We report two cases of hemopericardium occurring in seven-month-old and 12-year-old boys, who had no history of major trauma. The possible cause of the hemopericardium for the infant was falling from a bed which was 75 cm high two weeks prior to the admission. The 12-year-old boy had fallen from a chair and damaged his chest 4 weeks previously. Their coagulation tests were all normal. By means of pericardiotomy, we drained 120 ml and 1200 ml of blood, respectively. The boys have now been well over follow-up periods of 24 and 18 months, respectively

Amongst all primary cardiac tumors, myxomas are the commonest, and their commonest site of origin is the left atrium. Myxomas originating in the left ventricle are rare. When seen, they usually present with a history of systemic embolisation and/or syncopal episodes, with constitutional symptoms being absent. We report here a child with left ventricular myxoma who presented with a prolonged febrile illness.

In a young infant with divided left atrium, unroofed coronary sinus syndrome, and persistent left superior caval vein, the pulmonary venous blood was successfully routed, after atrial septectomy, to the mitral valve by constructing a baffle using the partially detached diaphragm which had initially separated the pulmonary venous and vestibular components of the left atrium. The baffle was augmented with a patch of untreated autologous pericardium.

Ruptured aneurysm of an aortic sinus of Valsalva is a rare cause of left-to-right shunting. We show how resonance imaging can be used to make the diagnosis. This technique can successfully characterize the shunt as well as determine the presence of associated anomalies, such as ventricular septation and aortic regurgitation. It may be the only study required prior to therapeutic intervention.

A family is described where the father has the many skeletal, but none of the cardiac abnormalities associated with the Holt-Oram syndrome. His two daughters have similar skeletal anomalies, but with identical cardiac lesions, as does another patient, raising the possibility of an associated genetic marker.

We report right ventricular ultrastructure in a 20-year-old patient with Noonan's Syndrome. Initial left ventricular and septal myocardial hypertrophy, observed at 2 months without obstruction, progressed to biventricular involvement over 18 years. Histopathologic evidence of cardiomyocytic disarray, with ultrastructural evidence of myofibrillar disarray, confirmed typical biopsy findings of hypertrophic cardiomyopathy on the right ventricular side of the septum.

Cardiac rhabdomyoma is a rare tumor. Because of its rate of regression, mainly in the first year of life, conservative therapy is generally suggested when diagnosis occurs in infancy. We report surgical ablation of a rhabdomyoma in a 2-month-old boy in whom the tumor extended into the subaortic outflow tract. The tumor, which had a dual attachment to the aortic and mitral valves, was safely removed without any valvar damage. We suggest immediate surgical intervention for those rhabdomyomas which are life-threatening because of their size, location, or arrhythmogenicity.

Chick embryos are frequently used as animal models when researching the developing heart. In the past, every attempt to induce complete transposition (the combination of concordant arrioventricular and discordant ventriculo-arterial connections) failed in chicks, suggesting that it might be impossible to develop a chicken modle for this malformation. We demonstrate, to the best of our knowledge, the first well-documented case of complete transpositon occourrine in the chick

The association of left isomerism with interruption and azygos continuation of the inferior caval vein, anomalous connection of the right pulmonary veins to the right atrium, an interatrial commu nication of sinus venosus type, and tetralogy of Fallot with right aortic arch and anomalous coronary artery has been observed in a 6-month-old patient. As far as we are aware, the association of these congenital car diac malformations has not previously been reported in patients with isomeric left atrial appendages.

Cardiac rhabdomyoma is a rare tumor. Because of its rate of regression, mainly in the first year of life, conservative therapy is generally suggested when diagnosis occurs in infancy. We report surgical ablation of a rhabdomyoma in a 2-month-old boy in whom the tumor extended into the subaortic outflow tract. The tumor, which had a dual attachment to the aortic and mitral valves, was safely removed without any valvar damage. We suggest immediate surgical intervention for those rhabdomyomas which are life-threatening because of their size, location, or arrhythmogenicity.

Congenital herat disease is a significant cause of morbidity and mortality in the newborn. Its diagnosis may lead to a crisis in the affercted families; there are the percerived implications of having an abnormality of so vital on organ. To that may be added the assumed guilt or blame, grief and at times anger, frequently experienced by parents of abnormal infants. It often befalls the paediatric cardiologist to initiate counselling while providing the expert information concerning the abnormality and its optimum management

Such counselling differs from that needed for minor lesions as compared for more complex abnormalities where a fatal outcome may ensure. While it is important to provide an accurate diagnosis and management plan to the parents, early detailed information is often confusing and may not be assimilated at a time of great stress. The parents seem more concerned as to whether the infant will survive, what the long term outlook will be, whether he or she will attend school, play, work and so on. With the more severe cardiac abnormalities, especially where there is a family history, one need be aware of the often perceived guilt of the parents. At times, it may be necessary to help the parents retain sufficient ‘self-control’, delaying the grieving process to enable them to contribute to the decision making. Where the infant has died, a follow-up appointment can facilitate grieving and help deal with unresolved issues

Through skilled counselling, the cardiologist in addition to his/her diagnostic and management skills, may meaningfully influence the ongoing care of the infant. They may help avoid the development of unrealistic fears or an over-optimistic outlook, thereby fostering the normal development of the child

We report a case of anomalous origin of the coronary artery from the pulmonary trunk. The lesion was discovered in an eleven-month-old asymptomatic boy because of a continuous murmur.

Innovative cardiac surgery (‘blind’ or indirect infundibular resecion) for tetralogy of Fallot on a child of 4 years was followse by survial for 43 years without further surgery. The patinent remained well until about one year before death, when he developed clinical features of progressive biventricular failure associated with pulmonary hypertension and incompetence of the pulmonary and tricuspid value. Postmortem examination showed severe damage to one of the leaflets of the pulmonary valve, interpreted as due to indavertent avulsion during the original surgical procedure. A large ventricular septal defect was present but there was no residual subpulmonary infundibula obstruction

Anomalous origin of a coronary artery is occasionally associated with other congenital heart malformations. Presence of the anomalous artery can then complicate surgical repair. We report an unusually complex arrangement in a patient who died 16 hours following complete surgical repair despite appropriate management.