Chronic heart failure management in adult patients with congenital heart disease

Abstract

Purpose of reviewA growing number of adult patients with congenital heart disease (ACHD) are entering the healthcare system as a result of advances in the diagnosis and management of congenital heart defects. Heart failure is a common final pathway for this diverse patient population, representing the leading cause of mortality in ACHD patients. Herein, we review present guideline-directed management of heart failure in ACHD patients. Recent findingsThere exists a dearth of data to guide management of ACHD-related heart failure. Given this gap, recent guidelines have been limited in the recommendations they can provide for this patient population, with practitioners being consequently forced to generalize findings from studies of acquired heart disease patients based on mechanistic plausibility. The small number of studies directly assessing ACHD patients have been largely limited in their clinical relevance through being negative, small, observational, limited to specific subsets of ACHD patients or assessing nonvalidated outcomes. SummaryDespite the prevalence and impact of ACHD-related heart failure, there are limited evidence-based therapies for its management. Given the rising burden of this clinical problem, definitive trials assessing newer therapies are required to establish their potential role in heart failure amongst ACHD patients.

Discovery and functional investigation of BMP4 as a new causative gene for human congenital heart disease

“…As an array of cardiovascular developmental deformations, CHD is clinically categorized into > 30 distinct isoforms, encompassing double-outlet right ventricle (DORV) and ventricular septal defect (VSD) [2,[7][8][9][10][11][12][13][14]. Though certain mild/minor forms of CHD may resolve spontaneously [2], severe/complex forms of CHD usually lead to worse quality of life [15][16][17], reduced exercise performance [18][19][20][21], neurodevelopmental delay and structural brain anomaly [22][23][24][25][26], ischemic/thromboembolic stroke [27,28], acute renal injury/chronic kidney disease [29][30][31][32], hepatic fibrosis [33,34], pulmonary dysplasia/ pulmonary arterial hypertension [35][36][37], bacte-Am J Transl Res 2024;16 (5):2034-2048 rial endocarditis [38][39][40][41][42], chronic heart failure [43][44][45], supraventricular/ventricular arrhythmias [46][47]…”

Discovery of BMP10 as a new gene underpinning congenital heart defects

“…As a vast collection of cardiovascular developmental anomalies, CHD is clinically assorted to >26 diverse isoforms, including patent ductus arteriosus (PDA), aortic/pulmonary atresia, aortic/pulmonary stenosis, aortic coarctation, aortopulmonary window, atrial/ ventricular septal defect, tetralogy of Fallot (the Am J Transl Res 2024;16(1):109-125 commonest cyanotic CHD), atrioventricular septal defect, single ventricle, endocardial cushion defect, transposition of the major arteries, double outlet right ventricle, aortic arch interruption, abnormal coronary artery connection, cor triatriatum, and left heart hypoplasia/left ventricular noncompaction/spongy myocardium [2,[6][7][8][9][10][11]. Though some minor types of CHD do resolve spontaneously [2], severe types of CHD may give rise to degraded health-correlated quality of life [12][13][14][15], impaired exercise capacity [16][17][18], pulmonary arterial hypertension [19][20][21], acute brain injury and delayed neurodevelopment [22][23][24][25], thromboembolic/ischemic cerebral stroke [26][27][28], acute renal injury and chronic kidney disease [29][30][31], liver fibrosis and dysfunction [32], infective endocarditis [33][34][35][36][37], chronic/congestive heart failure [38][39][40], miscellaneous supraventricular and life-threatening ventricular dysrhythmias [41]…”