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Autopsy in adults with congenital heart disease (ACHD)

2020Journal article Open access
http://hdl.handle.net/10400.26/41925
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Authors

Angelini, Annalisa
di Gioia, Cira
Doran, Helen
Fedrigo, Marny
Ho, Siew Yen
Leone, Ornella
Sheppard, Mary N
Thiene, Gaetano
Dimopoulos, Konstantinos

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Abstract(s)

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.

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Keywords

Autopsy Cardiovascular pathology Congenital heart diseases Adult congenital heart diseases Protocol

URI

http://hdl.handle.net/10400.26/41925

Citation

Angelini, A., di Gioia, C., Doran, H., Fedrigo, M., Henriques de Gouveia, R., Ho, S. Y., Leone, O., Sheppard, M. N., Thiene, G., Dimopoulos, K., Mulder, B., Padalino, M., van der Wal, A. C., & Association for European Cardiovascular Pathology (AECVP) (2020). Autopsy in adults with congenital heart disease (ACHD). Virchows Archiv : an international journal of pathology, 476(6), 797–820

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Publisher

Springer Verlag

DOI

10.1007/s00428-020-02779-8

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INMLCF - Artigos Científicos

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