Idiopathic pulmonary arterial hypertension is a rare, progressive and life-limiting disease that is difficult to diagnose because of its nonspecific symptoms and reliance on invasive right-heart catheterization for diagnosis. Diagnostic delays contribute to a highly heterogeneous patient population. Current blood biomarkers reflect mainly secondary cardiac strain rather than pulmonary vascular pathology, but a new paper highlights the NOTCH3 extracellular domain as a pulmonary vasculature- and idiopathic-specific blood biomarker to support diagnosis and prognosis.
- Merve Keles
- Martin R. Wilkins
- Allan Lawrie
