Abstract
Background
In complete transposition of the great arteries (TGA) with previous atrial switch operation and congenitally corrected transposition of the great arteries (ccTGA), the morphological right ventricle and its tricuspid valve support the systemic circulation. This results in late complications, including systemic right ventricle (sRV) failure, systemic atrioventricular valve regurgitation, and rhythm disturbances, affecting long-term outcome. We aimed to describe contemporary survival and determine predictive factors of death in an adult cohort with a sRV.
Methods and results
All adult patients (>16 years of age) with a sRV under active follow-up between January 2000 and December 2018 in our tertiary center were included. Demographic and clinical details were obtained from records. Baseline clinical status, transthoracic echocardiography, cardiopulmonary exercise testing and BNP were taken at the earliest available assessment during the study period.
Two hundred thirty-six patients, 141 with TGA/ 95 ccTGA, were included (mean age at baseline 31±12 years, 45% female sex). Over a median follow-up of 10.2 years IQR [5.6–14.9], 27 patients died, and 2 patients underwent heart transplantation. Cause of death was heart failure in 13 patients, sudden cardiac death in 2, and unspecified in 12. Survival at 5, 10 and 15 years of follow-up was 97.5%, 90.0% and 81.0%, respectively (Figure 1A).
On univariate Cox regression analysis, history of atrial arrhythmia, heart failure, and pulmonary arterial hypertension, baseline NYHA functional class ≥2, BNP≥120ng/L, peak VO2 and moderate/severe sRV function were predictive of death/heart transplantation. In contrast, previous permanent pacing, moderate or severe pulmonary stenosis, and use of beta-blockers were not associated with an increased risk of death. On multivariate analysis, NYHA ≥2 (HR=5.3 95% CI [1.1–25.6], p=0.03) and peak VO2 (HR=0.95 95% CI [0.91–0.99], p=0.01) were the strongest independent predictors of death. Patients with NYHA class≥2 and/or a pVO2<60% of predicted had a 3.6-fold higher risk of death compared to the remainder, even after adjustment for cardiac treatment at baseline (p=0.03, Figure 1B)
Figure 1. (A) Long term survival with 95% confidence intervals. (B) Survival according to the presence of symptoms (NYHA functional class ≥2) and/or a peak VO2 <60% predicted.
Conclusions
Mortality remains considerable in a contemporary cohort of adults with a sRV. Heart failure appears to be the leading cause of death, whereas only a few patients benefit from transplantation. Symptomatic patients with moderate or severe reduction in peak VO2 are at increased risk of adverse outcome in this setting and merit a more proactive approach and management.
Acknowledgement/Funding
Fundaciόn Alfonso Martín Escudero
Exercise training improves exercise capacity in adult patients with a systemic right ventricle: a randomized clinical trial
