Staged correction of double discordance, situs inversus, and absence of true pulmonary arteries.
The case of a young boy with an extremely rare and complex cardiac malformation is described. Situs inversus, dextrocardia, double discordance with pulmonary atresia, absence of true pulmonary arteries, and systemico-pulmonary collateral arteries was the original diagnosis. Three preparatory operations were necessary to reconstruct and unifocalize the pulmonary vascular tree. At the age of 7 years complete correction with intraoperative stenting of the left neopulmonary artery was successfully performed. After more than 3 years from the definitive operation, the patient is alive with no physical limitations.
Brunelli
Federico
F
Division of Cardiac Surgery, Private Non-Profit Hospital Poliambulanza, Brescia, Italy. cch-segreteria.poli@poliambulanza.it
Amaducci
Andrea
A
Tasca
Giordano
G
Mhagna
Zen
Z
Amari
Bruno
B
D002648:Child; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000601:surgery; D012086:Reoperation