Regional right ventricular remodeling and function in children with idiopathic pulmonary arterial hypertension vs those with pulmonary valve stenosis: Insights into mechanics of right ventricular dysfunction

Background Right ventricular (RV) pressure overload in the context of pulmonary stenosis (PS) has a much better prognosis than in the context of idiopathic pulmonary arterial hypertension (iPAH), which may be related to differences in global and regional RV remodeling and systolic function. We compa...

Full description

Saved in:
Bibliographic Details
Published in:Echocardiography (Mount Kisco, N.Y.) N.Y.), 2017-06, Vol.34 (6), p.888-897
Main Authors: Driessen, Mieke M.P., Meijboom, Folkert J., Hui, Wei, Dragulescu, Andreea, Mertens, Luc, Friedberg, Mark K.
Format: Article
Language:English
Subjects:
Child
Echocardiography - methods
Echocardiography, Doppler - methods
Familial Primary Pulmonary Hypertension - complications
Familial Primary Pulmonary Hypertension - physiopathology
Female
Heart Ventricles - diagnostic imaging
Heart Ventricles - physiopathology
Humans
idiopathic pulmonary hypertension
Male
pediatric
pulmonary stenosis
Pulmonary Valve Stenosis - complications
Pulmonary Valve Stenosis - physiopathology
Retrospective Studies
right ventricular function
Ventricular Dysfunction, Right - complications
Ventricular Dysfunction, Right - diagnostic imaging
Ventricular Dysfunction, Right - physiopathology
Ventricular Remodeling - physiology
Citations: Items that this one cites
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Background Right ventricular (RV) pressure overload in the context of pulmonary stenosis (PS) has a much better prognosis than in the context of idiopathic pulmonary arterial hypertension (iPAH), which may be related to differences in global and regional RV remodeling and systolic function. We compared RV mechanics in children with PS to those with iPAH, aiming to identify mechanisms and markers of RV dysfunction. Methods Eighteen controls, 18 iPAH and 16 PS patients were retrospectively studied. Age, BSA, and sex distribution were comparable. Two‐dimensional echocardiography, blood flow and tissue Doppler, and longitudinal RV deformation were analyzed. ANCOVA—including RV systolic pressure (RVSP) and length as covariates—was used to compare patient groups. Results RV systolic pressure was higher in iPAH vs PS (96.8±25.4 vs 75.4±18.9 mm Hg, P=.011). Compared to controls, PS patients showed mild dilation (P
ISSN:0742-2822
1540-8175
DOI:10.1111/echo.13529