[Scimitar syndrome with left pulmonary hypertension and right pulmonary artery stenosis in childhood--report of a case of successful surgical repair].
An one-year old child with Scimitar syndrome (anomalous drainage of the right pulmonary vein to inferior vena cava with right lung hypoplasia) associated with left side pulmonary hypertension and right pulmonary artery stenosis underwent surgical repair. The anomalous pulmonary vein was anastomosed to right atrium with 10 mm PTFE graft interposition and drained to left atrium through equine pericardial intra-atrial baffle. The stenotic portion of right pulmonary artery was enlarged with porcine pericardial patch. To our knowledge, this is the first successful surgical case of Scimitar syndrome with pulmonary hypertension in childhood reported in literature. A repair of anomalous pulmonary venous drainage to inferior vena cava in childhood is difficult, and a meticulous selection of surgical procedure is necessary.
Fujita
Y
Y
Yoshimura
T
T
Miyamura
H
H
Kanazawa
H
H
Imaizumi
K
K
Takahashi
Y
Y
Fukuda
J
J
Aono
T
T
Eguchi
S
S
D001807:Blood Vessel Prosthesis; D003251:Constriction, Pathologic / Q000150:complications / Q000601:surgery; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications*; D007223:Infant; D011651:Pulmonary Artery / Q000601:surgery*; D012587:Scimitar Syndrome / Q000150:complications / Q000601:surgery*