P. Macchiarini et al., DOUBLE-LUNG TRANSPLANTATION IN SITUS-INVERSUS WITH KARTAGENERS-SYNDROME, Journal of thoracic and cardiovascular surgery, 108(1), 1994, pp. 86-91
En bloc double lung transplantation with bilateral bronchial anastomos
es was successfully performed in three patients with complete situs in
versus and end-stage Kartagener's syndrome. Dextrocardia was not a tec
hnical problem for institution of cardiopulmonary bypass, but a large
azygos vein draining the systemic venous return was systematically pre
served. The major technical difficulty was restoration of airway conti
nuity, because patients with situs inversus have an inverse direction
and length of the main stem bronchi. The right and left main bronchi o
f the recipients were approached in the aortocaval sinus and transecte
d approximately at 1.5 cm from the carina. The donor right main stem b
ronchus was divided at its origin and the donor left main stem bronchu
s was divided proximal to the upper lobe takeoff. The different bronch
ial angulation was not an obstacle, and airway continuity was reestabl
ished twice with an end-to-end anastomosis and once with a telescopic
technique. Because of the midline position of the left atrium and pulm
onary artery, the anastomoses with the respective recipient's structur
es were made as in patients with situs solitus. One patient required a
right lower lobectomy because the position of the right side of the h
eart interfered with lobar expansion. One patient died of obliterative
bronchiolitis 36 months after the operation. The remaining two are al
ive and doing well after 48 and 6 months, respectively.