Infected cardiac thrombus is rare in children, with antibiotic or antifungal agents used as the first line of treatment. Persistence is an indication for surgical intervention. We describe two children who were treated successfully with a combination of antibiotic and antithrombotic agents. Use of antithrombotic agents promotes degeneration of fibrin, thus reducing the mass and facilitating the diffusion of the antibiotic and/or antifungal agents.

We report an infant with ectopic atrial tachycardia, due to an aneurysm of the right atrial appendage, who developed congestive heart failure. Although catheter ablation was transiently successful, tachycardia recurred 2 days later. The aneurysm of the right atrial appendage was resected successfully by surgery, and thereafter she did well, reverting to normal sinus rhythm.

Rheumatic carditis is a major manifestation of acute rheumatic fever. Conventional therapy includes the use of salicylates and steroids. To date, however, such therapy has not been proven to have a clear benefit in reducing valvar heart disease. We report the use of high-dose intravenous immunoglobulin in two chidlren with acute rheumatic carditis in whom we have been able to document the beneficial effect.

Cardiac rhabdomyoma represents the commonest primary cardiac tumour. Its natural history is generally favourable, with resolution in the majority of cases. Surgical resection has been described for intractable arrhythmia, severe obstruction of the inflow or outflow tracts sufficient to compromise cardiac output, and systemic embolization. We describe an alternative palliative strategy, which was associated with regression of the tumour.

We describe a 31-year-old man with Down's syndrome who presented with severe chronic hypothyroidism and a massive pericardial effusion. Following partial aspiration of this effusion, he rapidly deteriorated and died. Findings at autopsy revealed him to have an atrioventricular septal defect with shunting at the atrial level. We postulate that, by releasing extrinsic pressure on his right heart by the aspiration, there was sudden shunting of blood from left-to-right, resulting in hypotension, shock, and subsequent death. We highlight the difficulties in management of such a case, and suggest alternative strategies.

Increasingly, paediatric cardiologists are called upon to diagnose cardiac malformations prenatally. In the main, the types of malformation seen during fetal life will be similar to those documented postnatally, but the frequency with which they are encountered, as well as the views that can be used for diagnosis, will be different. This review aims to describe the anatomic spectrum of malformations seen in 917 fetal hearts examined consecutively following prenatal diagnosis. The distribution of anomalies is illustrated in terms of a simple sweep through the fetal thorax passing from the four-chamber plane to the outflow tracts, and then to more cranial views of the mediastinum. Two-thirds of the anomalies described would have been detectable in the four-chamber plane. Some, such as tricuspid valvar abnormalities, will alter the normal appearances of the four-chambers dramatically. In terms of the overall spectrum, however, such obvious abnormalities only form a minor part of the total number. Others, such as atrioventricular septal defect, will often require closer inspection of intracardiac anatomy, but will make up a large proportion of the entire cohort. Up to one third of the anomalies in the series would have required views more cranial to the four-chamber plane of section. In these, it would have been necessary to examine the nature of the left ventricular outflow tract, the crossing of the two outflows, or else the arterial arches in order to secure detection. In the fetus, these and other planes must be considered by the echocardiographer in order completely to detect and document the entire spectrum of cardiac abnormalities likely to be encountered.

Cardiac tamponade occurring late after interventional closure of defects within the oval fossa is a very rare but life-threatening complication. We describe such an occurrence after use of a Cardioseal device to close an interatrial communication. Two arms of the device had perforated left atrial wall. The device was removed at surgery, and the defect closed uneventfully. All available means should be used to identify this complication.

A newborn infant with tetralogy of Fallot and absent pulmonary valve was successfully corrected in two stages. Absent pulmonary valve syndrome presenting in early infancy manifests severe respiratory symptoms that still make challenging both management and surgical treatment. This is ascribed to tracheobronchial compression by the extremely dilated pulmonary arteries, and to the resultant pulmonary obstructive lesions. We report herein the first findings of 99m Technegas ventilation lung scanning in an infant with the syndrome to assess the pulmonary obstructive lesions.

In a neonate born prior to term with a weight of 1825 grams, and diagnosed prenatally as having atrioventricular septal defect and Down's syndrome, we found the aortic arch to be interrupted between the left carotid artery and the left subclavian artery, with the arterial duct being the only route of distal perfusion. Three days later, however, echocardiographic interrogation revealed marked collateral connections between the aortic arch and the descending aorta, the picture then mimicking coarctation rather than interruption of the aortic arch. The rapid development of the collateral arteries was confirmed by magnetic resonance imaging and during cardiac surgery.

Anomalous origin of the left coronary artery from the pulmonary arteries is a rare and life-threatening defect, usually needing prompt surgical correction during infancy. We describe the case of a young asymptomatic patient with this defect who underwent surgical reimplantation despite the absence of signs of myocardial ischemia, due to the presence of proximal stenosis of the anomalous coronary artery.

Patients with complex congenital cardiac malformations who have been converted to the Fontan circulation with partial exclusion of the hepatic veins may develop progressive cyanosis because of formation of intrahepatic veno-venous malformations. We describe transcatheter closure of a major intrahepatic fistula in such a setting using an Amplatzer septal occluder delivered by the left jugular venous approach in a 5 year old boy.

We describe a neonate with DiGeorge syndrome undergoing diagnosis and successful repair of interrupted right aortic arch and origin of the left pulmonary artery from the aorta. We discuss a link between this lesion and persistence of a left fifth arch.

At some point during vertebrate evolution from species dwelling in water to living on land, the ancestral double or right aortic arches became single and left-sided in mammals, including humans, as the result of synchronous developments in cardiovascular and respiratory embryogenesis. Since left-sided aortic arches are unique to mammals, hemodynamics related to the placenta, specifically the requirement for a large arterial duct connecting to the descending aorta, may have led to switching from the right-sided to the left-sided arch. Additionally, development of a trilobar right lung and its bronchial tree, also unique to mammalian evolution, restricted the space above the high eparterial bronchus to a single large vessel. Consequently, mammals that mutated to the left-sided aortic arch avoided respiratory, digestive or circulatory problems that are often associated with an isolated right-sided aortic arch – something which could be considered a successful mistake. Due to natural selection, and survival of the fittest, the left-sided arch became the norm in mammals.

In congenital cardiac malformations where a large arterial duct is not mandatory in fetal life, as in Fallot's tetralogy or common arterial trunk, a right-sided aortic arch continues to occur, perhaps as an atavistic reversion to the anatomy seen in ancestral vertebrates.

We report a 17 year old boy with a large fistula from the right coronary artery to the right ventricle. The fistula consisted of two major branches. The smaller branch could be embolised with three detachable platinum microcoils without problems. The high flow of blood in the larger branch, however, prevented conventional occlusion using coils. Interventional occlusion of this branch was achieved in a second attempt. Following creation of an arterio-venous wire-loop, we advanced a balloon catheter into the distal end of the fistula. Under temporary occlusion of the fistula, complete closure was achieved by delivery of 7 detachable platinum microcoils. Thus, even large coronary arterial fistulas can be closed safely with these new platinum detachable microcoils. The procedure, however, requires temporary control of the flow of blood by balloon occlusion.

The authors achieved biventricular repair in a 1-year-old boy having double outlet right ventricle and unusual straddling of the mitral valve. There was a substantial abnormal muscular bar present within the right ventricle, and some of the tendinous cords of the mitral valve were attached to this structure. Preoperative echocardiography was particularly informative in enabling the surgeon to arrange the optional operative procedures. The postoperative course was smooth and uneventful.

We describe a 970-gram infant born prematurely at 28 weeks gestation. On the third day of life, she presented in heart failure with Shone's syndrome and severe coarctation of the aorta. Despite initiation of intravenous prostaglandins, she developed progressive severe biventricular dysfunction and acidosis. Balloon angioplasty of the coarctation was successfully performed on the fifth day of life, with a dramatic improvement in ventricular function. At seven weeks of age, after improvement of ventricular function, she underwent surgical resection of the coarctation and arch advancement. At six months of age she is thriving and developing normally.

We describe a 2-year-old girl with tetralogy of Fallot and pulmonary atresia, palliated as a neonate with a right modified Blalock Taussig shunt, who developed severe cyanosis following total correction in the absence of corresponding evidence of parenchymal lung disease on the chest X-ray. Selective pulmonary angiography showed new intrapulmonary shunting involving only the right middle and lower lobes only. The cyanosis resolved rapidly subsequent to inhalation of nitric oxide. To our knowledge, this is the first documented case of rapid onset of localised intrapulmonary right-to-left shunting, involving only two lung lobes, following biventricular repair for complex congenital heart disease.

Anastomosis of the ascending aorta to the right pulmonary artery, the so-called Waterston shunt, was undertaken as a palliative procedure for children with cyanotic congenital heart disease due to obstruction of the pulmonary outflow tract with reduced pulmonary blood flow. We present the clinico-pathological correlations in two patients who underwent construction of Waterston shunts as neonates, and subsequently died of ruptured pulmonary aneurysms in adult life. Rupture should, therefore, be recognized as a late complication of this procedure, and be considered in the long-term follow-up of such patients, especially when the shunted lung is hypertensive.

Isolated noncompaction of the ventricular myocardium is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. The diagnosis can be made echocardiographically, and the entity may be associated with problems of cardiac rhythm. We describe two illustrative cases, with Wolf-Parkinson-White syndrome in one, and left bundle branch block in the other. We emphasize that children with problems of rhythm and findings suggestive for left ventricular cardiomyopathy, either hypertrophic or dilated; should be investigated to exclude isolated noncompaction of the ventricular myocardium.