Weak Radial Artery Pulse: An unusual congenital cause.

We present an 11year-old boy with a weak right radial pulse, and describe the successful application of vascular ultrasound to identify the ulnar artery dominance and a thin right radial artery with below normal Doppler flow velocity that could explain the discrepancy. The implications of identifying this anomaly are discussed.

Clinical characteristics of neonates with inborn errors of metabolism detected by Tandem MS analysis in Oman.

We reviewed the clinical profile of our neonates diagnosed to have inborn errors of metabolism (IEM) by Tandem Mass Spectrometry (TMS) over a seven years period, and compared the results with published reports. We also attempted to evaluate various clinical situations wherein the screening test would yield a high pick up rate. Among the 166 neonates studied (10 aged 1 day, 79 aged 2-7 days and 77 aged 8-28 days), significant abnormalities on TMS suggestive of IEM were detected in 38 babies (23%), most common diseases diagnosed were maple syrup urine disease (10 neonates), propionic acidemia (8 neonates), urea cycle diseases (6 neonates) and isovaleric acidemia (4 neonates).

Right atrial rhabdomyoma acting as the substrate for Wolff-Parkinson-White syndrome in a 3-month-old infant.

We report a 3-month-old infant girl who presented with supraventricular tachycardia, who was detected to have a large right atrial rhabdomyoma and evidence of Wolff-Parkinson-White (WPW) syndrome with an accessory pathway corresponding to the position of the tumour. In view of the resistant cardiac arrhythmia and large size of the tumour, the infant underwent surgical excision of the tumour. The evidence of WPW syndrome disappeared from the surface electrocardiogram immediately upon removal of the tumour.

Survival analysis of patients with idiopathic dilated cardiomyopathy in Oman.

Objective: To study the 10-year survival of adults with idiopathic dilated cardiomyopathy (IDC) in Oman.

Methods: Ninety-seven patients aged >13 years with IDC attending the Cardiology Unit, Sultan Qaboos University Hospital, Muscat, Oman from 1992-1995 were prospectively studied, in order to identify the outcome and factors contributing to death.

Results: Among 97 patients, 2 died from acute heart failure at presentation.

Dizziness during pregnancy due to cardiac myxoma.

A woman in the second trimester of pregnancy with recurrent episodes of dizziness is presented. She had a large left atrial myxoma that interfered with left ventricular filling, compromising cardiac output accounting for the symptoms. Surgical excision of the tumor was performed successfully during pregnancy.

Antiphospholipid syndrome presenting as dilated cardiomyopathy in an 11-year-old boy.

We report on an 11-year-old Omani boy who presented with acute heart failure due to dilated cardiomyopathy. Examination revealed the presence of left ventricular thrombi on echocardiography and a marked elevation of anticardiolipin antibodies. Subsequently the boy exhibited features of antiphospholipid syndrome including multiple thrombotic episodes involving the brain, and renal and iliac veins.

IgA nephropathy presenting clinical features of poststreptococcal glomerulonephritis.

IgA nephropathy and poststreptococcal glomerulonephritis are common forms of primary glomerulonephritis in children. This paper reports a 5-year-old Omani boy who had a chance occurrence of these two different glomerular diseases. Our patient presented with clinical features of poststreptococcal glomerulonephritis and then developed recurrent macroscopic hematuria, polyarthritis, bloody diarrhea, and erythematous swelling of the penis.

Myclonic seizures in a young girl with Fishers variant of Guillain-Barre syndrome.

A 10-year-old girl with Fischer`s variant of acute Guillain-Barre syndrome is described. She had predominantly sensory involvement with autonomic dysfunction, ophthalmoplegia and myoclonic jerks. Myoclonus persisted for 2 weeks and the pupillary involvement was evident even after 2 months.

Malignant familial long QT syndrome.

We report a family with congenital long QT syndrome, an inherited disorder of myocardial repolarization in which affected individuals have prolongation of corrected QT interval on the electrocardiogram and a tendency to develop ventricular arrhythmia, leading to syncope, convulsion or sudden death. Our family is characterized by several affected members (11/16), early onset of symptoms, malignant course prior to diagnosis and good response to beta-blocker therapy. The genetic basis of long QT syndrome has been traced to defective proteins encoding cardiac ion channels.

Accidental detection of a giant right atrial aneurysm in an asymptomatic infant.

We present a 4-month old infant boy accidentally detected to have cardiomegaly on a chest radiograph, and on further investigation discovered to have a giant right atrial aneurysm and a secundum atrial septal defect. Congenital right atrial aneurysm or diverticulum is a rare anomaly, usually presenting with atrial or supraventricular tachycardia. Previously reported patients did not have any associated congenital heart defect.

An unusual presentation of metabolic cardiomyopathy due to Pompe's disease.

An Omani infant boy who presented in the neonatal period with cardiac failure secondary to hypertrophic cardiomyopathy is reported. He subsequently progressed to show features of a metabolic disorder with multisystem involvement and was diagnosed to have Type II glycogenosis (Pompe's disease). The differential diagnosis and management of metabolic cardiomyopathy are outlined.

Accidental tricyclic antidepressant poisoning in a 2 year-old boy.

We present a 2-year old boy with accidental ingestion of a tricyclic antidepressant and outline the clinical features, management and prevention. Despite ingesting a high dose of amitriptyline (20mg/kg) and showing serum levels above the toxic range (1380 ng/ml), our patient did not develop any life threatening complications. Gastric lavage followed by instillation of activated charcoal, repeated at 6 hours, along with supportive measures led to complete recovery in 48 hours.