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The great majority of myocardial tissues in cyanotic tetralogy of Fallot indicates pre-existing ultrastructural hypertrophic and degenerative changes. The changes are more pronounced in older patients subjected to long-standing cyanosis and pressure overload and may account for or may coexist with the higher incidence of myocardial dysfunction and ventricular arrhythmia.

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.

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