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.

Ventriculocoronary Fistulas with Hypoplastic Left Heart in a Neonate: Imaging with Cardiac CT

Fistulous communications between the ventricular cavities and the coronary arterial tree can be found in the presence of hypoplasia of the left ventricle, especially when the ventricular septum is intact and mitral stenosis and aortic atresia subtype are present. The cardiac CT provides excellent anatomic information especially in the evaluation of extracardiac vessels and coronary arteries. In this case study, we report a newborn with ventriculocoronary fistulas (VCFs) with the hypoplastic left disease diagnosed with cardiac CT. Transthoracic echocardiography of a term baby showed hypoplastic left heart syndrome (HLHS) with mitral stenosis and aortic atresia. The patient immediately underwent a Sano variation of the Norwood procedure. On the postoperative second day, the clinical status of the patient deteriorated. A prospective electrocardiogram-gated axial technique was performed within a single heartbeat for the patient and large VCFs were detected and a second operation were performed to close the VCFs that failed. On the nineteenth day after the operation, the baby passed away. According to us, cardiac CT can also be performed free-breathing and without anesthesia in the neonatal period for the definition of complex cardiac anatomy with the lower radiation dose from the latest scanners, radiation risk of CT should be weighed against the anesthesia risk of cardiac MRI and intraoperative risk of conventional cardiac angiography. Pre-operative cardiac CT may increase surgical success.

Aortic atresia with interrupted aortic arch and bilateral arterial ductus: a successful initial palliation

A combination of aortic valve atresia and an interrupted aortic arch is a unique disease in which perfusion to the brain and myocardium depends on coexisting lesions or type of interruption. We report a case of aortic valve atresia with type B interrupted arch, bilateral arterial ductus in a neonate who was successfully palliated using a hybrid approach by placing stents in both arterial ductus and banding of branch pulmonary arteries.