We report a girl, aged 11 years, who presented with cyanosis. Cardiac catheterization showed occlusion of the infrahepatic segment of the inferior caval vein, with drainage of the hepatic veins into the left atrium. Transoesophageal echocardiography revealed an anomalous Eustachian valve that baffled the vein to the left atrium. This lesion is an extremely rare cause of cyanosis.

Temporary wires are routinely sutured to both the atrial and ventricular epimyocardium after open heart surgery. Despite their rarity, complications related to removal of such pacemaking wires may cause life threatening situations. We describe here a patient who developed ventricular fibrillation immediately after removal of temporary epicardial pacemaking wires.

In this review, I will address the problem as to whether children with disease of the aortic valve should undergo repair, or be put forward for the Ross procedure. So as to put this debate in the correct framework, we need to evaluate all the options for aortic valvar disease. These include not only the Ross procedure, involving the insertion of a pulmonary autograft, and valvar repair, but also the option of replacing the valve with a biological or mechanical prosthesis.

We describe an infant with an atrioventricular septal defect and separate valvar orifices for the right and left ventricles, the so-called primum defect, who presented with serious pulmonary hypertension. Treatment with sildenafil was changed to intravenous epoprostenol due to lack of any measurable effects of the initial therapy as shown by echocardiography. The girl died three days after the change in treatment. We suggest that a mismatch between ventilation and perfusion contributed significantly to her death.

We report a case where fetal echocardiography identified both complete heart block and ventricular tachycardia. The mother tested positive for anti-Ro antibodies. Prenatal detection of this unusual combination of arrhythmias prompted early postnatal evaluation, which revealed prolongation of the QT interval. Autoimmune mediated congenitally complete heart block associated with such prolongation of the QT interval has a poor prognosis. The child was successfully treated with beta blockers and implantation of a pacemaker.

We describe our experience with prenatal diagnosis of tetralogy of Fallot with supracardiac totally anomalous pulmonary venous connection. We also suspected obstruction in the ascending vertical vein as it crossed the right bronchus and coursed superiorly to join the right superior caval vein. This finding was confirmed on postnatal echocardiography, and at autopsy.

Ventricular noncompaction is a rare unclassified cardiomyopathy occurring because of arrest of the normal intrauterine compaction of the loose luminal component of the ventricular myocardium. There is limited data regarding its diagnosis and outcome in children. It is recognised, however, that right ventricular involvement is extremely rare. We report a case in which only the right ventricular myocardium was noncompacted, a situation which led to heart failure soon after birth.

Severe right ventricular failure complicated a postoperative systemic inflammatory response in a 33-year-old woman after surgical repair of congenital cardiac malformations. Volume loading, and high doses of catecholamines, failed to produce improvement, but treatment with vasopressin improved all haemodynamic parameters, and also allowed reduction of the other inotropes. After 10 days, the patient was discharged in stable condition from the intensive care unit.

I have previously reported the successful use of the 0.9 millimetre excimer laser catheter in 2 neonates with pulmonary valvar atresia and intact ventricular septum. In this report, I describe the expanded role of the excimer laser in a premature infant weighing 1.8 kilograms who presented with an occluded right pulmonary artery secondary to an organized thrombus. Successful reconstruction of the right pulmonary artery was performed by sequential use of lasers of 0.9, 1.4, and 2 millimetres diameter, followed by conventional balloon angioplasty. As is now the situation in adults, laser catheter intervention should now be an integral part of the armamentarium of the paediatric cardiac interventionalist.

There are now many surgical alternatives available for repairing the aortic valve, depending on whether the primary problem is one of stenosis or insufficiency. Patients with insufficiency of the common truncal valve have a unique anatomic substrate, which in some cases allows an innovative strategy of repair not available for the typical patient with a diseased aortic valve. Surgeons should be aware that there are a large number of techniques described for repair of the aortic valve. Some of these are shown in Table 1. For repair of the truncal valve, in contrast, fewer techniques have been described. As shown in Table 2, they are significantly different than those available for repair of the aortic valve.

A term infant rapidly developed profound cyanosis and metabolic acidosis shortly after an uncomplicated vaginal delivery. Echocardiography identified a flail antero-superior leaflet of the tricuspid valve, which was producing severe tricuspid insufficiency. The clinical state deteriorated despite maximal medical management, and the patient was placed on venoarterial extracorporeal membrane oxygenation. Within twenty-four hours, the metabolic acidosis corrected, inotropic support was discontinued, and the patient was weaned to minimal ventilator settings. Successful repair of the tricuspid valve was performed two days later.

Congenital arteriovenous fistulas between the thoracic arteries and the systemic veins are rare, and in clinical terms may mimic patency of the arterial duct. We present a neonate with a large arteriovenous fistula between the left sixth intercostal artery and the left brachiocephalic vein, to the best of our knowledge a unique site of drainage. To our knowledge, ours is also the earliest presentation and treatment of a thoracic arteriovenous fistula.

Idiopathic aneurysms of the inferior caval vein are uncommon in children and adults. We describe a 14 year old boy with a saccular aneurysm of the inferior caval vein, in whom no surgical intervention was required to repair the aneurysm. The patient is being followed-up periodically for the evaluation of any increase in the diameter of the inferior caval vein.

Cardiac involvement is a rare complication of infection by the influenza B virus. It usually presents with ventricular dysfunction, arrhythmias, or both. We report a 13-year-old boy with clinical, electrocardiographic, and laboratory findings of myocardial ischaemia during an otherwise silent acute infection with influenza B. Coronary endothelial injury constituted a potential underlying mechanism, and microthrombosis was promoted by high levels of lipoprotein(a) in the serum.

We report obstruction of the reconstructed left ventricular outflow tract diagnosed 18 months after an arterial switch operation that included closure of a ventricular septal defect. We speculate that either turbulences created by the patch used to close the ventricular septal defect, or postoperative modifications of ventricular geometry, were responsible for producing this unusual complication.