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 ≥20mmHg and/or pulmonary-To-systemic flow-ratio ≥1.2, and mean pulmonary capillary wedge pressure <15 mmHg, underwent cardiac catheterization with intravascular ultrasound (IVUS) imaging between 1994 and 1997 with follow-up until 2015. PA-stiffness indices evaluated were PA-Area-compliance (PA-compliance) and PA-Area-distensibility (PA-distensibility). During follow-up, advanced PAH was determined by echocardiography and cardiac catheterization. During a median follow-up of 19 years, in 31 (76%) patients PVD had reversed and 10 (24%) had advanced PAH. Six (15%) died due to PVD. In addition to conventional haemodynamics, PA-compliance and PA-distensibility were associated with advanced PAH at long-Term follow-up (adjusted OR [95% CI] 0.56 [0.37-0.85] and 0.52 [0.31-0.86]), and mortality (adjusted HR [95% CI] 0.60 [0.41-0.87] and 0.67 [0.49-0.90]). Also in a subgroup of patients with favourable haemodynamics, baseline PA-compliance and PA-distensibility were lower in patients with advanced PAH at follow-up (P = 0.002 /P = 0.030). 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.

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Keywords Congenital heart disease, Outcome, Paediatrics, Pulmonary arterial hypertension, Pulmonary vascular disease, Stiffness
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Journal European Heart Journal Cardiovascular Imaging
Ploegstra, M.-J. (Mark-Jan), Brokelman, J.G.M. (Jody G. M), Roos-Hesselink, J.W, Douwes, J.M. (Johannes M), van Osch-Gevers, L, Hoendermis, E.S, … Berger, R.M.F. (Rolf M. F). (2018). Pulmonary arterial stiffness indices assessed by intravascular ultrasound in children with early pulmonary vascular disease. European Heart Journal Cardiovascular Imaging, 19(2), 216–224. doi:10.1093/ehjci/jex015