Abstract 16862: Hypertrophic Cardiomyopathy in Octogenarians: Is Age Just a Number?

Abstract

Introduction: Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiomyopathy and is typically diagnosed in youth or early adulthood. The clinical characteristics of hypertrophic cardiomyopathy among the very old have not been described. Methods: This is a single-center, retrospective observational study. We queried the Yale Inherited Cardiomyopathies database, obtaining data on HCM patients that were 80 years or older at time of inclusion (5/1/2018) or time of death. Care was taken to exclude patients with other diagnoses that could mimic the HCM phenotype. Results: Results are presented in Table 1. The majority had non-obstructive disease with apical hypertrophy in a substantial majority. All had echocardiographic diastolic dysfunction with concurrent symptomatic heart failure (NYHA II-IV). LA enlargement was substantial with mean LA ESV index of 53.2+-37.5 ml/m2. Atrial fibrillation was common (50%). High risk features such as SCD, ventricular arrhythmias and extreme hypertrophy were absent from this cohort, although syncope and family history of SCD were reported. Genetic test yield was 33%. Conclusions: Non-obstructive HCM with diastolic dysfunction, symptomatic heart failure, and arrhythmia are common among the very old with HCM. These features may reflect a combination of age related diastolic dysfunction and inherited HCM. Despite excellent survival symptomatic structural heart disease, arrhythmia, and stroke risk was the norm. The male:female ratio >1 usually seen in HCM is reversed, suggesting some degree of survival bias. Genetic testing remains useful in this age category. In summary, although HCM is not incompatible with reaching advanced old age, diastolic dysfunction drives significant HCM related morbidity.