[Raghib's syndrome associated with cor triatriatum--a rare surgical case report].
A 27-year-old male with a history of congenital heart disease was admitted for pre-operative evaluation of a cardiac malformation. Echocardiography and cardiac catheterization revealed an incomplete endocardial cushion defect with a persistent left superior vena cava which drained into the left atrium but echo-cardiographic evidence of an abnormal intra-atrial septum was not found. The patient was placed on cardiopulmonary bypass and prepared for the surgical correction of his primary cardiac lesion. Intra-cardiac examination during reconstruction of Raghib's syndrome also revealed the presence of cor triatriatum. Reghib's syndrome is characterized by the combination of abnormal drainage from the left superior vena cava into the left atrium, the presence of an atrial septal defect and the absence of a coronary sinus. To prevent secondary complications such as a brain abscess, we redirected blood flow from the left superior vena cava to the right atrium utilizing a trimmed woven dacron vascular graft that was placed in the intra-atrial position. Mitral valvoplasty, excision of the obstructing diaphragm and atrial septation were also performed successfully. Although the literature has described the surgical repair of Raghib's syndrome, its correction in combination with cor triatriatum is considered to be extremely rare.
Fujimura
Y
Y
Katoh
T
T
Koizumi
S
S
D000328:Adult; D003310:Cor Triatriatum / Q000150:complications* / Q000601:surgery; D003330:Coronary Vessel Anomalies / Q000150:complications / Q000601:surgery*; D006344:Heart Septal Defects, Atrial / Q000150:complications / Q000601:surgery*; D006801:Humans; D008297:Male; D013577:Syndrome; D014683:Vena Cava, Superior / Q000002:abnormalities*