National Fontan Operation outcomes at or below 2-years-of-age compared to older than 2- years-of-age

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.

Isolated Cleft of the Posterior Mitral Leaflet in 8 Year Old Patient

Cleft of the mitral valve is a rare cause of the congenital mitral regurgitation. Isolated cleft is uncommon finding and is more often found associated with some form of endocardial cushion defect. Cleft of the septal leaflet is found much more often than the defect in the posterior leaflet. Mitral clefts can extend to the mitral annulus or they can be less ''deep'' and show only minor regurgitation. Clinical importance of the isolated cleft of the mitral valve (ICMV) is that it can be successfully corrected by surgery and that it can have good long-term results.

Cystic Lung Disease in Down Syndrome: A Case Report and Literature Review

Subpleural lung cysts (SPC) are seen in children with Down syndrome (DS). The incidence and the long term course of these lesions are not known. It is important for pediatricians and pediatric radiologists to be aware of these lung lesions since the DS patients’ longevity has increased and they have greater frequency to encounter the clinicians. Autopsy and the radiology series have shown that these lesions are often found in association with congenital heart disease, particularly the endocardial cushion defect and prematurity.