Pulmonary arterial hypertension with below threshold pulmonary vascular resistance.
Eur Respir J
; 56(1)2020 07.
Article
em En
| MEDLINE
| ID: mdl-32341105
ABSTRACT
Pulmonary vascular resistance (PVR) >3â
Wood units is a criterion of the haemodynamic definition of pulmonary arterial hypertension (PAH). However, this cut-off is conservative and arbitrarily defined. Data is lacking on the natural history, response to therapy and survival of patients diagnosed with precapillary pulmonary hypertension (PH) with mild or borderline elevation of PVR.In Australia, PAH therapy could be prescribed solely on mean pulmonary arterial pressure (PAP) and pulmonary arterial wedge pressure (PAWP) criteria. Using the Australian and New Zealand Pulmonary Hypertension Registry, we aimed to study a population diagnosed with PAH between January 2004 and December 2017 with the pre-defined haemodynamic characteristics of mean PAP ≥25â
mmHg, PAWP ≤15â
mmHg and PVR <3â
Wood units.Eighty-two patients met the pre-defined haemodynamic inclusion criteria (mean age 63±11â
years; 67 females). Underlying aetiologies included idiopathic disease (n=39), connective tissue disease (CTD; n=42) and HIV infection (n=1). At diagnosis, mean PAP was 27â
mmHg (interquartile range (IQR) 25-30â
mmHg), PAWP 13â
mmHg (IQR 11-14â
mmHg) and PVR 2.2â
Wood units (IQR 1.9-2.7â
Wood units). Baseline 6-min walk distance (6MWD) was 352â
m (IQR 280-416â
m) and 77% of subjects were in New York Heart Association (NYHA) functional class 3 or 4. All patients were commenced on initial monotherapy with an endothelin receptor antagonist (ERA; n=66) or phosphodiesterase type-5 inhibitor (PDE5i; n=16). At first re-evaluation, 6MWD increased by 46â
m (IQR 7-96â
m) and 35% of subjects demonstrated improvement in NYHA functional class. After a median follow-up of 65â
months (IQR 32-101â
months), 18 out of 82 subjects (22.0%) had died, with estimated 1-year and 5-year survival rates of 98% and 84%, respectively. Death attributed to PAH occurred in six out of these 18 patients (33.3%, 7% of total cohort).Patients with precapillary PH and "borderline" PVR falling outside the current definition have adverse outcomes. Such patients appear to respond to PAH therapy; however, this requires further study in randomised trials.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Infecções por HIV
/
Hipertensão Arterial Pulmonar
Tipo de estudo:
Clinical_trials
Limite:
Aged
/
Female
/
Humans
/
Middle aged
País/Região como assunto:
Oceania
Idioma:
En
Revista:
Eur Respir J
Ano de publicação:
2020
Tipo de documento:
Article
País de afiliação:
Austrália