Cetta, Frank

This clinical case report describes surgical management of a giant left ventricular fibroma and postoperative venoarterial extracorporeal membrane oxygenation support in a newborn male. Transthoracic echocardiography performed at 9-month follow-up showed an ejection fraction of 33% and a cardiac index of 4.5 liters per minute per square meter.

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.

Background: Persistence or recurrence of stenosis is a complication of initial coarctation repair. This study aims to report short-term outcomes of surgical management of recurrent coarctation and initial repair analysis. Methods: We retrospectively reviewed our experience with 51 patients undergoing recoarctation surgical repair between 2008 and 2019 using antegrade cerebral perfusion technique. Results: Surgical correction included prosthetic patch aortoplasty in 23 (45%), resection with wide end-to-end anastomosis in 15 (29%) and a tube interposition graft in 13 (25%) patients. Median age at initial correction and reintervention were 12 month and 9 years. Median interval from primary repair to reintervention was 60 months. Initial repair analysis revealed 33% of patients had initial correction in the neonatal period, 72,5% of patients were done via a left thoracotomy approach and 63% of patients had end-to-end anastomosis at initial surgery. Conclusion: Our study demonstrates that surgical repair of recurrent coarctation of the aorta using antegrade cerebral perfusion technique can be performed safely and with excellent results.

Objective To review the long-term functional outcomes of patients with Ebstein anomaly who had cardiac surgery at our institution. Methods From April 1, 1972 to January 1, 2006, 539 patients with Ebstein anomaly had 604 cardiac operations at Mayo Clinic Rochester. The 506 late survivors form the basis of this report. Patient records were reviewed and all patients were mailed a medical questionnaire or contacted by telephone. Patients who had associated complex congenital heart disease were excluded (pulmonary atresia, conotruncal abnormalities, and atrioventricular discordance and ventriculoarterial discordance). Results At the initial operation at our institution, the mean age was 24 years (range 8 days to 79 years) and 53% were female. Survival at 5, 10, 15, and 20 years was 94%, 90%, 86%, and 76%. Survival free of late reoperation was 86%, 74%, 62%, and 46% at 5, 10, 15, and 20 years respectively. Surveys were returned by 285 patients. Two hundred and thirty-seven (83%) of patients were in NYHA class I or II and 34% were taking no cardiac medication. Five patients (2%) reported having endocarditis, 1 patient (<1%) reported a stroke, and 103 patients (36%) had atrial fibrillation or flutter. There were a total of 202 pregnancies among 82 women. Recurrence of congenital heart disease was low. Only 9/232 (0.04%) of liveborn children had congenital heart disease. Conclusions Patients have good long term survival and functional outcomes, after operation for Ebstein anomaly. Atrial arrhythmias are relatively common both before and after operation. Pregnancy appears to be well tolerated with low recurrence risk of congenital heart disease.

This clinical case demonstrated surgical management of a giant congenital left ventricular aneurysm in a 2-month-old female using the Dor procedure. Transthoracic echocardiography performed at 6-month follow-up showed an ejection fraction of 66%.

Intraconal or transseptal course of the left main coronary artery is a rare form of coronary artery anomalies which has been linked to sudden death. We present details of the surgical technique that we used to address this challenging anomaly in two cases.