Capturing Adult Congenital Heart Disease: Framework for Development of an Adult Congenital Heart Disease Mortality Risk Model

Ann Thorac Surg. 2022 Nov;114(5):1762-1770. doi: 10.1016/j.athoracsur.2021.09.063. Epub 2021 Nov 3.

Abstract

Background: As a formative step toward development of adult congenital heart disease (ACHD) risk-adjusted metrics, we describe The Society of Thoracic Surgeons (STS) ACHD population, procedural frequency, and early mortality.

Methods: Adults (≥18 years) with CHD (2014-2019) were identified in the STS Adult Cardiac Surgery (ASCD) and the Congenital Heart Surgery (CHSD) Databases. After deduplication, variable mapping, data concatenation, and harmonization of preprocedure factors, procedures were grouped, and unadjusted mortality was catalogued for the overall cohort and the cohort excluding patients with an isolated bicuspid aortic valve (BAV).

Results: Among 171 186 ACSD and 18 281 CHSD records, 152 731 unique records met inclusion criteria. Twenty-eight congenital diagnoses accounted for 86% of the overall cohort, and prevalence of ACHD increased over the study time frame. ACHD patients underwent operations to treat both acquired and CHD. Most common procedures overall and after excluding isolated BAV were aortic valve replacement (AVR), ascending aortic surgery, and coronary artery bypass grafting (CABG). After excluding isolated BAV, major procedure combinations occurred in 46% (31% had 2 major procedures and 3% had ≥4), and the most prevalent combinations included AVR + CABG (n = 2352), AVR + subaortic stenosis repair (n = 1481), and AVR + ascending aortic surgery (n = 1239). Unadjusted 30-day mortality was procedure dependent, 2.8% overall and 3.6% with isolated BAV excluded.

Conclusions: The ACHD surgical population is heterogenous, and patients undergo surgery for CHD-related and adult/acquired procedure combinations. Early mortality is variable and influenced by surgical complexity. Excluding isolated BAV patients and developing procedure-based ACHD mortality risk models may be ideal but will require empirically derived grouping and collaboration.

MeSH terms

  • Adult
  • Aortic Valve / abnormalities
  • Aortic Valve / surgery
  • Aortic Valve Stenosis* / surgery
  • Bicuspid Aortic Valve Disease*
  • Heart Defects, Congenital* / surgery
  • Heart Valve Diseases* / diagnosis
  • Heart Valve Prosthesis Implantation*
  • Humans
  • Retrospective Studies
  • Treatment Outcome