Congenital Heart Disease-Associated Pulmonary Hypertension

Clin Chest Med. 2021 Mar;42(1):9-18. doi: 10.1016/j.ccm.2020.11.005. Epub 2021 Jan 12.

Abstract

Presently, with increasing survival of patients with congenital heart disease (CHD), pulmonary arterial hypertension (PAH) associated with CHD is commonly encountered in children and adults. This increased prevalence is seen despite significant advances in early diagnosis and surgical correction of patients with structural CHD. PAH is the cause of significant morbidity and mortality in these patients and comes in many forms. With the increased availability of targeted therapies for PAH, there is hope for improved hemodynamics, exercise capacity, quality of life, and possibly survival for these patients. There may also be opportunities for combined medical and interventional/surgical approaches for some.

Keywords: Congenital heart disease; Eisenmenger syndrome; Pulmonary arterial hypertension; Pulmonary vascular disease.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Child
  • Child, Preschool
  • Exercise*
  • Heart Defects, Congenital / complications*
  • Hemodynamics*
  • Humans
  • Hypertension, Pulmonary* / epidemiology
  • Hypertension, Pulmonary* / etiology
  • Hypertension, Pulmonary* / surgery
  • Hypertension, Pulmonary* / therapy
  • Longevity
  • Middle Aged
  • Patients
  • Quality of Life*
  • Young Adult