Treatment of heart failure in adult congenital heart disease: a position paper of the Working Group of Grown-Up Congenital Heart Disease and the Heart Failure Association of the European Society of Cardiology

Abstract

Heart failure in congenital heart disease: prevalence and outcomeImproved medical care of congenital heart disease patients increased survival into adulthood from 15% in the 1960s to over 85% in the current era.As a consequence, the prevalence of adult congenital heart disease (ACHD) increased rapidly, 1 which is estimated to be .1 million ACHD patients in North America and 1.2 million in Europe.The growing number and aging of ACHD patients led to an overall increase in hospitalizations over the last decade and a substantial increase in patients presenting with heart failure (HF) ( 20%). 2 The incidence of first HF-admission was 1.2 per 1000 patientyears in the Dutch national 'CONCOR' registry.Patients admitted with HF had a five-fold higher risk of death than those not admitted.From the same registry, the mortality was 2.8% during a follow-up period of 24 865 patient-years.Chronic HF (26%) and sudden death (19%) were recorded most frequently.The median age at death from HF was 51.0 years (range: 20.3 -91.2 years).3 In another ACHD cohort, sudden death (26%) was the most common cause of death, followed by progressive HF (21%) and perioperative death (18%).4 Although patients with ACHD may not readily report symptoms, clinical HF is documented in 22.2% of patients with a Mustard repair for transposition of the great arteries (TGAs), 32.3% with congenitally corrected transposition of the great arteries (ccTGA), and 40% of patients after Fontan palliation. Pathophysiology of heart failure in adult congenital heart diseaseHeart failure with impaired systolic ventricular functionThe aetiology and triggers of impaired systolic ventricular function in ACHD patients are summarized in Table 1. Heart failure with preserved systolic ventricular functionThis occurs less often in ACHD patients, but is associated with specific conditions such as Shone complex and restrictive right ventricular (RV) physiology in the context of pulmonary atresia, ventricular septal defect (VSD), and major aorto-pulmonary collateral arteries. Genetic and neurohormonal backgroundHeart failure in ACHD is the result not only of a structural defect but also of defective contractility or conduction.An intriguing and emerging hypothesis is that genes involved in morphogenesis duringThe opinions expressed in this article are not necessarily those of the Editors of the European Heart Journal or of the European Society of Cardiology.