Abstract 16262: Pulmonary Hypertension in Hypertrophic Cardiomyopathy: Prevalence, Clinical Profile, and Prognosis
Background: The clinical profile and prognosis of patients with hypertrophic cardiomyopathy (HCM) and pulmonary hypertension (PH) are unknown. We hypothesized that PH in patients with HCM is associated with mortality.
Methods: We identified 2,565 adults with HCM who underwent clinical evaluation at our institution from 1993 to 2012. Pulmonary artery systolic pressure (PASP) derived from echocardiography was available in 1,747 patients (68%). These patients (54±16 yrs; 53% male) were stratified into 3 groups based on PASP: Group 1, n=937 (54%) with PASP<35mmHg; Group 2, n=564 (32%) with PASP 35 to 49mmHg; Group 3, n=246 (14%) with PASP≥50mmHg. Group characteristics were compared using a t-test and survival was evaluated with Kaplan-Meier curves. Multivariable proportional hazards models were used to assess the association of PASP with mortality after adjustment for age, gender, family history of sudden death, atrial fibrillation, and NYHA III/IV status.
Results: Group 3 vs Group 1 patients were older (61±14 vs 52±15 yrs), consisted of more females (68% vs 39.2%), and more likely to be NYHA III/IV (71% vs 42%; p<0.001 for all). On echocardiogram, Group 3 patients had larger left atria (70±45 vs 43±16 ml/m2), higher filling pressures (E/e’: 26±12 vs 16±7), more ≥moderate mitral regurgitation (52% vs 12%; p<0.001 for all), and greater left ventricular outflow tract gradients (46 [IQR 0, 96] vs 18 mmHg [IQR 0, 52]). During follow-up (mean 2.4±3.6 yrs; median 0.6 yrs [IQR 0, 3.7]) there were 168 deaths. Kaplan-Meier analysis demonstrated worse survival according to PASP level (Figure 1). Furthermore, PASP was independently associated with higher mortality (HR 1.23 per 10mmHg; 95%CI 1.12-1.34).
Conclusions: Over 10% of the patients with HCM evaluated in our center have significant PH which likely reflects a greater burden of the disease. Higher PASP was independently associated with lower survival in patients with HCM, and may represent a valuable tool for risk stratification.
- © 2013 by American Heart Association, Inc.