Autopsy in adults with congenital heart disease (ACHD)

Area of Science:

• Cardiovascular Pathology
• Medical Autopsy
• Congenital Heart Disease

Background:

• The adult congenital heart disease (ACHD) population now exceeds the pediatric congenital heart disease (CHD) population, comprising over 90% of all CHD cases.
• Approximately 75% of ACHD patients have undergone surgical or percutaneous interventions, leading to complex cardiac remodeling and diverse sequelae.
• Autopsy protocols for ACHD patients must account for native disease, palliative/corrective interventions, and evolving treatment eras.

Purpose of the Study:

• To provide pathologists with a systematic approach for conducting autopsies in adult congenital heart disease (ACHD) patients.
• To describe common congenital heart defects (CHDs) encountered in ACHD autopsies, including surgical/interventional procedures and pathological findings.
• To emphasize the importance of clinicopathological correlation in determining the cause of death in ACHD.

Main Methods:

• Review of common congenital heart disease types and associated surgical/interventional procedures.
• Description of major pathological manifestations in the context of ACHD.
• Proposal of a systematic dissection and autopsy protocol tailored for ACHD cases.

Main Results:

• Detailed overview of prevalent CHDs and their surgical/interventional histories in the ACHD population.
• Identification of key pathological findings and challenges specific to ACHD autopsies.
• Presentation of a structured autopsy methodology for improved diagnostic accuracy.

Conclusions:

• Standard autopsy methods are insufficient for the heterogeneous ACHD population.
• A systematic, clinicopathologically correlated approach is crucial for accurate diagnosis in ACHD autopsies.
• This paper provides a practical framework to guide pathologists through complex ACHD cases.