Orthotopic Heart Transplantation for Congenital Heart Disease: An Alternative for High-Risk Fontan Candidates?

Abstract

Objective-Evaluation of incremental risk factors for early mortality in children undergoing orthotopic heart transplantation (OHT) for congenital heart disease. Methods-Between 1988 and 2002, 43 patients (mean age 9.1Ϯ7.2 years) underwent 44 OHT for complex TGA (6), DORV (4), single ventricle (21), and other end-stage structural heart disease (11). Two discernible ventricular chambers were present in 18 pts (41.8%). Previous reconstructive or palliative procedures had been previously accomplished in 35 pts (83.3%), including atrial switch (5), systemic-to-pulmonary shunts (10), cavopulmonary anastomosis (9), Fontan completion (6), and others (5). Results-30-day survival for the 2-ventricle subgroup was 94.4Ϯ5.4% compared with 67.2Ϯ9.5% for the single ventricle subgroup (Pϭ0.04) (overall 78.6%Ϯ3.3%). OHT following single ventricle staging to bi-directional cavopulmonary anastomosis exhibited 100% early survival, as opposed to 62.5Ϯ17.1% for OHT after systemic-to-pulmonary shunts, and 33.3Ϯ19.2% for OHT following failing Fontan (Pϭ0.010). HLHS diagnosis (0.0085) and failing Fontan (Pϭ0.003) were identified as independent predictors of early mortality by regression logistic modeling, while Fontan stage represented the only predictor of overall mortality by Cox proportional hazard. Overall 10-year survival was 54.3Ϯ11%. Conclusions-OHT for structural congenital heart disease with single ventricle physiology entails substantial early mortality and bi-directional cavopulmonary anastomosis enables the best transition to heart transplant. OHT should be considered in the decision making process as an alternative to Fontan completion in high-risk candidates, since rescue-OHT after failing Fontan seems unwarranted.

Transplantation in the single ventricle population

“…In previous eras, these patients with immediate compromise who are usually extremely sick (47), were not considered for transplant and therefore underwent Fontan take-down. Mortality of OHT in this scenario is reported to be as high as 44-66% (29,48). In our institution, OHT proved to be viable although technically challenging, in a limited cohort in this situation (49).…”

“…However, destabilization of multiple homeostatic mechanisms leads to high peri-operative risk which must be considered when counselling patients (4,28). Finding better ways to monitor progression in this apparently "stable" group will in the future better inform timing of OHT, which has led some to suggest that OHT is not indicated in this cohort (29).…”

“…SV physiology in adults has been shown to carry a 3-fold higher risk of in-hospital mortality following OHT compared with biventricular pathologies (16), prompting discussion of appropriate allocation of limited donor resources (21,29,37). Early complications, relating to transplant complexity, contribute substantially to the mortality of both adults and children and have been reported across the literature (24,28,38).…”

“…Early and late survival is reported to be optimal when OHT is performed following Glenn shunt but before Fontan completion rather than at shunt stage, or even worse, Fontan stage (29). Following the Glenn shunt, the detrimental effect of shunt physiology including ventricular overload and coronary steal have been reduced and the multi-organ consequences of Fontan completion which contribute to early post-operative mortality are avoided (24).…”

Pulsatile venous waveform quality in Fontan circulation-clinical implications, venous assists options and the future

“…For many of these patients, heart transplantation has become the next surgical "stage". However, donor shortage and the high-risk nature of transplantation for these complex and often very ill patients demand a search for alternative forms of treatment (2). Unfortunately, the progress to develop alternative surgical strategies for patients with single ventricle physiology has reached a plateau with minimally derived physiologic benefit from minute adjustments to surgical technique.…”