ArticleThe European respiratory journal2020
Pulmonary arterial hypertension with below threshold pulmonary vascular resistance.
Article in The European respiratory journal, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 21 citations in OpenAlex.
- Association of pulmonary hypertension-targeted therapy and survival in precapillary pulmonary hypertension with mean pulmonary arterial pressure between 21 and 24 mmHg.ERJ open research · 2025Article
- The latest definition and classification of pulmonary hypertension.International journal of cardiology. Congenital heart disease · 2024Review
- Updated Clinical Classification and Hemodynamic Definitions of Pulmonary Hypertension and Its Clinical Implications.Journal of cardiovascular development and disease · 2024Review
- Pulmonary Hypertension: A Contemporary Review.American journal of respiratory and critical care medicine · 2023Review
- Case report: Progressive pulmonary artery hypertension in a case of megalencephaly-capillary malformation syndrome.Frontiers in genetics · 2023Article
- Mild pulmonary hemodynamic alterations in patients with systemic sclerosis: relevance of the new 2022 ESC/ERS definition of pulmonary hypertension and impact on mortality.Respiratory research · 2022Article
- Hemodynamic indices in pulmonary hypertension: a narrative review.Cardiovascular diagnosis and therapy · 2022Review
- Review
- Cardiovascular Diseases That Have Emerged From the Darkness.Journal of the American Heart Association · 2021Article
- Pulmonary hypertension phenotypes in patients with systemic sclerosis.European respiratory review : an official journal of the European Respiratory Society · 2021Review
- mTOR Signaling in Pulmonary Vascular Disease: Pathogenic Role and Therapeutic Target.International journal of molecular sciences · 2021Review
- Cardiopulmonary Hemodynamics in Pulmonary Hypertension and Heart Failure: JACC Review Topic of the Week.Journal of the American College of Cardiology · 2020Review
- Adding an important piece to the pulmonary vascular resistance puzzle in pulmonary arterial hypertension.The European respiratory journal · 2020Article
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Authors and funding
18 authors at 11 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Pulmonary vascular resistance (PVR) >3 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 ≥25 mmHg, PAWP ≤15 mmHg and PVR <3 Wood units.Eighty-two patients met the pre-defined haemodynamic inclusion criteria (mean age 63±11 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 27 mmHg (interquartile range (IQR) 25-30 mmHg), PAWP 13 mmHg (IQR 11-14 mmHg) and PVR 2.2 Wood units (IQR 1.9-2.7 Wood units). Baseline 6-min walk distance (6MWD) was 352 m (IQR 280-416 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 46 m (IQR 7-96 m) and 35% of subjects demonstrated improvement in NYHA functional class. After a median follow-up of 65 months (IQR 32-101 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.
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