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Diagnosis and Pathophysiological Mechanisms of Group 3 Hypoxia-Induced Pulmonary Hypertension

  • Kel Vin Woo
  • David M. Ornitz
  • Gautam K. SinghEmail author
Pediatric and Congenital Heart Disease (G Singh, Section Editor)
  • 89 Downloads
Part of the following topical collections:
  1. Topical Collection on Pediatric and Congenital Heart Disease

Abstract

Purpose of review

Group 3 hypoxia-induced pulmonary hypertension (PH) is an important and increasingly diagnosed condition in both the pediatric and adult population. The majority of pulmonary hypertension studies to date and all three classes of drug therapies were designed to focus on group 1 PH. There is a clear unmet medical need for understanding the molecular mechanisms of group 3 PH and a need for novel non-invasive methods of assessing PH in neonates.

Recent findings

Several growth factors are expressed in patients and in animal models of group 3 PH and are thought to contribute to the pathophysiology of this disease. Here, we review some of the findings on the roles of vascular endothelial growth factor A (VEGFA), platelet-derived growth factor B (PDGFB), transforming growth factor-beta (TGFB1), and fibroblast growth factors (FGF) in PH. Additionally, we discuss novel uses of echocardiographic parameters in assessing right ventricular form and function.

Summary

FGF2, TGFB, PDGFB, and VEGFA may serve as biomarkers in group 3 PH along with echocardiographic methods to diagnose and follow right ventricle function. FGFs and VEGFs may also function in the pathophysiology of group 3 PH.

Keywords

Pulmonary hypertension Hypoxia Group 3 pulmonary hypertension Echocardiography Right ventricle Fibroblast growth factors 

Notes

Compliance with Ethical Standards

Conflict of Interest

Kel Vin Woo declares no potential conflicts of interest.

David M. Ornitz reports a grant from the NIH.

Gautam K. Singh is a section editor for Current Treatment Options in Cardiovascular Medicine.

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

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Authors and Affiliations

  • Kel Vin Woo
    • 1
    • 2
  • David M. Ornitz
    • 2
  • Gautam K. Singh
    • 1
    Email author
  1. 1.Department of PediatricsWashington University School of MedicineSt. LouisUSA
  2. 2.Department of Developmental BiologyWashington University School of MedicineSaint LouisUSA

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