Comprehensive Noninvasive Evaluation of Right Ventricle-Pulmonary Circulation Axis in Pediatric Patients with Pulmonary Hypertension

Abstract

Purpose of review: The purpose of this review is to detail the advanced echocardiographic studies that evaluated outcomes in pediatric PH using these advanced imaging techniques: (1) right heart size and function (using 2D and 3D echocardiography), (2) myocardial mechanics, and (3) right ventricular (RV) to pulmonary arterial coupling.

Recent findings: Advanced echocardiographic imaging tools in pediatric PH include RV/left ventricular ratio, 3D echocardiographic assessment of RV volumes and ejection fraction, right atrial and RV strain, and RV-pulmonary arterial coupling ratio that allow for evaluation of clinical outcomes in this patient population. The right atrial to RV axis provides insights to RV diastolic function that has been poorly understood in pediatrics. The RV-pulmonary arterial coupling ratio helps us understand the mechanics of the RV response to afterload. The advanced imaging tools provide clinical outcome evaluation in pediatric PH patients and provide insight into the RA-RV axis and the RV-pulmonary circulatory unit.

Keywords: 3D echocardiography; Myocardial mechanics; Pediatric pulmonary hypertension; Right atrial function; Right ventricular function; Ventricular vascular coupling ratio.