Development of heart failure in young patients with congenital heart disease: a nation-wide cohort study

Abstract

Objective: Heart failure (HF) is a common cause of hospitalisation and death in adults with congenital heart disease (CHD). However, the risk of HF in young patients with CHD has not been determined.

Methods: By linkage of national patient registers in Sweden, we identified 21 982 patients with CHD born between 1970 and 1993, and compared these with 10 controls per case. Follow-up data were collected from birth until 2011 or death.

Results: Over a mean follow-up of 26.6 years in patients with CHD and 28.5 years in controls, 729 (3.3%) and 75 (0.03%) developed HF, respectively. The cumulative incidence of HF in all CHD was 6.5% and in complex CHD 14.8% up to age 42 years. Thus, one patient in 15 with CHD runs the risk of developing HF before age 42 years, a risk that is 105.7 times higher (95 % CI 83.2 to 134.8) compared with controls. For patients with complex CHD (such as conotruncal defects, univentricular hearts, endocardial cushion defects), one in seven will develop HF, a HR of 401.5; 95% CI 298 to 601 as compared with controls. The cumulative probability of death in patients with CHD, after HF diagnosis, was 63.4% (95% CI 57.5 to 69.3).

Conclusions: An extremely high risk of developing HF (more than 100-fold) was found in patients with CHD, compared with matched controls, up to the age of 42 years. Patients with complex congenital heart malformations carried the highest risk and have to be considered as the main risk group for developing HF.

Keywords: congenital; epidemiology; heart defects; heart failure.

Figure 1

Cumulative incidence of HF in the study population, with death as a competing event. Solid black line indicates HF among controls, dashed black line indicates death among controls, solid red line indicates HF among patients with CHD and dashed red line indicates death among patients with CHD. CHD, congenital heart disease; HF, heart failure.

Figure 2

Cumulative incidence of heart failure in patients with CHD and controls according to a hierarchical classification. Lesion group 1 was defined as patients with conotruncal defects, including common arterial trunk, transposition of the great arteries, tetralogy of Fallot or aortopulmonary septum defect. Lesion group 2 was defined as patients with nonconotruncal defects, including endocardial cushion defects, common ventricle and hypoplastic left heart syndrome. Lesion group 3 was defined as patients with coarctation of the aorta. Lesion group 4 was defined as patients with ventricular septal defects. Lesion group 5 was defined as patients with atrial septal defects. Lesion group other included all other heart and circulatory system anomalies and all other CHD diagnoses that were not included in the other five lesion groups. CHD, congenital heart disease.

Figure 3

Mortality (95% CI) in patients and controls with HF (right panel), compared with those without HF (left panel). Red lines indicate patients with congenital heart disease, blue lines represent controls and dashed lines indicate 95% CIs. HF, heart failure.