Transplantation for Congenital Heart Disease: Focus on the Impact of Functionally Univentricular Versus Biventricular Circulation.

BACKGROUND: Varying single center data exist regarding the posttransplant outcomes of patients with single ventricle circulation, particularly following the Fontan operation. We sought to better elucidate these results in patients with congenital heart disease (CHD) through combining two national databases. METHODS: The United Network for Organ Sharing (UNOS) transplantation database was merged with the Pediatric Health Information System (PHIS), an administrative database with 71% of UNOS patients matched. Patients undergoing transplantation at a PHIS hospital from 2006 to 2017 were categorized as single ventricle or biventricular strategy based on their diagnoses and procedures in 90% of patients. When known, single ventricle patients were further analyzed by their palliative stage post-Glenn or post-Fontan (known in 31%). RESULTS: A total of 1,517 CHD transplantations were identified, 67% with single ventricle strategy (1,016). Single ventricle, biventricular, and indeterminate patients had similar survival (log-rank P > .1). Risk factors for mortality in patients with CHD were extracorporeal membrane oxygenation (ECMO) support at transplant (hazard: 2.27), ABO blood type incompatibility (hazard: 1.61), African American recipient (hazard 1.42), and liver dysfunction (hazard 1.29). A total of 130 confirmed Fontan and 185 confirmed bidirectional Glenn patients underwent transplantation, each with survival equivalent to biventricular patients (log-rank P > .500). For Fontan patients, renal dysfunction (hazard: 5.40) and transplant <1 year after Fontan (hazard 2.82) were found to be associated with mortality. CONCLUSIONS: Single ventricle patients, as a group, experience similar outcomes as biventricular patients with CHD undergoing transplantation, and this extends to Fontan patients. Risk factors for mortality correlate with end-organ dysfunction as well as race and ABO blood type incompatibility in the CHD population.

3D-printed models optimize preoperative planning for pediatric cardiac tumor debulking.

BACKGROUND: This study reports the first known application of 3-dimensional (3D) printing of cardiac tumors to preoperatively plan debulking in infants. 3D-printed cardiac tumor models were used to identify the spacial relationship between the tumors and coronary arteries as well as understand the depth and infiltration of the tumors. METHODS: Physical 3D cardiac tumor models of two children were obtained using medical imaging, image 3D rendering and modeling, and 3D printing. The hearts were 3D-printed in an opaque material while the tumors were made transparent to allow optimal visualization of the cardiovascular anatomy within the tumor. The surgical team used these models to plan exposure of the tumor, as well as, the extent of debulking. RESULTS: Patient 1 had a cardiac tumor arising from the anterior surface of the right ventricle causing significant right ventricular outflow tract obstruction and involving the right and left coronary artery courses. Patient 2 had a cardiac tumor arising from the left ventricle and extending beyond the left atrium compressing the airway preventing extubation, and surrounding the left coronary artery system. In both patients, 3D-printed models were used to maximize debulking and avoid injury to the coronaries. CONCLUSIONS: 3D-printed cardiac tumor and anatomic models were effectively used to preoperatively plan two pediatric tumor debulkings. Both patients had tumors that were integrally involved with the coronary arteries. The 3D models helped devise a safe surgical strategy for maximal tumor debulking while protecting the coronary circulation.