The morphologic variations of mixed totally anomalous pulmonary venous connection are many and varied. In this review, we give an account of all cases previously described as mixed totally anomalous pulmonary venous connection, analyzing in detail those cases where an accurate anatomical description was provided. We identified 182 suitable cases, from 54 investigations, and reviewed the clinical presentation, anatomic variations, diagnostic features, and management of the patients described.

Cross-sectional echocardiography, and cardiac catheterization, provided the necessary diagnostic information, and defined the anatomy before surgery in 139 patients. Magnetic resonance imaging and computerized tomographic angiography had been used for further clarification of the pulmonary venous anatomy. An obstructive pattern of drainage, involving one or more pulmonary veins, had been described in over half of the patients. We then grouped the lesions into categories that have a bearing on the appropriate surgical approach, discussing the appropriate repair for each group. For the overall group, the operative mortality remains high, at 22.9%. We submit that an increased appreciation of various types of mixed totally anomalous pulmonary venous connection may well contribute to improved future surgical management.

Isolated ventricular non-compaction is an increasingly commonly diagnosed myocardial disorder characterised by excessive and prominent trabeculation of the morphologically left, and occasionally the right, ventricle. This is associated with high rates of thromboembolism, cardiac failure, and cardiac arrhythmia. Recent improvements in understanding the embryonic processes underlying ventricular formation have led to the hypothesis that ventricular non-compaction is due to a failure of normal ventriculogenesis, leading to abnormal myocardium which may present clinically many years later. Experimental work in animal models provides several candidate transcription factors and signalling molecules that could, in theory, cause ventricular non-compaction if disrupted.

Background: Surgical closure of ventricular septal defects has been performed for many years, and is considered as the gold standard for treatment. It remains associated with morbidity and mortality. Transcatheter techniques have been developed in the last 10 years as a possible alternative to conventional surgery. Methods: The procedure is performed under general anaesthesia, and with continuous fluoroscopic and transesophageal echocardiographic guidance. Devices of the Amplatzer family, two in particular, have achieved a large popularity in clinical practice, and are currently the devices most commonly used to close muscular and perimembranous ventricular septal defect percutaneously. Results: Data from literature show that successful closure of muscular defects is obtained in around 96% of patients, with a rate of major complication of around 2%. Pooling data from the literature shows that successful closure of perimembranous defects is also obtained in 96% of patients, again with major acute complications in around 2%. The major problem is the occurrence of complete atrioventricular block, reported in 1.7% of subjects. Acquired defects can occur as residual leaks after surgical closure, or as consequence of myocardial infarction. There are very few data concerning percutaneous closure of postoperative residual defects. As for the surgical approach, in patients with post-myocardial defects the success rate of percutaneous closure is around 88%, with a mortality of 22%. Conclusions: Nowadays, in experienced hands, percutaneous closure is a safe and effective procedure. In selected patients, closure of congenital or acquired muscular and perimembranous ventricular septal defects can be considered a real alternative to the standard surgical approach, with the advantage of a significantly reduced rate of mortality and complications.

“When the history of congenital heart disease comes to be written, Brown will be given a high and honourable place along with Farre, Peacock, Fallot, Théremin, Vierordt, and Maude Abbott, as one whose labours have contributed vitally to the subject.”1