The major diagnoses carried by children undergoing
cardiac transplantation worldwide are
congenital heart defects,
cardiomyopathies, and retransplantation. The leading diagnosis in infancy is
congenital heart disease, whereas
cardiomyopathy predominates in older children. In view of this wide spectrum of diagnoses, the perioperative management of these children requires medical, interventional, and surgical expertise in treatment of complex
congenital heart defects, end-stage
heart failure, and
cardiac transplantation. According to the Pediatric
Heart Transplantation Survey database, the majority of children listed for
cardiac transplantation eventually require higher levels of cardiac support before
transplantation. The team caring for these children should be prepared to escalate support in a timely fashion in order to avoid end-organ dysfunction or a catastrophic event that will remove the patient from the
cardiac transplantation list. The first step is advanced hemodynamic monitoring in a specialized pediatric cardiac intensive care unit and initiation of inotropic support. Further escalation of care should be based on careful analysis of the hemodynamic profile, end-organ function, and
biochemical markers of perfusion and myocardial stress. A patient who continues to deteriorate in spite of inotropic support requires
positive pressure ventilation, and if deterioration continues, mechanical circulatory support is initiated.
Cardiac transplantation is a challenging operation, and even more so in children with complex
congenital heart defects. The abnormal cardiovascular anatomy requires planning and anticipation of possible pitfalls as hypoplasia of the aortic arch, abnormal pulmonary arteries, and abnormal systemic and pulmonary venous connections. The time required to remove adhesions in children with prior cardiac operations increases the ischemic time of the graft and the risk of
primary graft dysfunction. Assessment of pulmonary vascular resistance in children with
congenital heart defects is problematic, and even children with a normal transpulmonary gradient and pulmonary vascular resistance are at increased risk of postoperative
pulmonary hypertension and right ventricular graft failure. The postoperative course is directly linked to the patient's preoperative physical condition and perioperative course. The induction of immunosuppression and the use of
plasmapheresis in children with a positive cross-match may lead to further hemodynamic compromise. If severe
primary graft dysfunction evolves, early initiation of extracorporeal membranous
oxygenator is indicated to avoid irreversible end-organ dysfunction.