Adult congenital heart disease and pulmonary arterial hypertension: the Texas Adult Congenital Heart Program experience

Postgrad Med. 2011 Nov;123(6):32-45. doi: 10.3810/pgm.2011.11.2493.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Algorithms
  • Antihypertensive Agents / therapeutic use
  • Bosentan
  • Endothelin Receptor Antagonists
  • Epoprostenol / therapeutic use
  • Familial Primary Pulmonary Hypertension
  • Heart Defects, Congenital / complications*
  • Hospitals, Pediatric
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / drug therapy
  • Hypertension, Pulmonary / epidemiology
  • Hypertension, Pulmonary / etiology*
  • Phenylpropionates / therapeutic use
  • Phosphodiesterase 5 Inhibitors / therapeutic use
  • Prevalence
  • Pyridazines / therapeutic use
  • Sulfonamides / therapeutic use
  • Texas
  • Transition to Adult Care


  • Antihypertensive Agents
  • Endothelin Receptor Antagonists
  • Phenylpropionates
  • Phosphodiesterase 5 Inhibitors
  • Pyridazines
  • Sulfonamides
  • Epoprostenol
  • ambrisentan
  • Bosentan