Prevalence and influence of pulmonary hypertension in patients with obstructive hypertrophic cardiomyopathy undergoing septal myectomy

Objectives: Pulmonary hypertension (PH) is an independent predictor of all-cause mortality among patients with obstructive and nonobstructive hypertrophic cardiomyopathy (HCM). However, there is little information on the influence of coexisting PH on long-term survival following septal myectomy. This study investigates the prevalence of PH among patients with obstructive HCM undergoing septal myectomy and analyzes patient survival and the course of PH after operation. Methods: We included 1342 patients with obstructive HCM who had Doppler echocardiographic estimates of the right ventricular systolic pressure (RVSP) before and after transaortic septal myectomy. PH was defined as RVSP ≥35 mm Hg, with ≥50 mm Hg categorized as moderate-to-severe PH. A multivariable Cox proportional hazards model was used to identify characteristics associated with survival, and longitudinal trends in RVSP were modeled with generalized least squares analysis. Results: Patients underwent operations from 1989 to 2019. The median age was 57.9 years (interquartile range, 47.4-66.7 years); 49.5% were women. Preoperatively, PH was present in 47.8% of patients, and 14.4% had moderate-to-severe PH. Higher preoperative RVSP was independently associated with overall mortality in the multivariable Cox model. Among patients with moderate to severe preoperative RVSP elevation, postoperative RVSP decreased from baseline by a median of 12 mm Hg. Conclusions: Preoperative PH is independently associated with late mortality following septal myectomy, and the magnitude of preoperative RVSP was associated with a postoperative decrease in pulmonary pressure. The influence of PH on late postoperative survival may influence the timing of operation in patients who are candidates for septal myectomy.