CAR/01(P) PATENT DUCTUS ARTERIOSUS STENTING IN NEW BORN BABIES Prabhat Kumar Head Deptt of Pediatric, Cardiology Military Hospital (Cardio thoracic centre), AFMC, Pune drprabhat_cardio@yahoo.co.in Introduction: Neonates with duct dependant circulation present as life threatening emergency. There survival depends on patency of the ductus arteriosus ( PDA ) till the time of cardiac surgery. We present our experience of three new born babies who were successfully managed with ductal stenting. Case report : Three babies with birth weight of 2.4 kg - 3.6 kg, noticed to have cyanosis on day of birth were transferred to our centre . Systemic examination was normal except a grade II / VI systolic murmur over left II intercostal space. Echocardiography showed PDA of 2 mm diameter. One patient had Pulmonary atresia while two had hypoplastic left heart syndrome( HLHS ) . Prostaglandin E1 infusion was immediately started at the rate of. 01 mcg / kg / min. Ductal stenting was performed due to its obvious advantages. In patient with pulmonary atresia a coronary stent with diameter 3.5 mm and length of 20 mm and in HLHS a stent size 10 mm X 30mm were deployed. It was ensured that length of the stent was longer than the duct so that it covered full length of duct including both ends adequately . In future these patients will get single ventricle repair. Result and Conclusion: The procedure of ductal stenting is performed at very few centers. The successful procedure highlights the importance of early recognition of new born babies with duct dependant circulation and their referral to a tertiary care centre. CAR/02(P) CONGENITAL HEART DISEASE: CLINICOECHOCARDIOGRAPHICAL PROFILE OF INFANTS ATTENDING PRIVATE MEDICAL COLLEGE Ravinder K. Gupta, Vikas Sharma , Prenika Shangloo , Ashok Bakaya Department of Pediatrics & Cardiology Acharya Shri Chander College of Medical Sciences and Hospital(ASCOMS), Sidhra, Jammu drrk_gupta2000@yahoo.com Objective: To detect different types of congenital heart diseases among the infants attending private medical college and to study the clinical and echocardiographical profile. Design: Prospective Study Setting: Indoor and Outdoor wings of Department of Pediatrics and Cardiology, ASCOMS and Hospital, Sidhra, Jammu. Methods and Subjects: The prospective study was conducted over a period of two years ,i.e Mar 2008 to Feb. 2010. One hundred twenty infants suspected to have features of congenital heart disease were enrolled for the study. Besides detailed history and clinical examination, relevant investigations were done. Echocardiography was done in all the cases. Results: Among the 120 infants enrolled for the study 100 were having congenital heart disease as per echocardiographic findings. In rest 20 patients echocardiography was normal. In this study males slightly outnumbered female (54 males:46 females). There were about 12 neonates. About 40% presented in late infancy. About 32% presented with cyanosis and rest 68% were considered as acyanotic heart disease. About 62% had isolated cardiac lesions while rest 38% had complex lesions. Acyanotic group consisted of VSD (32%) ASD (16%) PDA (12%) while cyanotic group had tetralogy of fallot (TOF) (12%), transposition of great arteries (6%), pulmonary stenosis (6%), pulmonary atresia (4%), complex lesions (4%).Eight neonates had cyanotic heart disease. Six cases of Down’s syndrome were part of the study and the cardiac lesion among three such cases was VSD while rest had ASD. Recurrent chest infections, feeding difficulties, failure to gain weight and excessive sweating while feeding were the common presenting features in acyanotic group. Cyanosis, exertional dyspnea (hypercyanotic spells) and poor weight gain were the presenting features in the cyanotic group. About nine cases of VSD, three cases of PDA were complicated by congestive failure. Conclusion: Acyanotic congenital heart diseases far outnumber the cyanotic counterparts. VSD is the commonest acyanotic heart disease and TOF is the commonest in cyanotic variety. Infants with feeding difficulty, poor weight gain and recurrent chest infections should make one suspect of congenital heart disease. Cyanotic heart disease can present in early infancy. CAR/03(O) COMPARISON OF BOSENTAN VS SILDENAFIL IN CHILDREN WITH PULMONARY HYPERTENSION Brig Mukti Sharma, Maj Suprita Kalra, Col KS Rana Department of Pediatrics, AFMC Pune kalrasuprita@gmail.com Introduction: Pulmonary hypertension(PAH) is a rare cause of morbidity & mortality in children with incidence ranging from one to two new cases per million.Use of sildenafil,a phophodiesterase inhibitor for PAH has been a common practice due to ease of administration & low cost . Aim & objectives: This was a pilot study conducted to compare the efficacy of Bosentan vs Sildenafil in children with PAH.Bosentan is a specific and competitive antagonist of endothelin receptor types ETA and ETB causing selective pulmonary vasodilatation. Materials & methods: 21 children with idiopathic PAH were studied in a tertiary care centre from Jun 2008 to Jun 2010.Children with congenital heart disease with left to right shunt &/or any underlying pulmonary disease were excluded. These children were in the age group of newborn to 12 yrs with mean age group being 2 yrs ± 9.3yrs.Male to female ratio was 1:1.5.The children were randomized to sildenafil or Bosentan group depending upon the affordability. Echocardiographic measurement of TR gradients was taken as marker of PAH. Baseline Echocardiography was done in all children & repeated at the end of 2 wks & 4 wkly thereafter. Results: In 10 children (group 1) initial pulmonary pressures were 40-60mmHg & >60 mmHg in remaining (group II).In children given Bosentan at the end of 2 wks the TR gradients decreased by an average of 10-20 mmHg in group I & 20-30mm in group II.In children on sildenafil in group I l there was only a negligible fall in pulmonary pressure & by 10-20mm Hg in group II. After 2 months the total TR gradients dropped by 30 -40mm in Bosentan group & by 10-20mm in sildenafil group. Conclusion:This study shows that use of Bosentan in children with Pulmonary hypertension has a modest advantage over Sildenafil however a larger study will be required to validate this statement. CAR/04(P) GROWTH OF ECONOMICALLY DISADVANTAGED FOLLOWING CARDIAC SURGERY Praveen S Lal, Pankaj Kasar, J Vimala, R Suresh Kumar Institute of Cardiovascular Diseases, The Madras Medical Mission, Chennai. praveenslal99@gmail.com CHILDREN Introduction: Malnutrition and consequent poor somatic growth are rampant in economically underprivileged children. Children with CHD from underprivileged families have the double burden of poverty and heart disease. Very little is known about the impact of CHD surgery on their subsequent growth. Aims and Objectives: To assess the impact of congenital heart surgery on anthropometric scores of growth in economically disadvantaged children. Materials and Methods: A cohort of 100 economically disadvantaged children was followed up after cardiac surgery for their nutritional recovery. Weight, height and body mass index for age were measured just before surgery and at a median period of 48.1 months (range 9 to 59.9 months) after surgery. Z scores of the age adjusted variables were compared using paired‘t’ tests and the McNemar chi square test wherever appropriate. Results: The mean weight for age of the cohort increased from 14.7±5.76 to 23.8±7.83. In malnourished children (weight for age Z score less than -2) the mean weight changed from -3.1 to -1.6 (p <0.05). The median improvement in weight for age Z score was 0.85. The change in other parameters was not statistically significant. The proportion of malnourished children decreased from 61% to 27% after surgery. Subgroup analysis of the children with malnutrition showed significant improvement in weight for age Z scores (p=0.002) compared to non-malnourished children, those with worse malnutrition faring better. Children with residual malnutrition tended to have extreme economic backwardness, surgery for cyanotic CHD or associated syndromes. Conclusion: Congenital heart surgery resulted in a salutary improvement in the growth of children from an economically underprivileged background. Residual malnutrition was likely to be associated with extreme economic backwardness, surgery for cyanotic congenital heart disease or coincidental syndromes. CAR/05(P) RECOMBINANT ACTIVATED FACTOR VII IN THE MANAGEMENT OF EXCESSIVE BLEEDING FOLLOWING OPEN-HEART SURGERY FOR CONGENITAL HEART DISEASE Praveen S Lal, Deepak Changlani, Uday Charan Murmu, G Selvakumar, J Vimala, R Suresh Kumar Institute of Cardiovascular Diseases, The Madras Medical Mission, Chennai. praveenslal99@gmail.com Introduction: Post-operative bleeding requiring the administration of blood products, haemostatic drugs, and re-exploration are associated with increased morbidity, mortality, and resource consumption. Recombinant activated Factor VII (rFVIIa) is new therapeutic agent, which offers an effective treatment strategy for patients with excessive bleeding. Aims And Objectives: To evaluate the efficacy of recombinant activated Factor VII for excessive bleeding following openheart surgery for congenital heart disease. Materials And Methods: This is a prospective study of 8 consecutive cases of excessive post-operative bleeding following surgery for congenital heart disease admitted to pediatric ICU from August 2009 to August 2010. Median age of patients was 1 year 4 months. Their demographic, procedural, and hematologic data were analyzed. Three patients underwent intracardiac repair for Tetralogy of Fallot, one patient underwent intracardiac repair for DORV/VSD, three patients underwent arterial switch operation for dTGA/VSD and one patient underwent truncus arteriosus repair. Median cardiopulmonary bypass time was 251 min. All patients had persistent intraoperative bleeding not responding to blood products or excessive postoperative chest tube drainage (>10ml/kg/hr). All eight patients received 8090mcg/kg single dose of rFVIIa intravenously over 2-3 min. Time to cessation of bleeding or chest tube drainage <1ml/kg/hr was assessed. Results: In the eight patients who underwent complex open-heart surgery for congenital heart disease, rFVIIa was effective in controlling excessive bleeding within a mean period of 45mins. None of the patients required surgical reexploration. Conclusion: Recombinant FVIIa is a useful drug in the pharmacologic management of excessive post-operative bleeding following open heart surgery for congenital heart disease. CAR/06(P) EVALUATION OF BALLOON ATRIAL SEPTOSTOMY V/S PGE1 INFUSION AS A PALLIATION IN NEONATES WITH TGA Brigadier Mukti Sharma, Major Gururaja R, Colonel Rakesh Gupta Department of Pediatrics, Armed Forces Medical College, Pune – 410040 yashguruafmc@rediffmail.com Introduction: Transposition of great arteries (TGA) with intact ventricular septum present as medical emergency. Such cases require palliation before definitive surgery. Palliation options available are prostaglandin infusion (PGE1) or balloon atrial septostomy (BAS). Our study aims to evaluate the effectiveness of BAS against PGE1 infusion as a palliation in neonates with TGA with intact septum. Aims and Objectives: To evaluate the effectiveness of BAS against PGE1 infusion as a palliation in neonates with TGA. Material and Methods: This study was conducted at a tertiary care centre over period of two years from Jan 2008. All consecutive neonates diagnosed as TGA with intact septum were enrolled in the study and analysed. Results: 15 neonates with TGA with intact septum were evaluated in the study. Based on their presentation and clinical profile, they were divided into 3 groups:- Group A: 4 neonates with PDA and ASD. These neonates did not require any form of palliation and directly underwent definitive surgery. 2 neonates survived and one neonate died while awaiting surgery due to sepsis. Group B: 3 neonates with restrictive ASD underwent BAS. All neonates survived during subsequent follow up. Group C: 8 neonates – 4 had non restrictive ASD and 4 Patent ductus arteriosus (PDA). Among these neonates, 02 presented in shock and were started on PGE1 after resuscitation. PGE1 was started in rest 6 babies pending definitive surgery. 2 neonates underwent Pulmonary artery banding to prepare the left ventricle. Rest were taken up for definitive surgery over next 46 weeks. 2 babies died of sepsis. Conclusions: There was no difference in the septostomy or PGE1 group in the outcome of definitive surgery. Both BAS and PGE1 infusions are equally effective as a palliation pending surgery. PGE1 infusion has the advantage in terms of convenience, less invasive and does not require high skill, but has side effects like apnoea and requires early definitive surgery. While septostomy is more invasive, requires highly skilled staff and cardiac cath lab, however neonate can be taken up for surgery in due course of time. CAR/07(P) NORMATIVE VALUES FOR HEART RATE VARIABILITY IN CHILDREN Vykunta Raju K.N, Sheffali Gulati, Navita Choudhary, Ashok Kumar Jariyal, K. K. Deepak C/o. Dr.Vykunta Raju K.N. DM, Consultant Pediatric Neurologist, Institute of Neurosciences, BGS Health and Education City, #67, Uttarahalli Road, Kengeri, BGS Global Hospital, Bangalore-560060 drknvraju@hotmail.com Purpose -HRV is beat to beat variation in heart rate under resting conditions. It has good tool to quantify the tone of autonomic nervous system to the myocardium. It has been associated with high predictive value in many diseases. As there is no normative data in children, we did this study to document normative HRV data in children. Methods- children of 2 to 15 years were included. Heart disease, diabetes and uremia were excluded. ECG was recorded in supine position for 5 min after 15 min of supine rest. Tests were done when child was comfortable. wave detection and RR intervals were done by software. Results: Mean age was 9.10 ±3.3 (4-15) years, 18(45%) were male. Values were skewed, therefore we expressed in median and range. Low Frequency (LF)Power(ms2)-602(91-2880), High Frequency(HF) Power(ms2)-1303(9110707), LF/HF-0.56(0.13-9.65), Standard deviation of differences between adjacent RR intervals -48(13-1850), Co. of variance-7(4-1073), Variance- 2447(387-12924), Standard deviation of the R-R intervals-37(7-389), Root square of the mean of the sum of the squares of differences between adjacent RR intervals -47(13-931),Number R-R interval differences ≥ 50 ms - 60(1306), Total power ms- 2192(529-15352), very low frequency nu-50(5-138),low frequency nu36(11-90), high frequency nu-55(9-85),Total power nu-145(83-231), percentage of very low frequency-21(5-59), percentage of low frequency-26(8-57) and percentage of high frequency 46(6-75). Discussion-our study results are comparable with Longin E et al (pediatr cardiol.2009.30:311-24). Conclusion-HRV normative data is useful reference date to assess autonomic dysfunction in pediatric neurological disorders like, Guillain -Barre syndrome, refractory epilepsy, autonomic neuropathy and others. CAR/08(P) SITUS INVERSUS WITH CHILDHOOD STROKE WITH INTRACRANIAL VASCULAR MALFORMATION AND DIABETES MELLITUS WITH HYPOTHYROIDISM Vykunta Raju K.N, Gupreet Singh, Sheffali Gulati, Vandana Jain C/o. Dr.Vykunta Raju K.N. DM, Consultant Pediatric Neurologist, Institute of Neurosciences, BGS Health and Education City, #67, Uttarahalli Road, Kengeri, BGS Global Hospital, Bangalore-560060 drknvraju@hotmail.com Purpose- We report a rare association of situs inversus child with type I diabetes mellitus presenting with childhood stroke with intracranial vascular malformation. Method and results: Four year old male child was a case of Diabetes mellitus since the age of 2 years, presented with weakness of right sided upper and lower limb with right sided complex partial seizures of 4 months duration. On examination, right sided upper motor neuron type of weakness and heart sound better heard on right side of the chest with normal rest of the examination. Investigation shows dextrocardia on chest X ray, which was confirmed by echocardiography, abdominal ultrasonogrpahy shows liver on left side and spleen on right side. His diabetes mellitus controlled with 1.5 U/kg/day human insulin. His thyroid function shows increased thyroid stimulating hormone -8 IU, for that started on thyroxin. Screening for celiac disease was normal. Liver and renal function tests were normal. Electroencephalograph shows, epileptiform discharges from left frontocentral areas. MRI and MRA revealed mild diffuse let cerebral atrophy with absent of left sided A1 segment of anterior cerebral artery and left posterior communicating artery. Carotid Doppler was normal. Metabolic work for stroke, complete blood counts, coagulation profile, lupus anticoagulant, lipid profile, arterial lactate was normal. Cause of stroke doesn’t revealed any apparent cause, however complete left cerebral involvement can be explained on basis of left middle cerebral involvement with absent left anterior cerebral artery. Conclusions- Intracranial vascular malformation should be suspected any child presenting with stroke with situs inversus. CAR/09(O) ELECTROCARDIOGRAPHIC CHANGES IN BIRTH ASPHYXIA AND ITS CO-RELATION WITH CARDIAC TROPONIN I Goel Manjusha, Mahawer J, Dwivedi R Department of Paediatrics, Kamla Nehru Hospital and Gandhi Medical College, Bhopal- 462001 manjushagoel@rediffmail.com Introduction: Perinatal asphyxia is one of the leading causes of neonatal mortality with the incidence varying from 1.8 to 7.7 per 1000 live births. The incidence of cardiac dysfunction in birth asphyxia varies from 24-31%. The present study was conducted to find out the ECG changes in birth asphyxia and cardiac troponin I level. Aims: The aim of this study is to determine if any relationship exists between cardiac troponin I (cTnI) level and ECG abnormalities in newborns with birth asphyxia. Objective: To compare the cardiac troponin I level & ECG findings in healthy neonates and neonates with birth asphyxia. Material and method: Forty term babies with birth asphyxia without any congenital malformation were selected as cases. They were compared with twenty healthy term babies without asphyxia. Myocardial dysfunction was evaluated using clinical examination, electrocardiography and cardiac troponin I level.ECG findings were graded according to Jedeikin’s criteria. Results: Among the 40 cases, 21 had evidence of myocardial involvement. Grade I ECG changes were present in 3 (7.5%), Grade II in 11 (27.5%), Grade III in 5 (12.5%), Grade IV in 1 (2.5%). While 9 babies in control group had Grade I ECG changes. Mortality increased with severity of ECG changes. All the cases and control had normal sinus rhythm. Cardiac troponin I level was <1 ng/ml in all cases and controls. Conclusion: ECG is useful in evaluating the severity of myocardial dysfunction and because of wide range of cardiac troponin I, rapid card test has less predictive value. CAR/10(O) DEVICE CLOSURE OF LARGE PDA IN INFANTS. CAN IT BE CLOSED IN INFANTS BELOW 6 Kgs? Chitra Narasimhan, I.B. Vijayalakshmi, Praveen Jayan, C.N.Manjunath Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bangalore- 560069. chitradr@gmail.com Transcatheter closure of PDA has practically replaced surgery. Despite the improvement in technique and hardware, closure of large PDAs in small infants, remains a challenge. Aim : To know the feasibility, efficacy of closing large PDA in infants especially in infants weighing 6 kgs. Materials and Methods: 748 cases of PDA were catheterized between January 1997 September 2010. PDA closure done with coils in 232, Duct Occluder in 309, PFM Nit Occlud in 8, surgery in 173 and managed medically in 26 cases of small PDA. 203/748 (27.1 %) were infants. 61 infants (30.1%), underwent device closure, 38 (18.7%) coil closure and remaining surgery. Of the 61 infants, 38 (62.3%) were < 6 kgs. PDA was closed with duct occluders in 30 and modified duct occluder in 8. Age ranged - 4 - 12 months (mean 8.2 months), weight - 3.9 - 6 kgs (mean 4.5 kgs), PDA measured 4.1 - 8.6 mm (mean 6.3 mm). Fluoroscopy time - 3-18 min (m 13 min). Results: No residual shunt or LPA stenosis in any patient. Average weight gain, one month, post procedure was 1 Kg + 250 mgs. Aortic obstruction occurred in two cases, managed with balloon dilatation (0.05%). One patient with single kidney had device embolisation, latter died due to uraemia (0.02%). 3 patients (0.08 %) had minor vascular complications. Conclusions: Transcatheter closure of very large PDA, in sick malnourished , infants, with LV failure is possible, safe and effective. Aortic and LPA stenosis can be avoided with suitable modifications in the device. CAR/11(P) RIGHT VENTRICULAR OUTFLOW OBSTRUCTION DUE TO FUNGAL MASS IN A NEONATE Aradhana Dwivedi, Daljit Singh, R Ghuliani, Prabhat kumar, Dept of Pediatrics, AFMC & Command Hospital, Pune-411002 aradhanakd@gmail.com Systemic fungal infection occurs in approximately one percent of infants in neonatal intensive care units. Fungal endocarditis is an uncommon manifestation of systemic fungal infections and is usually related to the presence of indwelling catheters in the right atrium. Candida is the usual organism isolated from these cases. Cases due to aspergillus though rare, are the second most common cause of fungal endocarditis. We report a 30 day old infant who developed right ventricular mass due to aspergillus fungus ball in the absence of any indwelling catheter. Case Summary: 4 weeks old infant was admitted with recurrent seizures and hypoglycemia. He required NICU care at birth due to perinatal asphyxia.His birth weight was 5 kg and liver was palpable 5 cm below right costal margin.Other general and systemic examination and investigations to rule out various causes of recurrent hypoglycemia revealed no abnormality. Baby was managed with dextrose infusion. On day 5 of admission, the baby developed features of respiratory distress and shock. Clinical examination and x-ray chest were normal. Echocardiography revealed a mass 2 cm in circumference occupying tricuspid valve and rt ventricle outflow. Baby succumbed to his illness on day 7 of admission. Post mortem was carried out and histopathology revealed the mass to be aspergillus mycelia. A very few cases have been reported in literature of a fungal mass presenting as right ventricular outflow obstruction. CAR/12(O) PERCUTANEOUS DEVICE CLOSURE OF ATRIAL SEPTAL DEFECTS IN CHILDREN <5 YEARS IN A SINGLE CENTRE. Krishnamoorthy KM, Sivasankaran S, Titus T, Bijulal S, Ajithkumar VK, Harikrishnan S, Namboodiri KKN, Anees T, Sanjay S, Tharakan JA. Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum kmkm@sctimst.ac.in Introduction: Device closure (DC) of secundum atrial septal defects (ASD) is generally not performed in small children. Objectives: To study the feasibility and results of DC of ASD in children <5 years. Methods: Children <5 years who underwent DC of ASD were studied. Morphology of ASD, haemodynamics and procedural details were noted. Results: Among 850 patients who had DC in the last 11 years, 116 were <5 years (13.6%). 78 were girls (67.2%). Mean age was 4.62+1.6 years (range 1.5-5). Mean weight was 12.3+5.4 kg (range 6-22). Multiple ASD was noted in 7 patients prior to and 3 others during DC. DC was successful in 107 (92.2%). Defect size was 13.7+4.8 mm (range 7-20). Mean pulmonary artery pressure was 22.1+7.9 mm Hg (range 12-46). Mean left to right shunt was 1.94+0.64 l/min (range 1.4-3.5). Mean device size was 14.7+5.2 mm (range 8-22). 12 (10.3%) patients had DC under sedation alone, without intubation and transoesophageal echocardiography. Associated patent ductus arteriosus was closed by coil in 2 and device in another. Elective surgery was required in 9 patients (9.4%) due to improper device position (n=4), inadequate rims (n=2) and multiple defects (n=3). There were no major complications. 82 patients (95.3%) had immediate closure. Abolition of residual shunt and right ventricular regression was seen on follow-up of 3.2+4.1 years (range 6 months-9 years). None developed atrioventricular block. Conclusion: Percutaneous DC of ASD is a safe and effective option in carefully selected children <5 years. Size of the child is not a contraindication. CAR/13(O) CLINICO-EPIDEMIOLOGICAL& FOLLOW- UP STUDY(FOR ONE YEAR) ON VENTICULAR SEPTAL DEFECT IN CHILDERN M.L Gupta, Brijmohan, Vijay kumar Associate Professor, Department Of Pediatric Medicine; SMS Medical College, Jaipur vijay.ks83@gmail.com Aims: 1.To Identify various types of VSDs based on site &size of defect 2.To find out the proportion of isolated VSD& VSD associated with other cardiac lesion 3.To study the mortality& morbidity of a case of VSD over a follow up of one year duration Material& Methods: 100 Childern were included in this study in whom diagnosis of VSD was made on 2D Echocardiography done at SPMCHI; JAIPUR during a period of 2007-09 Results: 1.62% of the case were having isolated VSD&38% were having associated cadiac lesion.TOF(28.95%) followed by ASD(21.05%) were commonly associated lesion. 2.Most common type of VSD was perimembranous(68%) followed by muscular(13%) then multiple(9.6%). 3.Perimembranous &muscular VSD were mostly of small size while inlet VSDs were of large size. 4.Mortality in case with isolated VSD was 14.52% but it increased to 26% associated lesion was also included. Conclusion: .Small VSD and muscular VSD are having good prognosis, while large VSD& inlet VSD are having bad prognosis until surgical correction is done. .Most deaths inVSD occurs before age of one year. So early intervention is recomonded for improving survival of children with VSD. CAR/14(P) FIRST EPISODE OF RHEUMATIC CARDITIS IN CHILDREN: ECHOCARDIOGRAPHIC MORPHOLOGY AND PULMONARY ARTERY PRESSURES. Prasanth K.S, Vijayakumar B, Zulfikar Ahamed M*, Geetha S, Rajamohanan K, Lalitha Kailas Dept. of Paediatrics, SAT Hospital, Govt. Medical College, Thiruvananthapuram. drprasanthks01@gmail.com We have studied the morphologic pattern of cardiac involvement and the pulmonary artery pressure changes in the first episode of rheumatic carditis in children by echocardiography.We have observed that mitral valve involvement was universal and pulmonary artery pressure was elevated significantly in children with acute rheumatic carditis. Objectives: To characterize the morphologic pattern of cardiac involvement in the primary episode of rheumatic carditis in children and to compare with normal children, the pulmonary artery (PA) pressure changes in the primary episode of rheumatic carditis by echocardiography. Settings: Tertiary care teaching hospital. Design: Hospital based Descriptive Study. Methods: All 16 consecutive cases of primary episode of acute rheumatic carditis in the paediatric age group admitted to the paediatric wards of S.A.T. Hospital, Thiruvananthapuram from 2009 June to 2010 May were included in the study. For comparing the pulmonary artery pressure, an equal number of age and gender matched normal children were also inducted into the study. All chidren had undergone Doppler Echocardiography study by the same Paediatric Cardiologist. All the cases underwent echocardiographic examination within 24 to 48 hours of establishment of the diagnosis of first episode of rheumatic carditis and before starting anti-inflammatory treatment.In addition to PA pressure, various other echocardiographic indices were studied. Data was compared using SPSS version 11.0 and t test was used for analyzing the significance. Results: In children with acute rheumatic carditis, mitral valve regurgitation was present in 100% while isolated aortic regurgitation was present in none. None had pericardial involvement.The pulmonary artery pressures were significantly elevated in all children with initial episode of rheumatic carditis (p < 0.001).62.5% had mild pulmonary artery hypertension (PA pressure 30 - 50 mm Hg). Conclusion: In children with initial episode of rheumatic carditis PA pressure is elevated, and the effect of this on long term outcome needs to studied. CAR/15(P) ABSENCE OF THE RIGHT COMMON CAROTID ARTERY AND RIGHT INTERNAL CAROTID ARTERY-A RARE VASCULAR ANOMALY IN ASSOCIATION WITH DYKE DAVIDOFF MASSON SYNDROME. Pushpalatha, C.N Reddy, Abdul Razak Department of Paediatrics: Bowring and Lady Courzon Hospital, Bangalore dr.razak_007@hotmail.com Cerebral hemiatrophy or Dyke-Davidoff-Masson syndrome (DDMS) is a rare clinical picture, characterized by unilateral cerebral atrophy and facial asymmetry, hemiparesis, seizures and mental retardation(1). Although absence of the common carotid artery (CCA) is a rare anomaly, the specific configuration in our case makes it extremely rare. In our case there is absent Right CCA and Right ICA with separate origin of Right ECA from Arch of Aorta, this makes our case extremely rare. We report a case of DDMS in relation with a rare vascular anomaly in an 18month old boy who presented with left sided focal seizures, hemiparesis of the same side. CAR/16(P) SINGLE SYNCOPE IN A YOUNG GIRL : TURNS OUT TO BE A RARE CARDIAC TUMOR Barnali Mitra, Prabhat Kumar Resident Pediatrics, Department of Pediatrics, AFMC, Pune drprabhat_cardio@yahoo.co.in Introduction : Syncope in children are not uncommon. Mostly syncopes occurring in school assembly area or play ground turn out to be vaso vagal syncope which do not require special investigations and therapy. Myxoma arising from left ventricle in a young child presenting as syncope is not reported in children. Case Report: A 12 year old girl presented with history of an episode of syncope while playing in school. The patient reported to busy Pediatric outdoor where general examination of the child did not reveal any abnormality except a grade II / VI systolic murmur in left parasternal area. Patient was referred to Pediatric cardiologist for further evaluation. Echocardiography revealed a round pedunculated mass( 18 X 30 mm ) in left ventricle arising from middle of interventricular septum with a pedicle measuring 4 mm at its base. Echo images were interesting to observe as the large tumor was projecting through aortic valve with contraction of left ventricle in each systole.and could have embolised any moment. With a provisional diagnosis of myxoma patient was taken up for emergency surgery and complete excision of tumor was done through transmitral approach. Histopathology of the tumor confirmed it to be a myxoma. Conclusion : Syncope in children should always be investigated for a possible etiology . Vaso vagal syncope should be an exclusion diagnosis. Myxoma arising from left ventricle in a young girl presenting as syncope is reported in this case report.