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

Anna Titz; Laura Mayer; Paula Appenzeller; Julian Müller; Simon R Schneider; Michael Tamm; Andrei M Darie; Sabina A Guler; John-David Aubert; Frédéric Lador; Hans Stricker; Jean-Marc Fellrath; Susanne Pohle; Mona Lichtblau; Silvia Ulrich

doi:10.1093/ehjopen/oead069

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

Eur Heart J Open. 2023 Jul 3;3(4):oead069. doi: 10.1093/ehjopen/oead069. eCollection 2023 Jul.

Authors

Anna Titz^{1

2}, Laura Mayer^{1

2}, Paula Appenzeller¹, Julian Müller^{1

2}, Simon R Schneider^{1

2}, Michael Tamm³, Andrei M Darie³, Sabina A Guler⁴, John-David Aubert⁵, Frédéric Lador⁶, Hans Stricker⁷, Jean-Marc Fellrath⁸, Susanne Pohle⁹, Mona Lichtblau^{1

2}, Silvia Ulrich^{1

2}

Affiliations

¹ Department of Pulmonology, University Hospital Zurich, Rämistrasse 100, Zurich 8091, Switzerland.
² Department of Pulmonology, University of Zurich, Zurich, Switzerland.
³ Clinic of Respiratory Medicine and Pulmonary Cell Research, University Hospital Basel, Basel, Switzerland.
⁴ Department of Pulmonary Medicine and Department for BioMedical Research, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
⁵ Department of Pulmonology, University Hospital Lausanne, Lausanne, Switzerland.
⁶ Department of Pulmonology, University Hospital Geneva, Geneva, Switzerland.
⁷ Department of Angiology, Ospedale La Carità, Locarno, Switzerland.
⁸ Service of Pulmonology, Hospital Neuchâtelois, Neuchâtel, Switzerland.
⁹ Lung Center, Cantonal Hospital St Gallen, St Gallen, Switzerland.

Abstract

Aims: 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 pre-capillary PH (CpcPH).

Methods and results: This retrospective analysis of the Swiss PH Registry included incident patients with CpcPH registered from January 2001 to June 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 World Health Organization (WHO)-functional class (FC), 6 min walk distance (6MWD), and N-terminal pro-brain natriuretic peptide (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 analyses. Two hundred and thirty-one patients (59.3% women, age 65 ± 12 years, mPAP 48 ± 11 mmHg, PAWP 21 ± 5 mmHg, PVR 7.2 ± 4.8 WU) were included. Survival analyses showed a significantly longer survival for women [hazard ratio (HR) 0.58 (0.38-0.89); P = 0.01] and a higher mortality risk for mPAP > 46 mmHg [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 with patients stratified to low-intermediate risk [HR 2.44 (1.23-4.84); P = 0.01]. A total of 46.8% of CpcPH patients received PH-targeted pharmacotherapy; however, PH-targeted medication was not associated with longer survival.

Conclusion: Among patients with CpcPH, women and patients with an mPAP ≤46 mmHg 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.

Keywords: Combined post- and pre-capillary pulmonary hypertension; CpcPH; Left heart disease; PH-LHD; Pulmonary hypertension; Survival.