Uhl's anomaly: rare but does exist.

A 17-year-old boy presented with facial puffiness and swelling in the lower limbs for 6 months and one episode of syncope 15 days earlier. Transthoracic echocardiography showed a dilated right atrium and right ventricle with right ventricular systolic dysfunction. The free wall of the right ventricle was thinned out and devoid of myocardium and trabeculations. Cardiac magnetic resonance imaging showed an extremely dilated thin-walled right ventricle and absence of trabeculations, with no fat signal in the right ventricular wall, in contrast to that seen in arrhythmogenic ventricular dysplasia, which confirmed the diagnosis of Uhl's anomaly.

Unusual systemic venous Collateral channels to left atrium causing desaturation after Fontan operation closed percutaneously.

We present an unusual cause of progressive cyanosis in a child appearing 2 years after successful Fontan surgery for tetralogy of Fallot with hypoplastic right ventricle. The cause of cyanosis was identified as one large venous channel draining into the left atrium. The channel was closed by Amplatzer vascular plug resulting in improvement of oxygen saturation.

Intermediate and long-term followup of percutaneous device closure of fossa ovalis atrial septal defect by the Amplatzer septal occluder in a cohort of 529 patients.

OBJECTIVES: The aim of present study is to analyze the intermediate and long-term follow up results of percutaneous closure of fossa ovalis atrial septal defect (ASD) with Amplatzer septal occluder (ASO) in a large cohort of patients including children and adults. METHODS: Between May 1998 and July 2008, 529 patients (age group 2-77 years, median 28 years) underwent successful device closure with an ASO at single tertiary referral cardiac center in India.. This was out of an attempted 543 cases. The procedure was carried out in catheterization laboratory under transesophageal echocardiographic and fluoroscopy guidance. The mean size of ASD was 20 mm (7-40 mm) while size of septal occluder was 10-40 mm (mean 24 mm). Two devices were deployed in four patients. Three patients developed transitory pulmonary edema in immediate postprocedure period requiring ICU care for 48 hrs. All patients were advised for Aspirin (3-5 mg/kg, maximum 150 mg) once daily for 6 months. In patients with device 30 mm or larger, Clopidogril ( 75 mg once daily) was given for 3 months in addition to Aspirin. Clinical evaluation, echocardiogram were done on 3 months, 6 months and then at 1, 3, 5, 7 and 10 years of follow up. Transesophageal echocardiography (TEE) was performed in case of any doubt on clinical evaluation or on transthoracic echocardiography (n=10). RESULTS: Followup data is available for 496 patients (93.7%). Followup period is from 12 months to 120 months (median 56 months). On followup, device was in position in all patients, no residual shunt and no evidence of thrombosis. Interventricular septal motion normalized on day of procedure in 89% patients, in 6% over 3 months while flat septal motion persisted in 5% (n=25, all in age group > 40 years) of cases, though right ventricular dilatation persisted in 10% (n=50, age more than 40 years) of patients. Symptom-free survival was 96.7 % (480/496) in patients who came for followup. Only one 68 year old patient with preexistent tricuspid regurgitation developed congestive heart failure, and one patient (58 years old) had a history of hemiparesis after 1 year of device on telephonic interview. Ten patients were in atrial fibrillation (AF) before the procedure and remained in AF on followup. CONCLUSIONS: Our study showed that percutaneous closure of fossa ovalis ASD is a safe and effective procedure on intermediate and long-term followup in both the children as well as adults.both. Technical factors during the procedure and proper follow up are important. Our single centre intermediate and long term experience in a large number of patients support the use of device closure as an alternative to surgery.

Myocarditis presenting as junctional ectopic tachycardia.

Junctional ectopic tachycardia (JET) is an unusual arrhythmia in children. It is usually described as a congenital tachycardia or as occurring in the postoperative setting. Here we report the case of an 8-year-old girl who presented with JET in the context of viral myocarditis and its later subsidence which could have been in response to Carvedilol therapy.

64-slice multidetector-row computed tomographic angiography for evaluating congenital heart disease.

This study aimed to assess critically the role of 64-slice multidetector-row computed tomographic (MDCT) angiography for evaluating congenital heart disease. The study enrolled 60 consecutive patients (median age, 4.7 years; median weight, 16.5 kg) with congenital heart disease who underwent 64-slice MDCT angiography during the period June 2006 through September 2007. The results were classified as diagnostic categories, and the impact of the procedure on strategizing management was critically analyzed. In each of the groups, the current technique offered a clear advantage over conventional imaging and provided specific clues for surgical/interventional management. A management algorithm was evolved based on questions frequently asked about pulmonary artery anatomy. The correlation with surgical anatomy in all cases that involved surgery was excellent. Early results suggest that 64-slice MDCT angiography is a major breakthrough in cardiovascular imaging with an important diagnostic and decision-aiding role. Diagnostic cardiac catheterization, especially for evaluating great vessel anomalies, could be largely replaced by the described technique for congenital heart disease.