Babu Uthaman

The impact of interruption of anomalous systemic arterial supply on scimitar syndrome presenting during infancy.

We sought to evaluate the impact of interruption of anomalous systemic arterial supply (ASAS) on clinical course and management outcome of scimitar syndrome (SS) presenting during infancy.

Transcatheter closure of large pulmonary arteriovenous fistula including pulmonary artery to left atrial fistula with Amplatzer septal occluder.

We sought to evaluate the safety and efficacy of Amplatzer septal occluder (ASO) to close very large pulmonary arteriovenous fistula (PAVF).

Percutaneous Pericardial Biopsy: Technique, Efficacy, Safety, and Value in the Management of Pericardial Effusion in Children and Adolescents

Abstract. This study describes our technique of nonsurgical percutaneous pericardial biopsy (PPB), its efficacy and safety, and its diagnostic and therapeutic usefulness in the management of pericardial effusion (PE) in children and adolescents. In an 8-year period, 19 selected patients, aged 2 to 20 years, had PPB for evaluation and treatment of PE. Using this procedure we procured multiple biopsy specimens from various areas of the thickened parietal pericardium, and also procured pericardial fluid for investigation. The biopsy material helped establish a specific etiologic diagnosis in 63% and helped exclude tuberculosis and malignancy in the rest (37%). The histopathologic diagnoses were tuberculous pericarditis (37%), uremic pericarditis (16%), radiation pericarditis (5%), connective tissue disease (5%), and idiopathic pericarditis (37%). In tuberculous pericarditis, the biopsy specimen provided an immediate histopathologic diagnosis, and a higher culture positivity (71%) than pericardial fluid (29%). In addition, the procedure was therapeutically effective in relieving tamponade and in treating effusion. Our experience indicates that the PPB procedure has both diagnostic and therapeutic value, and it can be performed easily, safely, and without complications even in small children.

Impact of the Gulf war on congenital heart diseases in Kuwait

BACKGROUND There has been concern over the increase in the number of babies born with congenital heart diseases (CHD) in Kuwait after the Gulf War. METHODS We evaluated retrospectively the number of Kuwaiti infants who were diagnosed to have CHD within the first year of life. The comparison was made between those presented from January 1986 to December 1989 (preinvasion) and those presented after the liberation of Kuwait (from January 1992 to December 2000). The number of cases was considered per 10,000 live births in that year. RESULTS The numbers of cases were 2704 (326 before the invasion and 2378 after liberation). The mean annual incidence of CHD was 39.5 and 103.4 (per 10,000 live births) before and after the Gulf War, respectively (P<0.001). There was an increase in the number of babies with CHD during the immediate 3 years postliberation with a relative reduction in the trend from 1995 to 2000, in some types of CHD. CONCLUSIONS In our series, there was an increased incidence of CHD almost immediately following the end of the Gulf War period. The cause of this increase remains relatively obscure. Environmental pollution may be a contributing factor; others such as possible psychological trauma remain subject to speculation.

Detection and follow-up of mediastinal lymph node enlargement in tuberculous pericardial effusions using computed tomography

Tuberculous pericardial effusions are most often due to spread from the mediastinal lymph nodes. The prognosis is excellent with specific therapy; however the diagnosis maybe missed or delayed particularly when the presentation is with cardiac tamponade. After detecting enlarged lymph nodes in a patient with a chest computed tomographic (CT) scan we studied the use of CT scans in patients with proven tuberculous pericardial effusion as well as in patients with postcardiotomy syndrome or viral/idiopathic pericarditis who served as controls. (authors)

Do we have a less severe form of Kawasaki disease or is it the gammaglobulin effect

This study was undertaken to evaluate the incidence of coronary artery aneurysms (CAA) in Kawasaki disease (KD). We reviewed the clinical and echocardiographic findings of 135 children who presented to our center with KD between December 1986 and December 1997. The age of onset ranged between 3months to 13 years (median 2 years). The male to female ratio was 1.54:1. All patients received intravenous Gammaglobulin (IVGG) during the acute stage. The echocardiogram, which was done between 2-3 weeks of the onset of fever, was normal in 106 patients (78.5%). Follow-up studies over a period of 6 months to 1 year remained normal. Minimal right or left coronary artery wall ectasia without dilatation or aneurysm formation was seen in 16 (11.85%). Follow-up of these patients showed disappearance of these changes over 6 weeks to 6 months. One patient (0.74%) had generalised dilatation of all the coronary arteries during the acute stage. This has normalized over a period of 9 months. A total of 10 (7.4%) had CAA during the acute stage. On follow-up of 8 of these patients for an average 3 months to 1.5 years all CAA regressed completely. One patient had residual Giant CAA after 1 year follow-up. One patient with CAA was lost to follow-up. One patient (0.74%) had pericardial effusion and another one (0.74%) had mitral incompetence during the acute stage only, both had no coronary involvement. None of our patients had cardiac failure, arrhythmia, myocardial infarction or death. We conclude that coronary artery changes due to KD are less common and less severe in our patients than those seen in other studies. We speculate that this can be related partly to the early administration of IVGG. The difference in incidence of CAA secondary to KD among different racial groups warrants more detailed genetic studies.

Tuberculous pericardial effusion: features, tamponade, and computed tomography.

The clinical features with particular reference to tamponade and mediastinal adenopathy were studied in tuberculous pericardial effusion. Tamponade is a frequent complication and the recognition of tuberculous etiology can be difficult. Involvement of the pericardium is mostly from mediastinal lymph nodes that have not been studied. This was a prospective cohort study. All patients had large effusions, and underwent pericardiocentesis and chest computed tomography. Patients with tuberculosis had specific therapy. Others with viral/idiopathic effusion served as controls for the computed tomography studies. There were 26 patients with tuberculosis: 18 had tamponade on echocardiography. All had symptoms. Fever (n=23) and dyspnea (n=20) were the most frequent presenting symptoms. Pericardial rub was heard in 14, and 3 had enlarged cervical or axillary nodes. Pulmonary tuberculosis was present in 6. Tuberculin skin test measured 17 ±3.3 mm. The biopsy specimen showed a granuloma in 22 of 24. All 26 had mediastinal lymph nodes >10 mm with a mean size of 19.5 ±8.6 mm that disappeared (81%) or regressed (19%) on treatment (p<0.001). Aortopulmonary nodes were most frequently enlarged (65.4%) and hilar the least. Three required pericardiectomy. At follow-up all were doing well. None with viral/idiopathic effusion had lymph node enlargement. Fever, dyspnea, and tamponade were frequent with tuberculosis. The prognosis was good with specific therapy. Mediastinal nodes were enlarged in all and only with tuberculosis and not with viral/idiopathic effusion. Nodes disappeared or regressed with treatment. In the appropriate clinical context, mediastinal lymph node enlargement on chest computed tomography along with a strongly positive skin test results could help in the diagnosis of a tuberculous etiology of pericardial effusion.

Familial truncus arteriosus: A possible autosomal-recessive trait

Truncus arteriosus is a relatively uncommon congenital malformation. It accounts for approximately 1% of congenital heart diseases. The defect occurs sporadically but 22q11 deletion is frequently noted in such patients with conotruncal defects. We studied six cases of TA in four closely related families. Analysis of karyotypes in these cases was normal. Family 1 has one affected male infant who was born in 1998. Family 2 has two affected children (one male and one female) who were born in 1989 and 1995, respectively. They have four other normal children. Family 3 has two affected children (one male and one female) who were born in 1981 and 1984, respectively. They have three other normal children. Family 4 has one affected male born in 1998 and another healthy child. All parents of all affected children are double cousins. The data in this study are compatible with an autosomal-recessive inheritance, but multifactorial inheritance may also play a role.

Homozygous Familial Hypercholesterolemia with Generalized Arterial Disease

Objective: This report describes the clinical features and management of an 11-year-old boy with end-stage homozygous familial hypercholesterolemia (hoFH) and generalized arterial disease. Clinical Presentation and Intervention: The patient presented with recurrent anginal episodes. On examination, he was found to have multiple planar and tendinous xanthomas, an (LDL) cholesterol level of 24.6 mmol/l and family history of hypercholesterolemia. Resting electrocardiogram showed ST depression in the anterior and inferior leads. Coronary angiogram outlined 70% stenosis of the left main coronary, ostial stenosis of the right coronary artery and extensive atherosclerotic disease of the aorta and all its major branches. The lipid profile was grossly abnormal, but the other biochemical and hematological parameters were normal. The patient was managed with metoprolol 12.5 mg twice daily, nitroglycerin infusion, antithrombotics (aspirin 75 mg once daily and heparin infusion 150 units per hour), cholesterol-lowering drugs (simvastatin 10 mg once a day, cholestyramine 4 g twice a day) and analgesics. Conclusion: This case report emphasizes the need to diagnose early familial hypercholesterolemia in families with heart disease and the need to test the partners of affected persons so that the risk of conceiving children with hoFH can be counseled.

Pulmonary Arteriovenous Malformation Presenting with Severe Hypoxemia

Objective: To report a case of right pulmonary arteriovenous malformation (PAVM) affecting the right upper lobe, following the incidence of empyema thoracis in the contralateral lung. Clinical Presentation and Intervention: A 19-year-old, previously healthy male presented with acute respiratory distress, left pleuritic chest pain, fever and hypoxemia. Clinical findings, laboratory and radiological examinations including pulmonary angiogram were consistent with the diagnosis of left pneumonia complicated with parapneumonic pleural effusion and right upper lobe PAVM. The patient was intubated and ventilated because of persistent hypoxemia. He was successfully treated by percutaneous transcatheter embolization. Conclusion: This case shows that percutaneous transcatheter embolization is a safe and effective first option for the treatment of PAVM.