Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Tetralogy of Fallot with absent pulmonary valve is a rare congenital cardiac anomaly. We report on the echocardiographic features of a fetus with such an anomaly and aneurysm of the pulmonary artery associated with hydrops fetalis and polyhydramnios. The prognosis of tetralogy of Fallot with absent pulmonary valve and pulmonary aneurysm is grave, especially in a fetus with intrauterine heart failure. Serial sonographic follow-up is mandatory to detect the signs of hydrops fetalis. Early intervention and intensive treatment should be given to improve the perinatal outcome once the prenatal diagnosis is made.
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PMID:Prenatal diagnosis of tetralogy of Fallot with absent pulmonary valve accompanied by hydrops fetalis. 925 57

Tetralogy of Fallot with absent pulmonary valve syndrome is associated with absence of the left pulmonary artery in one-seventh of cases. Infants with this disorder commonly present very early in life with respiratory distress and heart failure. Because the pulmonary blood flow is primarily to the right lung only, severe respiratory distress occurs. Survival to late childhood without surgery is very rare due to clinical worsening in infancy. This report describes two adult natural survivors with this rare association who presented with extremely unusual unreported clinical features. The aneurysmally dilated main pulmonary artery was compressed on the left coronary ostium, causing angina in the first patient. The second patient had recurrent ventricular tachycardia originating from the dilated thinned out right ventricular outflow tract. Both patients had successful surgical correction with valved xenograft conduits.
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PMID:Unusual clinical presentation of rare adult survivors with a missing pulmonary valve associated with a missing left pulmonary artery. 2286 76