Autopsy in adults with congenital heart disease (ACHD)

Abstract

The adult congenital heart diseases (ACHD) population is exceeding the pediatric congenital heart diseases (CHD) population and is progressively expanding each year, representing more than 90% of patients with CHD. Of these, about 75% have undergone surgical and/or percutaneous intervention for palliation or correction. Autopsy can be a very challenging procedure in ACHD patients. The approach and protocol to be used may vary depending on whether the pathologists are facing native disease without surgical or percutaneous interventions, but with various degrees of cardiac remodeling, or previously palliated or corrected CHD. Moreover, interventions for the same condition have evolved over the last decades, as has perioperative myocardial preservations and postoperative care, with different long-term sequelae depending on the era in which patients were operated on. Careful clinicopathological correlation is, thus, required to assist the pathologist in performing the autopsy and reaching a diagnosis regarding the cause of death. Due to the heterogeneity of the structural abnormalities, and the wide variety of surgical and interventional procedures, there are no standard methods for dissecting the heart at autopsy. In this paper, we describe the most common types of CHDs that a pathologist could encounter at autopsy, including the various types of surgical and percutaneous procedures and major pathological manifestations. We also propose a practical systematic approach to the autopsy of ACHD patients.

Sudden death in young South European population: a cross-sectional study of postmortem cases

“…Prior medication information was obtained based on the description provided in the autopsy and toxicology report. The final cause of death was ascertained based on the data present in the autopsy report (after toxicological and the anatomopathological diagnosis), in the light of the Guidelines for autopsy investigation of sudden cardiac death (2017) 5 , 14 . Finally, deaths in which both autopsy and toxicology investigations were inconclusive, non-cardiac etiologies were excluded, and the heart had non-diagnostic pathological findings of uncertain significance at gross and histological examination, 15 were classified as possible SADS as the final cause of death 4 .…”

Prognostic Value of Liver and Spleen Stiffness in Patients with Fontan Associated Liver Disease (FALD): A Case Series with Histopathologic Comparison

“…A complete autopsy was performed according to the standard protocol adopted in our centre and previously published both for post-transplant [ 5 ] or adult CHD [ 6 ] cases. Histopathology was part of the complete autopsy, and the liver samples were collected and stained with Haematoxylin–Eosin and Masson’s Trichromic (Diapath S.p.A., Martinengo (BG), Italy) for evaluation both of the extracellular matrix and fibrous pattern.…”

Sudden Death in Congenital Heart Disease: The Role of the Autopsy in Determining the Actual Cause

“…Recent guidelines from the European Association of Cardiovascular Pathology describe the most common types of CHDs and include a description of the various types of surgical and percutaneous procedures and major pathological complications. This is a valuable guide to pathologists facing such an autopsy [ 17 ]. Small autopsy studies in the forensic area relate to SCD cases with known CHD in the majority but this study also showed that five cases had no diagnosis prior to autopsy, highlighting that the first presentation of CHD can be SCD like in the Danish study [ 15 ].…”