Long-term follow-up of persistent truncus arteriosus: Kuwait experience.

OBJECTIVE: To evaluate the long-term results of patients in Kuwait who were operated for persistent truncus arteriosus (PTA). SUBJECTS AND METHODS: The following data were collected for retrospective analysis from 24 medical records of consecutive patients with PTA in Kuwait between August 1993 and August 2009: demographics, morphology, management and outcome. Major associated abnormalities included interrupted aortic arch in 1 patient and abnormal coronary artery anatomy in 2. RESULTS: Of the 24 patients, 16 underwent total intracardiac repair. The age at operation ranged from 15 days to 5 years (mean 166.19 ± 438.63 days) and weight ranged from 2.5 to 15 kg (mean 4.3 ± 3.01 kg). The right ventricle to pulmonary artery continuity was established with aortic homograft in 11, pulmonary homograft in 4 and by implantation of a Contegra conduit in 1 patient. Four patients had moderate truncal valve regurgitation requiring concomitant truncal valve repair. After a mean follow-up period of 81.81 ± 61.58 months (range 3-166) there was no death. Eight of the 16 (50%) patients underwent redo homograft operations. One patient who had concomitant truncal valve repair subsequently underwent aortic valve replacement. CONCLUSION: The data showed that complete repair of PTA in the neonatal and early infancy period was the treatment with the best potential for survival. The homograft remained one of the conduits of choice to establish continuity between the right ventricle and the pulmonary artery in spite of the high incidence of conduit redo operations.

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.

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.

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.

Large pericardial effusion: the differentiation of tuberculous from chronic idiopathic effusion.

AIMS: Tuberculosis is a common cause of pericardial effusion in many parts of the world often presenting with tamponade. Its recognition is important but not always easy. This study was to prospectively compare the features of tuberculous with chronic idiopathic pericardial effusion. METHODOLOGY: We studied 47 patients with large pericardial effusions or tamponade. All had pericardiocentesis and chest CT studies. Twenty-eight had tuberculous and 19 viral or idiopathic effusion. Pericardial biopsy was done in 26/28 and tuberculin skin test in 23/28 with tuberculosis and all received specific treatment. RESULTS: Patients with idiopathic effusion were older with thinner pericardium. Tamponade was frequent in both groups (64%). Fever (p<0.001) and pericardial rub (p<0.002) were more with tuberculosis. The skin test measured 17 +/- 3.2 mm. On echocardiography pericardial deposits and strands in the pericardial space were more with tuberculosis. The fluid was similar in quantity and quality. Only and all 28 with tuberculosis had enlarged mediastinal lymph glands on CT measuring 19.5 +/- 8.3 mm. On follow up of 16 +/- 10.2 months glands disappeared in 81% and regressed in 19%. Lymphadenopathy was not seen in any patient with viral/idiopathic pericarditis. All patients in both groups were well at follow up. CONCLUSIONS: Tamponade is frequent with large tuberculous and chronic idiopathic effusions. The quantity and quality of the effusion were similar. Fever and pericardial rub were more frequent with tuberculosis as also deposits and strands on echocardiography and patients had a strongly positive skin test. Mediastinal lymph gland enlargement on chest CT was found only and in all with tuberculous effusion.

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.

Percutaneous pericardial biopsy: technique, efficacy, safety, and value in the management of pericardial effusion in children and adolescents.

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.

New nonsurgical technique for multiple pericardial biopsies.

We report a new, nonsurgical technique for obtaining multiple pericardial biopsies in patients with pericardial effusion. A short catheter is introduced by the Seldinger technique under fluoroscopy through the subxiphoid approach. The pericardial fluid is aspirated and the catheter replaced by an 8F teflon sheath with a curved tip. A bioptome is inserted through the sheath, and air is allowed to enter the pericardium. This method outlines the parietal percardium. The curved sheath directs the bioptome to the left or right wall. Eighteen consecutive patients had an average of eight pieces of pericardium removed. On histological examination, three patients had malignancy. Six had tuberculous granuloma, and mycobacterium tuberculosis was cultured from all six tissue specimens but only once from the fluid. Tissue smears showed acid fast bacilli in four out of six, whereas the fluid was negative in all. The biopsy yielded diagnostic information in nine out of ten patients with a thickened pericardium. There were no complications.