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Airway abnormalities are frequently associated with congenital heart disease in infants and children. Respiratory distress in these children frequently requires prolonged respiratory support. Wide-detector dynamic pulmonary computed tomography angiography (DP-CTA) is a non-invasive technique that completely evaluates vascular and airway abnormalities during a single breathe. Our purpose was to evaluate the efficacy of DP-CTA to provide unique actionable information for patient care in newborns and infants with congenital heart disease and persistent respiratory distress. 23 infants with congenital heart disease and persistent respiratory distress underwent DP-CTA. All were intubated at the time of the examinations. The most common cardiac anomalies were tetralogy of Fallot (6) and hypoplastic left heart syndrome variants (5). The most common cardiac surgeries prior to DP-CTA were Norwood (4) and hybrid (3) procedures. The protocol for DP-CTA for intubated infants is four gantry rotations in 1.4 s after intravenous contrast injection. 3D and multiplanar reconstruction with cine loops were created for combined cardiopulmonary imaging. Tracheobronchomalacia was present in 17 children. Lobar bronchomalacia was identified in six children. Branch pulmonary artery stenosis was the most common vascular finding (10 children). Medical management was changed or a surgical procedure performed based on the information resulting from DP-CTA in 16 of the 23 patients (70 %). DP-CTA is non-invasive, fast and provides unique information for the management of infants with congenital heart disease and persistent respiratory distress. DP-CTA is uniquely suited for comprehensive and simultaneous evaluation of airway and vascular abnormalities in infants.

Background: Major congenital heart disease (CHD) is seen in 10% to 15% of patients with congenital diaphragmatic hernia (CDH). Some neonates with CDH and major CHD are treated by surgery and some with extracorporeal membrane oxygenation (ECMO). Because of presumed poor survival, there is significant heterogeneity in management approaches for patients with CDH and associated CHD, and there is no published outcome data of patients who were placed on ECMO support., Methods: To examine outcomes of children with CDH with CHD supported with ECMO, Extracorporeal Life Support Organization (ELSO) registry was queried from 1998 to May 2010. There were a total of 3,342 deployments for CDH and 316 (9.5%) neonates with associated CHD. The median values are as follows: age at presentation, 1 (0 to 52) days; gestational age, 38 (29 to 42) weeks; birth weight, 3 (1.35 to 4.7) kg; admission to time to ECMO, 16 (0 to 1,220) hours; duration of ECMO, 194 (3 to 823) hours; time off of ECMO to extubation, 366 (0 to 7,934) hours; and time off of ECMO to death was 114 (0 to 7,272) hours., Results: Initially, ECMO support was venoarterial in 91% (283 of 316), 5 were converted from venovenous to venoarterial ECMO. Overall survival to hospital discharge for all patients with CDH and CHD was 47% (148 of 316). Survival to hospital discharge for hypoplastic left heart syndrome and single-ventricle physiology was 55% (33 of 60), 48% (43 of 89) for ventricular septal defect, and 40% (24 of 60) for coarctation of the aorta patients., Conclusions: Patients with CDH and CHD supported with ECMO have had better than predicted short-term outcomes. In this cohort, overall survival of patient's with CDH with or without CHD was similar. Patients with single-ventricle physiology had similar short-term outcome to those with 2 ventricle physiology., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

We report the aneurysmal dilation of the ascending aorta and the main pulmonary artery in 2 children with Kabuki syndrome. In 1 patient, there was progressive aneurysmal dilation of the ascending aorta necessitating aortoplasty. Histologic examination of the resected aorta revealed disrupted and fragmented elastic fibers in the medial layer, along with mucinous degeneration of the aortic wall. This is the first recognition and report of these findings as part of the Kabuki syndrome.

A neonate was diagnosed with hypoplastic left heart syndrome, heterotaxy syndrome, and interrupted inferior vena cava soon after birth. At 6 days old she underwent the Norwood procedure using Sano modification. Postoperatively she had persistent direct hyperbilirubinemia and was diagnosed with biliary atresia for which she underwent a Kasai procedure at 29 days old. At 10 months she underwent the Kawashima procedure. She is now 20 months old and has been thriving without any jaundice. This case report illustrates that even in the presence of major multiple congenital anomalies, staged reconstruction for hypoplastic left heart syndrome can be successfully performed.