Abstract
Background Hypertrophic cardiomyopathy (HCM) is a genetic heart disease that poses a risk for sudden cardiac death. It has not been studied systematically in Southeast Asian countries. The purpose of this study was to investigate the characteristics and patterns of fibrosis in HCM, as well as to determine the risk of sudden cardiac death in relation to age, sex, and myocardial fibrosis in Brunei Darussalam. Methods All patients diagnosed with HCM from 2011 to 2017 were studied in Brunei Darussalam. Data was acquired from clinical notes of these patients and studied for the demographic profile of the disease, clinical manifestations, and risks for sudden cardiac death. Results A total of 39 patients with HCM were identified, with a mean age at diagnosis of 47.46±12.27 years. Roughly half of HCM patients (51.3%) were found to be asymptomatic. A total of 30 patients underwent cardiac magnetic resonance (CMR). Myocardial fibrosis was found in 53.8%. This was more prevalent and significant in apical HCM compared to asymmetrical septal HCM, in 10 (83.3%) and nine (60%) patients (p=0.032), respectively. Apical and asymmetrical septal HCM were the most common types of HCM in the population, 15 (38.5%) and 16 (41%), respectively. Asymmetrical septal HCM presented with more murmurs than apical HCM patients, nine (56.3%) and one (6.7%) patients (p=0.001), respectively. Asymmetrical septal HCM has a higher median risk score compared to apical HCM, with medians of 2.05 (IRQ: 1.78) and 1.44 (IRQ: 0.85; p=0.025), respectively. There was no statistical difference in terms of clinical and imaging characteristics between male and female HCM patients. Conclusion Demographics and clinical presentation of the disease are comparable to those of other studies. However, the risk for sudden cardiac death was higher in asymmetrical septal HCM compared to apical HCM. We also found that myocardial fibrosis on CMR is more significant in apical HCM.