Objective: The traditional outcomes of the Fontan Operation (FO) in Endocardial Cushion Defect (ECD) patients have been suboptimal. Previous studies have been limited by the smaller number of ECD patients, longer study period with an era effect and do not directly compare short-term outcomes of FO in ECD patients with non-ECD patients. Our study aims to address these shortcomings. Methods: A retrospective analysis of the Kids Inpatient Database (2009, 2012, and 2016) for the FO was done. The groups were divided into those who underwent FO with ECD as compared to non-ECD diagnosis. The data was abstracted for demographics, clinical characteristics, and operative outcomes. Standard statistical tests were used. Results: 3380 patients underwent the FO of which 360 patients (11%) were FO-ECD. ECD patients were more likely to have Down syndrome, Heterotaxy syndrome, transposition/DORV, and TAPVR as compared to non-ECD patients. FO-ECD had a higher discharge-mortality (2.84% vs. 0.45%, p=0.04). The length of stay (16 vs. 13 days, p=0.05) and total charges incurred ($ 283, 280 vs. 234, 106, p=0.03) for the admission were higher in the FO-ECD as compared to non-ECD patients. In multivariable analysis: ECD diagnosis, cardiac arrest, acute kidney injury, and post-operative hemorrhage were predictors of mortality. Conclusion: Contemporary outcomes for FO are excellent with very low overall operative mortality. However, the outcomes in ECD patients are inferior with higher operative mortality than non-ECD patients. Occurrence of post-operation complications, associated TAPVR and a diagnosis of ECD were predictive of a negative outcome.

Introduction: Opinion is divided about optimal early timing of the Fontan Operation (FO). While some studies have suggested 3 years-of-age, others have shown good outcomes below 2 years-of -age. We analyzed the impact of age ≤2-years as compared age >2-years on short-term outcome of the FO using a large national database. Methods: A retrospective analysis of the Kids Inpatient Database (2009-16) for the FO was done. The groups were divided into those who underwent FO at age ≤2-years (EF) as compared to age >2-years (LF). The data was abstracted for demographics, clinical characteristics, and operative outcomes. Standard statistical tests were used. Results: 3381 patients underwent FO during this period of which 1482 (44%) were EF. The mean ages of the EF and LF were 1.6 and 4.3, respectively (p< 0.001). LF were more likely to be non-White, female, and have Heterotaxy syndrome. HLHS was more common in EF. There was no difference in the discharge mortality, length of stay, disposition (majority went home), and mean total charges incurred. The overall discharge mortality was low at 0.7% (24/3381). In multivariate analysis: cardiac arrest, acute kidney injury, mechanical ventilation >96 hours, endocardial cushion defect and non-White ethnicity were predictors of a mortality and not age. Conclusion: Contemporary outcomes for FO are excellent with equivalent short-term outcomes in both the age groups. Occurrence of postoperative complications, non-white ethnicity and endocardial cushion defect diagnosis were predictive of a negative outcome.

Objectives: Innominate artery compression syndrome (IAS) is caused by an anterior compression of the trachea by an abnormally originating innominate artery. One option to relieve such a compression is an anterior aortopexy (AA). In this paper we describe our technique of an AA via a partial upper median sternotomy. Methods: A retrospective review of a prospectively maintained database of patients with IAS (July 2017 to November 2020) treated with AA via a partial upper median sternotomy at University teaching hospitals in the US was done. Results: Nine consecutive patients underwent AA for IAS during the study period. The median age was 9 months (IQR 3- 16.5). The male to female ratio was 1.25. All patients had > 70% compression by flexible bronchoscopy. 2 patients had previous surgeries. The follow-up was a median of 6 (IQR 4- 8.5) months. The indications for the operation were: reflex apnea (4/9 patients), recurrent intubation (4/9 patients), and severe stridor (1/9). IAS was a technical success (defined as ≤ 20 % residual stenosis) in 78 % (7/9) of the patients. Complete symptom resolution after an AA was seen in 71% (5/7) of the patients. 2 patients had an unsuccessful AA, requiring a tracheal resection and an innominate artery reimplantation, respectively. Conclusion: An upper partial sternotomy approach provides a very versatile approach to an AA for IAS. Besides facilitating an adequate AA, it provides options for direct tracheal surgery or an innominate artery reimplantation in case an optimal result is not obtained by an AA.