Poothirikovil Venugopalan

Left atrial spontaneous echo contrast in patients with rheumatic mitral valve stenosis in sinus rhythm : relationship to mitral valve and left atrial measurements

We studied 37 consecutive patients with mitral stenosis in sinus rhythm using transthoracic and transesophageal echocardiography to relate the presence of spontaneous echo contrast (SEC) in the left atrium with mitral valve area and left atrial dimensions. We also compared the value of left atrial area by planimetry with that of left atrial dimension by M mode in predicting presence of SEC and monitored the effect of anticoagulation on SEC. Transesophageal echocardiography demonstrated spontaneous echo contrast in 9/37 (24%) patients and thrombus in none. SEC continued to be present despite anticoagulation. Mitral valve orifice area by pressure half time method (P=0.001) and by planimetry (P=0.01), and left atrial area by planimetry (P<0.05) were predictors to presence of SEC. Left atrial dimension by M mode examination failed to predict SEC. Cut off values were mitral valve orifice </=1.4 cm(2) (agreement 86%) and left atrial area >/=25 cm(2) (agreement 81%). On multivariate analysis mitral valve area was the only independent predictor. SEC persisted despite anticoagulation. This supports the view that more than one mechanism is involved in the development of SEC.

Prevalence and aetiology of heart failure in an Arab population

The epidemiology of heart failure in Arabia, with a population different from the West in cultural and ethnic origin, has not been studied before.

Beneficial effect of carvedilol on heart rate response to exercise in digitalised patients with heart failure in atrial fibrillation due to idiopathic dilated cardiomyopathy.

Fourteen digitalised patients diagnosed with heart failure (NYHA Functional class II) with idiopathic dilated cardiomyopathy in chronic established atrial fibrillation were administered carvedilol in addition to their anti‐heart failure medications in an attempt to improve their heart rate control. Fourteen matched patients who did not receive carvedilol acted as control subjects. Patients treated with carvedilol showed significantly reduced resting heart rates (10–36%), maximal heart rates on exercise (5–20%) and an increased exercise time (2–30%) on treadmill stress tests (all P ‐ 0.001). Ventricular ectopic activity was also diminished. This was associated with symptomatic improvement in effort intolerance and palpitations. NYHA functional class, left ventricular dimensions and ejection fractions did not improve during the study period of 3 months. Thus, addition of carvedilol to digoxin had a beneficial effect on exercise tolerance in patients with idiopathic dilated cardiomyopathy in atrial fibrillation by virtue of an improved heart rate control both at rest and on exercise. Carvedilol was well tolerated despite impaired myocardial function.Fourteen digitalised patients diagnosed with heart failure (NYHA Functional class II) with idiopathic dilated cardiomyopathy in chronic established atrial fibrillation were administered carvedilol in addition to their anti-heart failure medications in an attempt to improve their heart rate control. Fourteen matched patients who did not receive carvedilol acted as control subjects. Patients treated with carvedilol showed significantly reduced resting heart rates (10-36%), maximal heart rates on exercise (5-20%) and an increased exercise time (2-30%) on treadmill stress tests (all P=0.001). Ventricular ectopic activity was also diminished. This was associated with symptomatic improvement in effort intolerance and palpitations. NYHA functional class, left ventricular dimensions and ejection fractions did not improve during the study period of 3 months. Thus, addition of carvedilol to digoxin had a beneficial effect on exercise tolerance in patients with idiopathic dilated cardiomyopathy in atrial fibrillation by virtue of an improved heart rate control both at rest and on exercise. Carvedilol was well tolerated despite impaired myocardial function.

Improved prognosis of heart failure due to idiopathic dilated cardiomyopathy in children

We analyzed the outcome of 18 children with idiopathic dilated cardiomyopathy (IDC). There was a striking female preponderance (M:F = 1:3.5) and 15 (83%) presented below the age of 2 years. Follow up for a mean duration of 3.5 years revealed complete recovery in seven (43%) and an additional three (19%) became asymptomatic. Three (19%) died and another three (19%) continued to be symptomatic but in controlled heart failure. Two were lost to follow up. Among those who improved, the majority (75%) did so in 1.5 years. Three of five patients who received periodic dobutamine infusion showed improvement in quality of life. The survivals of 94% at 1 year and 87% at 3 years are significantly better than those previously reported. This is the first such study of IDC in children from the Arab peninsula.

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. A second resolving tumour in the interventricular septum was also detected during the investigation. Although there are no other stigmata of tuberous sclerosis at present, our infant requires close follow-up. Right atrial rhabdomyomas are rare and to our knowledge this is the first reported instance where we could document clearly that the tumour itself formed the substrate for the WPW syndrome.

The full spectrum of persistent fetal vasculature in Aicardi syndrome: an integrated interpretation of ocular malformations

Editor,—Aicardi syndrome (OMIM 304050), a rare genetic disorder of cerebro-ocular development, is characterised by infantile spasms, psychomotor retardation, agenesis of corpus callosum, and lacunar chorio- retinopathy.1 We present an infant girl with Aicardi syndrome. In addition to chorioretinal lacunae, ophthalmic examination revealed microphthalmos, persistent pupillary membrane, posterior synechiae, posterior polar cataract, optic nerve malformation, and epipapillary and epiretinal gliosis. We believe that all these ocular anomalies other than the chorioretinal lacunae, are manifestations of persisting fetal blood vessels and should, therefore, be referred to as “persistent fetal vasculature”.  We discuss the ocular manifestations of Aicardi syndrome and their embryogenesis. We also attempt to link the ocular malformations with those of the brain. ### CASE REPORT A 22 day old baby girl was referred for evaluation of microphthalmos. She was the eighth child born to non-consanguineous parents and was delivered at term by caesarean section. There was no family history of neurological or eye disease. The baby was microcephalic (head circumference 30 cm), but did not have any facial dysmorphism. Neurological examination was unremarkable. On ophthalmic examination, the right eye was found to be microphthalmic with a horizontal corneal diameter of 8 mm. The pupil was irregular with posterior synechiae and persistent pupillary membrane (Fig 1), and the lens had a small, paraxial dot opacity on the posterior surface. Funduscopy revealed a large dysplastic disc with an eccentric coloboma. Glial tissue spread out nasally in a fan-shaped manner along the retinal surface, from the centre of the disc all the way to the ora serrata and posterior surface of the lens. The macula was partially covered …

Plasma catecholamine levels parallel severity of heart failure and have prognostic value in children with dilated cardiomyopathy

Excessive and sustained sympathetic stimulation favours progression of heart failure, and plasma catecholamine estimation can assess the degree of this stimulation w1x. High plasma noradrenaline levels have been reported in children with heart failure but these studies have largely included patients with congenital and valvular heart diseases w2–4x. We studied plasma catecholamine levels in children with chronic heart failure from idiopathic dilated cardiomyopathy(IDC), and related these to the severity of heart failure and outcome.

Cardiac involvement in infantile Sandhoff disease

Abstract: An 18‐month‐old boy with enzyme assay‐confirmed infantile Sandhoff disease (MIM 268800) is reported. Besides the classical neurological features, this patient exhibited severe mitral regurgitation secondary to mitral valve prolapse and mild aortic regurgitation from aortic valve prolapse. He also had asymmetric hypertrophy of the interventricular septum without left ventricular outflow tract obstruction.

Spread of heart diseases seen in an open-access paediatric echocardiography clinic

All patients <13 years of age referred to the open-access paediatric echocardiography clinic at the Sultan Qaboos University Hospital, Muscat, Oman, during the five years from 1994 to 1998 were analysed. Among the 2633 patients studied, 1543 (58.6%) were normal, 845 (32.1%) had congenital heart disease, and 245 (9.3%) had acquired heart disease. The major congenital heart diseases identified were secundum atrial septal defect (22.5%), ventricular septal defect (22.5%), patent ductus arteriosus (15.7%), mitral valve prolapse (10.7%), pulmonary stenosis (9.7%) and atrioventricular septal defect (4.5%). Fifty-eight percent of the congenital heart diseases were identified in the first year of life. Among the acquired heart diseases, rheumatic heart disease (30.2%) and cardiac involvement secondary to haemoglobinopathies (16.7%), dilated cardiomyopathy (16.3%) and hypertrophic cardiomyopathy (12.7%) were significant. Although the presence of specific cardiac symptoms was associated with a high yield of abnormalities, such disorders were also discovered in a significant number of children with isolated cardiac murmur. The referral source did not influence significantly the frequency of heart diseases diagnosed in this study. Open-access echocardiography is important in early detection of heart disease in the paediatric population.

The outcome of idiopathic dilated cardiomyopathy and myocarditis in children from the west of Scotland.

We analysed retrospectively all infants and children with idiopathic dilated cardiomyopathy (IDC) and myocarditis at the Regional Cardiac Centre of the Royal Hospital for Sick Children, Glasgow, during 1980-1997. Among the 39 patients with IDC, 25 (64%) were infants aged < 1 year, eight (20.5%) had wheezing as the presenting symptom, and only six (15%) had a significant cardiac murmur. Thirty-eight of thirty-nine patients diagnosed in life were followed-up for 1 day to 15 years (median 3 years). Twelve of the thirty-nine (31%) died, six deaths were within a week of presentation and the rest within a year. The survival at 1 year and at 12 years was 0.69 (95% CI 0.54 to 0.84). Fourteen patients had histologically proven myocarditis, and all 9/14 (64%) detected at post-mortem and one of the five diagnosed in life died. Patients with myocarditis exhibited an actuarial survival of 0.29 (95% CI 0.04 to 0.53) at 1 year and at 9 years, significantly lower than IDC patients (log rank 9.8, P < 0.01). There was no difference in the outcome for patients with positive or negative Coxsackie titres or who presented in the 1980s and in the 1990s. No risk factor that independently influenced the outcome or survival could be identified in either group. Thus our study from a relatively well-defined population of the west of Scotland showed that a significant proportion of children with IDC and myocarditis died in the first week of illness and that patients with myocarditis had shorter survival.