P254 Congenitally corrected transposition of the great arteries with an ebstenoid tricuspid valve, aortic atresia, hypoplastic aortic arch and a dysplastic pulmonary valve

Abstract

Abstract Funding Acknowledgements No financial support received Introduction Congenitally corrected transposition of the great arteries (CCTGA) is a rare cardiac malformation accounting for around 0.5% of congenital heart disease. Common associations are ventricular septal defects (VSD), pulmonary stenosis and heart block. CCTGA associated with aortic atresia is very rarely described in the literature. Description We present a case of a term neonate weighing 2.5kg. Fetal echo had initially made the diagnosis at 21/40. The baby was born in good condition and the postnatal echo confirmed the diagnosis of CCTGA. There were 2 secundum atrial septal defects, the sub-systemic tricuspid valve was ebstenoid with remarkably trivial regurgitation, there were multiple muscular ventricular septal defects with predominantly left to right flow, although flow appeared bi-directional in some views. The atretic aortic valve was anterior and to the left of the dysplastic, bicuspid pulmonary valve. The pulmonary valve leaflet motion was restricted with peak velocity of 2.4m/s and mild incompetence. The hypoplastic aortic arch filled retrogradely via the arterial duct. There was discrete coarctation with peak velocity of 2.2m/s down the descending aorta. The persistent left SVC drained to the coronary sinus. Following several discussions there was agreement that the most appropriate management for him would be to receive palliative care. He was discharged home at a week of age and continued to have intensive support from his local hospice and the cardiac team, until he passed away peacefully at home at 16 days of age. Discussion The multidisciplinary team decided surgery was extremely high risk due to the anatomy described above particularly in the context of his dysplastic, bicuspid pulmonary valve, which would have provided systemic outflow if intervention had been undertaken. Figure 1: 4 chamber view showing reverse offsetting of the atrioventricular valves, arch view showing a hypoplastic aortic arch with restrograde flow from the arterial duct, short axis view showing the semilunar valves and their relative positions, long axis view showing aortic atresia with retrograde flow into the ascending aorta Abstract P254 Figure 1