Open AccessPoor outcome of patients with pulmonary arterial hypertension with insufficient response to phosphodiesterase‐5 inhibitors alone or in combination with other specific therapy: a registry‐based study
Author(s) -
Hjalmarsson Clara,
Butler Oisin,
Hesselstrand Roger,
Holl Katsiaryna,
Jansson Kjell,
Klok Rogier,
Rådegran Göran,
Söderberg Stefan,
Kjellström Barbro
Publication year - 2020
Publication title -
pulmonary circulation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.791
H-Index - 40
ISSN - 2045-8940
DOI - 10.1177/2045894020958557
Subject(s) - medicine , prohormone , brain natriuretic peptide , pulmonary hypertension , natriuretic peptide , phosphodiesterase , combination therapy , cardiology , lung transplantation , lung , heart failure , biochemistry , chemistry , hormone , enzyme
Phosphodiesterase‐5 inhibitors are commonly used in pulmonary arterial hypertension but, as suggested by the RESPITE study, phosphodiesterase‐5 inhibitor therapy (mono‐/combination) does not always have a satisfactory treatment effect. This study aimed to investigate the clinical course of pulmonary arterial hypertension patients not at treatment goal after at least 90 days of treatment with phosphodiesterase‐5 inhibitors, alone or in combination with other pulmonary arterial hypertension therapies. The study included 106 incident patients from the Swedish Pulmonary Arterial Hypertension Registry, treated with phosphodiesterase‐5 inhibitors for ≥90 days, who were not at a pre‐specified treatment goal, i.e. in World Health Organisation functional class III, with 6‐min walking distance 165–440 m, and N‐terminal prohormone of brain natriuretic peptide >300 ng/L. Changes in World Health Organisation functional class, 6‐min walking distance, N‐terminal prohormone of brain natriuretic peptide, and risk group between index and follow‐up were assessed. Of patients with complete follow‐up data, ( n = 53) 77% were on combination therapy and risk assessment yielded 98% at intermediate risk at index. At follow‐up, 11 patients transitioned from World Health Organisation functional class III to World Health Organisation functional class II, the median (Q1; Q3) change in 6‐min walking distance was 6 (−30; 42) meters and in N‐terminal prohormone of brain natriuretic peptide 47 (−410; 603) ng/L, while 89% remained at an intermediate risk. Of those without complete follow‐up data, 11 patients died and 2 underwent lung transplantation. In conclusion, pulmonary arterial hypertension patients treated with phosphodiesterase‐5 inhibitors, as single or combination therapy and not achieving the pre‐specified treatment goals after ≥90 days have an unfavourable clinical course.