Adult congenital heart disease and pulmonary arterial hypertension: The texas adult congenital heart program experience

Wayne J. Franklin, Dhaval R. Parekh, Zeenat Safdar

Research output: Contribution to journalArticle

Abstract

Congenital heart disease (CHD) is a common structural defect of the heart or major blood vessels. Patients with adult congenital heart disease (ACHD) have medical needs that are distinct from those of pediatric patients with CHD, and the transition into adult health care is important for management of the patient with ACHD. A large proportion of patients with CHD develop diseases and complications associated with the long-term stress of intracardiac shunts. Pulmonary arterial hypertension (PAH) is a significant complication of some CHD lesions. The treatment of these patients remains challenging due to their combined heart and lung disease, and multidisciplinary care is ofen necessitated for a variety of secondary conditions. A number of treatment options are available for the management of PAH associated with CHD, including prostanoids, phosphodiesterase type-5 inhibitors, and endothelin receptor antagonists. This article discusses the diagnosis and management of such ACHD patients with PAH.

Original languageEnglish (US)
Pages (from-to)32-45
Number of pages14
JournalPostgraduate medicine
Volume123
Issue number6
DOIs
StatePublished - Nov 1 2011

Keywords

  • Congenital heart disease
  • Echocardiography
  • Endothelin receptor antagonist
  • Heart failure
  • Phosphodiesterase type-5 inhibitor
  • Prostacyclin
  • Pulmonary arterial hypertension

ASJC Scopus subject areas

  • Medicine(all)

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