Systemic Semilunar Valve Repair/Replacement With Fontan Circulation: The Mayo Clinic Experience

Background: There is a paucity of literature regarding systemic semilunar valve (SSLV) dysfunction in patients with Fontan circulation. We sought to describe our center's 47-year experience with systemic semilunar valve replacement or repair (SSLVR) in patients with Fontan circulation. Methods: The Mayo Clinic Fontan Database is a comprehensive institutional database that stores clinical information of 1176 patients from 1973 to 2021. It was reviewed to identify patients who had a SSLV intervention at the time of or after Fontan. A cohort of 15 patients was identified and a retrospective review of their records was performed. Results: Fourteen patients had SSLV replacement (all mechanical) and one had a repair. SSLVR occurred up to 29 years following the Fontan (mean 11.3 ± 9 years, median 14 years). Thirteen of 14 with SSLVR were performed after Fontan and one was done at the time of initial Fontan. This was an older cohort and mean age at the time of Fontan was 8.7 ± 9.4 years (median 4 years). Indication for the operation was > moderate SSLV regurgitation in all patients. Six patients had decreased ventricular function (EF < 50%) prior to SSLVR and 8 had reduced function after SSLVR. Conclusion: Fortunately, the need for SSLV intervention after Fontan was rare, as evidenced by our small cohort extracted from a large single-institution database spanning a 47-year time period. Reduced preoperative and postoperative ejection fraction was common but did not seem to impact the outcome. Optimal timing for SSLV intervention after Fontan remains unclear.

Damus-Kay-Stansel procedure with ventricular septal defect enlargement

A full-term infant who had tricuspid atresia with transposed great arteries, a ventricular septal defect, subpulmonary stenosis with posterior malalignment of the conus septum, bicuspid pulmonary valve, and a high-takeoff left coronary artery was referred to our institution. The subpulmonary stenosis gradually progressed and cyanosis worsened. We successfully performed a Damus-Kay-Stansel procedure and a bidirectional Glenn shunt concomitant with ventricular septal defect enlargement. The conus septum was resected along with thick fibrous tissue through both semilunar valves (without ventriculotomy). Postoperative echocardiography demonstrated that both the ventricular septal defect and the subpulmonary space were enlarged effectively without semilunar valve regurgitation.

Norwood Procedure With Left Ventricle Exclusion in Complex Single Ventricle Patients: A Novel Technique

Background: Hypoplastic left heart disease and its variants complicated by severe semilunar and atrioventricular valve insufficiency are associated with a high mortality risk in utero and postnatally. Through the description of three cases, this report outlines the evolution of a successful strategy of morphological left ventricle isolation (ie, “left-sided Starnes”) in caring for this rare cohort of patients with hypoplastic left heart syndrome (HLHS). Methods: This study includes three patients: two patients prenatally diagnosed and one transferred from an outside hospital. All patients presented with complex univentricular heart disease with a nonfunctional left ventricle and severe left-sided atrioventricular and/or semilunar valve insufficiency. Results: Two patients were delivered with an operating room on standby due to the possibility of emergent surgery being necessary; however, all patients were able to be medically stabilized after birth. Patient 1 underwent successful left ventricle exclusion with a fenestrated polytetrafluoroethylene (PTFE) patch, despite severe mitral valve regurgitation. Patient 2 reinforced the efficacy of PTFE patch occlusion in addition to having aortic valve closure with a small washing jet. Patient 3’s clinical course was successfully predicted based on the previous patients and outlined for the family prenatally. All patients are currently growing well at home, awaiting Fontan with marked improvement in cardiac function. Conclusion: This rare cohort of patients with HLHS having severe left-sided atrioventricular and/or semilunar valve insufficiency appear to survive to birth and can be medically stabilized prior to semiurgent intervention. The Norwood procedure with left ventricle exclusion appears to be a successful strategy for these unique patients.