Hypoplasia of the transverse aortic arch is commonly associated with aortic coarctation. Persistent or recurrent obstruction can occur at this level after successful repair of the native coarcted segment. The purpose of this report is to present a new technique to treat such lesions, namely with implantation of a balloon-expandable stent. This approach was used successfully in 4 children with such hypoplasia occurring after repair of coarctation. Implantation led to both anatomical and physiological relief of obstruction in all. The patients tolerated the procedure, and there were no major adverse events.

We reviewed 16 patients with coronary arterial fistulas seen between 1976 and 1997, and aged 2 days to 16 years, with a median age of 3.2 years. Only four patients were symptomatic: two had heart failure, one had exertional dyspnoea, and one infective endocarditis. The fistulas originated from the right coronary artery in seven patients, from the left coronary artery in seven, from both coronary arteries in one patient, while the origin was not clearly defined in the final patient. Associated cardiac anomalies were discovered in six patients, with three of the fistulas being diagnosed at the same presentation. Cross-sectional echocardiography had revealed a dilated coronary artery in 7 out of 11 subjects. The ratio of pulmonary to systemic flows ranged between 0.9 to 3.0, with a median of 1.5. Ten patients were referred for corrective surgery without any mortality. Trans-catheter closure was successfully undertaken in one patient, while spontaneous closure of the fistula was noted in two patients. We conclude that coronary arterial fistulas, although rare and potentially serious, are generally treatable.

Intrapericardial teratomas are rare primary cardiac tumors of infancy and childhood. We describe three neonates with intrapericardial teratomas diagnosed during fetal life and treated after birth. Clinical and anatomic considerations suggest that cardiopulmonary bypass provides for safe tumor dissection and complete excision of the tumor, thereby decreasing the risk of recurrence.

Isomerism of the atriums has often been described with various complex cardiac malformations. As far as is known, a case of ‘ventricular isomerism’ has never been recorded. Described is a specimen where, on the basis of morphological criterions, there are two right ventricles.

The incidence of congenital heart disease is higher in monozygotic than dizygotic twins, with a higher concordance rate. Although coarctation of the aorta has previously been reported in monozygotic twins, to the best of our knowledge it has not been described in dizygotic twins. We report here such a concurrence in dizygotic twins conceived by in-vitro fertilisation. The finding provides support for both genetic and environmental factors in the aetiology of congenital heart disease. Furthermore, it highlights our lack of data regarding the outcome of in-vitro fertilisation.

Detailed post-mortem is crucial in infants who die suddenly and without a known cause. We report a rare case of histiocytoid cardiomyopathy with endocardial fibroelastosis, the second case in the world literature. The infant presented with sudden death, but the cardiac histological appearance was initially believed to be caused by Pompes disease.

We describe an alternative to the water-seal system, suitable for drainage of pericardial and eventually pleural effusions, which has been shown to safe, effective, not painful, and rather “friendly”, providing patients with a smoother clinical course.

We successfully visualized the brachiocephalic arteries and aortic arch in a fetus seen at 19 weeks of gestation with a common arterial trunk and interrupted aortic arch by means of color power Doppler angiography, a new diagnostic development of color Doppler echocardiography. Power Doppler imaging is more sensitive to the state of low flow in fetal vessels, thus providing better visualization of fetal vascular structures from an early gestational stage.

Tetralogy of Fallot is often found in association with a wide variety of other cardiac lesions, but is rarely found in association with lesions causing obstruction to the left ventricular inflow or outflow. Subaortic stenosis has only rarely been reported in association with tetralogy of Fallot. We report a patient with Marfan syndrome who underwent repair of tetralogy of Fallot at five years of age. Discrete and progressive subaortic stenosis developed two years after the surgical correction, in a previously normal and unobstructed left ventricular outflow tract. Surgical removal of the acquired fibrous subaortic shelf was successful. Clinical signs of obstruction within the left ventricular outflow tract after surgical repair of tetralogy of Fallot should prompt further investigation to exclude the onset of acquired subaortic stenosis.

A patient with pulmonary atresia and intact ventricular septum was found to have a right ventricular-dependent coronary circulation. In this infant both coronary arteries lacked their normal proximal connection with the aorta, perhaps the most egregious form of this prejudicial coronary circulation. Even more interesting was a direct collateral vessel originating from the descending thoracic aorta and connecting with the coronary circulation. This patient has undergone bilateral modified Blalock-Taussig shunts, and left ventricular function seems preserved.

Intrapericardial teratoma is a rare but recognised cause of respiratory distress in neonates. Patients often present with the compressive effects of the mass within the thorax. Prompt diagnosis should be followed swiftly by surgical resection. We report an unusual case of intrapericardial teratoma in a neonate presenting with collapse of the lung which was successfully treated by surgery.

We describe our treatment of a premature baby born weighing 1400 g with severe aortic stenosis, with a gradient of 80 mmHg across the valve. Efforts to advance a 6 mm angioplasty catheter into the stenotic aortic valve via the left ventricle failed. Anterograde angioplasty, instead, was performed using two 4 mm coronary angioplasty catheters. Six months subsequent to the intervention, the pressure gradient measured 25 mmHg, and there was no hemodynamically significant aortic insufficiency.

We present a case of tetralogy of Fallot associated with Scimitar syndrome. The patient was an 11-month old female who underwent successfully total repair of her lesion, including rerouting of the anomalous pulmonary vein to the left atrium. The diagnosis was suspected from the chest x-ray and echocardiography, and confirmed by angiography. To the best of our knowledge only 2 additional cases have previously been reported.