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The Outcome Of 1.5 And 2 Ventricle Repair In Patients Assigned To A Fontan Pathway Or Unable To Obtain Surgical Repair
Marcus P. Haw, Jawad Khazaal, Neal Hillman, Giedrius Baliulis, Joseph Vettukattil, Jorden Gosnell.
Helen DeVos Childrens Hospital, Grand Rapids, MI, USA.


OBJECTIVE: Complications of the Fontan circulation are well described. Heterotaxia with TAPVR is often associated with poor outcome. This single center retrospective study reports results of a contemporary series of surgeries aimed at avoiding a Fontan circulation or rehabilitating patients denied surgery. METHODS: 18 patients operated between 2013 and 2021. All had commenced or completed Fontan surgery, or had failed previous surgery. Patients were re-investigated by a multidisciplinary team. Surgical planning was aided by 3D visualization and printing in 11. Outcomes including late functional status, current ejection fraction, re-intervention and surgery rate and major complications, were recorded from patientsí charts. RESULTS: 3 had Ebsteinís anomaly, 4 had a previous Fontan operation, 8 were on a Fontan pathway, and 4 had previous failed surgery and were not transplant candidates. 1 patient required pre-operative ECMO. All 18 patients survived surgery and remain alive at a mean follow up of 3.9 years. 13 patients have no symptoms related to their cardiovascular condition. 4 patients are in NYHA II and one patient is in NYHA III. 10 patients have had catheter-based re-intervention and there have been 2 further cardiac surgeries. 2 patients had pacemakers. 16 patients have left ventricular ejection fraction (LVEF) > 50% and 2 patients LVEF > 40%. 1 patient suffered severe staphylococcal sepsis and has long term disability. CONCLUSION: All patients operated achieved septation with functional improvement without mortality. Supportive interventional cardiac catheterization was necessary. Functional improvement is maintained over the follow-up period in all but one patient.


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