Pulmonary vascular disease & thromboembolismDiagnostic or prognostic study
Phenotypic Characterization of Unclassified Pulmonary Hypertension
- analysis of pulmonary vascular disease phenomics participants with a validation cohort and exploratory cohorts
- dynamic right heart catheterization
- transpulmonary metabolomics
- exercise right heart catheterization in validation cohort
Authors' conclusion
The presence of PH without obvious cause most often reflects subclinical left heart and metabolic dysfunction consistent with unrecognized early-stage HFpEF. Dynamic provocation during right heart catheterization can unmask unrecognized HFpEF in over half of unclassified PH, which may help guide appropriate initiation of proven HFpEF therapies to improve symptoms and functional status.
Study context
- Population
- Symptomatic patients with no pulmonary hypertension (PH) or unclassified PH (PH despite normal pulmonary vascular resistance and pulmonary artery wedge pressure); exploratory cohorts of adult congenital heart disease and high output heart failurepulmonary vascular disease phenomics 1,046 · validation cohort 1,202 · adult congenital heart disease 1,005 · high output heart failure 159 · healthy controls 96
- Participants
- 53 / 216 / 96 / 122 / 312
- Comparison
- Unclassified PH vs no PH and healthy controls
- unclassified PH (pulmonary vascular disease phenomics)n = 53
- no PH (pulmonary vascular disease phenomics)n = 216
- healthy controlsn = 96
- unclassified PH (validation cohort)n = 122
- no PH (validation cohort)n = 312
Performance
As reported in the abstract
Prevalence of unclassified PH
P = 0.006
Further findings
| Outcome | Values |
|---|---|
| Increased flow in unclassified PH | unclassified PH (pulmonary vascular disease phenomics) 28% · unclassified PH (validation cohort) 11% |
| Exertional pulmonary artery wedge pressure elevation consistent with undiagnosed HFpEF · exercise testing | unclassified PH (validation cohort) 59% |
Phenotype of unclassified PH vs no PH
- Greater adiposity, higher HFpEF-ABA score probability, and more left heart remodeling in unclassified PH
- Lower glycine metabolites in unclassified PH
- Left heart remodeling, quality of life, exercise capacity, and glycine levels abnormal relative to healthy controls
- Pulmonary artery wedge pressure, pulmonary vascular resistance, and pulmonary artery compliance subtly abnormal at rest (validation cohort)
Original publication
Phenotypic Characterization of Unclassified Pulmonary Hypertension. Circ Heart Fail. Published 19 June 2026. doi:10.1161/circheartfailure.125.014480
Summarised from the published abstract; every number is checked against it