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

Extract

Heart failure in congenital heart disease: prevalence and outcome

Improved 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 patient-years 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.