Purpose of the Review
This is a comprehensive update on failing Fontan physiology and the role of heart and combined heart and liver transplantation in the current era.
Recent Findings
Single ventricle physiology encompasses a series of rare congenital cardiac abnormalities that are characterized by absence of or hypoplasia of one ventricle. This effectively results in a single ventricular pumping chamber. These abnormalities are rarely compatible with long-term survival if left without surgical palliation in the first few years of life. Surgical treatment of single ventricle physiology has evolved over the past 60 years and is characterized by numerous creative innovations. These include the development of arteriopulmonary shunts, the evolution of partial cavopulmonary connections, and the eventual development of the “Fontan” operation. Regardless of the type of Fontan modification, the long-term consequences of the Fontan operation are predominantly related to chronic central venous hypertension and the multi-organ consequences thereof. Atrial arrhythmias can further compromise this circulation.Patients with single ventricle physiology represent a special sub-segment of congenital cardiac transplants and are arguably the most challenging patients considered for transplantation.
Summary
This review describes in detail the challenges and opportunities of heart and liver transplantation in Fontan patients, as viewed and managed by the experienced team at the Ahmanson/UCLA Adult Congenital Heart Center.
Extremely low birth weight (ELBW) infants weighing less than 1 kilogram are at a high-risk for delayed patent ductus arteriosus (PDA) closure. Percutaneous PDA closure offers a less invasive approach when compared with surgical PDA closure, which may provide faster recovery times and less transfusion requirements. However, this procedure involves transporting tenuous, unstable patients from the neonatal intensive care unit (NICU) to the catheterization laboratory which introduces many potential risks for the neonate. Performing percutaneous PDA closure at the bedside offers a successful alternative to performing the procedure in the catheterization laboratory and avoiding risk associated with transporting ELBW neonates.
Parturients may present with evidence of acute heart failure or respiratory distress during the peripartum period. This case report documents utilization of "stand-by" extracorporeal membrane oxygenation (ECMO) for a 40-year-old woman with a history of severe left ventricular dysfunction who presented for elective dilation and evacuation of triplets at 20 weeks' gestation. The patient's medical history was significant for hypertension, diabetes mellitus, methamphetamine use (acute/chronic), and cardiac-respiratory arrest before her previous emergent cesarean delivery. The patient underwent general anesthesia with the placement of peripheral venous and arterial cannulas for "stand-by" ECMO. The patient remained stable throughout the procedure, and "stand-by" ECMO was not initiated; the patient was discharged 5 days' postprocedure. The use of "stand-by" ECMO in the parturient with severe cardiopulmonary dysfunction is still in its infancy. Centers managing populations of both high-risk parturients and nonparturients may consider development of algorithms for implementation and utilization of ECMO.
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