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

To evaluate the long-term results of patients in Kuwait who were operated for persistent truncus arteriosus [PTA]. 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. 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. 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

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. 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. 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