Abstracts of the Annual meeting of the PCSI, 2008

OBJECTIVE : To determine the incidence of discrepancies in the echo reports from peripheral centers compared to our center. METHODS : One thousand consecutive patients with new registrations who came to pediatric echo room were studied. All echoes were performed by Consultant Pediatric Cardiologists on a GE Vivid-7 machine. Outside echo reports were reviewed separately. Subsequently, our reports were co-related with the cardiac CT, catheterization and surgical findings when available. RESULTS : Outside echo reports were not available in 106 (10.6%) cases. Of the remaining 894 cases, significant discrepancies were observed in 127 (14.2%) cases. Mean age was 3.4 years with 67 (56.8%) cases being infants. Errors were noticed in relation to great vessels origin/malposition (n=22;17%), pulmonary venous return (n=22;17%), intra/extra-cardiac shunts (n=15;12%), semilunar valve disease (n=15;12%), coarctation of aorta (n=11;8.7%), coronary anomalies (n=6;4.7%), atrioventricular valve disease (n=6;4.7%) and presence of CHD in previously diagnosed rheumatic heart disease (n=3;2.4%). Aortopulmonary window (APW) was missed in 4 (3%) patients. Three patients referred as dilated cardiomyopathy turned out to have anomalous left coronary artery from pulmonary artery (n=2) and coarctation of aorta (n=1). Eighteen (14%) patients referred as ASD were found to have other diagnoses like APW,TAPVC etc. Ten (7.9%) patients with diagnosis of primary pulmonary arterial hypertension

(all), RBBB / polyphasic QRS complexes (17), and WPW syndrome (5). Echocardiography showed inferior displacement of septal leaflet (all), atrial shunt (24), ventricular shunt (6), RVOT obstruction (3), significant Tricuspid regurgitation (25), LTGA (2), and Situs inversus / dextrocardia (1). Management options: Group A (n=18) follow up, Group B (n=11) medications (antiarrhythmics 5, antifailure drugs 5), Group C (n=7) Electrophysiologic study and RF ablation and Group D (n= 7) surgery. All patients in group C showed accessory pathways (right posteroseptal 3, right free wall 1, right midseptal 1, left free wall 1, multiple 1). Inducible arrhythmia was found in 5. RF ablation was successful in 6. Indication for surgery (Tricuspid valve repair 2,tricuspid valve replacement 2, 11/2 ventricle repair 2, modified BT shunt 1) was severe TR with progressive cardiomegaly or severe hypoxia. There was no mortality. At a median follow up of 16 months all patients in Group A remained in functional class I/ II, 1 patient in Group B underwent RFA but continued to have SVT on antiarrhythmics. One patient in Group C was on antiarrhythmics drugs. Operated patients were in functional class I/II. CONCLUSIONS: Initially asymptomatic Ebstein's anomaly tends to stay so subsequently. Surgery/RF ablation in indicated cases could be performed with zero mortality and good functional outcome in the intermediate term.
Cardiopulmonary exercise testing after surgical repair of tetralogy of fallot -A preliminary report G Madhusudan, P Atul, L Varatharajan, Jayanthi L, P Sreeja, J Vimala, R Suresh Kumar. Department of Pediatric Cardiology, ICVD, Madras Medical Mission, Chennai. INTRODUCTION: Cardiopulmonary exercise testing (CPT) is a powerful tool for objective evaluation of the cardiovascular, respiratory and muscular systems under controlled metabolic stress. Impaired CPT parameters could be a predictor for adverse events on long term follow-up. OBJECTIVE: To assess cardiopulmonary exercise parameters after surgical repair of Tetralogy of Fallot (TOF) and to compare them with surgical Atrial Septal Defect (ASD) closure patients, and normal controls.

METHODS:
In an ongoing study, 10 patients > 10 years (Group1, range 10 -36 years, median 19.5 years) who had undergone Intra Cardiac Repair (ICR) for TOF at a median age 10 years, underwent CPT using upright bicycle ergometry with MedGraphics BreezeSuite PFX Ultima TM , till exhaustion. Their exercise parameters [percentage of maximum oxygen consumption (VO2max) of the predicted, oxygen consumption at anaerobic threshold (VO2AT)/VO2max, respiratory exchange ratio (RER), minute ventilation/carbon dioxide production (VE/VCO2) ratio and normalized maximal exercise performance (MEP) (Watt/kg)] were compared with a similar number of post operative ASD patients (Group 2), and normal controls (Group 3). RESULTS: Comparison of exercise parameters [ Figure 1]. CONCLUSION: The early results indicate uniformly impaired exercise parameters in surgically repaired TOF patients,even though the results were statistically significant only for VO2 max%. This impairment is probably disease specific and not an effect of sternotomy/ thoracotomy as post operative ASD patients behaved like controls. This hypothesis and its correlation with ECG and Echo abnormalities is being studied in a larger series for validation Extracorporeal membrane oxygenation as an evolving treatment modality for low cardiac output state in developing countries Singh Vishal, Priya Poonkodi, Sharma Rajesh, Varma Amit.

Department of Pediatric Cardiac Surgery and Intensive Care
Escort's Heart Institute and Research Centre, New Delhi.
BACKGROUND: Veno-arterial extracorporeal membrane oxygenation (ECMO) is a common modality of circulatory assist device used in children. We assessed the outcome of children who had rescue ECMO following repair of congenital cardiac defects (CCD) in our hospital . METHODS: From September 07 to September 08 , 13cases underwent veno arterial extracorporeal membrane oxygenation (ECMO) and all the cases had intra thoracic cannulation .Of these 8 had undergone arterial switch operation (ASO) for D-Transposition of great arteries (D-TGA) with severe left ventricular (LV) dysfunction, 5 cases had elective ECMO post cardiac surgery due to regressed left ventricle. 2 cases required emergency ECMO following cardiac arrest in the intensive care unit (ICU) and one case required ECMO for acute respiratory distress syndrome (ARDS) due to gram negative sepsis. Three cases required ECMO following prolonged cardiac arrest after intracardiac repair for Tetralogy of Fallot and Rastelli procedure for tetralogy of fallot and pulmonary atresia . Two cases required emergency ECMO following prolonged cardiac arrest after severe pulmonary artery hypertensive (PAH) crisis with severe right ventricular (RV) dysfunction. RESULTS: The mean age and weight were 184.45±181.69 days and 6.79±3.91 kg respectively. ECMO was successfully weaned off in 69.2 % ( 9/13) and the mean duration of ECMO was 97 hours. One case required premature weaning off ECMO due to excessive bleeding. Eight cases were successfully discharged and the mean duration of ICU stay was 22 days after weaning off ECMO. Four patients, three with RV dysfunction following TOF repair and TAPVC repair and one with ARDS due to gram negative sepsis could not be weaned off ECMO and expired. One patient had ventricular dysrhythmia on ECMO and three cases had choreoathetoid movements following ECMO weaning which responded very well to oral sodium valproate. CONCLUSION: ECMO has a promising role as a temporary bridge in patients who need support both for acute cardiac or respiratory dysfunction.

AIM:
To assess the effect of vasopressin on hemodynamics when used as an infusion for refractory hypotension and post cardiac arrest. MATERIAL AND METHODS: A prospective study conducted over a duration of 6 months from February 08 to august 08 in a tertiary pediatric cardiac critical care unit. Twenty patients (median age 4.5 months ) who underwent surgical correction for a varied spectrum of complex congenital heart disease and who required vasopressin infusion for more than 60 mins were included. Vasopressin infusion was started following cardiac arrest in 9 patients & in 11 cases was instituted for refractory hypotension resistant to routine catecholamine infusion. Vasopressin was administered as a bolus during cardiac arrest at 0.4 units /kg/dose, if the patient was unresponsive to multiple adrenaline boluses and the infusion was administered within the dose range 0.0001 to 0.003 units/kg/min.

RESULTS:
The mean arterial blood pressure (ABP) improved from 42±15.3 mmHg to 58±16.8mmHg after one hour and after 24 hours the mean ABP improved to 68±19.3 mmHg. There was no significant increase in the heart rate following vasopressin infusion. Also in 9 cases the inotropes were successfully tapered & inotrope score was decreased from 23.97 to 20.82 within 24 hours of AVP infusion. There was no significant alteration observed in urine output, serum sodium, and platelet count. The median duration of vasopressin infusion was 55.5 hrs (12hrs to 237 hrs). No significant adverse effects involving the digital and splanchnic circulation or renal or hepatic function were observed. Two cases expired while on vasopressin infusion, due to severe low cardiac output state and three cases due to drug resistant gram negative sepsis eventually. CONCLUSION: Vasopressin infusion does have a role in improving the hemodynamics in cases with advanced shock and cardiac arrest, especially in catecholamine resistant shock. There was no significant difference in the angiographic PDA size or hemodynamic parameters. The post procedure LVEF correlated best with basal LV dimensions which in turn correlated with the size of the PDA. A cut off of 2.5 for the z score of basal LVIDD predicted LV dysfunction with a sensitivity of 62% and specificity of 71%. CONCLUSION: Larger PDA results in adverse LV remodelling which may lead to LV systolic dysfunction after closure. The basal LV function parameters are the best predictors of post procedural LV dysfunction.

Cardiac functions in children with HIV infection
Vascular function in patients after coarctation repair and relevance of age at surgery

INTRODUCTION:
The bidirectional cavo pulmonary shunt, is usually the first stage procedure for hearts with single ventricle physiology. This report studied the short term outcome following the procedure performed in infancy as compared to that performed later in life.

MATERIALS AND METHODS:
Between January 2003 and September 2008, 75 patients underwent the bidirectional cavo pulmonary shunt at our unit. Of these 28 (37%) were performed in infancy (Group I); mean age 7.8 months. Group II comprised 47 patients. Group II patients were further divided into II (a)-one to five years, II (b)-five to ten years and II (c)-more than ten years. Mean SpO 2 was 71.6% on room air in Group I, while the same in Group II was 80.4%. 23 patients underwent the pulsatile bidirectional cavo pulmonary operation of which 8 (28%) belonged to Group I. Variables assessed were anthropometric data, superior caval pressures, duration of ventilation, ICU stay and hospital stay and morbidity. Patients were followed up at intervals of three, six and twelve months. RESULTS: Outcome variables in the two groups and the three sub groups were assessed and statistically compared. There was no hospital mortality. The study showed no significant differences in the immediate and short-term outcomes following the procedure in either group. Oxygen saturations were higher at three months (85.4%) and at one year (79.2%) in those who underwent the procedure in infancy as compared to the pre operative levels.

CONCLUSION:
The bidirectional cavo pulmonary shunt can be safely performed in infancy with significant improvement in the systemic oxygen saturation. This could translate into improved quality of life and growth without adversely affecting the immediate hospital course.
Early onset pulmonary hypertension in atrial septal defects-association with a left superior vena cava to coronary sinus Sripadh Upadhya, Amit Misri, Sunita Maheshwari. Department of Pediatric Cardiology, Narayana Hrudayalaya, Bangalore.
BACKGROUND: Early development of pulmonary hypertension (PAH) is unusual in atrial septal defects (ASD). Traditional teaching is that PAH develops in the 2 nd to 3 rd decade. Left superior vena cava (LSVC) is seen in 1% of general population and upto 10% of secundum ASDs. We report on a series of ASDs with early onset of PAH ie at less than 3 years of age with a high incidence of a LSVC to coronary sinus (CS). METHODS: Eighty four patients (pts) were operated for secundum ASD at an age less than 3 years, 16 of whom had evidence of pulmonary hypertension (PAH). Potential etiologies for PAH were identified based on an analysis of associations. RESULTS: Among these 16 pts < 3 years of age with ASD/PAH, 5 (31%) pts had LSVC which was draining in to a dilated CS into the right atrium, 2 (12%) pts had partial anomalous pulmonary venous drainage (PAPVC) with LSVC to CS, 3 (19%) pts had PAPVC, 1 (6%) pt had mitral regurgitation and in 5 (31%) cause of PAH was not known. Early onset of PAH is known in ASD and mitral regurgitation as well as in ASD and PAPVC. Thus out of 12 patients with ASD/PAH and no known cause of PAH, LSVS to CS was present in 5 (42%). Among the rest of the 68 pts <3 years with no PAH only one pt had a LSVC.
Thus, in our study group of ASD/PAH 31% of pts had LSVC to CS, where as only 1.4% of pts with ASD & no PAH had a LSVC.
CONCLUSION: In our study 31% of patients with ASD/early PAH had a LSVC to CS as opposed to the norm of a 1-10% incidence. One could hypothesize that the left SVC and dilated CS increases the L-R shunt across the ASD and thus increases the early development of PAH. In ASD's with early PAH one must look for a LSVC-if found it could be a contributor to the PAH and early closure should be considered.  (9), complex single ventricle (9). 6/18 patients had a right aortic arch. 14 were on PGE1 pre operatively (pre-op). The mean age at presentation was 9.6±8.9 days. The mean weight was 2.98±0.63 kg (range 2.1-3.7 kg). The midline approach was used in 17 patients. Polytetrafluoroethylene graft was used in all patients. mBTS was left sided in 5/18 (27.77%). 10/18 (55%) patients had 3.5 mm; 7/18 had 4 mm and 1 had 3mm shunt. The ductus was simultaneously ligated in 6/18 (33%). Four neonates underwent offpump pulmonary arterial plasty concurrently. The mean inotrope duration was 40±43hours. The mean ventilation duration was 24±0.98 hrs. Eight of 18 cases were extubated in the operative room. The mean intensive care unit stay was 3±0.97 days. The mean hospital stay was 6±1.14 days. The mean oximetry post op was 79.3%. Post op medications included sildenafil in 3, lasix in 2 and aspirin in all neonates. There was one reexploration for duct ligation for pulmonary overcirculation. Perioperative mortality was 2 (11.1%). Both babies had documented bacterial sepsis and expired of its complications. At discharge, duct was not seen in 10/16 surviving neonates. The mean duration of follow up was 6±4.66 months. There were 3 late mortalities. One had severe congenital supra-glottic stenosis, where as two had aspiration pnemonia. There were no shunt blocks. Two of 13 survivors have undergone corrective surgery to date. In our experience, neonatal mBTS with or without ductal ligation has an acceptable early outcome.
Minimally invasive surgery through right anterior small thoracotomy (RAST) for congenital heart defects TEE has been demonstrated to be a safe procedure with a significant major positive impact on patient outcome. Data on the safe use and efficacy of the pediatric TEE probe in infants weighing less than 5kg is limited. We present our experience. Between June 2007 to August 2008, there were 186 intracardiac CHD surgeries in our center out of which 68 were on infants less than 5kg. Of these TEE was planned on 46. The mean age of patients (pts) was 3.16±2.66months. Mean weight of pts was 3.76±0.93kg (2.28-5kg). 40/46 (87%) eventually underwent TEE. The TEE probe could not be inserted in 4 pts (10%) because of retrognathia. The TEE probe had to be removed prior to imaging in 2: in one case TEE probe was compressing the anomalous pulmonary venous common chamber. In the second case there were dampening of arterial trace after insertion of TEE probe in a patient with vascular ring. On 2 occasions, there was accidental extubation of the TEE probe. The mean arterial pressures increased by 5 mm of Hg after removal of TEE probe in 5 babies weighing less than 3 kg. The mean airway pressure rose by 3mm H2O in 12/40. There was no incidence of endotracheal tube dislodgement, perforation of esophagus or bleeding. TEE findings prompted a return to CPB in 4 of 40 (10%) pts. Return to CPB occurred in 1/11 pts with ventricular septal defect, 2/17 patients with transposition, 1/5 with total anomalous pulmonary venous drainage. All post-CPB diagnoses were confirmed during reoperation. In our experience, infants less than 5kg could safely undergo intra op TEE. The use of nasal intubation and proper case selection in infants less than 3kg contributed to the safety. The role of TEE in neonatal and infantile CHD surgery was shown to be undisputed.  (n=37) and mixed in 9% (n=37). Obstruction in pulmonary venous circuit was seen in 32% (n=131). Post-operative mortality within 4 weeks of surgery was 12% (n=49), highest being in infracardiac type (11 out of 37). The mortality was highest in younger patients, with 19% in less than 1 month old (8 out of 42). The cumulative mortality in less than one year old was 14% (46 out of 323), making younger age an important risk factor for post-operative mortality. Emergency surgery was performed in 18% (n=76) with 9% mortality (n=7). Preoperative ICU stay of >10 days, irrespective of cause, was an important risk factor associated with postoperative mortality. Other significant risk factors for post-operative mortality were aortic cross-clamp time >100 min, CPB time >30 min and pulmonary venous obstruction. CONCLUSIONS: Mortality after total anomalous pulmonary venous connection repair remains highest in young patients (likely due to more severe obstruction) and in those with infracardiac type, especially with pulmonary venous obstruction. Overall mortality of 12% as compared to western mortality statistics of 5-8% may be related to relatively delayed diagnosis and possible late referrals and higher duration of preoperative ICU stay. Delineation of Pulmonary artery anatomy plays a very important role in surgical management of complex congenital heart defects. If echocardiography is unable to visualize branch pulmonary arteries, they can be visualized by either conventional angiography or noninvasive CT angiography. In this study we compared these three modalities of imaging in defining the branch pulmonary artery size. Twenty eight patients (age 3months -34 years, weight 3.1kg -75kg, body surface area 0.2-1.9m2) with various heart defects who had undergone echocardiogram, angiogram and CT angiogram were included in this study. Branch pulmonary artery size was measured in all three modes of imaging. Echocardiography could not show branch pulmonary arteries in nine patients out of whom CT angiography could visualize branch pulmonary arteries in six. Conventional angiography could show branch pulmonary arteries in one more out of the remaining three patients. Statistical analysis by Fischer's test was done to compare the three modes of imaging .Fischer's test applied for comparison of conventional angiography and CT angiography revealed no statistically significant difference in their ability to visualize branch pulmonary arteries (p>0.05) . We found positive correlation between these three techniques for measuring left pulmonary artery but the right pulmonary artery measurements correlated between angiography and CT angiographic measurements. CT angiography is as good as conventional angiography in visualizing and assessing size of branch pulmonary arteries and has an advantage of being noninvasive. CT angiography can be considered as a good alternative to conventional angiography if echocardiography proves inadequate.
Utility of tele-echocardiography in pediatric cardiology as an effective diagnostic tool: A pilot project Ritesh Sukharamwala, Suresh PV, Bidari LH 1 , Sunita Maheshwari.

Department of Pediatric Cardiology Narayana Hrudayalaya
Institute of Cardiac Sciences, Bangalore and 1 Ashwini Hospital, Bijapur, Karnataka.

INTRODUCTION:
Echocardiography diagnosis of congential heart disease (CHD) in India is limited by lack of trained echocardiographers with experience in the diagnosis of CHD. Tele-echocardiography has the potential to bring real-time diagnoses to pediatric facilities without in-house pediatric cardiologists. We analysed our tele-echo data to see if it made a significant difference to accurate diagnosis of CHD. METHODS: At our institution tele-echocardiography is done in two ways 1) live demonstration of echo via a video camera where the cardiologist can report real time echo loops and images or 2) prerecorded movie clips stored on a central server and retrieved by the cardiologist at a later time. From May 2006-July 2008, 218 echoes were reviewed via tele-echocardiography at 3 places, namely Bijapur, Nasik and Malaysia. We prospectively evaluated the utility of teleechocardiography in 47 children evaluated in the last 4 months. RESULTS: Among the 47 patients cardiac diagnoses included VSD (n-7), ASD (n=7), PDA (n=3), TOF (n=3), TGA (n=2), TAPVC (n=1), complex CHD (n=2), myocarditis (n=1), cardiomyopathy (n=1) and normal cardiac study (n=5). After Tele-echocardiography 17 children required urgent referral for surgery, 5 children required change in treatment and 25 children did not require any changes in treatment.
Out of 17 children referred, 8 were underwent successful surgery.
Of the 29 children, who did not require urgent referral were asked to follow-up. Overall, the accuracy of tele-echo exceeded 95% in the study. CONCLUSION: Real-time transmission of neonatal and pediatric echocardiograms has the potential to improve patient care, aid sonographer education, prevents unnecessary transports and has a positive impact on early referral of CHDs for surgery. BACKGROUND: Tissue Doppler and Strain rate imaging are relatively load independent. We studied the usefulness of Tissue Doppler and Strain rate imaging in detecting temporal changes in regional and global function of left and right ventricle in transposition of great arteries (TGA). METHODS: Standard echocardiography including Doppler studies was performed with 5 or 10 MHz probe (GE Vivid 7 dimension Machine). The images were analyzed offline by a customized software package (Echo Pac PC,GE Vivid Ultrasound). Intra and interobserver variability was assessed to be within standard limits. The correlation with surgical outcome will be presented.

RESULTS:
A total of 35 patients (aged 3-123 days) were included in this study. Twelve LV and 2 segments of RV in each patients were analyzed for peak systolic velocity, systolic strain rate and strain. There were no significant intergroup differences in of any of these parameters in patients with or without VSD, or in the groups with regressed or prepared LV. Paradoxically, peak systolic velocity and peak strain rates were statistically significantly higher in patients older than 28 days. CONCLUSION: Conventionally classified as regressed LV do not show reduced tissue velocities and strain rates. Peak systolic velocity and peak strain are higher in patients more than 28 days. These findings suggest that possibly intrinsic contractility of the LV myocardium is preserved in a majority of patients with TGA. BACKGROUND: As advances in the medical and surgical management improves survival in patients with congenital heart disease, increasing knowledge about neurodevelopmental and psychosocial outcomes and the factors that affect them will provide strategies to optimize long-term outcome in this high-risk population. OBJECTIVE: To evaluate the neurodevelopmental and psychosocial outcome in children with various types of congenital heart defects (both cyanotic & acyanotic) who underwent corrective surgical procedures from birth till 15 years of age. This investigation is part of an institutional effort to examine the neurodevelopment of children who underwent corrective surgical procedures for congenital heart defects.

PATIENTS AND METHODS:
We performed a battery of neuropsychological tests on a sample of 70 children with congenital heart defects (35 patients each in cyanotic group and acyanotic group) between 2006 and 2008 at 6 months and 1 year following surgical correction.

RESULTS:
The overall psychosocial and Neurodevelopmental outcome was found to be satisfactory in both the groups when assessed by standard psychosocial instruments for respective age. There was no significant difference in the overall outcome between the cyanotic and acyanotic group. The subgroup of patients who performed below average had their surgical corrective procedure at more than two years of age and psychological counseling was offered to all the patients. CONCLUSION: Further regular long term follow up is required to identify the specific psychosocial problems if any in these patients. More research regarding age at surgical repair and the psychosocial outcome is needed. There is an important need for further studies of quality of life in patients with different types of congenital heart diseases. , length of stay (late -11.5 days versus early -12 days) and inotrope score were similar in both groups respectively. There were no differences in the incidence of renal failure, acute lung injury and sepsis in the two groups. CONCLUSION: Preliminary observations suggest that primary arterial switch in infants ≥ 3 weeks of age using simple, inexpensive ICU strategies is feasible. These observations need to be validated in larger numbers of older infants to decide "how old is safe?".
Clinical screening for congenital heart disease immediately after birth: A prospective study BACKGROUND: A large proportion of neonates born with congenital heart disease (CHD) are missed at birth in Indian hospitals. OBJECTIVE: To develop a clinical strategy for detection of congenital heart disease (CHD) in the newborn through a combination of clinical signs and pulse oxymetry that best predicts the presence of CHD. METHODS: All consecutive newborns born in a secondary level hospital between June 2006 and June 2008 were prospectively screened for CHD 48 hours after birth. The on-site pediatrician performed clinical screening for CHD using a pre-designed format of 10 clinical signs. Pulse oxymeter saturations (right upper and lower extremity) were recorded by a nurse. Echocardiography was performed on site in all newborns. A 6-week clinical follow-up evaluation was also performed.

RESULTS AND ANALYSIS:
Of the 4190 babies screened, 325 had CHD (overall prevalence: 7.7%). Nine (0.21%) had major CHD (3 cyanotic & 6 acyanotic), two of whom (one ALCAPA and one large VSD) were missed during the initial evaluation and were detected on followup; four of these had a normal clinical evaluation and only one had low resting oxygen saturation. Univariate analysis of predictors of CHD included murmur, central cyanosis, precordial pulsation, and respiratory rate >60/minute. On multivariate analysis, murmur (OR=5.8; 95% C.I 3.44 -9.76) and respiratory rate >60/min (OR=2.54; 95% C.I 1.0 -6.47) were associated with presence of CHD. The overall low sensitivity of the clinical signs (7.2% for murmur) perhaps was due to small number of patients with significant CHD. Pulse oximetry was not found to be a significant predictor of CHD possibly due to technical and human factors. CONCLUSIONS: Clinical evaluation immediately after birth has a very low sensitivity for predicting newborns with CHD. Presence of murmur in a newborn warrants a referral for echocardiography. A 6-week repeat clinical evaluation is recommended to ensure that major CHD does not go undetected. BACKGROUND: There is little data on prevalence of 22q11 microdeletion syndromes among children with conotruncal malformations in Indians. Presence of this deletion has significant impact on the perioperative management and long-term outcome.

OBJECTIVES:
To determine prevalence and identify the best phenotypic correlates of chromosome 22q11 microdeletion among patients with conotruncal malformations.

METHODS:
This was a prospective, hospital-based, observational study conducted at a tertiary care center on consecutive children (≤ 2 years) with conotruncal cardiovascular anomalies that were identified and classified by pediatric cardiologist using echocardiography. A pediatric geneticist independently evaluated patients for dysmorphic features and phenotype. Karyotyping, and Fluorescence In Situ Hybridization (FISH) using the critical region probe for 22q11, was then performed. BACKGROUND: It is frequently challenging to determine operability in patients with large atrial septal defects (ASD) and pulmonary arterial hypertension (PAH) using conventional means. OBJECTIVES: To examine the utility of decline in arterial partial pressure of oxygen (PaO 2 ) after exercise as a marker of pulmonary vascular obstructive disease (PVOD) in patients with ASD with PAH. METHODS: A symptom limited treadmill exercise was performed in three groups of patients: Healthy volunteers (n = 6), large ASD with no PAH (n = 5) and 14 patients with ASD and PAH (oxygen saturation ≥ 90%, predicted PA systolic pressure of ≥ 50 mm Hg by Doppler echocardiography in absence of additional lesions or lung disease). Patients with advanced PVOD (sats < 90%) were excluded. A radial artery cannula was inserted before exercise and arterial blood gas samples were drawn before and as soon as the test was terminated (peak exercise). A decline in PaO2 ≥ 10 mmHg after exercise was considered significant. The patients with ASD and PAH underwent cardiac catheterization and detailed hemodynamic data sets were obtained on room air, oxygen and, a mixture of oxygen and nitric oxide (30-40 ppm). RESULTS: None of the controls (healthy or ASD and no PAH) had a significant fall in PO 2 . The data obtained from patients with ASD and PAH (n = 14, age: 33.6±8.6 yrs) is shown in the table 1. CONCLUSIONS: A decline in PaO 2 following exercise appears to predict a high PVRI (basal, post O 2 and post NO) in patients with ASD and PAH. This test appears promising for assessment of operability in borderline situations.

RESULTS
Pulse oximetry as a screening tool for detecting congenital heart defects in the british midlands -An interim analysis Abhay Bhoyar 1,2 , John G C Wright 2 , Andrew Ewer 1,3 .

OBJECTIVE:
To determine the accuracy of Pulse oximetry (PO) for detecting critical and clinically significant CHD in newborn.

STUDY DESIGN:
This population based prospective multicenter delayed cross sectional study was conducted in 6 the hospitals in the British Midlands. Asymptomatic newborns ≥35 weeks of gestation had arterial oxygen saturations measured within 24 hours of birth. A cut off of <95% in either limb or a difference of ≥3% between the limb readings was considered as abnormal. If PO was low and the clinical examination was unremarkable, the PO was repeated 1-2 hours later for a definitive definition of abnormality. Echocardiogram and interrogation of various databases were used as gold standards to confirm CHD. RESULTS: Of the 17500 deliveries in the 6 hospitals from February 2008 to September 2008, 13733 (78.5%) babies have been recruited and screened using PO. The mean birth weight was 3.31 Kg and the male to female ratio was 1.04. The number of parents declining the consent for the study was relatively higher amongst the Asian and the Black populations, 20% and 25% respectively Vs White Retrospective study of all patients who underwent surgery for CHD over a 7-year period (June 2001 to July 2008).

MATERIAL AND METHODS:
Out of 3624 patients who underwent

OBJECTIVES & METHODS:
Amplatzer vascular plug (AVP) is an underutilized item in the armamentarium of interventional cardiologists, though it is less thrombogenic than the duct occluder. We describe 9 cases of unusual interventions using the AVP.

RESULTS:
The patient age ranged from 2 -20 years. The diagnosis included coronary aterio-venous fistula (3 cases), baffle leak following Fontan operation (1 case), Coil embolization of AP collaterals (2 plugs), LPA aneurysm (1 case) and post operative BT shunt (Through arterial approach 1 case and through Glenn circuit 1 case). In 3 of the cases, delivery of duct/septal occluder failed due to inability to adequately advance the delivery sheath or the occluder. 4mm -12 mm AVP were used. In all the cases, the delivery of AVP was relatively unproblematic as it could be advanced over a 5F or 6 F right coronary guiding catheter. The procedure was successful in all the cases. Complete occlusion of shunt could be achieved with AVP alone, despite concerns over less thrombogenicity. CONCLUSION: In selected cases, Amplatzer vascular plug offers an alternative innovative solution for the interventionists. The better profile and manoeuvrability was useful in all the cases.
Feasibility and midterm outcome of transcatheter closure of small subaortic VSD with aortic cusp prolapse This study looked at the feasibility and medium term outcome of transcatheter closure of small subaortic ventricular septal defect with aortic cusp prolapse +/-aortic regurgitation (AR) as an alternative to surgical closure. METHODS: Between July 2004 to February 2006, prospectively collected data of 65 patients who underwent TEE and cardiac catheterization were analysed. 52 had perimembraneous and 13 had doubly-committed subarterial type VSD. Patients with weight more than 10 kg, haemodynamically insignificant shunt and presence of aortic valve prolapse +/-trivial or mild aortic regurgitation were initially included. Standard technique was performed to close the VSD using AMVOD. The patients were followed up for a minimum of 2 years. RESULTS: The mean age was 249 months (range 14-300). All of them had cusp prolapse with the degree of prolapse trivial in 50 (77%), mild in 10 (15%), moderate in 4 (6%) and severe in 1 (2%). 11 were excluded on TEE. 43 out of 50 (86%) had successful closure. 7 (14%) developed severe aortic regurgitation necessitating device retrieval. 4 cases were abandoned due to procedural difficulties. 36 (84%) patients showed no worsening of AR. Only 7 (16%) patients had worsening of AR, 3 from nil to trivial, 3 from trivial to mild and one progressed to severe AR requiring aortic valve replacement. 6 (P=0.015).Multivariate analysis revealed that likelihood of failure was higher in patients with suboptimal margins (OR=35.93; P=0.000), device larger than 20mm in size (OR=6.36; P=0.005) and procedures done during early phase (OR=6.80; P=0.001). There was no significant association between failure and age of patient or device type (ASO or BSO). Ten patients had minor complications and four patients had major complications including two device embolization and one death due to cardiac perforation. CONCLUSION: Efficacy of Transcatheter closure of ASD in a newly established unit has improved over the period of time and complications are comparable to published data in other centres.
Stenting of native coarctation of aorta in children using adult iliac stents AN Patnaik, D Seshagirirao. Department of Cardiology Nizam's Institute of Medical Sciences, Hyderabad.
Balloon angioplasty followed by stent is now an accepted strategy for the treatment of native coarctation of aorta in older children and adolescents world over. For the last 5 years we used self expanding nitinol stents (primarily recommended for iliac angioplasty in adults) in this condition. There were 32 patients (24 males; mean age 10.2± 1.43 years; youngest 6 year old). Successful procedure is defined as reduction of gradient to less than 20 mmHg or increase in the ratio of the diameter of the coarctation area to the diameter of the descending aorta to at least 0.8. In 32 patients 32 stents were used with overall success rate of 31/32 (96.8 %). The peak systolic pressure gradient (mean (SD)) decreased from 48.8 (23.5) to 2.2 (1.86) mm Hg (p<0.05). The diameter of the stenotic lesion increased from 5.6 (1.6) mm to 12.5 (2.6) (p <0.05). In one patient 2 stents were used because the first stent migrated downwards leaving the lesion partially uncovered. In the only unsuccessful case the lesion was 12 mm long, tubular and did not yield even at 16atm pressure. This case was sent for elective surgery at a later date. There were no deaths or cerebrovascular events. Two cases had femoral artery access related problems (hematoma-1, loss of pulse-1). Twenty five of the 31 successful cases were on regular clinical and echocardiographic follow-up. On mean follow-up of 1.25 years one had recoartation and successful balloon dilation was done. Another case with recoartation and persistent hypertension underwent elective surgical repair. There were no aortic aneurysms in any case. Stent implantation using this readily available low cost adult peripheral stent gave gratifying acute and early term results in treatment of coarctation of aorta in older children and adolescents.
A study of percutaneous closure of ventricular septal defect by duct occluder BACKGROUND: Majority of patients with muscular VSDs amenable to device closure can be closed with conventional muscular VSD occluder (MVSDO). However in certain cases either the defect location and shapes or the device design dose not appear very appropriate particularly which are located more apically and having narrow and long right ventricular (RV) exit. The Block-aid Duct Occluder (BDO) can be a good and suitable device in such cases. OBJECTIVE: Retrospective analysis of safety and efficacy of Blockaid duct occluder for the treatment of apical and mid muscular conical VSD PATIENT AND DESIGN: Six patients aged 4 to 9 years with height of 115.8 + 10.76 cm (range 99-130 cm) and weight of 16.83 + 3.76 kg (range 14-25 kg) with apical or mid muscular VSDs underwent transcatheter closure using BDO. The device consists of nitinol wire mesh that is shaped in to a cylindrical plug with a collar and with a polyester fabric inserted in it. Selection of delivery system and duct occluder size was according to LV Angiographic delineation of defects. RESULTS: Transthoracic echocardiography showed VSD size of 4.71 + 1.56 (LV), 3.27 + 0.90 (RV). The RVSP was 41.66 + 9.46. The angiographic VSD diameter was narrowest 3.19 + 0.85 and largest 5.04 + 2.56. A 6 to 9 French sheath was used to deliver the device. BDO of sizes 6x4, 8x6, and 10x8 were used according to the VSD sizes. Successful device delivery and complete closure occurred in all patients. Fluoroscopy time was 14.13 + 8.36 min (range 8.54-32.12).
No complications occurred. CONCLUSIONS: BDO is an important adjunct for closure of muscular VSDs and further studies are required to document its efficacy, safety, and long term results in a larger number of patients.
BACK GROUND: From relatively simple procedure like patent ductus arteriosus (PDA) closure to comparatively difficult ones like ventricular septal defect (VSD) closure, pediatric cardiac interventions have come a long way. With ever evolving technology plus increasing experience, two transcatheter interventions in the same patient (twin procedures) can be now performed with good results. We report our experience of twin procedures from 2005-2006. METHODS: All patients whose age was less than 18 years and had underwent more than one cath intervention for two different diagnosis at the same sitting were considered. Patients who had undergone two procedures either for two different disease or same disease but at different sittings were excluded. RESULTS: Nine patients whose age ranged from 6 months to 18 years with mean of 6.5 years underwent twin procedures. Out of these 5 (55%) had PDA and atrial septal defect (ASD) closure, 2 (22%) had ASD closure and balloon pulmonary valvuloplasty (BPV), 1 (11%) patient had BPV and balloon aortic valvuloplasty (BAV) and 1 (11%) patient had BAV and balloon coarctoplasty. All the procedures were successful with no immediate or short term complications. CONCLUSION: Twin procedures, whenever possible, are an effective alternative to surgery. Short post procedural ICU care and ability to do them off bypass are advantages. However cost implications and the amount of metal inserted into body may be issues for discussion.
Interventional therapy of pulmonary arteriovenous malformation using PDA or ASD occluder MATERIALS AND METHODS: The patients included 4 females and one Male with age range of 7-21years. Angiography showed diffuse capillary pulmonary arterio-venous malformation, multiple saccular and single saccular in 2,2,and 1 cases respectively. Pulmonary arteryvenous malformation was occluded by transcatheter technique in all patients using PDA or ASD occluder. RESULTS: Technical success was achieved in all patients. Hypoxemia symptoms were relieved. The arterial mean oxygen saturation was increased from 75.2% to 92.7%. CONCLUSION: Interventional therapy by using the PDA or ASD occluder is an effective treatment for pulmonary-arteriovenous malformation which have the huge saccular and the large feeding artery.
Transcatheter occlusion of ruptured sinus of valsalva aneurysm with amplatzer duct occluder BACKGROUND: Ruptured sinus of Valsalva aneurysm (RSVA) can be associated with ventricular septal defects or isolated lesions. Percutaneous transcatheter closure of RSVA has been an alternative strategy to surgery. METHODS: From January 2000 to May 2006, 10 patients (4 males, 6 females) aged from 7 years to 69 years (mean ages 37+/-18.8 years) were involved in the present report. The diagnosis of RSVA was made based on a combination of several imaging modalities. Of them, 9 patients were identified as congenital cause and one did as acquired RSVA. Two-dimensional and color Doppler echocardiography revealed the rupture of right coronary sinus into right ventricle in 5 cases and into right atrium in 3 cases, while non-coronary sinus ruptured into right atrium in 2 cases. Aortogram showed that the estimated size of the defect was 6.2+/-2.3 mm (2-10 mm). After the establishment of the arterio-venous wire loop, Amplatzer duct occluder (ADO) was deployed by antegrade venous approach in all patients. RESULTS: ADO with 1-3mm larger than the defect was used. All defects were successfully occluded without any complications. On the follow-up, echocardiography showed neither residual shunt nor aortic regurgitation, and there was also no device embolization, infective endocarditis in any of the patients. CONCLUSIONS: Transcatheter closure is a feasible and effective alternative for both congenital and acquired RSVA. However, longterm follow-up is mandatory.