Abstract

<h3>Introduction</h3> The relationship between New York Heart Association functional class (NYHA class) and mortality is well established in heart failure, but not in obstructive hypertrophic cardiomyopathy (oHCM). <h3>Hypothesis</h3> Among patients with oHCM, worse NYHA class is correlated with increased mortality risk. <h3>Methods</h3> The Sarcomeric Human Cardiomyopathy Registry (SHaRe) enrolled patients from 10 HCM specialty centers worldwide. The current study used the data through March 2019, analyzing patients ≥18 years old with oHCM (left ventricular outflow tract (LVOT) peak gradient >30 mmHg <i>or</i> septal reduction therapy) and documentation of NYHA class. Patients were followed from the date of index NYHA class assessment (first documentation of NYHA class I, II, III or IV) to last SHaRe visit or death. The risks of all-cause mortality and a composite endpoint of death and heart transplant were compared across index NYHA classes using log-rank tests. <h3>Results</h3> We analyzed 2495 patients, mean age 47.6 years at diagnosis and 42% females. Patient characteristics varied across NYHA class (Table). Over median follow-up of 3.9 years, there were 216 deaths: 52 initially class I (5% of class I; 24% of deaths), 97 class II (9% of class II; 45% of deaths), and 67 in class III/IV (13%; 31% of deaths). Risks of all-cause mortality and the composite outcome of death or heart transplant increased with worse NYHA class (both p<0.001; Table). <h3>Conclusion</h3> Worse NYHA class is correlated with increased all-cause mortality in oHCM. Monitoring functional status will assist in assessing treatment response and informing prognosis.

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