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Pathologic features of myocardial hamartomas causing childhood tachyarrhythmias
Insights
A rare myocardial hamartoma in infants can cause fatal tachyarrhythmias. Surgical excision, guided by electrophysiologic mapping, offers a potential cure for this condition.
Area of Science:
- Pediatric Cardiology
- Cardiac Pathology
- Electrophysiology
Background:
- A distinct clinicopathologic lesion causes tachyarrhythmias in infants, with a historically high fatality rate (96%).
- Previous pathogenic considerations included viral-induced lesions, cardiomyopathy, neoplasm, and Purkinje cell developmental disorders.
Purpose of the Study:
- To describe the clinicopathologic features of a specific lesion causing infant tachyarrhythmias.
- To evaluate the efficacy of electrophysiologic mapping and surgical excision for treating this condition.
Main Methods:
- Review of morphologic findings in 11 infant patients and 26 literature cases.
- Electrophysiologic mapping and surgical excision in 9 of the 11 studied patients.
Main Results:
- Nine of 11 infants survived following surgical excision, with follow-up up to 6 years.
- The lesion, believed to be a myocardial hamartoma, presents a tumor-like growth pattern without mitotic figures.
- The condition is potentially curable through surgical intervention.
Conclusions:
- Myocardial hamartoma is a distinct clinicopathologic entity in infants causing potentially fatal tachyarrhythmias.
- Electrophysiologic mapping and surgical excision are effective treatment modalities, leading to long-term survival.
Abstract:
We have observed in 11 infants, aged 2 years or less, a distinct clinicopathologic lesion responsible for tachyarrhythmias that were fatal in 96% (25/26) of previously reported cases. Nine of the 11 patients, who underwent electrophysiologic mapping and surgical excision of the lesion, have survived, with follow-up periods ranging from 1 month to 6 years. The morphologic findings in these 11 patients and in the 26 cases cited in the literature are reviewed. Pathogenic considerations have included viral-induced lesion, cardiomyopathy, neoplasm, and developmental disorder of Purkinje cells. We believe this lesion to be a myocardial hamartoma. Supportive evidence includes prevalence in infants, tumorlike growth pattern without mitotic figures, and association of other developmental abnormalities. Through electrophysiologic mapping, this myocardial hamartoma is potentially accessible to surgical excision and long-term cure.