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

• Published in: European heart journal cardiovascular imaging 2018-02, Vol.19 (2), p.216-224
• Main Authors: Ploegstra, Mark-Jan, Brokelman, Jody G M, Roos-Hesselink, Jolien W, Douwes, Johannes M, van Osch-Gevers, Lenny M, Hoendermis, Elke S, van den Bosch, Annemien E, Witsenburg, Maarten, Bartelds, Beatrijs, Hillege, Hans L, Berger, Rolf M F
• Format: Article
• Language: English
• Subjects: Cardiac Catheterization - methods; Child; Cohort Studies; Disease Progression; Female; Follow-Up Studies; Humans; Hypertension, Pulmonary - diagnostic imaging; Hypertension, Pulmonary - mortality; Hypertension, Pulmonary - physiopathology; Male; Prospective Studies; Pulmonary Artery - diagnostic imaging; Pulmonary Wedge Pressure; Survival Rate; Time Factors; Ultrasonography, Interventional - methods; Vascular Resistance; Vascular Stiffness; Ventricular Dysfunction, Right - diagnostic imaging; Ventricular Dysfunction, Right - physiopathology
• ISSN: 2047-2404; 2047-2412
• DOI: 10.1093/ehjci/jex015

Abstract 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