L-carnitine has been used in dilated cardiomyopathy secondary to carnitine deficiency in children, with favourable results. There are no reports on the effects of L-carnitine in children with idiopathic dilated cardiomyopathy. We undertook a prospective study to evaluate the effects of L-carnitine in children with idiopathic dilated cardiomyopathy. Thirteen children, mean age 3.29 +/- 1.44 years, with idiopathic dilated cardiomyopathy underwent echocardiographic evaluation while on conventional treatment alone, and with additional L-carnitine (50 mg/kg/day). To obviate the effects of spontaneous improvement, eight patients (Group 1) were restudied three weeks after stopping the drug, and five (Group 2) were restudied three weeks after addition of carnitine. Conventional treatment was continued throughout. After repeat echocardiographic examination, the parameters were compared statistically. With addition of carnitine, besides symptomatic improvement, the mean left ventricular ejection fraction improved from 36.9 +/- 16.1 percent to 46.9 +/- 14.5 percent (p < 0.001) and the mean pre-ejection period/left ventricular ejection time ratio from 39.07 +/- 14.8 to 43.2 +/- 8.1 (p < 0.01) in the entire group. These changes were concordant in both the subgroups. It was concluded that L-carnitine therapy in children with idiopathic dilated cardiomyopathy led to modest improvement in left ventricular function.

Cardiomyopathy, Dilated↗

PubMedDate not supplied

Balloon dilatation of simple aortic coarctation in neonates and infants.

Twenty-one infants with isolated native coarctation of aorta (mean age 4.73 +/- 2.85 months, range 15 days-12 months) underwent balloon dilatation in the last seven years at our institute. Seven of them were less than three months and 14 were older than three months. The procedure was successful in relieving the coarctation (gradient < 20 mm Hg) in five of seven (71%) infants up to three months and 12 of 14 (86%) above three months. Peak systolic gradients decreased from 70.8 +/- 20.1 to 14.5 +/- 11 mm Hg in the younger infants and from 47.7 +/- 11.7 to 11.4 +/- 9.9 mm Hg in infants above three months. Post-dilatation angiogram showed the coarctation segment to be equal to the isthmus in both the groups, 0.96 +/- 0.14 and 0.96 +/- 0.12 respectively. One three months old infant died immediately after a successful dilatation possibly due to coronary embolism, and another neonate died a few days later of unrelated pre-existing septicemia. Left ventricular function improved in all the infants with depressed preprocedure ejection fraction. Restenosis defined as peak instantaneous echo-Doppler gradient above 20 mm Hg with a significant narrowing on two-dimensional echocardiography occurred in all the five infants up to three months over a period of 1 to 12 months. Follow-up information over a period of 4 to 24 months was available in 11 of 14 older infants of which three had restenosis. Gradients progressively increased in all the four infants (2 of either group) with an initial partially successful result. A small isthmus relative to the descending thoracic aorta at diaphragm is an important predictor of restenosis/inadequate result. This discrepancy was seen much more in smaller infants, post-dilatation coarctation index being 0.68 +/- 0.11 vs 0.81 +/- 0.16 (p = 0.07). Four of the infants underwent a repeat successful dilatation. Balloon dilatation is safe and effective in infants above three months of age. In infants up to three months the procedure is feasible and leads to improvement in left ventricular function, but restenosis occurs rapidly in all of them.

Aorta, Thoracic↗

PubMedDate not supplied

Myocardial dysfunction in children with dengue haemorrhagic fever.

BACKGROUND: Dengue fever/dengue haemorrhagic fever/dengue shock syndrome is a serious health problem in tropical countries. Intravascular fluid depletion due to capillary leak is presumed to be the cause of hypotension in dengue haemorrhagic fever. The treatment guidelines of the World Health Organization lay stress primarily on monitoring and fluid replacement therapy. During the 1996 epidemic in New Delhi, we observed problems in fluid management of such children and prospectively looked for myocardial dysfunction as an additional factor for hypotension. METHODS: Fifty-four children (< 12 years old) admitted to the All India Institute of Medical Sciences, New Delhi after 15 October 1996 with various grades of the disease, who were fit to be shifted to the echocardiography laboratory, were examined clinically and subjected to a detailed M-mode, 2-dimensional and colour doppler echocardiography. Ejection fractions (Teichholz/Modified Simpson's) and shortening fractions were calculated. RESULTS: Ejection fraction by modified Simpson's rule was reduced (< 50%) in 9/54 (16.7%) children; 2 of these had significant reductions (< 35%). These 9 children belonged to all stages of clinical severity. Three of these 9 children who had a repeat echocardiogram within 2 months of the illness had improved ejection fractions. CONCLUSION: The role of myocardial dysfunction remains to be defined as there was no correlation with clinical severity. Myocardial functions need to be assessed in patients with this disease, especially those who have persistent hypotension in spite of adequate hydration.

Child↗

PubMedDate not supplied

Anomalous drainage of right superior vena cava into the left atrium.

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Cardiac Surgical Procedures↗