Predictors of the response to phosphodiesterase-5 inhibitors in pulmonary arterial hypertension: an analysis of the Spanish registry.


Journal

Respiratory research
ISSN: 1465-993X
Titre abrégé: Respir Res
Pays: England
ID NLM: 101090633

Informations de publication

Date de publication:
15 Sep 2023
Historique:
received: 28 06 2023
accepted: 05 09 2023
medline: 18 9 2023
pubmed: 16 9 2023
entrez: 15 9 2023
Statut: epublish

Résumé

Achieving and maintaining a low-risk profile is associated with favorable outcome in pulmonary arterial hypertension (PAH). The effects of treatment on risk profile are variable among patients. To Identify variables that might predict the response to treatment with phosphodiesterase-5 inhibitors (PDE-5i) in PAH. We carried out a cohort analysis of the Spanish PAH registry in 830 patients diagnosed with PAH that started PDE5i treatment and had > 1 year follow-up. 644 patients started PDE-5i either in mono- or add-on therapy and 186 started combined treatment with PDE-5i and endothelin receptor antagonist (ERA). Responders were considered when at 1 year they: (1) were alive; (2) did not present clinical worsening; and (3) improved European Society of Cardiology/European Respiratory Society (ESC/ERS) risk score or remained in low-risk. Univariate and multivariate logistic regression models were used to analyze variables associated with a favorable response. Two hundred and ten patients (33%) starting PDE-5i alone were classified as responders, irrespective of whether it was mono- or add-on therapy. In addition to known predictors of PAH outcome (low-risk at baseline, younger age), male sex and diagnosis of portopulmonary hypertension (PoPH) or HIV-PAH were independent predictors of favorable response to PDE-5i. Diffusing capacity for carbon monoxide (DLco) ≤ 40% of predicted was associated with an unfavorable response. When PDE-5i were used in upfront combination, 58% of patients were responders. In this group, diagnosis of idiopathic PAH (IPAH) was an independent predictor of favorable response, whereas connective tissue disease-PAH was associated with an unfavorable response. Male sex and diagnosis of PoPH or HIV-PAH are predictors of favorable effect of PDE-5i on risk profile when used as mono- or add-on therapy. Patients with IPAH respond more favorably to PDE-5i when used in upfront combination. These results identify patient profiles that may respond favorably to PDE-5i in monotherapy and those who might benefit from alternative treatment strategies.

Sections du résumé

BACKGROUND BACKGROUND
Achieving and maintaining a low-risk profile is associated with favorable outcome in pulmonary arterial hypertension (PAH). The effects of treatment on risk profile are variable among patients.
OBJECTIVE OBJECTIVE
To Identify variables that might predict the response to treatment with phosphodiesterase-5 inhibitors (PDE-5i) in PAH.
METHODS METHODS
We carried out a cohort analysis of the Spanish PAH registry in 830 patients diagnosed with PAH that started PDE5i treatment and had > 1 year follow-up. 644 patients started PDE-5i either in mono- or add-on therapy and 186 started combined treatment with PDE-5i and endothelin receptor antagonist (ERA). Responders were considered when at 1 year they: (1) were alive; (2) did not present clinical worsening; and (3) improved European Society of Cardiology/European Respiratory Society (ESC/ERS) risk score or remained in low-risk. Univariate and multivariate logistic regression models were used to analyze variables associated with a favorable response.
RESULTS RESULTS
Two hundred and ten patients (33%) starting PDE-5i alone were classified as responders, irrespective of whether it was mono- or add-on therapy. In addition to known predictors of PAH outcome (low-risk at baseline, younger age), male sex and diagnosis of portopulmonary hypertension (PoPH) or HIV-PAH were independent predictors of favorable response to PDE-5i. Diffusing capacity for carbon monoxide (DLco) ≤ 40% of predicted was associated with an unfavorable response. When PDE-5i were used in upfront combination, 58% of patients were responders. In this group, diagnosis of idiopathic PAH (IPAH) was an independent predictor of favorable response, whereas connective tissue disease-PAH was associated with an unfavorable response.
CONCLUSION CONCLUSIONS
Male sex and diagnosis of PoPH or HIV-PAH are predictors of favorable effect of PDE-5i on risk profile when used as mono- or add-on therapy. Patients with IPAH respond more favorably to PDE-5i when used in upfront combination. These results identify patient profiles that may respond favorably to PDE-5i in monotherapy and those who might benefit from alternative treatment strategies.

Identifiants

pubmed: 37715261
doi: 10.1186/s12931-023-02531-1
pii: 10.1186/s12931-023-02531-1
pmc: PMC10503122
doi:

Substances chimiques

Cyclic Nucleotide Phosphodiesterases, Type 5 EC 3.1.4.35
Phosphodiesterase 5 Inhibitors 0

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

223

Informations de copyright

© 2023. BioMed Central Ltd., part of Springer Nature.

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Auteurs

Agustin R Garcia (AR)

Department of Pulmonary Medicine, Hospital Clínic-IDIBAPS, University of Barcelona, Barcelona, Spain.
Biomedical Research Networking Center on Respiratory Diseases (CIBERES), Madrid, Spain.

Isabel Blanco (I)

Department of Pulmonary Medicine, Hospital Clínic-IDIBAPS, University of Barcelona, Barcelona, Spain.
Biomedical Research Networking Center on Respiratory Diseases (CIBERES), Madrid, Spain.

Lluis Ramon (L)

Department of Pulmonary Medicine, Hospital Clínic-IDIBAPS, University of Barcelona, Barcelona, Spain.
Biomedical Research Networking Center on Respiratory Diseases (CIBERES), Madrid, Spain.

Jesús Pérez-Sagredo (J)

Department of Pulmonary Medicine, Hospital El Bierzo, León, Spain.

Francisco J Guerra-Ramos (FJ)

Department of Pulmonary Medicine, Complejo Hospitalario Universitario Insular-Materno Infantil, Las Palmas, Spain.

Clara Martín-Ontiyuelo (C)

Department of Pulmonary Medicine, Hospital Clínic-IDIBAPS, University of Barcelona, Barcelona, Spain.
Biomedical Research Networking Center on Respiratory Diseases (CIBERES), Madrid, Spain.

Olga Tura-Ceide (O)

Department of Pulmonary Medicine, Hospital Clínic-IDIBAPS, University of Barcelona, Barcelona, Spain.
Biomedical Research Networking Center on Respiratory Diseases (CIBERES), Madrid, Spain.

Francisco Pastor-Pérez (F)

Cardiology Department, Hospital Clínico Universitario Virgen de la Arrixaca, Murcia, Spain.

Pilar Escribano-Subías (P)

Pulmonary Hypertension Unit, Cardiology Department, Hospital Universitario 12 de Octubre, Madrid, Spain.
Biomedical Research Networking Center on Cardiovascular Diseases (CIBERCV), Madrid, Spain.

Joan A Barberà (JA)

Department of Pulmonary Medicine, Hospital Clínic-IDIBAPS, University of Barcelona, Barcelona, Spain. jbarbera@clinic.cat.
Biomedical Research Networking Center on Respiratory Diseases (CIBERES), Madrid, Spain. jbarbera@clinic.cat.

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