Pulmonary arterial hypertension in congenital heart disease.
Pulmonary arterial hypertension (PAH) is a recognized complication of congenital systemic to pulmonary arterial cardiac shunts. The prognosis of PAH in this situation is better than primary or other secondary forms of PAH. Our knowledge of the pathophysiology of PAH complicating congenital heart disease has evolved over the past decade. Despite differences in etiology and pathobiology, therapies that have proven successful for primary PAH may benefit this group of patients.
Granton
John T
JT
Department of Medicine, University of Toronto, Division of Respirology and Critical Care Medicine Programme, University Health Network, 10 EN-220, 200 Elizabeth Street, Toronto, Ontario, Canada M5G 2C4. john.granton@uhn.on.ca
Rabinovitch
Marlene
M
D004541:Eisenmenger Complex / Q000150:complications* / Q000628:therapy; D016232:Endothelins / Q000037:antagonists & inhibitors*; D004730:Endothelium, Vascular / Q000503:physiopathology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000628:therapy*; D011379:Prognosis; D011651:Pulmonary Artery; D012307:Risk Factors; D014661:Vasoconstriction / Q000502:physiology*; D014665:Vasodilator Agents / Q000627:therapeutic use*
D016232:Endothelins; D014665:Vasodilator Agents