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.

Keywords: ACHD; AP-ACHD classification; SF-36; atrial arrhythmia; congenital heart disease; quality of life.

Figure 1

Probability of the composite outcome for various AP-ACHD classes based on Kaplan–Meier estimates. Kaplan–Meier estimates derived from maximum likelihood estimation of hazard function for each separate AP-ACHD class. For instance, the probability of the composite outcome occurring to patients in the IIIB AP-ACHD class is 20% at 20 months of follow-up (median follow-up duration). The cohort did not include any patient classified as IID. Hence, the probability of the outcome cannot be estimated for this subgroup. AP-ACHD, anatomic and physiological classification of adult congenital heart disease.

Figure 2

Cumulative incidence curves of the composite outcome by (A) AnatC, and (B) PhyS. Cumulative incidence curves of the composite outcome by (A) AnatC and (B) PhyS. AnatC, anatomic class; PhyS, physiological stage.