congenital cardiology practices. The primary aims are to compare cardiovascular practice variation by guideline adherence (medical therapy and athletic participation), and evaluate medication effectiveness by comparing rates of annual change in aortic root and ascending aorta dimensions/z scores over 3 years. Additional endpoints include aortic valve disease progression (stenosis/regurgitation), incidence of aortic dissection, surgery, death, and incidence of adverse drug events.
Recruitment in progress; data analysis pending.
We describe the development of regional pediatric clinical care guidelines and a patient care registry designed to refine medical management of bicuspid aortopathy.
Bicuspid aortic valve and its ascending aortopathy
2023, Current Opinion in Pediatrics
The Global Burden of Valvular Heart Disease: From Clinical Epidemiology to Management
2023, Journal of Clinical Medicine

: Grant Support/Funding: none

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Effect of Losartan or Atenolol on Children and Young Adults With Bicuspid Aortic Valve and Dilated Aorta

Highlights

Section snippets

Methods

Results

Discussion

Disclosures

Declaration of Competing Interest

Acknowledgments

Am J Cardiol

Am J Cardiol

J Am Coll Cardiol

Am J Cardiol

J Am Soc Echocardiogr

Am J Cardiol

J Am Coll Cardiol

Am J Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

Lancet

J Am Soc Echocardiogr

J Am Soc Echocardiogr

Am J Cardiol

Ann Thorac Surg

J Thorac Cardiovasc Surg