Annals of Pediatric Cardiology
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   2008| January-June  | Volume 1 | Issue 1  
    Online since June 4, 2008

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Stenting the ductus arteriosus: Case selection, technique and possible complications
Mazeni Alwi
January-June 2008, 1(1):38-45
DOI:10.4103/0974-2069.41054  PMID:20300236
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.
  30 10,078 1,584
Long QT syndrome: A therapeutic challenge
Maully Shah, Christopher Carter
January-June 2008, 1(1):18-26
DOI:10.4103/0974-2069.41051  PMID:20300233
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.
  8 23,179 1,466
Anomalous origin of the left coronary artery from the pulmonary artery in infancy with preserved left ventricular function: Potential pitfalls and clues to diagnosis
Renu P Kurup, Rachel Daniel, Raman Krishna Kumar
January-June 2008, 1(1):65-67
DOI:10.4103/0974-2069.41061  PMID:20300243
Left ventricular dysfunction is almost invariably associated with anomalous origin of the left coronary artery from pulmonary artery (ALCAPA) that presents during infancy. We report three cases of infants who presented with ALCAPA with relatively well-preserved left ventricular systolic function with a view to illustrate the mechanisms that help maintain left coronary perfusion and discuss the specific echocardiographic clues that suggest diagnosis in these circumstances.
  6 4,779 567
Balloon pulmonary valvotomy as interim palliation for symptomatic young infants with tetralogy of Fallot
KS Remadevi, Balu Vaidyanathan, Edwin Francis, B.R.J Kannan, Raman Krishna Kumar
January-June 2008, 1(1):2-7
DOI:10.4103/0974-2069.41049  PMID:20300231
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.
  6 10,767 988
Bacterial pericarditis presenting as hemorrhagic pericardial effusion in a 6-year-old girl
Anita Saxena, Navneet Singh, S Ramakrishnan, Shyamsunder Kothari
January-June 2008, 1(1):68-69
DOI:10.4103/0974-2069.41062  PMID:20300244
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.
  3 6,364 385
Simplified double barrel repair with autologous pericardium for tetralogy of fallot with hypoplastic pulmonary annulus and anomalous coronary crossing right ventricular outflow
Krishnanaik Shivaprakasha
January-June 2008, 1(1):34-37
DOI:10.4103/0974-2069.41053  PMID:20300235
  3 5,987 480
Alternatives to conduits
Krishna S Iyer
January-June 2008, 1(1):46-49
DOI:10.4103/0974-2069.41055  PMID:20300237
  3 4,927 502
Three dimensional echocardiography in congenital heart defects
Girish S Shirali
January-June 2008, 1(1):8-17
DOI:10.4103/0974-2069.41050  PMID:20300232
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.
  3 12,738 1,422
Perventricular closure of muscular ventricular septal defects: How do I do it?
Karim A Diab, Qi-Ling Cao, Ziyad M Hijazi
January-June 2008, 1(1):27-33
DOI:10.4103/0974-2069.41052  PMID:20300234
  2 8,281 840
Arterial tortuosity syndrome: A rare entity
Ashutosh Marwah, Sejal Shah, PV Suresh, Sunita Maheshwari
January-June 2008, 1(1):62-64
DOI:10.4103/0974-2069.41060  PMID:20300242
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.
  1 9,570 481
Making of a pediatric cardiac surgeon, in India
Rajesh Sharma
January-June 2008, 1(1):50-53
DOI:10.4103/0974-2069.41056  PMID:20300238
  1 7,204 534
Awareness of infective endocarditis prophylaxis in parents of children with congenital heart disease: A prospective survey
Parrimala Nath, V Kiran, Sunita Maheshwari
January-June 2008, 1(1):54-55
DOI:10.4103/0974-2069.41057  PMID:20300239
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.
  - 5,521 498
Giant coronary artery aneurysm with a thrombus secondary to Kawasaki disease
Sushant Patil, Salil Shirodkar, Robin J Pinto, Bharat Dalvi
January-June 2008, 1(1):59-61
DOI:10.4103/0974-2069.41059  PMID:20300241
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.
  - 5,375 490
Summary of the new guidelines for prevention of Infective Endocarditis: Implications for the developing countries
P Bobhate, Robin J Pinto
January-June 2008, 1(1):56-58
DOI:10.4103/0974-2069.41058  PMID:20300240
  - 3,789 669
Editors' note
Bharat Dalvi, Raman Krishna Kumar
January-June 2008, 1(1):1-1
DOI:10.4103/0974-2069.41048  PMID:20300230
  - 3,402 335
Drug therapy: Sildenafil for post-operative pulmonary hypertension and Eisenmenger syndrome - A brief review of literature and survey of expert opinion
Balu Vaidyanathan
January-June 2008, 1(1):70-74
DOI:10.4103/0974-2069.41063  PMID:20300245
  - 6,331 839
Selected summaries
B.R.J Kannan
January-June 2008, 1(1):75-77
  - 3,229 343