Pulmonary arterial stiffness indices assessed by intravascular ultrasound in children with early pulmonary vascular disease:Prediction of advanced disease and mortality during 20-year follow-up

Aims Prognosis in children with pulmonary vascular disease (PVD) is closely linked to right ventricular (RV) failure due to increased RV-afterload. Pulmonary arterial (PA) stiffening is known to occur early in the course of PVD and constitutes a main component of RV-afterload. This study aimed to evaluate the clinical value of PA-stiffness in children with PVD by determining its association with advanced pulmonary arterial hypertension (PAH) and mortality at long-term follow-up. Methods and results Forty-one children with various stages of arterial PVD, defined as mean PA-pressure >= 20 mmHg and/or pulmonary-to-systemic flow-ratio >= 1.2, and mean pulmonary capillary wedge pressure Conclusion In children with PVD, PA-stiffness indices assessed by IVUS predict advanced PAH and mortality at long term follow-up. Especially in patients with favourable haemodynamics, assessment of intrinsic PA-stiffness may enhance the prognostication of disease progression and survival