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Double aortic arch associated with atresia of the left arch proximal to the left common carotid artery has been considered a theoretical possibility. To our knowledge, we report the first patient with this anatomy confirmed by surgical observation.

A 1-year-old female child was diagnosed to have scimitar syndrome associated with partial anomalous pulmonary venous connection of the right lung at the superior vena cava-right atrial junction, right atrium, and inferior vena cava. To our knowledge, this pattern of pulmonary venous connection has not been previously described in this syndrome.

A monozygotic twin boy with supravalvar aortic stenosis had reduction in the degree of obstruction with body growth. His twin brother had normal cardiac anatomy. To our knowledge, this is the first report of improvement without intervention in a patient with supravalvar aortic stenosis and of discordance of supravalvar aortic stenosis in monozygotic twins.

An infant girl is described who had cor triatriatum and partial anomalous pulmonary venous connection of the left pulmonary veins to the coronary sinus, the first report of this combination of lesions. The infant also had a Dandy-Walker malformation and multiple facial and intrathoracic hemangiomas. The cardiac diagnosis was made by two-dimensional echocardiography. Cardiac catheterization and angiography confirmed the findings and also demonstrated a persistent left superior vena cava draining to the coronary sinus. The infant underwent successful surgical repair. Partial anomalous pulmonary venous connection and left superior vena cava not infrequently are associated with cor triatriatum. Although two-dimensional echocardiography is sensitive for the detection of cor triatriatum, preoperative cardiac catheterization is necessary to identify unequivocally systemic and pulmonary venous connections.