Tetralogy of Fallot or Pulmonary Atresia with Ventricular Septal Defect after the Age of 40 Years: A Single Center Study.

Julia Hock,Peter Ewert,Claudia Pujol,Alfred Hager,Renate Oberhoffer,Laurent Schwall,Oktay Tutarel

doi:10.3390/jcm9051533

Abstract

Background: The population of adults with tetralogy of Fallot (TOF) or pulmonary atresia with ventricular septal defect (PA/VSD) is growing and aging. Data regarding older patients are scarce. Prognostic outcome parameters in adults with TOF or PA/VSD ≥ 40 years were studied. Methods: This was a retrospective study of patients ≥ 40 years of age during the study period (January 2005–March 2018). Major adverse cardiac events (MACE) were a combined primary endpoint including death from any cause, prevented sudden cardiac death, pacemaker implantation, arrhythmia, and new-onset heart failure. Additionally, MACE II (secondary endpoint) was a combination of death from any cause and prevented sudden cardiac death. Results: 184 (58.7% female, mean age 45.3 ± 7.2 years) patients were included (159 (86.4%) TOF and 25 (13.6%) PA/VSD). During a median follow-up of 3.1 years (IQR: 0.6–6.5), MACE occurred in 35 and MACE II in 13 patients. On multivariable analysis, New York Heart Association class [HR: 2.1, 95% CI: 1.2–3.6, p = 0.009] emerged as an independent predictor for MACE, and age at corrective surgery [HR: 13.2, 95% CI: 1.6–107.1, p = 0.016] for MACE II. Conclusions: Adults with TOF or PA/VSD ≥ 40 years are burdened with significant morbidity and mortality. New York Heart Association class and age at corrective surgery were independent predictors of outcome.

Highlights

Due the advances in the treatment of congenital heart disease (CHD), more children survive to adulthood, leading to a new group of patients: adults with congenital heart disease (ACHD)
Tetralogy of Fallot (TOF) was one of the first CHD repaired by surgery beginning in the 1950s [3], leading to a better life expectancy
While in 2005, thirty-one patients (27 tetralogy of Fallot (TOF), 4 pulmonary atresia with ventricular septal defect (PA/VSD)) over the age of 40 were under follow-up, this number increased to 93 patients (78 TOF, 15 PA/VSD) in the year 2017

Summary

Introduction

Due the advances in the treatment of congenital heart disease (CHD), more children survive to adulthood, leading to a new group of patients: adults with congenital heart disease (ACHD). There is a growing number of ACHD patients over the age of 60 years with high mortality rates and higher utilization of healthcare resources compared with younger patients [1,2]. Acquired morbidities, such as coronary artery disease, seem to be key determinants of outcome in this older population in conjunction with the underlying CHD. Despite advances in treatment during the last six decades, TOF patients continue to be afflicted with increased mortality in comparison to the general population as they grow older [4]. New York Heart Association class and age at corrective surgery were independent predictors of outcome

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