Abstract
OBJECTIVES: Pulmonary hypertension (PH) is known to be associated with increased mortality in patients suffering from left ventricular disease. The aim of this study was to assess the incidence of PH among patients diagnosed with hypertrophic cardiomyopath (HCM) and to evaluate its prognostic significance. METHODS: The study cohort consisted of 2781 patients with HCM. Among them, 226 patients had PH (8.1%), and 2555 patients did not have PH (91.8%). The fourteen demographic and clinical variables were matched between the two groups using a 1:3 propensity score matching (PSM) method. Kaplan-Meier survival curves and Cox proportional hazard regression models were used to evaluate the correlation between PH and mortality. Moreover, a competing risk regression analysis was conducted to assess the competing risk. RESULTS: Before matching, there were 519 (18.7 %) patients with all-cause mortality, including 292 (10.5 %) patients who experienced cardiovascular mortality and 128 (4.6 %) patients who experienced SCD. There was a significant difference in the Kaplan-Meier survival curves for all-cause mortality (log-rank P < 0.0001), cardiovascular mortality (log-rank P < 0.0001) and SCD (log-rank P = 0.0005). After matching, there were also significant differences in cardiovascular mortality (log-rank P = 0.011) and SCD (log-rank P = 0.042), but only a similar trend was observed for all-cause mortality (log-rank P = 0.052). Cox regression analyses suggested that PH was an independent risk predictor for cardiovascular mortality [HR: 1.666; 95 % CI: 1.145-2.424; P = 0.008]. CONCLUSION: HCM patients with PH characterized by increased cardiovascular mortality and SCD, as well as a similar trend in all-cause mortality. Moreover, PH is an independent risk factor for cardiovascular mortality.