JUSTIFICATION:The indications and doses of most drugs used for heart ailments in children are extrapolated from data in adult patients. Separate guidelines are needed for neonates, infants and children because of the differences in underlying heart diseases and metabolic clearance of some of these drugs.PROCESS:Consensus emerged following expert deliberations at the National Meeting on Management of Congenital Heart Diseases in India, held on 13th September 2008, at the All India Institute of Medical Sciences, New Delhi, India, supported by Pediatric Cardiac Society of India.OBJECTIVES:To review the literature and frame evidence based guidelines for (i) indications, doses, adverse effects and safety profile of commonly used drugs in pediatric cardiology practice; and (ii) to provide an algorithm for treatment in various clinical settings.RECOMMENDATIONS:Consensus review and recommendations are given for drugs used in children for heart failure, hypertension, thrombosis, supraventricular tachycardia and intensive care. Guidelines are also given for use of intravenous immunoglobulins and sildenafil in children.

19520 Background: To evaluate the efficacy and safety of low dose Thalidomide and Dexamethasone in previously untreated patients with Multiple Myeloma. Methods: 34 patients (M/F: 21/13), of median age 57.8 years (range 37–74) with previously untreated symptomatic Myeloma were recruited between October 2005 and June 2007 and were treated with Thalidomide 100mg daily and Dexamethasone (40mg daily x 4days) once monthly for 6 months. All patients were investigated with serum protein electrophoresis, skeletal survey and bone marrow aspiration & biopsy. In addition, b2-microglobulin (18/34) and immunoglobulin electrophoresis (24/34) were done in a subset. Patients had IgG (12), IgA (8), IgM (1) & light chain (3) Myeloma. Response was determined as per the EBMT criteria (CR defined as Plasma cells

AbstractCase records of 67 children who presented beyond infancy (57 male, 10 female) with congenital valvar aortic stenosis were reviewed to assess progression utilizing the Doppler derived peak gradient as the index of severity. Age at presentation ranged from 1–13 years, and mean follow-up was 67 ± 29 months (range 12–142 months). The patients were divided into three groups depending on the rate of progression. Those with the most rapid rate of progression were significantly older when aortic stenosis was detected and at the time of presentation compared to the other two groups, who progressed minimally or not at all (p < 0.002 and p < 0.01 respectively). There was no significant correlation between the progression of stenosis and other clinical or Doppler echocardiographic variables.

A case of Salmonella typhimurium endocarditis of a Blalock-Taussig shunt in an infant is described for its rarity. Wider appreciation of such infections is warranted.

Background. Rapid two-stage arterial switch operation is advocated in infants with simple transposition presenting late. Accurate assessment of left ventricular preparation is crucial to successful outcome. The role of echocardiography alone in surgical decision making remains unclear.Methods. Seventeen patients with simple transposition (mean age, 4 months) underwent pulmonary artery banding and modified Blalock-Taussig shunt (first stage) to prepare the left ventricle for the arterial switch operation (second stage). Serial echocardiography was performed in the interval phase to assess left ventricular growth. Sixteen patients underwent arterial switch operation after a mean interval of 10.4 +/- 4 days, with 14 successful conversions. There was one mortality (5.9%) and two conversions to a Senning repair.Results. In all patients a mean increase in left venhicular mass (40.8 +/- 17.8 g/m(2) to 81.4 +/- 25.4 g/m(2)) and posterior wall thickness (3.37 +/- 0.47 mm to 4.63 +/- 0.58 mm) was recorded. Left ventricular end-diastolic internal diameter increased in all except the two switch failures. In all the successful cases the left ventricle had assumed a circular shape on cross-section with the interventricular septum contracting in synergy with the left ventricular mass. In the two failures, however, the interventricular septum had remained flat.Conclusions. Echocardiography can be used reliably in surgical decision making in rapid two-stage arterial switch operation. Increase in left ventricular mass, left ventricular posterior wall thickness, and left ventricular end-diastolic internal diameter toward normal combined with an acquisition of circular left ventricular configuration with the interventricular septum contracting in synergy with the left ventricular mass appear to best predict successful outcome.

A new technique of pulmonary vein wedge angiography to delineate pulmonary artery anatomy is described. Conventional pulmonary vein wedge angiography requires an interatrial communication--natural or created by a transseptal puncture. In retrograde pulmonary vein wedge angiography, the left atrium and pulmonary veins are entered from arterial route using a specially designed catheter. This technique can be specially utilized for patients in whom there is no interatrial communication.

We report a patient with a large submitral aneurysm together with non-specific aorto-arteritis involving the descending thoracic and abdominal aorta.

Fifty-four patients (18 males and 36 females, ages 2 to 37 years) with nonspecific aortoarteritis (NSAA) were studied. Evaluation revealed hypertension in 35, congestive heart failure (CHF) in 24, mild to moderate mitral regurgitation in six, and mild aortic regurgitation in two. Erythrocyte sedimentation rate was raised (> 35 mm in the first hour) in 38 patients. The arterial lesions included type I in seven, type II in eight, and type III in 34. Pulmonary artery involvement was present in 4 (type IV) of the 20 patients in whom it was studied. Selective coronary angiography was done in 11 patients and revealed 90% left main stenosis in one patient. Hemodynamic data revealed raised (> 7 mm Hg) mean right atrial pressure in nine, raised mean pulmonary artery pressure (> 20 mm Hg) in 29, and raised left ventricular filling pressure (> 12 mm Hg) in 27 patients. Radionuclide ventriculography revealed reduced (< 45%) left ventricular ejection fraction (LVEF) in 27 patients. The myocardial morphology as evaluated on right ventricular endomyocardial biopsy revealed normal histology in nine, features of inflammatory myocarditis in 24, and nonspecific changes suggestive of dilated cardiomyopathy in six patients. Marked right ventricular endocardial thickening was present in three. All patients with CHF had some histologic abnormality. We emphasize that myocardial involvement including myocarditis is common in NSAA and may precipitate CHF in these patients.

We conducted electrophysiologic (EP) studies and estimated the sinus node function and atrioventricular (AV) conduction in 10 patients with suspected coronary arterial disease (age range 35–55 years) before and during diltiazem therapy (60 mg thrice daily for 5–7 days). The effect of beta blockade (0.1 mg/kg of intravenous propranolol) was evaluated in both EP studies. The mean spontaneous sinus cycle length (SCL) and the AV nodal Wenckebach cycle lengths (AVWB) were significantly higher (p<0.05) after propranolol alone (913±131 and 504±197 ms, respectively) compared with baseline values (SCL: 827±149 ms, AVWB: 439±173 ms). Diltiazem alone failed to influence the SCL and AVWB significantly. Following the combination (diltiazem + propranolol), SCL (945 ± 147 ms) and AVWB (533 ± 148 ms) were significantly higher (p<0.05) than baseline and post diltiazem values (SCL: 840 ± 150 ms; AVWB 457 ± 103 ms). None of the other parameters (sinoatrial conduction time, corrected sinus node recovery time, AH and HV intervals, AV nodal and atrial effective refractory periods) were significantly influenced by propranolol, diltiazem, or the combination. No patient developed AV block, sinus arrest/sinoatrial exit block, or symptomatic sinus bradycardia following beta blockade after diltiazem administration. Oral diltiazem therapy alone and after beta blockade does not appear to adversely influence the sinus node function and AV conduction in patients below the age of 55 years. The combination of diltiazem and beta blocker thus appears safe in selected patients with coronary arterial disease.

The clinical records of the first 17 consecutive patients (20 lesions) in whom percutaneous transluminal coronary angioplasty was done using the ultra-low profile "balloon-on-wire probe" passed through an intracoronary probing catheter were reviewed. All patients had high-grade "difficult" lesions. In 15 lesions (12 patients) other balloon systems (over-the-wire low profile balloons (n = 9), and balloon-on-wire used alone, (n = 6)) had failed to cross the lesion. Acute ischemia due to complete occlusion at the site of the lesion during attempts to cross was seen in 3 of these patients. The intracoronary probing catheter was used to deliver the probe across the lesion in all these cases. Successful dilatation was achieved in 14 lesions (93%). In 5 lesions (5 patients) this combination was used as the initial strategy. Three of these had chronic total occlusions. Successful dilatation was achieved in 3 lesions (60%) using this combination and in 1 lesion over-the-wire balloon finally succeeded. There was 1 failure. The intracoronary probing catheter in combination with probe balloon wire offers a promising method to increase the success rate in patients with high grade "difficult" lesions. This combination is especially useful in situations where other balloon systems fail to cross the lesion.