RESUMO
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.
RESUMO
Extracorporeal membrane oxygenation (ECMO) is used as a salvage therapy in children with irreversible myocardial failure who may be candidates for heart transplantation (HTx) (at the Hospital for Sick Children). We retrospectively assessed outcomes of children wait-listed for HTx from ECMO, and risk factors for patients (pts) bridged to HTx from January 1990 through December 2005. Of 205 patients supported with cardiac ECMO, 46 were wait-listed for HTx. Sixteen patients died before HTx: eight died while wait-listed on ECMO; eight were delisted (clinical deterioration; all died); five were delisted (improved), and 25 (54%) underwent HTx from ECMO. Of 25 patients who underwent HTx (median age 7.0 years [10 days to 17 years]), 13 had myocarditis or cardiomyopathy, and 12 had congenital heart disease. Median ECMO duration was 6.7 days (3-18 days). Median follow-up was 4.3 years (0.2-10.6 years). Four patients died <1 week post-HTx, and 21 survived until hospital discharge (84%). Post-transplant survival was 67% and 52% at 1 and 5 years, respectively. Risk factors for early death were older age, higher body surface area, higher creatinine before and during ECMO, fungal infections, and exposure to blood products. In summary, few risk factors preclude HTx candidacy from ECMO. The impact of newer assist technology on ECMO, wait-list mortality, and HTx outcomes remains to be elucidated.
