The Adult Congenital Heart Disease Anatomic and Physiological Classification: Associations with Clinical Outcomes in Patients with Atrial Arrhythmias

Abstract

The implications of the adult congenital heart disease anatomic and physiological classification (AP-ACHD) for risk assessment have not been adequately studied. A retrospective cohort study was conducted using data from an ongoing national, multicentre registry of patients with ACHD and atrial arrhythmias (AA) receiving apixaban (PROTECT-AR study, NCT03854149). At enrollment, patients were stratified according to Anatomic class (AnatC, range I to III) and physiological stage (PhyS, range B to D). A follow-up was conducted between May 2019 and September 2021. The primary outcome was a composite of death from any cause, any major thromboembolic event, major or clinically relevant non-major bleeding, or hospitalization. Cox proportional-hazards regression modeling was used to evaluate the risks for the outcome among AP-ACHD classes. Over a median 20-month follow-up period, 47 of 157 (29.9%) ACHD patients with AA experienced the composite outcome. Adjusted hazard ratios (aHR) with 95% confidence intervals (CI) for the outcome in PhyS C and PhyS D were 1.79 (95% CI 0.69 to 4.67) and 8.15 (95% CI 1.52 to 43.59), respectively, as compared with PhyS B. The corresponding aHRs in AnatC II and AnatC III were 1.12 (95% CI 0.37 to 3.41) and 1.06 (95% CI 0.24 to 4.63), respectively, as compared with AnatC I. In conclusion, the PhyS component of the AP-ACHD classification was an independent predictor of net adverse clinical events among ACHD patients with AA.

Discovery of GJC1 (Cx45) as a New Gene Underlying Congenital Heart Disease and Arrhythmias

“…Based on specific anatomic deformities, CHD has been clinically classified into over 25 distinctive entities, including tetralogy of Fallot (TOF), Ebstein’s anomaly, atrial septal defect (ASD), truncus arteriosus, ventricular septal defect (VSD), coarctation of the aorta, interrupted aortic arch, double outlet right ventricle (DORV), endocardial cushion defect (ECD), atrioventricular septal defect (AVSD), hypoplasia of the left ventricle, aortic stenosis, transposition of the great arteries, anomalous pulmonary venous connection, and patent ductus arteriosus [ 2 ]. Although slight lesions may resolve spontaneously [ 2 ], severe CHD, which accounts for almost one-third of all types of CHD [ 1 ], requires timely medical intervention or surgery, and otherwise may lead to diminished exercise performance and degraded health-associated quality of life [ 3 , 4 , 5 , 6 , 7 ], delayed central nervous development and brain damage [ 8 , 9 , 10 ], ischemic cerebral stroke [ 11 , 12 ], pulmonary arterial hypertension and impaired pulmonary function [ 13 , 14 , 15 , 16 ], chronic kidney disease and acute renal injury [ 17 , 18 , 19 , 20 ], infective endocarditis [ 21 , 22 , 23 , 24 ], aortic dissection and rupture [ 25 ], chronic heart failure [ 26 , 27 ], cardiac dysrhythmias [ 28 , 29 , 30 ], and cardiac premature demise [ 31 , 32 , 33 , 34 ]. Over the past several decades, significant improvement in drug therapy and surgical treatment of CHD has been achieved, which remarkably changes the natural history of severe CHD, allowing over 90% of CHD newborns to survive into adulthood [ 1 ].…”

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...…”

Association of Health Status Metrics with Clinical Outcomes in Patients with Adult Congenital Heart Disease and Atrial Arrhythmias

“…Atrial arrhythmias are the most common cardiovascular comorbidity encountered in the course of ACHD, affecting up to 20% of these patients [ 3 ]. In the ACHD setting, atrial arrhythmias have been described as a major source of impaired quality of life (QoL) and burdening symptoms [ 3 , 4 , 5 , 6 ].…”