In a young infant with divided left atrium, unroofed coronary sinus syndrome, and persistent left superior caval vein, the pulmonary venous blood was successfully routed, after atrial septectomy, to the mitral valve by constructing a baffle using the partially detached diaphragm which had initially separated the pulmonary venous and vestibular components of the left atrium. The baffle was augmented with a patch of untreated autologous pericardium.

We report right ventricular ultrastructure in a 20-year-old patient with Noonan's Syndrome. Initial left ventricular and septal myocardial hypertrophy, observed at 2 months without obstruction, progressed to biventricular involvement over 18 years. Histopathologic evidence of cardiomyocytic disarray, with ultrastructural evidence of myofibrillar disarray, confirmed typical biopsy findings of hypertrophic cardiomyopathy on the right ventricular side of the septum.

Cardiac rhabdomyoma is a rare tumor. Because of its rate of regression, mainly in the first year of life, conservative therapy is generally suggested when diagnosis occurs in infancy. We report surgical ablation of a rhabdomyoma in a 2-month-old boy in whom the tumor extended into the subaortic outflow tract. The tumor, which had a dual attachment to the aortic and mitral valves, was safely removed without any valvar damage. We suggest immediate surgical intervention for those rhabdomyomas which are life-threatening because of their size, location, or arrhythmogenicity.

We report a case of anomalous origin of the coronary artery from the pulmonary trunk. The lesion was discovered in an eleven-month-old asymptomatic boy because of a continuous murmur.

Anomalous origin of a coronary artery is occasionally associated with other congenital heart malformations. Presence of the anomalous artery can then complicate surgical repair. We report an unusually complex arrangement in a patient who died 16 hours following complete surgical repair despite appropriate management.

Cardiovascular involvement is commonly reported in various muco- polysaccharidoses. We report a first case of Sanfilippo syndrome type A in a 12-year-old white female who has developed combined progressive mitral valvar regurgitation due to prolapse and aortic regurgitation.

We report a case of tricuspid atresia in which the symptoms underwent spontaneous improvement during childhood, and did not return until the age of 56 after which the patient went into progressive congestive failure and atrial fibrillation. He died at the age of 60, without ever having surgical intervention. Autopsy confirmed the presence of tricuspid atresia due to absence of the right atrioventricular connection, and also revealed partial left juxtaposition of the atrial appendages, an atrial septal defect in the oval fossa, rudimentary right ventricle, double outlet left ventricle with anterior aorta (anatomically corrected malposition), subvalvar and valvar pulmonary stenosis, dilation of the pulmonary arteries, patent arterial duct and major systemic to pulmonary collateral arteries.

It was considered that the development of the systemic to pulmonary arterial anastomoses probably accounted for the initial remission of symptoms, whilst the obstruction of the pulmonary outflow tract prevented the development of any significant degree of pulmonary hypertension in the presence of double outlet left ventricle.