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 resultsWe 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 (

Heart Failure and Patient‐Reported Outcomes in Adults With Congenital Heart Disease from 15 Countries

“…A recent study by Arnaert et al identified several risk factors associated with the presence of HF in adults with CHD including: older age, infective endocarditis, atrial arrhythmia, pacemaker, end‐organ dysfunction, advanced New York Heart Association class, faster heart rate, ventricular dysfunction, and pulmonary hypertension severity. 12 Consistent with our findings, they reported a higher prevalence of HF in patients with cyanotic CHD (41%), Fontan circulation (30%) and systemic right ventricle (25%). 12 Another study by Cohen et al identified factors associated with hospitalization for HF during the following year: age ≥50 years, male sex, CHD lesion severity, HF admission over the previous 12 months, pulmonary arterial hypertension, chronic kidney disease, coronary artery disease, systemic arterial hypertension, and diabetes.…”

“… 12 Consistent with our findings, they reported a higher prevalence of HF in patients with cyanotic CHD (41%), Fontan circulation (30%) and systemic right ventricle (25%). 12 Another study by Cohen et al identified factors associated with hospitalization for HF during the following year: age ≥50 years, male sex, CHD lesion severity, HF admission over the previous 12 months, pulmonary arterial hypertension, chronic kidney disease, coronary artery disease, systemic arterial hypertension, and diabetes. 13 The factors identified with HF in our study, including older age, complex CHD, arrhythmias, implantable cardioverter‐defibrillators, and comorbidities are coherent with the prior literature.…”

Late Fontan Circulatory Failure. What Drives Systemic Venous Congestion and Low Cardiac Output in Adult Fontan Patients?

“…The Fontan circulation provides a unique solution for patients with a single functional or anatomical ventricle, expanding life expectancy and quality of life. However, every Fontan circulation carries within itself the seeds of its own decay ( 69 ). Increased systemic venous pressures (and the subsequent development of Fontan-associated liver disease and abnormal lymphatics) and low cardiac output at rest, but especially during exercise (causing exercise intolerance), are the key components of every Fontan circulation and deteriorate slowly over time.…”

Identification of SOX18 as a New Gene Predisposing to Congenital Heart Disease

“…As a collective diagnosis for cardiovascular developmental deformities, CHD is anatomically categorized into >20 different clinical subtypes, encompassing pulmonary stenosis (PS), patent ductus arteriosus (PDA), atrial septal defect, and hypoplastic left heart [ 1 , 4 , 5 , 6 ]. Although some mild CHD can resolve spontaneously, severe CHD often leads to worse quality of life associated with health [ 7 , 8 , 9 ], reduced exercise tolerance [ 10 , 11 , 12 ], brain injury and neurodevelopmental anomaly [ 13 , 14 , 15 , 16 ], thromboembolism [ 17 , 18 ], infective endocarditis [ 19 , 20 ], pulmonary arterial hypertension [ 21 , 22 , 23 ], chronic kidney disease and acute kidney injury [ 24 , 25 , 26 ], impaired liver function [ 27 ], restrictive lung dysfunction [ 28 ], congestive heart failure [ 29 , 30 , 31 , 32 , 33 ], miscellaneous cardiac dysrhythmias [ 34 , 35 , 36 , 37 , 38 , 39 ], and cardiac demise [ 40 , 41 , 42 , 43 , 44 ]. Striking improvement has been achieved in pediatric cardiac surgical procedures and perioperative intensive care as well as transcatheter interventional treatment over recent decades, which dramatically alters the natural history of CHD, allowing ~95% o...…”