Myocardial Perfusion Abnormalities in Patients with Hereditary Hypertrophic Cardiomyopathy: A Study of Three Cases and Review of the Literature

Abstract

Introduction: Hypertrophic cardiomyopathy (HCM) belongs to the very heterogeneous group of cardiomyopathies. This study aimed to study myocardial perfusion abnormalities on scintigraphy and assess the risk of sudden death from ventricular arrhythmia in hereditary sarcomeric HCM. Patients and Methods: This is a retrospective and prospective descriptive study over 18 months (January 01, 2021, to July 31, 2022) on the records of patients over 18 with the diagnosis of hypertrophic sarcomeric cardiomyopathy with or without ventricular rhythm disorders and who have undergone myocardial scintigraphy. Results: Three patients were included. The average age of our patients was 66 years old. Dyspnea is the primary symptomatology found in our patients. One patient presented with syncope and unsustained ventricular tachycardia. His risk score for sudden death from ESC at five years is estimated at 6.45%, and the patient received an ICD in primary prevention. The average sudden death risk score of our patients was 3.78%. The mean LV wall thickness was 20 mm. The mean maximum left intraventricular gradient was 39 mmHg. Myocardial fibrosis was present in all our patients. Myocardial scintigraphy was normal in all cases. Conclusion: Hypertrophic cardiomyopathy is a very heterogeneous group of cardiomyopathies. The rhythmic risk is multifactorial and constitutes a significant prognostic factor.