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

  • Pei-Ni JoneEmail author
  • Dunbar D. Ivy
Pediatric and Congenital Heart Disease (G Singh, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Pediatric and Congenital Heart Disease


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.


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


Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflicts of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Authors and Affiliations

  1. 1.Pediatric Cardiology, Children’s Hospital ColoradoUniversity of Colorado School of MedicineAuroraUSA

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