Double aortic arch: an elusive diagnosis in a child with CHD

An 18-month-old male with pulmonary atresia and ventricular septal defect presented with stridor after neonatal systemic-to-pulmonary artery shunt surgery, that persisted on follow-up. CT angiography revealed a vascular ring with balanced double aortic arch.

Unguarded Tricuspid Orifice with Congenitally Corrected Transposition of the Great Arteries and Aortic Atresia- A Highly Unusual Case

Unguarded tricuspid orifice is a very rare anomaly. It is characterised by the absence of one or more of the tricuspid valve leaflets resulting in severe tricuspid regurgitation and right heart failure. It is rarely an isolated anomaly but more often associated with pulmonary atresia and intact ventricular septum. When the ventricles are inverted however, the result of outflow tract obliteration is not pulmonary atresia, but aortic atresia. This anomaly has been described in the literature in only 2 cases so far. We present a case of a neonate with unguarded tricuspid orifice with absence of all tricuspid leaflets, congenitally corrected transposition of the great arteries and aortic atresia. The severe tricuspid regurgitation and right ventricular enlargement would have required a Norwood-like procedure combined with a right ventricular plication. Due to the complexity of this lesion no surgical therapy was attempted in consent with the parents.

3D Virtual Reality Imaging of Major Aortopulmonary Collateral Arteries: A Novel Diagnostic Modality

Background Major aortopulmonary collateral arteries (MAPCAs), as seen in patients with pulmonary atresia, are arteries that supply blood from the aorta to the lungs and often require surgical intervention. To achieve complete repair in the least number of interventions, optimal imaging of the pulmonary arterial anatomy and MAPCAs is critical. 3D virtual reality (3D-VR) is a promising and upcoming new technology that could potentially ameliorate current imaging shortcomings. Methods A retrospective, proof-of-concept study was performed of all operated patients with pulmonary atresia and MAPCAs at our center between 2010 and 2020 with a preoperative computed tomography (CT) scan. CT images were reviewed by two congenital cardiac surgeons in 3D-VR to determine additional value of VR for MAPCA imaging compared to conventional CT and for preoperative planning of MAPCA repair. Results 3D-VR visualizations were reconstructed from CT scans of seven newborns where the enhanced topographic anatomy resulted in improved visualization of MAPCA. In addition, surgical planning was improved since new observations or different preoperative plans were apparent in 4 out of 7 cases. After the initial setup, VR software and hardware was reported to be easy and intuitive to use. Conclusions This study showed technical feasibility of 3D-VR reconstruction of children with immersive visualization of topographic anatomy in an easy-to-use format leading to an improved surgical planning of MAPCA surgery. Future prospective studies are required to investigate the clinical benefits in larger populations.