Johnson, Jonathan N.

AbstractWe performed a retrospective review of outcomes after heart transplantation during long‐term follow‐up of a surgical cohort of 1138 Fontan patients who were followed at the Mayo Clinic. Follow‐up information was obtained from medical records and a clinical questionnaire that was mailed to patients not known to be deceased at the initiation of the study. Forty‐four of 1138 Fontan patients with initial or subsequent evaluation at Mayo had cardiac transplantation between 1988 and 2014 (mean age at transplantation was 23.2 ± 12 yr, median was 19.8 yr; mean interval between Fontan and transplantation was 13.0 ± 7.7 yr, median was 13.1 yr). Two patients had combined organ transplantation (one heart–lung, one heart–liver). Twelve of the 44 (27%) patients had PLE prior to transplantation. There was no difference in post‐bypass Fontan pressures or incidence of late reoperations for AVV repair/replacement between transplanted and non‐transplanted patients. There were 16 (36%) deaths in the transplantation cohort; seven occurred within 30 days of transplantation. Overall one, five, 10, and 15 yr post‐transplantation survival was 80%, 72%, 69%, and 55%, respectively. Although this is a challenging group of patients, intermediate‐term results suggest that cardiac transplantation remains a reasonable option for patients with a failed Fontan circulation.

AbstractSVC obstruction is a rare acute complication after bicaval cardiac transplantation and can be catastrophic for the patient. Herein, we report the case of a child who developed SVC thrombosis causing acute SVC syndrome nine days after heart transplantation. Thrombolysis was successfully performed using the AngioJet™ system and subsequent balloon angioplasty with re‐establishment of flow. This procedure allowed for revascularization without repeat sternotomy or stent implantation. The patient is well without recurrence at last follow‐up.

AbstractSirolimus is used in heart transplant patients with CAV and CNI‐induced nephropathy. However, little is known regarding the tolerability, rejection rate, and effect on renal function when used empirically in children. We describe our experience with the empiric use of a sirolimus‐based immunosuppressive regimen in pediatric heart transplantation recipients. We reviewed records of patients in whom conversion was attempted to a CNI‐free sirolimus‐based regimen. Rejection episodes and measures of renal function were recorded. We attempted to convert 20 patients, of which 16 were successful. In total, six of 20 patients (30%) experienced adverse effects. Of the 16 converted, four patients converted to sirolimus due to CNI‐induced disease (three nephropathy, one CAV), while 12 patients (mean age 5.5 yr, range 0.1–21 yr; 33% female; 33% with a history of congenital heart disease) were empirically switched to sirolimus at a mean of 2.3 yr after transplant. Follow‐up was available for a mean of 2.5 yr after conversion (range 0.5–8.3 yr). The rate of rejection while taking CNIs was 0.18 rejection episodes per patient‐year (total of five episodes), compared with 0.03 rejection episodes per patient‐year (total of one episode) while on sirolimus. Renal function, in terms of GFR, significantly improved after sirolimus conversion at latest follow‐up (from 86 ± 37 mL/min to 130 ± 49 mL/min, p = 0.02). Here, we demonstrate the potential benefit of empiric use of sirolimus in pediatric heart transplant patients in a CNI‐free regimen. Larger and longer studies are needed to further clarify risks of rejection and adverse effect profiles.

We present the case of a 36‐year‐old woman with increasing shortness of breath, a new 3/4 diastolic murmur, and a complex history of LV outflow tract obstruction. She has undergone multiple surgeries including the replacement of her old LV apex to ascending aorta conduit with a 20‐mm Gore‐Tex tube graft, addition of a 24‐mm homograft sutured between the conduit and the LV apex, and insertion of a 21‐mm Freestyle porcine valve conduit between the Gore‐Tex tube graft and allograft at age 23. The current assessment showed a failing Freestyle conduit prosthesis leading to left heart decompensation. Due to substantial surgical risk, the patient underwent successful implantation of a Melody valve into the Gore‐Tex tube and exclusion of the failing Freestyle bioprosthesis with a NuMed CP stent in a hybrid procedure. The case nicely illustrates the collaborative potential of cardiovascular surgeons and interventional cardiologists in the new arena of a hybrid operating room. Complex hybrid procedures like the current one, especially those including percutaneous placements of valves, offer therapeutic options for patients that are otherwise too high risk for conventional open heart surgery. © 2013 Wiley Periodicals, Inc.