We aim to describe the clinical course of a series of patients with hypoplastic left heart syndrome and refractory systolic heart failure supported with a HeartWare ventricular assist device (HVAD) following Fontan palliation. This is a retrospective review of three consecutive patients supported with a HVAD following Fontan palliation through February 2016. Data include patient characteristics, operative variables, postimplantation hemodynamic/device parameters, event outcomes, and duration of HVAD support. Patient ages were 11.7, 13.5, and 17.5 years, respectively, at the time of HVAD implant. The duration of HVAD support was 148, 272, and 271 days, respectively, of which 86, 222, and 211 were outpatient days. Inflow cannula position was the morphologic right ventricle with depth adjustment and manipulation of the tricuspid subvalvar apparatus to ensure good inflow. Echocardiographic, hemodynamic, and noninvasive oximetric monitoring resulted in high RPM settings for all patients. Despite various complications, all patients were successfully transplanted and discharged home alive. We present three patients bridged to transplantation using the HVAD following Fontan palliation. We demonstrate potential for durable support with transition to outpatient care while awaiting heart transplantation in a subset of patients status post Fontan surgery.

Objectives The purpose of this study was to evaluate the association of open and closed Fontan fenestration status with event-free survival. Methods All patients who underwent a fenestrated Fontan procedure at our institution from January 1994 through June 2007 were reviewed. Patient information was obtained from medical records. Patients were assigned to one of two study groups, Open vs Closed, based on their most recent fenestration status. Clinically relevant morbid events were tabulated and Kaplan-Meier event analysis used to create event-free probability curves with log-rank comparisons. Results 161 patients were classified as Open and 51 as Closed. The median (inter-quartile range) time to event was 1.1 (0.1,3.3) years after the Fontan procedure; whereas, median time to closure was 1.2 (0.7,3.3) years. Median time to event was 1.5 (0.1,4.6) years in the Closed group and 1.1 (0.1,3.3) years in the Open group. Event-free probability analysis revealed no significant difference between groups (P=0.15). Follow-up median arterial oxygen saturation was higher in the Closed group – 96.0 (94.0,97.0)%, compared to the Open group – 91.0 (86.0,95.0)% (P<0.0001). Conclusions Fenestration closure was associated with higher arterial oxygen saturation, but not higher event-free survival. Time to event was slightly less than time to fenestration closure, suggesting potential merit in the evaluation of earlier fenestration closure. Adoption of specific fenestration management guidelines might help improve overall outcomes and enhance the quality of future studies.

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