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Conduction System Hamartoma: Autopsy Case Series
Beyza Keskin Ozturk1, Eylul Gun2
1From the Department of Pathology, The Council of Forensic Medicine, Istanbul, Turkey.
Insights
Conduction system hamartoma, a rare benign heart lesion, was identified in four infants during postmortem exams. This entity, linked to congenital heart disease and lung infections, can cause fatal arrhythmias and sudden cardiac death.
Area of Science:
- Cardiovascular Pathology
- Pediatric Pathology
Background:
- Conduction system hamartoma is a rare, benign lesion originating from cardiac conduction tissue.
- Previously termed histiocytoid cardiomyopathy, it is now classified by the WHO as conduction system hamartoma.
- This entity can lead to fatal arrhythmias and sudden cardiac death, particularly in young children.
Purpose of the Study:
- To investigate cases of conduction system hamartoma detected during postmortem histopathological examinations.
- To analyze the histopathological features, demographic data, and autopsy findings of these cases.
- To emphasize the importance of recognizing this condition for accurate diagnosis and management.
Main Methods:
- Retrospective review of histopathology reports from autopsies conducted between 2012 and 2022.
- Evaluation of four cases with conduction system hamartoma.
- Analysis included histopathological characteristics, demographics, autopsy findings, causes of death, and cardiac anomalies.
Main Results:
- Four cases of conduction system hamartoma were identified, with a female-to-male ratio of 3:1 and a mean age of 5.8 months.
- Microscopy showed sharply demarcated cell groups with foamy cytoplasm.
- Congenital heart disease and lung infection were the identified causes of death in all cases.
Conclusions:
- Conduction system hamartoma is an important entity to recognize in pediatric autopsies, especially in cases under two years of age.
- Awareness of this lesion is crucial for pathologists to prevent misdiagnosis and understand its role in sudden cardiac death.
- Extra cardiac sampling during autopsy may be necessary in suspected cases to confirm the diagnosis.
Abstract:
Conduction system hamartoma is a benign hamartomatous lesion arising from Purkinje and Purkinje-like cells of the heart. We aimed to investigate the cases that we detected during postmortem histopathological examination. The histopathology reports of the cases autopsied between 2012 and 2022 were reviewed retrospectively. The cases were evaluated in terms of histopathological features, demographic data, autopsy findings, causes of death, microbiological results, and accompanying cardiac anomalies. There was a total of 4 cases. The female-to-male ratio was 3/1. The mean age of the cases was 5.8 months. The heart weight was found within the normal range when evaluated according to age and gender. Microscopy revealed sharply demarcated cell groups or layers with histiocyte-like cells with foamy cytoplasm. Congenital heart disease and lung infection were given as causes of death in all cases. The lesion, which was previously called histiocytoid cardiomyopathy and many different names, is known as "conduction system hamartoma" in the latest World Health Organization classification (5th edition). Being aware of this entity that causes fatal arrhythmias and sudden cardiac deaths is important for pathologists. Autopsy findings should be evaluated by taking extra samples from the heart if necessary, especially in suspected cases below 2 years of age.
