Key Points
Question
Is humanitarian global pediatric cardiac surgery in low- and middle-income countries cost-effective?
Findings
This economic evaluation found that for a cohort of 424 children who underwent operations in 10 low- and middle-income countries in 2015, the cost-effectiveness of the intervention was $171 per disability-adjusted life-year averted.
Meaning
Humanitarian pediatric cardiac surgery in low- and middle-income countries is highly cost-effective.
Patients with failing Fontan physiology and liver cirrhosis are being considered for combined heart and liver transplantation. We performed a retrospective review of our experience with en bloc combined heart and liver transplantation in Fontan patients > 10 years old from 2006 to 18 per Institutional Review Board approval. Six females and 3 males (median age 20.7, range 14.2-41.3 years) underwent en bloc combined heart and liver transplantation. Indications for heart transplant included ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia, and/or lymphatic abnormalities. Indication for liver transplant included portal hypertension and cirrhosis. Median Fontan/single ventricular end-diastolic pressure was 18/12 mm Hg, respectively. Median Model for End-Stage Liver Disease excluding International Normalized Ratio score was 10 (7-26), eight patients had a varices, ascites, splenomegaly, thrombocytopenia score of ≥ 2, and all patients had cirrhosis. Median cardiopulmonary bypass and donor ischemic times were 262 (178-307) and 287 (227-396) minutes, respectively. Median intensive care and hospital stay were 19 (5-96) and 29(13-197) days, respectively. Survival was 100%, and rejection was 0% at 30 days and 1 year post-transplant. En bloc combined heart and liver transplantation is an acceptable treatment in the failing Fontan patient with liver cirrhosis.
Fontan-associated liver disease (FALD) encompasses abnormalities in liver structure and function that result from Fontan circulation unrelated to other disease processes. 1 FALD induces liver fibrosis, which is universal in all Fontan patients. 2,3 Fibrosis does not start just with Fontan circulation but begins at some point before Fontan along the patient's single ventricle palliation pathway. 4 FALD can lead to cirrhosis and portal hypertension and complicate the management of patients with "Fontan failure." 5 In some centers, heart transplantation is not offered to patients with Fontan failure and liver fibrosis due to the high morbidity and mortality risk. 6 Combined heart and liver transplantation (CHLT) may be appropriate in certain cases. Liver biopsy in FALD may help to characterize and stage liver fibrosis as part of a comprehensive evaluation of patients considered for heart and/or CHLT. 7-9 However, liver biopsy may not accurately
Objective: Transition from pediatric to adult care is a critical time for patients with congenital heart disease. Lapses in care can lead to poor outcomes, including increased mortality. Formal transition clinics have been implemented to improve success of transferring care from pediatric to adult providers; however, data regarding outcomes remain limited. We sought to evaluate outcomes of transfer within a dedicated transition clinic for young adult patients with congenital heart disease.Design, Setting, and Patients: We performed a retrospective analysis of all 73 patients seen in a dedicated young adult congenital heart disease transition clinic from January 2012 to December 2015 within a single academic institution that delivered pediatric and adult care at separate children's and adult hospitals, respectively.Intervention and Outcome Measures: Demographic characteristics including congenital heart disease severity, gender, age, presence of comorbidities, presence of cardiac implantable electronic devices, and type of insurance were correlated to success of transfer. Rate of successful transfer was evaluated, and multivariate analysis was performed to determine which demographic variables were favorably associated with transfer.Results: Thirty-nine percent of patients successfully transferred from pediatric to adult services during the study period. Severe congenital heart disease (OR 4.44, 95% CI 1.25-15.79, P 5 .02) and presence of a cardiac implantable electronic device (OR 4.93, 95% CI 1.18-20.58, P 5 .03) correlated with transfer. Trends favoring successful transfer with presence of comorbidities and private insurance were also noted.Conclusions: Despite a dedicated transition clinic, successful transfer rates remained relatively low though comparable to previously published rates. Severity of disease and presence of implantable devices correlated with successful transfer. Other obstacles to transfer remain and require combined efforts from pediatric and adult care systems, insurance carriers, and policy makers to improve transfer outcomes.
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