Long-term outcome of patients with combined post- and pre-capillary pulmonary hypertension

Abstract

Background Pulmonary hypertension (PH) is a complex clinical condition and left heart disease is the leading cause. Little is known about the epidemiology and prognosis of combined post- and precapillary PH (CpcPH). Methods This retrospective analysis of the Swiss PH Registry included incident patients with CpcPH registered from 01/2001 to 06/2019 at 13 Swiss hospitals. Patient baseline characteristics (age, sex, mean pulmonary artery pressure (mPAP), pulmonary artery wedge pressure (PAWP), pulmonary vascular resistance (PVR) and risk factors including WHO-functional class (FC), 6 minute walk distance (6MWD) and NT-proBNP, treatment, days of follow-up and events (death or loss to follow-up) at last visit were analysed by Kaplan Meier and Cox regression analysis. Results 231 patients (59.3% women, age 65±12y, mPAP 48±11mmHg, PAWP 21±5mmHg, PVR 7.2±4.8WU) were included. Survival analyses showed a significantly longer survival for women (HR 0.58 (0.38, 0.89); p=0.01) and higher mortality risk for mPAP>46mmHg (HR 1.58 (1.03, 2.43); p=0.04), but no association with age or PVR. Patients stratified to high risk according to four-strata risk-assessment had an increased mortality risk compared to patients stratified to low-intermediate risk (HR 2.44 (1.23, 4.84); p=0.01). 46.8% of CpcPH-patients received PH-targeted pharmacotherapy, however, PH-targeted medication was not associated with longer survival. Conclusion Amongst patients with CpcPH, women and patients with an mPAP≤46mmHg survived longer. Furthermore, risk stratification by using non-invasively assessed risk factors such as WHO-FC, 6MWD and NT-proBNP, as proposed for pulmonary arterial hypertension, stratified survival in CpcPH and might be helpful in the management of these patients.