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

Mark-Jan Ploegstra; Jody G M Brokelman; Jolien W Roos-Hesselink; Johannes M Douwes; Lenny M van Osch-Gevers; Elke S Hoendermis; Annemien E van den Bosch; Maarten Witsenburg; Beatrijs Bartelds; Hans L Hillege; Rolf M F Berger

doi:10.1093/ehjci/jex015

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

Eur Heart J Cardiovasc Imaging. 2018 Feb 1;19(2):216-224. doi: 10.1093/ehjci/jex015.

Affiliations

¹ Center for Congenital Heart Diseases, Department of Paediatric Cardiology, Beatrix Children's Hospital, University of Groningen, University Medical Center Groningen, The Netherlands.
² Department of Cardiology, Erasmus University Medical Center, Rotterdam, The Netherlands.
³ Department of Paediatric Cardiology, Erasmus University Medical Center, Sophia Children's Hospital, Rotterdam, The Netherlands.
⁴ Center for Congenital Heart Diseases, Department of Cardiology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
⁵ Department of Epidemiology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.

PMID: 28329155
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 <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.

Keywords: congenital heart disease; outcome; paediatrics; pulmonary arterial hypertension; pulmonary vascular disease; stiffness.

MeSH terms

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