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

Anastasios Kartas,Alexandra Frogoudaki,Haralambos Karvounis,George Giannakoulas,Dimosthenis Avramidis,Andreas S Papazoglou,Ioannis Doundoulakis,Georgios P Rampidis,Tereza Mousiama,Sotiria Apostolopoulou,Dimitrios V Moysidis,Stefanos Despotopoulos,Despoina Ntiloudi,Elena Vrana,Afrodite Tzifa,Diamantis Kosmidis,Sotiria Liori,Amalia Baroutidou,Athanasios Koutsakis

doi:10.3390/diagnostics12020466

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.

Highlights

The advances of modern medicine have extended the survival of patients with congenital heart disease (CHD) well into adulthood
The study is being conducted at four centers in the two largest cities in Greece, which serve the majority of the country’s adults with congenital heart disease (ACHD) population
AP-ACHD, anatomic and physiological classification of adult congenital heart disease; BMI, body mass index; CHA2DS2-Vasc; HAS-BLED (Hypertension, Abnormal renal/liver function, Stroke, Bleeding history or predisposition, Labile INR, Elderly, Drugs/alcohol); IQR, interquartile range; NT-proBNP, N-terminal pro-BNP; mEHRA, modified European Heart Rhythm Association; SD, standard deviation; SF-36, Short Form-36. In this retrospective analysis of a prospectively followed-up cohort of ACHD patients with AA on apixaban, the physiological stage (PhyS) component of the AP-ACHD classification was independently associated with a composite of fatal and major non-fatal adverse outcomes

Summary

Introduction

The advances of modern medicine have extended the survival of patients with congenital heart disease (CHD) well into adulthood. As these patients live longer, the focus of care expectantly shifts towards indexes of morbidity [1]. Reliable risk assessment in ACHD with AA is necessary This process can be challenging, owing to the multifactorial aspects that affect prognosis in these patients. Anatomic complexity was originally described in 2001 by the Bethesda Task Force [7]. Since it relies solely on anatomy, that classification scheme does not reflect the hemodynamic, arrhythmic, or functional disorders which may complicate CHD. Despite the potential of this new classification, little data exist on its association with adverse outcomes, especially in patients with comorbid AA [12,13,14]

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Results

Conclusion