Congenital
Heart Transplantation in Situs Inversus Maintaining Dextrocardia

https://doi.org/10.1053/j.optechstcvs.2018.06.004Get rights and content
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We are currently utilizing a technique of heart transplantation in situs inversus/dextrocardia that maintains dextrocardia after implantation. Concerns may be technical difficulty and long-term complications. We have therefore obtained follow-up in our cohort up to 10 years.

Between July 2007 and February 2017, we performed heart transplantation in five patients with situs inversus and dextrocardia. All had bilateral superior venae cavae (SVC). In all, the recipient left SVC was anastomosed to the donor SVC, and the recipient right SVC was anastomosed to the donor right atrial appendage. The normal donor heart was rotated 120° to the right to maintain a position of dextrocardia in the recipient.

Three patients are alive and well to this date. One patient, who also had a pneumonectomy for recurrent infections at the time of transplant, died early postoperatively from a disseminated fungal infection. Another patient did well initially, however died from post-transplant lymphoproliferative disease 4 years posttransplantation. A third patient required stent implantation 4 years postoperatively to treat stenosis of the right SVC to right atrial anastomosis. There were no other complications related to the surgical technique and no other reinterventions.

This technique maintains excellent long-term outcomes. It uses the existing pericardial cavity most effectively. It avoids pericardial resection, lung compression, or right ventricular compromise. Anastomotic obstruction of the SVC connection to the right atrium may occur, which is amenable to transcutaneous stenting.

Keywords

Situs inversus
Dextrocardia
Cardiac transplantation
Pediatric

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