Pulmonary hypertension is associated with worse survival in hypertrophic cardiomyopathy

Aims Pulmonary hypertension (PH) is associated with increased mortality in various forms of left-sided heart disease. However, the implications of PH in hypertrophic cardiomyopathy (HCM) have not been elucidated. The objective of this study was to determine the prevalence and prognostic implications of PH in HCM. Methods and results The study cohort consisted of 1570 (54 +/- 15 years; 53% male) adults with HCM followed for a median of 3.3 years. PH [pulmonary artery systolic pressure (PASP) > 36 mmHg] was identified in 38% of patients who were older (57 +/- 15 vs. 52 +/- 15 years, P < 0.0001), more likely to be female (59 vs. 40%, P, 0.0001), and were characterized by a higher prevalence of New York Heart Association (NYHA) class 3 or 4 symptoms (61 vs. 45%, P < 0.0001) and atrial fibrillation (29 vs. 15%, P, 0.0001) vs. those without PH. Only 12% had moderate or severe PH (PASP > 50 mmHg). In multivariate Cox regression analyses adjusted for age, sex, NYHA class 3 or 4 symptoms, and atrial fibrillation, PASP was an independent predictor of all-cause mortality in patients with non-obstructive HCM (HR 1.59 per 10 mmHg PASP increase, 95% CI 1.28-1.96, P < 0.0001) and in those with obstructive physiology who did not undergo septal reduction therapy (SRT) (HR 1.15 per 10 mmHg PASP, 95% CI 1.01-1.31, P = 0.035). However, PH was not predictive of outcomes in patients with obstructive HCM who underwent SRT. Conclusions Over one-third of adults evaluated in our referral HCM clinic demonstrated concomitant PH, and this was associated with increased mortality except in those with obstructive HCM who underwent SRT.