Pulmonary vascular disease & thromboembolism
The NOTCH3 extracellular domain is a serum biomarker for pulmonary arterial hypertension
Authors' conclusion
Measurement of serum NOTCH3-ECD can therefore provide a highly sensitive, specific and noninvasive test for predicting the presence, disease severity, progression and survival of individuals with IPAH.
Study context
- Population
- Individuals with WHO group 1.1 pulmonary hypertension (idiopathic pulmonary arterial hypertension (IPAH)) and healthy individuals3 cohorts
- Participants
- 341 vs 376
- Comparison
- Serum NOTCH3 extracellular domain (NOTCH3-ECD) in IPAH vs healthy controls
- IPAHn = 341
- Healthy controlsn = 376reference
- Outcome
- Serum NOTCH3-ECD level
Methodological detailsthree geographically distinct cohorts
- Three geographically distinct cohorts
Performance
As reported in the abstract
P < 0.001
Further findings
| Outcome | Values |
|---|---|
| Diagnostic accuracy of serum NOTCH3-ECD for IPAH | NOTCH3-ECD AUC 0.96 (95% CI 0.95-0.98) · Sensitivity 90% · Specificity 93% · Cutoff 13.0 ng ml-1 |
3-year mortality in IPAH
- Mortality risk increase per 3 ng ml-1 NOTCH3-ECD above the diagnostic cutoff
- 18%
Correlations and prognostic calculators
- NOTCH3-ECD correlated with mean right atrial pressure, PVR, mean PA pressure, tricuspid regurgitant velocity, 6-min walk distance and NYHA class
- Adding NOTCH3-ECD improved REVEAL 2.0, REVEAL 2.0 Lite and COMPERA 2.0
- Predicted IPAH in treatment-naive individuals; correlated with disease progression over 6 years
Original publication
The NOTCH3 extracellular domain is a serum biomarker for pulmonary arterial hypertension. Nat Med. Published 9 January 2026. doi:10.
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