Congenital long QT syndrome (LQTS) is one of the most common cardiac channelopathies and is characterized by prolonged ventricular repolarization and life-threatening arrhythmias. The mortality is high among untreated patients. The identification of several LQTS genes has had a major impact on the management strategy for both patients and family members. An impressive genotype-phenotype correlation has been noted and genotype identification has enabled genotype specific therapies. Beta blockers continue to be the primary treatment for prevention of life threatening arrhythmias in the majority of patients. Other therapeutic options include pacemakers, implantable cardioverter defibrillators, left cardiac sympathetic denervation, sodium channel blocking medications and lifestyle modification.

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Temporary epicardial pacing is commonly used for the diagnosis and treatment of arrhythmias in the acute post operative period after surgery for congenital heart disease. Temporary epicardial pacemakers have become increasingly sophisticated over the years and have evolved from simple single chamber devices with few programmable parameters to complex dual chamber devices capable of adjustable parameters similar to permanent pacemakers. This review will describe the various indications for temporary pacing, technical considerations for both the choice of pacing wires and pacemaker modes, complications with temporary pacing and our current practice with temporary pacing.

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Three dimensional echocardiography (3DE) is a new, rapidly evolving modality for cardiac imaging. Important technological advances have heralded an era where practical 3DE scanning is becoming a mainstream modality. We review the modes of 3DE that can be used. The literature has been reviewed for articles that examine the applicability of 3DE to congenital heart defects to visualize anatomy in a spectrum of defects ranging from atrioventricular septal defects to mitral valve abnormalities and Ebstein's anomaly. The use of 3DE color flow to obtain echocardiographic angiograms is illustrated. The state of the science in quantitating right and left ventricular volumetrics is reviewed. Examples of novel applications including 3DE transesophageal echocardiography and image-guided interventions are provided. We also list the limitations of the technique, and discuss potential future developments in the field.

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Objectives: To describe the case selection, technique and immediate and short-term results of balloon pulmonary valvotomy (BPV) in young infants with tetralogy of Fallot (TOF). Background : Symptomatic young infants with TOF can either undergo corrective surgery or Blalock-Taussig (BT) shunt. Corrective surgery in early infancy is associated with significant morbidity and is not a realistic option in many centers. BT shunt carries the risk of branch pulmonary artery distortion and shunt occlusion. Methods : Infants less than three months with a significant valvar pulmonary stenosis (with or without associated infundibular and annular component) and oxygen saturation ≤ 80% were offered BPV. The right ventricular outflow tract (RVOT) was crossed with 4F Judkin's right coronary catheter and the valve was crossed with 0.014" coronary guide wire. Serial balloon dilatations were done with over the wire coronary balloons (3-4 mm) and Mini Tyshak balloons up to a balloon annulus ratio of 2:1, depending upon the improvement in saturation and formation of annular waist. Results : Seventeen infants less than three months of age with tetralogy of Fallot (median age: 33 days, range: 10-90 days, weight: 3.47 ± 0.87 kg, pulmonary annulus Z score: -5.59 ± 1.04) including eight neonates underwent palliative BPV between May 2004 and March 2007. The mean balloon annulus ratio was 1.4 ± 0.28 and fluoroscopy time was 26.18 ± 20.2 minutes. The mean oxygen saturation increased significantly from 73 ± 7% to 90 ± 3.68% following BPV (p = 0.0001). The only major complication was RVOT perforation and pericardial tamponade in one infant. The mean follow-up period was 23 ± 12 months. Two babies developed significant desaturation requiring surgery in the six months following BPV. There was a significant increase in pulmonary annulus. The z score for the pulmonary annulus improved from -5.59 ±1.04 before BPV to - 4.31 ± 1.9 at the time of last follow-up (p = 0.018). The mean Z score of hilar right pulmonary artery (RPA) increased significantly from -1.19 ± 1.78 before BPV to 0.7 ± 0.91 after BPV (p = 0.001). The mean Z score of hilar left pulmonary artery (LPA) increased significantly from -1.28 ± 1.41 to 0.03 ± 1.29 after BPV (p = 0.005). Eight patients underwent corrective surgery. Conclusions : Balloon pulmonary valvotomy is safe and effective. It significantly improves the growth of pulmonary annulus and branch pulmonary arteries. Thus it can be considered as an interim palliative procedure for symptomatic young infants with TOF and predominant valvar pulmonary stenosis.

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Intravascular or intracardiac stenoses occur in many forms of congenital heart disease (CHD). Therefore, the implantation of stents has become an accepted interventional procedure for stenotic lesions in pediatric cardiology. Furthermore, stents are know to be used to exclude vessel aneurysm or to ensure patency of existing or newly created intracardiac communications. With the further refinement of the first generation of devices, a variety of "modern" stents with different design characteristics have evolved. Despite the tremendous technical improvement over the last 20 years, the "ideal stent" has not yet been developed. Therefore, the pediatric interventionalist has to decide which stent is suitable for each lesion. On this basis, currently available stents are discussed in regard to their advantages and disadvantages for common application in CHD. New concepts and designs developed to overcome some of the existing problems, like the failure of adaptation to somatic growth, are presented. Thus, in the future, biodegradable or growth stents might replace the currently used generation of stents. This might truly lead to widening indications for the use of stents in the treatment of CHD.

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Tetralogy of Fallot (TOF) is an important lesion for all pediatric and congenital heart surgeons. In designing the most appropriate operation for children with TOF, the postoperative physiology should be taken into account, both in the short and long term. The balance between pulmonary stenosis (PS) and pulmonary insufficiency (PI) may be critical for preservation of ventricular function. A unified repair strategy that limits both residual PS and PI is presented, along with supporting experimental evidence, a strategy for dealing with coronary anomalies, and comments regarding best timing of operation.

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We present a 5 month old baby who was referred for an incidental detection of a murmur and was found to have tortuous pulmonary arteries with multiple peripheral pulmonary stenoses and bilateral inguinal hernia pointing towards the diagnosis of arterial tortuosity syndrome.

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The Fontan circulation results from routing of the systemic venous blood to the pulmonary circulation without a hydraulic source of a ventricle. Although a hypertrophied right atrium was thought to be essential for this circulation, the current form of the operation has neither the right atrium nor any valves in the venous circulation that is connected to the pulmonary arteries directly. Modifications in the operative model was one of the early steps in improving outcome. Use of fenestration, staging of Fontan completion and better perioperative management have led to a significant drop in mortality rates in the current era. Despite this, there is late attrition of patients with complications such as arrhythmias, ventricular dysfunction, and unusual clinical syndromes of protein-losing enteropathy (PLE) and plastic bronchitis. Management of failing Fontan includes a detailed hemodynamic and imaging assessment to treat any correctable lesions such as obstruction within the Fontan circuit, early control of arrhythmia and maintenance of sinus rhythm, symptomatic treatment for PLE and plastic bronchitis, manipulation of systemic and pulmonary vascular resistance, and Fontan conversion of less favorable atriopulmonary connection to extra-cardiac total cavopulmonary connection with arrythmia surgery. Cardiac transplantation remains the only successful definitive palliation in the failing Fontan patients.

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Ductal stenting is an attractive alternative to conventional shunt surgery in duct dependent congenital heart disease as it avoids thoracotomy and its related problems. With today's generation of coronary stents which have better profile, flexibility and trackability, ductal stenting may be achieved safely and with considerably less difficulty than previously described. As in Blalock-Taussig (BT) shunt, ductal stenting is indicated mainly in duct-dependent cyanotic lesions chiefly in the neonatal period. Unlike the Patent ductus arteriosus (PDA) as an isolated lesion, the ductus in cyanotic heart disease has a remarkable morphologic variability. The ductus tends to arise more proximally under the aortic arch, giving rise to a vertical ductus or occasionally it may arise from the subclavian artery. It also tends to be long and sometimes very tortuous, rendering stent implantation technically impossible. The ductus in these patients may also insert onto one of the branch pulmonary arteries with some stenosis at the site of insertion. The ductus in Tetralogy of Fallot with pulmonary atresia (TOF-PA) tend to exhibit these morphologic features and to a lesser degree in transposition of great arteries with ventricular septal defect and pulmonary atresia (TGA-VSD-PA) and the more complex forms of univentricular hearts. In the preliminary angiographic evaluation, it is important to delineate these morphologic features as the basis for case selection. Ductal stenting may be done by the retrograde femoral artery route or the antegrade transvenous route depending on the ductus morphology and the underlying cardiac lesion. The detailed techniques and essential hardware are described. Finally, major potential complications of the procedure are described. Acute stent thrombosis is the most serious and potentially catastrophic. Emergent treatment with thrombolytic therapy and mechanical disruption of thrombus are required. With proper case selection, appropriate technique and the right hardware ductal stenting provides reasonable short-medium term palliation in duct-dependent cyanotic heart disease.

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The prenatal ultrasonographic diagnosis of ectopia cordis associated with a complex intra-cardiac defect (common atrium, common atrioventricular valve with single ventricle) is illustrated in a 32-week gestation fetus. The fetus showed associated features of amniotic band disruption sequence. The cardiac autopsy findings correlated with the antenatal diagnosis. The association of ectopia cordis with amniotic band disruption is rare and infrequently reported in literature.

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Prenatal diagnosis of congenital heart disease is now well established for a wide range of cardiac anomalies. Diagnosis of congenital heart disease during fetal life not only identifies the cardiac lesion but may also lead to detection of associated abnormalities. This information allows a detailed discussion of the prognosis with parents. For continuing pregnancies, appropriate preparation can be made to optimize the postnatal outcome. Reduced morbidity and mortality, following antenatal diagnosis, has been reported for coarctation of the aorta, hypoplastic left heart syndrome, and transposition of the great arteries. With regard to screening policy, most affected fetuses are in the "low risk" population, emphasizing the importance of appropriate training for those who undertake such obstetric anomaly scans. As a minimum, the four chamber view of the fetal heart should be incorporated into midtrimester anomaly scans, and where feasible, views of the outflow tracts should also be included, to increase the diagnostic yield. Newer screening techniques, such as measurement of nuchal translucency, may contribute to identification of fetuses at high risk for congenital heart disease and prompt referral for detailed cardiac assessment.

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Objectives :To investigate the effectiveness and safety of prophylactic surgical ligation of patent ductus arteriosus (PDA) on mortality and morbidity of preterm infants weighing less than 1000 g at birth. Materials :The study conducted a systematic search of available database from 1996-2008. Retrieved articles were assessed for eligibility and data was abstracted independently by two reviewers. Decisions to include studies for review and the methodological quality of included studies were asssessed in duplicate based on predetermined criteria. No language restrictions were applied. Results :Only one eligible study that enrolled 84 extremely low birth weight infants was identified. Prophylactic surgical ligation of PDA resulted in a statistically significant reduction of severe stage II or III necrotizing enterocolitis, [RR 0.25, 95% CI (0.08, 0.83), P value 0.02, number needed to treat 5]. The study, however, found no statistically significant difference in mortality, intraventricular hemorrhage, bronchopulmonary dysplasia, and retinopathy of prematurity. Conclusions :Current evidence does not support the use of prophylactic surgical ligation of PDA in the management of the preterm infants.

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A prospective survey of parents of the children with congenital heart disesease was conducted to determine their awareness as regards the importance of oral hygiene and prophylaxis against infective endocarditis (IE). The results of this study demonstrated that only 8% of the parents were aware of the importance of good oro-dental hygiene and need for IE prophylaxis.

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Background and Objectives : Initial experience with transcatheter closure of patent ductus arteriosus (PDA) using detachable coils and Amplatzer duct occluder devices is reported. We evaluated the outcome, complications, and influence of the learning curve, and also assessed the need of surgical backup for such interventional procedures. Methods: From January 2000 to December 2004, 121 patients underwent transcatheter closure of PDA. Aortic angiogram was performed to evaluate the size, position, and shape of the duct for appropriately choosing the occluder device type and size. A second aortic angiogram was performed 10 minutes after device deployment. Echocardiography was repeated at intervals of 24 hours, then at 1, 3, and 6 months after the procedure to assess complications. Stepwise multiple regression analysis was used to assess the role of experience in improving the outcome of the procedure. Results: Of 121 cases, four patients had pulmonary artery embolization of the occluder device which was successfully retrieved in the catheterization laboratory, while two others had embolization that required surgical intervention. Four patients had temporary residual leak, nine had protrusion of the device into the aorta without significant Doppler pressure gradient or hemolysis on follow-up, and five had partial hemodynamically insignificant obstruction to the left pulmonary artery. Statistical analysis showed that the effect of the learning curve and experience was responsible for 93% improvement in the procedural outcome over the five-year study period. Conclusion: Transcatheter occlusion of PDA is safe and effective alternative to surgery. Complications occurred in those with unfavorable duct anatomy and with the use of multiple coils. Surgical backup was important for such interventional procedures. Experience played a major role in the proper choice of device type and size which greatly influenced the outcome of the procedure.

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Pediatric cardiac surgery is undergoing a metamorphosis, with more and more critical patients being operated in our country today. Although the principles of physiology have not changed, it is imperative that care providers continue to stay abreast with developments and newer drugs that may help modify the outcome. The team dynamics have also become more complex, which necessitates the need for all care providers (surgeons, cardiologists, anesthesiologists, and intensivists) to better understand the interactions and benefits of newer drugs. Vasopressin has been used in our adult patients for more than a decade and recently has found its rightful place in the pediatric armoury. The objective of this article is to review the physiology of vasopressin and the rationale of its use in critically ill children with shock, in context of the available published data.

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Although coronary artery aneurysms occur in Kawasaki disease, giant aneurysms are rare. We report a very large coronary artery aneurysm, measuring 25 mm and involving left anterior descending artery, in a 2-year-old child with Kawasaki disease. The challenges in management of such a patient have been highlighted.

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Hemorrhagic pericardial effusion is known to occur due to tuberculosis, malignancy, uremia or trauma. We present a rare case of a 6 year old girl with bacterial pericarditis who had hemorrhagic pericardial effusion with cardiac tamponade.

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