The association of truncus arteriosus communis with interrupted aortic arch and mitral atresia is an exceptionally rare congenital defect. We describe the initial decision-making and management of this lesion, which eventually achieved a Fontan palliation.

Anomalous origin of the left coronary artery from the right pulmonary artery in single ventricles is a very rare congenital anomaly. Failure to recognise it preoperatively may lead to adverse outcomes, including death. We report the case of a neonate with a univentricular heart in the form of a double-outlet right ventricle, mitral atresia with discrete coarctation of the aorta, and an incidental intraoperative finding of an anomalous origin of the left coronary artery from the right pulmonary artery. The patient underwent a successful repair with an uneventful postoperative course.

Mitral atresia is commonly seen as a part of the spectrum of hypoplastic left heart syndrome, and it is usually associated with multiple levels of systemic outflow tract obstruction. Isolated mitral atresia with a normal aortic valve is extremely rare. We report the rare combination of mitral atresia, transposition of the great arteries, and unobstructed systemic and pulmonary blood flow.

We describe a case of a baby girl born with hypoplastic left heart syndrome consisting of mitral atresia, aortic atresia, hypoplastic ascending aorta, and left ventricle. The pulmonary arteries were hypoplastic, measuring 3 mm. Fluorescence in situ hybridisation analysis demonstrated a microduplication of chromosome 22q11.2. Subsequent array comparative genomic hybridisation showed a gain of 2.3 Mb in one copy of chromosome 22q at band 22q11.21. The proband underwent a successful Norwood procedure with Sano shunt and subsequently underwent bi-directional Glenn shunt and Fontan procedure. This report highlights the association between hypoplastic left heart syndrome with hypoplastic pulmonary arteries and chromosome 22q11.21 microduplication.

As we described in the previous review,1 double inlet ventricle is usually found with the atrial chambers connected to a dominant left ventricle, less frequently to a dominant right ventricle, and rarely to a solitary and indeterminate ventricle. As we have also discussed in this supplement,2 double inlet to the left ventricle was, for many years, considered the exemplar of so-called “single ventricle”, despite the fact that such patients unequivocally possess one big and one small ventricle. Echocardiographic interrogation has served to resolve this controversy, showing that such patients make up a significant proportion of those having functionally univentricular hearts. Such echocardiographic investigation has also served to resolve similar controversies regarding patients having tricuspid atresia. For some time, it was argued that patients with tricuspid atresia also had “univentricular hearts”,3 but the logic used to underscore this approach was just as flawed as that used to justify the use of “single ventricle” in patients with double inlet atrioventricular connection.4,5 The increasing use of the Fontan procedure has served to demonstrate that these patients, along with many having mitral atresia in the setting of hypoplastic left heart syndrome, also have functionally univentricular arrangements. As we will show in this review, however, the anatomical substrates found in patients with atrioventricular valvar atresia are much more complex than those seen in the setting of double inlet ventricle. This is because atrioventricular valvar atresia can be produced either by absence of one atrioventricular connection, or by presence of an imperforate valvar membranes closing completely one or other of the two normal atrioventricular junctions. This important difference, combined with multiple segmental combinations, produces a bewildering array of potential anatomical substrates, with the complications magnified by the fact that, when one atrioventricular connection is absent, the other atrioventricular junction can be shared between the two ventricles, the so-called uniatrial and biventricular arrangement.6 In our review, we will first describe the anatomical options, before concentrating our attention on the more frequent patterns seen in clinical practice.