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.
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.



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).



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.
Center for Congenital Heart Diseases, Department of Paediatric Cardiology, Beatrix Children's Hospital, University of Groningen, University Medical Center Groningen, The Netherlands.
Mark-Jan
MJ
Ploegstra
Center for Congenital Heart Diseases, Department of Paediatric Cardiology, Beatrix Children's Hospital, University of Groningen, University Medical Center Groningen, The Netherlands.
Jody G M
JGM
Brokelman
Department of Cardiology, Erasmus University Medical Center, Rotterdam, The Netherlands.
Jolien W
JW
Roos-Hesselink
Center for Congenital Heart Diseases, Department of Paediatric Cardiology, Beatrix Children's Hospital, University of Groningen, University Medical Center Groningen, The Netherlands.
Johannes M
JM
Douwes
Department of Paediatric Cardiology, Erasmus University Medical Center, Sophia Children's Hospital, Rotterdam, The Netherlands.
Lenny M
LM
van Osch-Gevers
Center for Congenital Heart Diseases, Department of Cardiology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
Elke S
ES
Hoendermis
Department of Cardiology, Erasmus University Medical Center, Rotterdam, The Netherlands.
Annemien E
AE
van den Bosch
Department of Cardiology, Erasmus University Medical Center, Rotterdam, The Netherlands.|Department of Paediatric Cardiology, Erasmus University Medical Center, Sophia Children's Hospital, Rotterdam, The Netherlands.
Maarten
M
Witsenburg
Center for Congenital Heart Diseases, Department of Paediatric Cardiology, Beatrix Children's Hospital, University of Groningen, University Medical Center Groningen, The Netherlands.
Beatrijs
B
Bartelds
Department of Epidemiology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
Hans L
HL
Hillege
Center for Congenital Heart Diseases, Department of Paediatric Cardiology, Beatrix Children's Hospital, University of Groningen, University Medical Center Groningen, The Netherlands.
Rolf M F
RMF
Berger
D006328:Cardiac Catheterization / Q000379:methods; D002648:Child; D015331:Cohort Studies; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging* / Q000401:mortality / Q000503:physiopathology; D008297:Male; D011446:Prospective Studies; D011651:Pulmonary Artery / Q000000981:diagnostic imaging*; D011669:Pulmonary Wedge Pressure; D015996:Survival Rate; D013997:Time Factors; D018084:Ultrasonography, Interventional / Q000379:methods*; D014655:Vascular Resistance; D059289:Vascular Stiffness; D018497:Ventricular Dysfunction, Right / Q000000981:diagnostic imaging* / Q000503:physiopathology
congenital heart disease; outcome; paediatrics; pulmonary arterial hypertension; pulmonary vascular disease; stiffness