Background and aims Sudden cardiac death (SCD) is a relatively common cause of death in hypertrophic cardiomyopathy (HCM). HCM patients presenting at >60 years of age of life are considered at low risk for disease-related morbidity/mortality, including SCD. The aim of the study was to investigate the demographics of SCD in a large cohort of patients that died suddenly with a post-mortem diagnosis of HCM.
Methods We reviewed a database of 5100 consecutive cases of SCDs referred to our specialist cardiac pathology centre between 1994 and March 2017 and identified 196 (4%) cases with HCM. Two groups were considered: Group 1 (SCD at 60 years of age) and Group 2 (SCD at >60 years of age). All cases underwent detailed post-mortem evaluation including histological analysis by an expert cardiac pathologist. Clinical information was obtained from referring coroners.
Results Group 1 and Group 2 comprised of 176 (90%) and 20 (10%) individuals, respectively. Male gender was prevalent in Group 1 (n=134, 76%), but not in Group 2 (n=9, 45%), (p=0.007). Death occurred at rest in 130 (75%) individuals in Group 1 and in 17 (85%) individuals in Group 2. Previous cardiac symptoms were present in 46 (26%) individuals in Group 1 and in 5 (25%) individuals in Group 2, p=0.56. The mean heart weight was similar in the two groups (554±184 in Group 1 vs 593±209 in Group 2, p=0.38). Left ventricular (LV) fibrosis was present in 106 (60%) individuals in Group 1 and in 9 (45%) in Group 2, p=0.29. Associated critical coronary artery stenosis were found in 3 (3%) cases in Group 1 and in 2 (10%) cases in Group 2, p=0.47.
Conclusions SCD in elderly patients with HCM is a rare but possible event. SCD victims with HCM who died at less than 60 years of age are predominantly males, whereas older patients are more frequently females. Cardiac symptoms and diagnosis prior to death are reported in 1 in 4 individuals suggesting that SCD is often the first manifestation of the disease.
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