Systolic dysfunction of the subpulmonary left ventricle is associated with the severity of heart failure in patients with a systemic right ventricle
Introduction
Patients with congenitally corrected transposition of great arteries (ccTGA) or transposition of great arteries (TGA) after Mustard or Senning operation usually pose a particular challenge in their management during adulthood due to systemic right ventricular (SRV) dysfunction, systemic tricuspid valve regurgitation, baffle obstruction or leaks, arrhythmias and sudden cardiac death [1].
Impaired systolic function of the SRV is associated with limited exercise capacity [[2], [3], [4], [5]], heart failure and increased mortality [6,7]. Subpulmonary left ventricular (LV) dysfunction has also been reported in this population [8], however its contribution to the development of symptoms remains unclear. Routine echocardiographic evaluation of the LV systolic function in patients with SRV is not fully established and little is known on whether it has any clinical significance.
The aim of the present study was to assess the relation between systolic dysfunction of subpulmonary LV and the severity of heart failure symptoms in patients with a SRV.
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Study design and population
From our digitally recorded echocardiographic database within a large tertiary center for adult congenital heart disease, we identified all patients with the diagnosis of SRV who had at least one transthoracic echocardiography (TTE) study from October 2014 to August 2018. If a patient had more than one TTE during this time span, the most recent TTE was used for the analysis.
Demographic and clinical data (age, body surface area, body mass index, cardiovascular risk factors, NYHA functional
Patient characteristics
We identified 157 patients with a SRV: 56.7% male, median age 40.6 [33.1–46.8] years (Table 1). Eighty-nine (56.7%) had TGA and had previously undergone Mustard or Senning operations, while 68 (43.3%) had a diagnosis of ccTGA. The majority of patients (133, 84.7%) were in NYHA functional class 1–2. One hundred patients (63.7%) were receiving heart failure treatment and 35 (22.3%) had an implanted cardioverter-defibrillator. Median B-type natriuretic peptide (BNP) concentration was 79.5
Discussion
This study, on a large cohort of patients with a SRV, shows that subpulmonary LV systolic dysfunction is independently associated with NYHA functional class 3–4, and this association is stronger than for SRV dysfunction. LV FAC rather than MAPSE provides a reasonably accurate quantitative echocardiographic assessment of subpulmonary LV systolic function when compared to CMR-derived LV EF. These findings demonstrate that subpulmonary LV function has a significant impact on the symptoms and
Conclusions
Subpulmonary LV dysfunction is independently associated with NYHA class 3–4 heart failure in patients with SRV. LV FAC is more accurate than SRV FAC in predicting worse functional class and suggests that the LV plays an important role in defining the clinical picture, and potentially, prognosis of these patients. Subpulmonary LV size and function should be accurately assessed in SRV patients and be taken into account in clinical decision making and timing interventions.
The following are the
Funding
This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Disclosures
All authors have no relationships with industry relevant to the content of this paper to disclose.
References (27)
- et al.
Relation of systemic ventricular function quantified by myocardial performance index (Tei) to cardiopulmonary exercise capacity in adults after Mustard procedure for transposition of the great arteries
Am. J. Cardiol.
(2005) - et al.
Factors associated with exercise capacity in patients with a systemic right ventricle
Int. J. Cardiol.
(2019) - et al.
Systemic right ventricular longitudinal strain is reduced in adults with transposition of the great arteries, relates to subpulmonary ventricular function, and predicts adverse clinical outcome
Am. Heart J.
(2012) - et al.
Right ventricular function with standard and speckle-tracking echocardiography and clinical events in adults with D-transposition of the great arteries post atrial switch
J. Am. Soc. Echocardiogr.
(2012) - et al.
Consensus recommendations for echocardiography in adults with congenital heart defects from the International Society of Adult Congenital Heart Disease ( ISACHD )
Int. J. Cardiol.
(2018) - et al.
Right ventricular systolic dysfunction at rest is not related to decreased exercise capacity in patients with a systemic right ventricle
Int. J. Cardiol.
(2018) - et al.
Right ventricular dysfunction assessed by cardiovascular magnetic resonance imaging predicts poor prognosis late after myocardial infarction
J. Am. Coll. Cardiol.
(2007) - et al.
Right ventricular function and prognosis in stable heart failure patients
J. Card. Fail.
(2014) - et al.
Dyssynchrony and electromechanical delay are associated with focal fibrosis in the systemic right ventricle - insights from echocardiography
Int. J. Cardiol.
(2016) - et al.
Left ventricular outflow tract obstruction and its impact on systolic ventricular function and exercise capacity in adults with a subaortic right ventricle
Int. J. Cardiol.
(2017)
Comparison of transthoracic echocardiography versus cardiovascular magnetic resonance imaging for the assessment of ventricular function in adults after atrial switch procedures for complete transposition of the great arteries
Am. J. Cardiol.
Echocardiogram versus cardiac magnetic resonance imaging for assessing systolic function of subaortic right ventricle in adults with complete transposition of great arteries and previous atrial switch operation
Am. J. Cardiol.
Global longitudinal strain may identify preserved systolic function of the systemic right ventricle
Can. J. Cardiol.
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This author takes responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation.