Universität zu Köln

Wissmuller, Max, Xanthouli, Panagiota ORCID: 0000-0002-7743-2472, Benjamin, Nicola, Grunig, Ekkehard, Richter, Manuel J., Gall, Henning, Ghofrani, Hossein Ardeschir, Herkenrath, Simon, Skowasch, Dirk, Pizarro, Carmen, Halank, Michael, Hohmann, Christopher, Hellmich, Martin, Gerhardt, Felix and Rosenkranz, Stephan (2022). Profiles and treatment patterns of patients with pulmonary arterial hypertension on monotherapy at experienced centres. ESC Heart Fail., 9 (5). S. 3873 - 3886. SAN FRANCISCO: WILEY PERIODICALS, INC. ISSN 2055-5822

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Abstract

Aims Guideline recommendations highlight the critical role of combination therapy for the treatment of pulmonary arterial hypertension (PAH). Conversely, registry data demonstrate that a considerable number of PAH patients remain on monotherapy. The reasons for this discrepancy remain elusive. The aim of this study was to assess the patient profiles, treatment patterns, and disease characteristics of patients diagnosed with PAH who were kept on monotherapy at experienced pulmonary hypertension (PH) centres and to capture potential reasons for monotherapy. Methods and results We analysed the patient profiles of 182 patients on monotherapy with PAH-targeted drugs, managed at experienced PH expert centres (Cologne, Giessen, Heidelberg, and Dresden). Patients were identified based on their latest follow-up visit and analysed retrospectively from the time of PAH diagnosis to last follow-up. Patients were dichotomized by age, and patient characteristics, treatment patterns, response to therapy, change in risk status, and drug tolerability were recorded during the course of their disease. Patients' mean age was 69.1 +/- 13.1 years at the most recent follow-up (Key Time Point 1) and 64.5 +/- 14.9 years at the time of diagnosis (Key Time Point 2). The mean time on monotherapy was 60.7 +/- 53.8 months; 35.7/64.3% of patients were male/female. The majority (66.5%) had idiopathic PAH, followed by PAH associated with connective tissue disease (17.0%) and portopulmonary PH (8.2%). Among patients on monotherapy, there were five main clusters: (i) patients with failed escalation attempts mostly because of intolerability (26.9%); (ii) low risk on monotherapy, favourable response, and no reason for escalation (24.2%); (iii) patients with mild PAH (36.3%); (iv) elderly patients with PAH and multiple co-morbidities (38.5%); and (v) patients with associated forms of PAH where the level of evidence for combination therapies is considered low (16.5%). There were substantial differences between patients above or below the median age (68 years). The most frequently used monotherapy for PAH was phosphodiesterase type 5 inhibitors (75.3%). Conclusions A considerable number of PAH patients are on monotherapy at large PH expert centres, characterized by specific reasons that justify this kind of treatment. Nevertheless, as comprehensive treatment strategies have shown improved long-term outcomes even in mildly symptomatic patients, each case of monotherapy should be justified.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Wissmuller, Max UNSPECIFIED UNSPECIFIED UNSPECIFIED Xanthouli, Panagiota UNSPECIFIED orcid.org/0000-0002-7743-2472 UNSPECIFIED Benjamin, Nicola UNSPECIFIED UNSPECIFIED UNSPECIFIED Grunig, Ekkehard UNSPECIFIED UNSPECIFIED UNSPECIFIED Richter, Manuel J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Gall, Henning UNSPECIFIED UNSPECIFIED UNSPECIFIED Ghofrani, Hossein Ardeschir UNSPECIFIED UNSPECIFIED UNSPECIFIED Herkenrath, Simon UNSPECIFIED UNSPECIFIED UNSPECIFIED Skowasch, Dirk UNSPECIFIED UNSPECIFIED UNSPECIFIED Pizarro, Carmen UNSPECIFIED UNSPECIFIED UNSPECIFIED Halank, Michael UNSPECIFIED UNSPECIFIED UNSPECIFIED Hohmann, Christopher UNSPECIFIED UNSPECIFIED UNSPECIFIED Hellmich, Martin UNSPECIFIED UNSPECIFIED UNSPECIFIED Gerhardt, Felix UNSPECIFIED UNSPECIFIED UNSPECIFIED Rosenkranz, Stephan UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-689150
DOI:	10.1002/ehf2.13804
Journal or Publication Title:	ESC Heart Fail.
Volume:	9
Number:	5
Page Range:	S. 3873 - 3886
Date:	2022
Publisher:	WILEY PERIODICALS, INC
Place of Publication:	SAN FRANCISCO
ISSN:	2055-5822
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language INITIAL COMBINATION THERAPY; CONTRACTILE FUNCTION; HEART-FAILURE; AMBRISENTAN; PHENOTYPES; MORBIDITY; TADALAFIL; MORTALITY Multiple languages Cardiac & Cardiovascular Systems Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/68915