Right and left isomerism: the cardiac surgeon's view.
We have treated by palliative or corrective surgery 12 patients with the complex cardiovascular anomalies associated with right isomerism (asplenia syndrome) or left isomerism (polysplenia syndrome). A systemic-pulmonary artery shunt was performed in 4 patients, with 2 early deaths. Anatomical intracardiac repair was successfully done in 2 patients, while an orthoterminal repair using a modified Fontan procedure was attempted in 5 patients, only 1 of whom survived. A 1-year-old girl with diffuse pulmonary arteriovenous fistulas associated with left isomerism was not considered a good candidate for operation after an exploratory sternotomy. The surgical options and technical problems related to the complex intracardiac morphology of these developmental syndromes are presented.
D000293:Adolescent; D000328:Adult; D001166:Arteriovenous Shunt, Surgical; D002648:Child; D002675:Child, Preschool; D003914:Dextrocardia / Q000601:surgery; D005260:Female; D006330:Heart Defects, Congenital / Q000601:surgery*; D006343:Heart Septal Defects / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010166:Palliative Care; D011379:Prognosis; D011667:Pulmonary Veins / Q000002:abnormalities; D013154:Spleen / Q000002:abnormalities; D013577:Syndrome