Anomalous origin of the anterior descending coronary artery from the pulmonary trunk is very rare. We suspected such anomalous origin in a boy aged 2.5 years owing to the echocardiographic demonstration of mitral regurgitation and abnormal diastolic flow in the ventricular septum. The diagnosis was confirmed by angiography. The anomalous artery was successfully reimplanted into the aorta.

We describe the findings in a neonate with aortic atresia, interruption of the aortic arch between the carotid arteries, and aortopulmonary window. This unusual association changes the anticipated physiology, and helps in the survival. We emphasize the fact that this combination simulates common arterial trunk, both clinically and on echocardiography.

We report replacement of the aortic valve in a patient aged 9 years with mucolipidosis III and severe aortic insufficiency. Histopathology demonstrated abnormalities of the matrix and lysosomal inclusion bodies. As life expectancy increases for patients with lysosomal storage disorders, approaches to intervention for valvar disease become increasingly important.

Pulmonary atresia with intact septum is itself a rare congenital abnormality, albeit known to be associated with other cardiac and non-cardiac anomalies. The combination of right- and left-sided obstructive lesions, however, is extremely rare. We describe a patient having pulmonary atresia with intact septum associated with aortic coarctation, which to the best of our knowledge has been previously described on but one occasion.

We report an infant of 3 1/2 months with complete obstruction of the venous component of the scimitar syndrome, a large atrial septal defect within the oval fossa, a common left pulmonary vein, and pulmonary hypertension. To the best of our knowledge, ours is the first description of complete congenital obstruction of a scimitar vein.

Although cardiac tumours are rare, such tumours are increasingly being diagnosed with increasing frequency and great accuracy by antenatal ultrasound. Cardiac haemangiomas account for less than one-twentieth of all primary cardiac tumours, with most being diagnosed in the neonatal period. We report 3 instances of successful neonatal resection of cardiac haemangioma subsequent to prenatal diagnosis. Such diagnosis is important in perinatal management, since early surgical intervention provides a good prognosis.

We describe our experience with 2 patients having Costello syndrome, aged 11 and 36 months, who suffered systolic anterior motion of the aortic leaflet of the mitral valve and obstructive cardiac hypertrophy requiring surgery, comparing their cardiac characteristics to those described previously. We conclude that the heterogeneous nature of the cardiac hypertrophy in this syndrome can be considered and managed as the sum of a diffuse hypertrophy accessible to beta-blockade, and an asymmetric hypertrophy accessible to surgical myomectomy.

Congenital unilateral pulmonary venous atresia is a rare anatomical defect. Patients present with pulmonary symptoms, and diagnosis may be ellusive. Pulmonary arterial wedge angiography is the gold standard with which to achieve diagnosis, and surgical intervention is often required for symptomatic patients.

Early postoperative arrhythmias are a known complication of cardiac surgery. It is unusual, however, to encounter postoperative junctional and ectopic atrial tachycardias in the same patient. We describe our experience with a 2-year-old girl who suffered both these tachycardias after repair of a ventricular septal defect, the abnormal rhythms being controlled solely with intravenous administration of amiodarone.

We report the occurrence of non-obstructive totally anomalous pulmonary venous connection to the superior caval vein in a child conceived by intracytoplasmic injection of sperm, a type of assisted reproductive technology. Totally anomalous pulmonary venous connection is an uncommon congenital anomaly, in which all the pulmonary veins connect to the morphologically right atrium, or one of its tributaries. To our knowledge, this malformation has not been previously described in a child conceived on the basis of assisted reproductive technology.

The syndrome of twin-to-twin transfusion is known potentially to be associated with the development of right ventricular obstruction, albeit rarely at supravalvar levels, in the recipient twin. We report the case of a recipient twin with diffuse hypoplasia and calcification of the pulmonary arterial tree, confirmed by postnatal thoracic angioscan. When aged 2 years, the child was moderately symptomatic with mild cyanosis, in spite of suprasystemic right ventricular systolic pressure as revealed by follow-up echocardiography.