An unusual case of congenitally unguarded tricuspid valve orifice associated with pulmonary valve atresia and intact ventricular septum.

We report on a premature neonate who presented with cyanosis soon after birth. She was found on echocardiography to have an unguarded tricuspid valve orifice associated with pulmonary valve atresia and an intact interventricular septum. Owing to the small-sized pulmonary arteries, she was initially managed conservatively with prostaglandin infusion, and at one year of age underwent a successful bidirectional cavo-pulmonary (Glenn) shunt operation.

Endovascular covered stent treatment for descending aorta pseudoaneurysm following coarctation of the aorta repair in an infant.

The development of a pseudoaneurysm is a rare complication following repair of a coarctation of the aorta. Surgical management of pseudoaneurysms is associated with high morbidity and mortality. We describe the successful endovascular deployment of a covered stent in a sick infant with a descending aorta pseudoaneurysm, following the repair of an aortic coarctation. We highlight the challenges we encountered. Endovascular repair is a safe palliative alternative to re-do open surgery in unstable infants with large pseudoaneurysms following aortic coarctation repair. The role of endovascular stents as the final definitive therapy will remain limited by the deployable, small-size stents in small, growing children.

Transcatheter occlusion of giant congenital coronary cameral fistulae: a case series.

BACKGROUND: A coronary cameral fistula is a rare connection between a coronary artery and a cardiac chamber or vein bypassing the cardiac capillary bed system. Most of these fistulae are congenital and solitary, although they can be acquired and multiple. CASES PRESENTATION: Case 1: A 10-year-old black South African boy presented with a long-standing history of fatigue; he had a heart murmur, and a bounding pulse and wide pulse pressure. An echocardiogram demonstrated a large coronary cameral fistula involving his left coronary artery and his left ventricle. This was also confirmed on ascending aortogram. Surgical ligation was done and his symptoms improved afterward, but a small residual fistula remained. Case 2: A 7-year-old black South African boy had decreased effort tolerance and a heart murmur on the mid-sternal border. He had cardiomegaly on chest roentgenogram and a dilated left coronary artery origin on echocardiogram. An ascending aortogram confirmed a large left coronary cameral fistula draining to the left ventricle. Case 3: A 28-year-old black South African woman with decreased effort tolerance and chest pain on exertion had a continuous murmur over the lower sternal border. Echocardiography demonstrated a dilated right coronary artery with a fistulous connection to her right ventricle. An ascending aortogram demonstrated a tortuous coronary cameral fistula arising from her right coronary artery to her right ventricle. All three patients were successfully treated percutaneously using the Amplatzer vascular plug type II device. CONCLUSION: The availability of numerous vascular closure devices has made transcatheter occlusion the treatment of choice for the majority of coronary cameral fistulae, rather than the traditional surgical ligation.

Evaluation of the extracardiac conduit modification of the Fontan operation for thrombus formation using magnetic resonance imaging.

BACKGROUND: The prevalence of thrombosis after the Fontan procedure depends upon the surgical technique used and the method of detection employed. Current investigations for thrombosis lack sensitivity and specificity or, in the paediatric population, require a general anaesthetic. We undertook a study to examine the feasibility of using magnetic resonance imaging (MRI) to detect thrombosis within the conduit, cardiac chambers and pulmonary arteries after the extracardiac conduit modification of the Fontan procedure. METHODS: Of the 50 children who had undergone this procedure at our institution between 1997 and 2002, 26 were eligible for, and 13 underwent, MRI study. The mean age was 10.2 years (range 8.2-16.8 years, median 9.5 years) and the average time from operation was 63 months (range 29-79 months, median 68 months). The mean age at Fontan operation was 4.9 years (range 2.1-10.5 years). Ten were on low dose aspirin, two were on warfarin and one was not anti-coagulated. In all cases, satisfactory imaging of the venous pathways and pulmonary arteries was obtained and there were no thrombi detected. CONCLUSIONS: We conclude that MRI is a potentially useful tool for the detection of thrombus in patients who have undergone the Fontan operation.

Cryptogenic Cirrhosis and Hepatopulmonary Syndrome in a Boy with Hepatic Hemangioma in Botswana: A Case Report and Review of the Literature.

Hepatic hemangiomas are considered to be the most common benign tumors of the liver. They are often found incidentally while investigating for other causes of liver disease. Hemangiomas that are less than 10 cm are not expected to cause any problems. Typically, they do not enlarge and, apart from regular follow-up, no definitive treatment is indicated. This is a posthumous case report of a male child with a medium-sized hemangioma from infancy, complicated by cryptogenic cirrhosis and hepatopulmonary syndrome. It demonstrates the challenges of managing a child with such complicated conditions in a resource-limited setting.

Unusual variant of scimitar syndrome associated with an absent right pulmonary artery, stenosis of the inferior vena cava, hemi-azygous continuation and the VACTERL association.

We report on a two-month-old infant with an unusual form of scimitar syndrome, associated with an absent right pulmonary artery, obstructed inferior vena cava, hemi-azygous continuation and the VACTERL association. The infant posed a major management problem and eventually died from a lower respiratory tract infection.

Development of a subaortic aneurysm secondary to disseminated tuberculosis in a child.

Subvalvular aneurysms of the left ventricle are very rare and often the cause is uncertain. Most of the cases of subvalvular left ventricular aneurysms described in the literature are due to congenital weakness of the fibromuscular annuli. We describe a unique case of a child with a tuberculous subaortic aneurysm observed at different stages of development by serial transthoracic echocardiography. The patient underwent successful cardiac surgery after the initial conservative treatment for tuberculosis.