Between 1983 and 1995, 546 Blalock-Taussig shunt procedures were performed in 472 patients: 128 (23-0%) were classical shunts, 90 of them on the same side as to the aortic arch, and 418 (77.0%) were modified shunts, 182 on the same side of the arch. At the time of surgery, 78 patients were aged below one week, 270 from one week to 12 months, and 198 patients were over one year of age. The mean pre-operative arterial saturation (71.7%± 16.5%) was significantly increased to 83%±17.9% immediately after the procedure (p=0.017). The overall hospital mortality rate was 2.9% (16/546), with rates of 2.3% (3/128) for the classical, and 3.1% (13/418) for the modified shunts (p= not significant). The rate was significantly higher, however, for classical shunts when the pulmonary arterial diameter was less than 4 mm (15.4% versus zero; p=0.047), though this relationship was reversed for modified shunts (zero versus 3-6%; p=0.338). Early mortality was significantly influenced by the age at surgery, 5/78 (6.4%) in patients aged below 1 week, 3.7% between 1 week and 1 year, and 0.5% over 1 year (p=0.019). Early mortality was also significantly increased in patients weighing 3kg or less, 8/156 (5.1%), versus 3/303 (1.0%), p=O.O37. Overall, 51 shunts failed (9.3%), 10 early and 41 late. Early failure was significantly increased in patients weighing 3kg or less, 8/156 (5.1%) versus 3/303 (1.0%), p=0.0l6. The overall early failure rate was 1.4% (3/215) when heparin was administered intra-operatively and for 48 hours postoperatively, in contrast to an early failure rate of 3.4% (7/203) when heparin was not used (p = 0.294). Overall rates of failure during follow-up were 9-1% (17/188) in heparinized patients versusl3-6% (24/177), (p=0.173) in non-heparinized patients. Failure of classical shunts was 10.2% (13/128), compared with 6.7% (28/418) for modified shunts (p=0.195). Failure was more common overall if the pulmonary arterial diameter was less than 4 mm, 14.7% (9/61), as opposed to 8.7% (26/300) when the diameter was 4 mm or greater, (p=0.l44). Administration of aspirin during follow-up after the modified shunt procedure reduced failure from 11 % (18/163) to 6.7% (10/150), p=0.176. Classical or modified Blalock-Taussig shunts, either on the same side or opposite to the aortic arch, can be performed on patients of any age with minimum postoperative complications and low operative mortality. The use of intra-and post-operative heparin appears to reduce the overall rate of failure, and the administration of aspirin during follow-up appears to reduce failure of modified Blalock-Taussig shunts.

A child with 11, beta-hydroxylase deficiency, an unusual variant of congenital adrenal hyperplasia, presented with dilated cardiomyopathy. Congestive heart failure and dilatation of ventricles resolved with hydrocortisone replacement therapy. This case highlights the importance of complete history and physical examination of children with dilated cardiomyopathy.

The study describes the pattern of childhood neuromuscular disorders seen in a decade (1982-1992) at King Khalid University Hospital, Riyadh, Saudi Arabia. Similar data are scanty outside Europe and North America, and lacking in Saudi Arabia. Eighty-four children (< or = 16 years) were assigned to an entity of neuromuscular disease following review of the clinical, biochemical and neurophysiological data, and after re-examination of the histological and histochemical features of the muscle biopsies. Of the 84 ascertained cases, 40 (48%) had different forms of muscular dystrophy (MD), 26 (31%) had one of the various types of spinal muscular atrophy (SMA) and two (2.4%) hereditary motor and sensory neuropathy type I. The rest were miscellaneous cases including one (1.2%) with dermatomyositis. Of the dystrophies, severe childhood autosomal recessive muscular dystrophy (SCARMD) was more prevalent (30%) than Duchenne type (25%), conforming with observations from North African countries known to have a high incidence of consanguineous marriages. Family history of other cases of SCARMD included three males and three females, one of whom died at 15 years, and consanguinity was evident in 63%. Congenital MD, inherited in an autosomal recessive pattern, was also common (30%). A history of consanguinity was present in 55%. Of the 26 cases of SMA, type I (Werdnig-Hoffman disease) was the most prevalent (69%). Consanguinity was ascertained in 65% of SMA families and histories revealed another 14 affected siblings. Autosomal recessive forms seem to constitute the bulk of neuromuscular disorders in Saudi Arabia.

Umbilical venous catheterization in neonates is an intravascular infusion route for resuscitation and maintenance fluids, blood and blood products, parenteral nutrition, and hypertonic solutions that can be used as an alternative when peripheral venous access is not possible. When used, special precautions should be taken and guidelines followed to prevent rare but often fatal complications.

AS Al Jarallah, Arrhythmogenic Right Ventricular Dysplasia (Arvd) in Childhood: Case Report with a Review of the Literature. 1997; 17(3): 350-353 Over the last few decades of advances in the field of cardiology, many newly identified diseases have been described. One of these newly recognized conditions was arrhythmogenic right ventricular dysplasia (ARVD), which was first described in 1978 by Frank et al. 1 The clinical spectrum of this condition was described by Marcus et al. in 1982. 2 Estimates of the prevalence of ARVD vary sharply in the literature. Thiene et al. found a startling 20% incidence of ARVD in a study of sudden deaths in young adults originating from northeast Italy. 3 In contrast, in two retrospective studies of sudden deaths in young American adults, the incidence of ARVD was found to be 0.5%-3.0%. 4 In this condition, areas of fatty and fibrous tissues replace the normal myocardium of the right ventricle. The involved myocardium evokes ventricular arrhythmias of right ventricular origin. The diagnosis of ARVD in the past was based on the presence of ventricular arrhythmia with left bundle branch block configuration and morphologic changes or motion abnormalities in the free wall of the right ventricle. 2 Recently, standardized diagnostic criteria have been proposed, based on the presence of major and minor criteria encompassing structural, histological, electrocardiographic, arrhythmia, and genetic factors. 5 Although the long-term prognosis of ARVD is generally good, the prevalence is unknown because of its rarity, especially in the pediatric age group. A male child with ARVD is reported here, with a review of the literature. Case ReportA 12-year-old boy presented with an 18-month history of progressive shortness of breath and recurrent vomiting. Nine months prior to admission, he was seen in a private clinic with severe dyspnea and tachycardia (heart rate of 216 beats/min), where he was diagnosed with heart failure and treated with digoxin, furosemide and spironolactone. There was no history of rheumatic fever or endocarditis and no family history of heart disease.General examination revealed a puffy child, mildly dyspneic, and pink in color. Weight was in the 50th percentile, height in the 10th percentile, temperature was normal, respiratory rate was 22 per minute. Pulse was 110 per minute, regular, good volume, with no radio-femoral delay. Blood pressure was 110/75 mmHg in the right upper arm. Precordial examination revealed no chest deformity, with quiet precordium, no thrill. First and second heart sounds were muffled, third heart sound was audible (gallop rhythm) with a grade 3/6 pansystolic murmur at the left lower sternal border and the lung fields were clear with equal air entry. Abdomen was slightly distended with mild ascites and tender hepatomegaly (6 cm below the right costal margin). There was pitting edema involving both lower limbs.Chest x-ray showed globular enlargement of the heart (cardiothoracic ratio 70%) (Figure 1). The 12-leads electrocardiography showed sinus rh...

Epilepsy can sometimes be mimicked in children by uncommon conditions. We present a 6-year-old boy with congenital deafness and long QT syndrome (Jervell and Lange-Nielsen syndrome) triggered by swimming. He had bradycardia and a history of early seizures and deaths in the family. He had been misdiagnosed and treated as idiopathic generalized tonic-clonic epilepsy for three years without control.