We describe the clinical features of idiopathic restrictive cardiomyopathy in a female infant. A marked elevation of left ventricular end-diastolic pressure, and profoundly abnormal myocardial relaxation, were detected with the use of Doppler blood flow echocardiography, coupled with the relatively new technique of Doppler tissue echocardiography. There was no clinical evidence of ongoing heart failure, but she had signs of myocardial ischaemia, and unfortunately died suddenly at the age of 13 months.

We describe a patient with advanced perinatal acquired immunodeficiency syndrome who had early clinical manifestation of severe dilated cardiomyopathy with congestive heart failure. The picture was completely reversed after six years treatment and follow-up, and the child is now doing well at the age of seven, with normal left ventricular dimension and contractility as shown by echodopplercardiography. To the best of our knowledge, this is the first reported case of full recovery from cardiomyopathy in children with perinatally acquired infection by the human immunodeficiency virus.

We report a rare variant of anomalous origin of the left coronary artery from the non-adjacent sinus of the pulmonary trunk. The patient also had the right coronary artery arising from the non-facing sinus of the aorta.

In a fetus at 20 weeks gestation, we found a large fistula from the left coronary artery to the left atrium in association with tetralogy of Fallot. Postnatally, the therapeutic issues were complex because of prematurity, low birth weight, decreased flow of blood to the lungs, and volume overload of the left ventricle because of the huge fistula. At three months, the baby underwent repair of tetralogy of Fallot, with surgical ligation of the fistula, but the baby died postoperatively.

Tetralogy of Fallot is only rarely associated with aortic valvar disease. We present a child who had uncomplicated repair of tetralogy of Fallot at 16 months of age, and who developed mild aortic stenosis three years later, with mild to moderate aortic incompetence being seen two years subsequent to that event. No aortic valvar disease had been noted prior to surgery. No intervention is planned at this stage.

Our long-term follow-up has revealed that symmetrical development of the breasts is significantly impaired in pre-pubescent female patients subsequent to an anterolateral thoracotomy. Although standard posterolateral and anterolateral right-sided thoracotomies are used for correction of “simple” cardiac lesions such as patency of the arterial duct, coarctation of the aorta, or atrial septal defect, the required partial transection of large muscle groups, and injury to the developing tissues of the breast, may contribute to an unfavourable cosmetic outcome. Over the years, many surgeons, mostly specialising in thoracic procedures, have advocated mini- or axillary thoracotomies. In an attempt to improve surgical and cosmetic outcome, we have now adopted such a muscle-sparing approach, using a small horizontal midaxillary incision. We have now successfully employed the technique to close atrial septal defects in the oval fossa in 17 pre-pubescent females.

We report two siblings with isolated ectopic hearts. Neither child had associated congenital diseases. To the best of our knowledge, this is the first reported familial occurrence of ectopic hearts.

We treated a neonate with pulmonary atresia and a ventricular septal defect complicated by straddling of the atrioventricular valves by constructing a central aorto-pulmonary shunt. The postoperative course was complicated by obstruction of the shunt, which was treated by implantation of a coronary stent. Six months after the stenting, a Glenn anastomosis was created and the stented shunt removed. Analysis showed that the shunt was completely covered by a vascularized neointima. The stent had not produced injury to the shunt, with struts of the stent covered nicely by neoendothelium, with sparse inflammation surrounding the artificial implants.

We report a 12-year-old girl who had multiple congenital cardiac lesions, specifically an arterial duct, left pulmonary arterial stenosis, an atrial septal defect in the oval fossa, and mild Ebstein's malformation of the tricuspid valve. Therapeutic transcatheter intervention was performed to stent the left pulmonary artery, occlude the arterial duct with a coil, and place a device to close the atrial septal defect. Subsequent to the catheterization, she complained of hoarseness, which was shown to be due to entrapment of the left recurrent laryngeal nerve between the coil used to close the arterial duct and the stent placed in the left pulmonary artery. Laryngoscopy confirmed paralysis of the recurrent laryngeal nerve.

A young woman with hypertrophic cardiomyopathy presented with intractable chest pain due to a myocardial bridge over the left anterior descending artery, producing severe compression during systole. Percutaneous intracoronary stenting was performed. The patient, however, developed severe and diffuse restenosis within 30 days of the procedure. Myotomy may provide a more effective treatment option for such patients with symptomatic myocardial bridging.

In a 4-year-old boy with severe mitral regurgitation, cross sectional echocardiography combined with Doppler interrogation confirmed the presence of isolated perforation of the aortic leaflet of the mitral valve. The perforation was closed with a patch of fresh autologous pericardium. Serial echocardiograms taken postoperatively showed no regurgitation across the mitral valve.

A man, aged 32, who had undergone surgical correction of complex congenital cardiac disease, had chronic severe right heart failure. Increasing reliance on diuretics led to deteriorating renal function, and he was rejected as a candidate for cardiac transplantation. By inserting a Tenckhoff catheter, and training him to drain his ascites at home, we achieved a dramatic improvement in both his cardiac and renal function. As far as we can ascertain, this is the first time this technique has been used for such a patient. We discuss the mechanisms of improvement, and suggest that other such patients with congenital cardiac disease might benefit from similar management.

We report a novel technique using an Amplatzer atrial septal occluder to close a defect located in the inferior-posterior portion of the interatrial septum that extended into the mouth of the inferior caval vein. Because of the close relation of the defect to the inferior caval vein, the right atrial disc was opened into the inferior caval vein and pushed toward the right atrium by use of the delivery cable. There was no residual shunting immediately and 3 months after the intervention. We conclude that even defects located infero-posteriorly within the oval fossa may be successfully closed by transcatheter techniques using the Amplatzer device.

At 32 weeks, a fetus was found to be hydropic with congenitally complete heart block in an asymptomatic mother who was positive to anti-Ro antibodies. Maternal therapy with oral salbutamol was successful in prolonging gestation for a period of 3 weeks so that preterm delivery was delayed until the 35th week of gestation. Following treatment with inotropes and diuretics, a permanent transvenous pacemaker was implanted at 8 weeks of age. The child is now thriving at 20 months of age.

We describe the clinical history of a nine years old girl with Shone's syndrome. She underwent balloon angioplasty of the aortic coarctation in infancy, and later developed severe sub-aortic stenosis and moderate mitral valvar stenosis. The mitral valve was therefore replaced with a mechanical prosthesis, and the sub-aortic shelf was resected. Immediately after the operation, she developed signs of myocardial ischemia. Coronary angiography showed compression of the middle part of the circumflex artery by the mechanical prosthesis, the obstructed segment being successfully dilated using a coronary arterial stent.

The potential for development of 2 to 1 atrioventricular conduction in children with prolongation of the QT interval has been previously reported secondary to electrolytic disturbances. We report here a child who developed 2 to 1 atrioventricular conduction with prolongation of the QT interval following treatment with amiodarone for refractory supraventricular tachycardia. We highlight the importance of electrocardiographic monitoring to assess for those at risk of amiodarone toxicity, which may be manifested by prolongation of the QT interval and the simultaneous loss of atrioventricular conduction, and of equal importance the need for prompt conversion to an alternative anti-arrhythmic agent.

Haemoptysis may occur in patients with tetralogy of Fallot and major aorto-pulmonary collateral arteries. We describe such a patient in whom bleeding from a major aorto-pulmonary collateral artery produced severe pulmonary haemorrhage. Interventional closure of the artery could not be performed because it perfused the native pulmonary arteries. Instead, we inserted a conduit between the right ventricle and the native pulmonary arteries, followed by percutaneous closure of the collateral artery. Our patient demonstrates the increasing necessity for combined surgical and interventional procedures.