Pulmonary atresia with ventricular septal defect

Pulmonary atresia with ventricular septal defect (PA-VSD) is a rare cyanotic congenital heart malformation characterized by underdevelopment of the right ventricular outflow tract and atresia of the pulmonary valve, ventricular septal defect (VSD) and pulmonary collateral vessels. Clinical features depend on the anatomic variability of the lesion and patients may be minimally symptomatic, severely cyanotic or may develop congestive heart failure. PA-VSD may represent a severe form of Tetralogy of Fallot (see this term).

Hypertension

The presence of chronic increased pressure in the systemic arterial system.

Total: 2

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PMID (PMCID)
18006164	MIXED_SAMPLE	Adult
	Sildenafil therapy in complex pulmonary atresia with pulmonary arterial hypertension.
	Lim ZS, Vettukattill JJ, Salmon AP, Veldtman GR. Int J Cardiol. 2008;129(3):339-43.
	pulmonary atresia with ventricular septal defect and major aorto-pulmonary collaterals (MAPCAs) or Tetralogy of Fallot with MAPCAs frequently have a clinical course complicated by development of pulmonary arterial (PA) hypertension.
10815306	FEMALE	Adult
	Permissive hypoxemia permitted postoperative weaning from artificial ventilation after repair of pulmonary atresia.
	Golej J, Burda G, Hermon M, Marx M, Boigner H, Trittenwein G. Wien Klin Wochenschr. 2000;112(6):293-6.
	In a 20-year-old female, conduit repair and unifocalisation of pulmonary atresia with ventricular septal defect and systemic to pulmonary arterial collaterals were performed despite preexisting one-sided pulmonary hypertension.