Heart failure related to adult congenital heart disease: prevalence, outcome and risk factors

Abstract

Aims: Information on the prevalence, outcome and factors associated with heart failure in patients with adult congenital heart disease (CHD) (ACHD-HF) is lacking. We aimed at assessing the prevalence and outcome of ACHD-HF, the variables associated with ACHD-HF, and the differences between major anatomical/pathophysiological ACHD subgroups.

Methods and results: We included 3905 patients (age 35.4 ± 13.2 years) under active follow-up in our institution (last visit >2010). Outcome of ACHD-HF cases was compared with sex- and age-matched cases. Univariable and multivariable binary logistic regression with ACHD-HF diagnosis as a dependent variable was performed. Overall prevalence of ACHD-HF was 6.4% (mean age 49.5 ± 16.7 years), but was higher in patients with cyanotic CHD (41%), Fontan circulation (30%), and a systemic right ventricle (25%). All-cause mortality was higher in ACHD-HF cases when compared with controls (mortality rate ratio 4.67 (2.36-9.27); P = 0.0001). In multivariable logistic regression analysis, age at latest follow-up [per 10 years; odds ratio (OR) 1.52; 95% confidence interval (CI) 1.31-1.77], infective endocarditis (OR 4.11; 95%CI 1.80-9.38), history of atrial arrhythmia (OR 3.52; 95%CI 2.17-5.74), pacemaker implantation (OR 2.66; 95% CI 1.50-4.72), end-organ dysfunction (OR 2.41; 95% CI 1.03-5.63), New York Heart Association class (OR 9.28; 95% CI 6.04-14.25), heart rate (per 10 bpm; OR 1.27; 95% CI 1.08-1.50), ventricular dysfunction (OR 3.62; 95% CI 2.54-5.17), and pulmonary hypertension severity (OR 1.66; 95% CI 1.21-2.30) were independently related to the presence of ACHD-HF. Some variables (age, atrial arrhythmia, pacemaker, New York Heart Association, and ventricular dysfunction) were related to ACHD-HF in all anatomical/physiological subgroups, whereas others were not.

Conclusions: ACHD-HF is prevalent especially in complex CHD and is associated with poor prognosis. Our data provide insight in the factors related to ACHD-HF including differences between specific anatomical and physiological subgroups.

Keywords: ACHD; All-cause mortality; Congenital; Heart failure.

Figure 1

Adult congenital heart disease (ACHD)‐heart failure (HF) (ACHD‐HF) prevalence heat map. Heat map indicating the absolute number and proportion of patients with ACHD‐HF subdivided per age category and per ACHD subgroup. Although the proportion of patients with ACHD‐HF is substantially higher (at younger age) for cyanotic patients, Fontan patients and patients with a systemic right ventricle (RV), the absolute number of patients is higher in the ACHD subgroups with a biventricular circulation and systemic left ventricle (LV).

Figure 2

Adult congenital heart disease (ACHD)‐heart failure (HF) (ACHD‐HF) and all‐cause mortality. Inverse Kaplan–Meier plots for all‐cause mortality in 186 ACHD‐HF patients and 186 matched controls. All‐cause mortality was 4.67 times higher in ACHD‐HF patients as compared with their matched controls. AR, at risk; E, events; HF, heart failure.

Figure 3

Multivariable binary logistic regression analysis. Multivariable analysis for the entire cohort indicating (in red) factors that are independently related with the presence of adult congenital heart disease‐heart failure (ACHD‐HF) [missing cases: 334 (8.6%)]. BMI, body mass index; NYHA, New York Heart Association.

Figure 4

Univariable binary logistic regression analysis. Univariable analysis of factors related with the presence of adult congenital heart disease (ACHD)‐heart failure (ACHD‐HF) for individual ACHD anatomical/physiological subgroups (numbered 1–6 for cyanotic/Eisenmenger; Fontan; systemic right ventricle; predominant right‐sided residual lesions; shunt lesions; and predominant left‐sided residual lesions, respectively) for each risk factor assessed in the multivariable analysis of the entire cohort. BMI, body mass index; NYHA, New York Heart Association.