Annals of Pediatric Cardiology
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REVIEW ARTICLES
Post operative temporary epicardial pacing: When, how and why?
Anjan S Batra, Seshadri Balaji
July-December 2008, 1(2):120-125
DOI:10.4103/0974-2069.43877  PMID:20300253
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.
  22,630 1,334 3
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.
  21,184 1,406 1
REVIEW ARTICLE
The use of Z-scores in paediatric cardiology
Henry Chubb, John M Simpson
July-December 2012, 5(2):179-184
DOI:10.4103/0974-2069.99622  PMID:23129909
Z-scores are a means of expressing the deviation of a given measurement from the size or age specific population mean. By taking account of growth or age, Z-scores are an excellent means of charting serial measurements in paediatric cardiological practice. They can be applied to echocardiographic measurements, blood pressure and patient growth, and thus may assist in clinical decision-making.
  14,303 1,034 18
STATE-OF-THE-ART PAPER
The role of stents in the treatment of congenital heart disease: Current status and future perspectives
Bjoern Peters, Peter Ewert, Felix Berger
January-June 2009, 2(1):3-23
DOI:10.4103/0974-2069.52802  PMID:20300265
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.
  11,962 1,714 35
REVIEW ARTICLES
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.
  11,478 1,381 3
MINI SYMPOSIUM: CONGENITAL MITRAL VALVE LESIONS
Congenital mitral valve lesions : Correlation between morphology and imaging
Bo Remenyi, Tom L Gentles
January-June 2012, 5(1):3-12
DOI:10.4103/0974-2069.93703  
Congenital malformations of the mitral valve are often complex and affect multiple segments of the valve apparatus. They may occur in isolation or in association with other congenital heart defects. The majority of mitral valve malformations are not simply classified, and descriptive terms with historical significance (parachute, mitral, or arcade) often lack the specificity that cardiac surgeons demand as part of preoperative echocardiographic morphological assessment. This paper examines the strengths and limitations of commonly used descriptions and classification systems of congenitally malformed mitral valves. It correlates pathological, surgical, and echocardiographic findings. Finally, it makes recommendations for the systematic evaluation of the congenitally malformed mitral valve using segmental echocardiographic analysis to assist precise communication and optimal surgical management.
  11,736 981 8
STATE-OF-THE-ART PAPER
The Fontan pathway: What's down the road?
Sachin Khambadkone
July-December 2008, 1(2):83-92
DOI:10.4103/0974-2069.43872  PMID:20300248
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.
  9,983 1,458 7
PERSPECTIVE
Tetralogy of Fallot: Current surgical perspective
Tom R Karl
July-December 2008, 1(2):93-100
DOI:10.4103/0974-2069.43873  PMID:20300249
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.
  9,255 1,860 8
CASE REPORTS
Carotid-subclavian bypass for subclavian steal syndrome following coarctation repair in infancy
Scott Chowning, Donald Stowell, UmaKumaran Ponniah
July-December 2012, 5(2):207-209
DOI:10.4103/0974-2069.99631  PMID:23129918
A 19 year old man presented with dizziness that was exacerbated while using left arm. On investigation, subclavian steal syndrome (SSS) was diagnosed. He underwent left carotid to subclavian bypass with relief of symptoms. Subclavian steal syndrome, although rare, should be considered in the differential diagnosis in patients with the history of subclavian artery manipulation in the past in proper settings.
  10,718 148 -
ORIGINAL ARTICLE
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.
  9,382 941 3
CLINICO-MORPHOLOGICAL CORRELATIONS
Sequential segmental analysis
Robert H Anderson, Girish Shirali
January-June 2009, 2(1):24-35
DOI:10.4103/0974-2069.52803  PMID:20300266
  8,156 1,937 18
POINT OF VIEW
Rheumatic fever pathogenesis: Approach in research needs change
Rajendra Tandon
July-December 2012, 5(2):169-178
DOI:10.4103/0974-2069.99621  PMID:23129908
Despite identifying that rheumatic fever (RF) is the result of an immunological reaction following group-A beta-hemolytic streptococcal infection, the pathogenesis remains elusive. RF has been incorrectly designated as causing pancarditis, since it does not cause myocarditis. Research directed toward myocarditis, targeting myosin to unravel the pathogenesis has not succeeded in more than 60 years. RF causes permanent damage to cardiac valves. The mitral valve (MV), derived from the wall of the left ventricle, is composed of a central core of connective tissue, covered on both sides by endothelium. The left ventricle does not have either myocardial or intermyocardial connective tissue involvement in RF. By exclusion, therefore, the primary site of RF damage appears to be the endothelium. Evaluation of the histopathology and immunopathology indicates that RF is a disease of the valvular and vascular endothelium. It is not a connective tissue disorder. Research to identify pathogenesis needs to be focused toward valvular endothelium.
  9,328 707 5
OPINION POLL
Digoxin in management of heart failure in children: Should it be continued or relegated to the history books?
Shreepal Jain, Balu Vaidyanathan
July-December 2009, 2(2):149-152
DOI:10.4103/0974-2069.58317  PMID:20808628
  8,648 1,273 3
CASE REPORTS
Ectopia cordis with endocardial cushion defect: Prenatal ultrasonographic diagnosis with autopsy correlation
K Balakumar, K Misha
July-December 2010, 3(2):166-168
DOI:10.4103/0974-2069.74048  PMID:21234197
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.
  9,272 146 -
TECHNIQUE IN FOCUS
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.
  7,992 1,423 11
HEMODYNAMIC ROUND
Understanding the physiology of complex congenital heart disease using cardiac magnetic resonance imaging
Mahesh Kappanayil, Rajesh Kannan, Raman Krishna Kumar
July-December 2011, 4(2):177-182
DOI:10.4103/0974-2069.84666  PMID:21976882
Complex congenital heart diseases are often associated with complex alterations in hemodynamics. Understanding these key hemodynamic changes is critical to making management decisions including surgery and postoperative management. Existing tools for imaging and hemodynamic assessment like echocardiography, computed tomography and cardiac catheterization have inherent limitations. Cardiac magnetic resonance imaging (MRI) is emerging as a powerful bouquet of tools that allow not only excellent imaging, but also a unique insight into hemodynamics. This article introduces the reader to cardiac MRI and its utility through the clinical example of a child with a complex congenital cyanotic heart disease.
  8,690 465 -
CASE REPORTS
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.
  8,687 456 -
REVIEW ARTICLES
Pathology of Takayasu arteritis: A brief review
Pradeep Vaideeswar, Jaya R Deshpande
January-June 2013, 6(1):52-58
DOI:10.4103/0974-2069.107235  PMID:23626437
Takayasu arteritis (TA) is a chronic idiopathic and granulomatous vasculitis, manifesting mainly as a panaortitis. Autoimmune cell-mediated immunity is probably responsible for the disease. The inflammation commences from the adventitia and progresses to the intima and leads to, both in adults and children, segmental stenosis, occlusion, dilatation, and/or aneurysm formation. This review focuses briefly on the etiopathogenesis, and describes the pathological and clinical features in adults and children.
  8,111 924 8
PERSPECTIVE
The role of the Fontan operation in the treatment of congenitally corrected transposition of the great arteries
Tom R Karl
July-December 2011, 4(2):103-110
DOI:10.4103/0974-2069.84634  PMID:21976866
Congenitally corrected transposition of the great arteries (ccTGA) is a complex cardiac anomaly with an unfavorable natural history. Surgical treatment has been available for over 50 years. Initial procedures used for ccTGA did not correct atrio-ventricular discordance, leaving the right ventricle in systemic position. In the past two decades anatomic repair has been considered to be a better option. Many cases subjected to anatomic repairs would also be suitable for the Fontan strategy, which probably has a lower initial risk. The rationale for use of the Fontan operation in management of congenitally corrected transposition is discussed in this review, with comparisons to other strategies.
  8,396 601 7
MINI SYMPOSIUM: RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE
Genes, autoimmunity and pathogenesis of rheumatic heart disease
L Guilherme, KF Köhler, E Postol, J Kalil
January-June 2011, 4(1):13-21
DOI:10.4103/0974-2069.79617  PMID:21677799
Pathogenesis of rheumatic heart disease (RHD) remains incompletely understood. Several genes associated with RHD have been described; most of these are involved with immune responses. Single nucleotide polymorphisms in a number of genes affect patients with RHD compared to controls. Molecular mimicry between streptococcal antigens and human proteins, including cardiac myosin epitopes, vimentin and other intracellular proteins is central to the pathogenesis of RHD. Autoreactive T cells migrate from the peripheral blood to the heart and proliferate in the valves in response to stimulation with specific cytokines. The types of cells involved in the inflammation as well as different cytokine profiles in these patients are being investigated. High TNF alpha, interferon gamma, and low IL4 are found in the rheumatic valve suggesting an imbalance between Th1 and Th2 cytokines and probably contributing to the progressive and permanent valve damage. Animal model of ARF in the Lewis rat may further contribute towards understanding the ARF.
  7,912 986 7
OPINION POLL
Role of beta-blockers in Marfan's syndrome and bicuspid aortic valve: A time for re-appraisal
Balu Vaidyanathan
July-December 2008, 1(2):149-152
DOI:10.4103/0974-2069.43885  PMID:20300261
  8,051 675 3
ORIGINAL ARTICLE
Role of prophylactic surgical ligation of patent ductus arteriosus in extremely low birth weight infants: Systematic review and implications for clinical practice
Rafat Mosalli, Khalid AlFaleh, Bosco Paes
July-December 2009, 2(2):120-126
DOI:10.4103/0974-2069.58313  PMID:20808624
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.
  7,873 836 5
MINI SYMPOSIUM: RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE
Global research priorities in rheumatic fever and rheumatic heart disease
Jonathan R Carapetis, Liesl J Zühlke
January-June 2011, 4(1):4-12
DOI:10.4103/0974-2069.79616  PMID:21677798
We now stand at a critical juncture for rheumatic fever (RF) and rheumatic heart disease (RHD) control. In recent years, we have seen a surge of interest in these diseases in regions of the world where RF/RHD mostly occur. This brings real opportunities to make dramatic progress in the next few years, but also real risks if we miss these opportunities. Most public health and clinical approaches in RF/RHD arose directly from programmes of research. Many unanswered questions remain, including those around how to implement what we know will work, so research will continue to be essential in our efforts to bring a global solution to this disease. Here we outline our proposed research priorities in RF/RHD for the coming decade, grouped under the following four challenges: Translating what we know already into practical RHD control; How to identify people with RHD earlier, so that preventive measures have a higher chance of success; Better understanding of disease pathogenesis, with a view to improved diagnosis and treatment of ARF and RHD; and Finding an effective approach to primary prevention. We propose a mixture of basic, applied, and implementation science. With concerted efforts, strong links to clinical and public health infrastructure, and advocacy and funding support from the international community, there are good prospects for controlling these RF and RHD over the next decade.
  7,601 820 29
ORIGINAL ARTICLES
Amplatzer vascular plugs in congenital cardiovascular malformations
Parag Barwad, Sivasubramanian Ramakrishnan, Shyam S Kothari, Anita Saxena, Saurabh K Gupta, Rajnish Juneja, Gurpreet Singh Gulati, Priya Jagia, Sanjiv Sharma
July-December 2013, 6(2):132-140
DOI:10.4103/0974-2069.115255  PMID:24688229
Background: Amplatzer vascular plugs (AVPs) are devices ideally suited to close medium-to-large vascular communications. There is limited published literature regarding the utility of AVPs in congenital cardiovascular malformations (CCVMs). Aims: To describe the use of AVPs in different CCVMs and to evaluate their safety and efficacy. Materials and Methods: All patients who required an AVP for the closure of CCVM were included in this retrospective review of our catheterization laboratory data. The efficacy and safety of AVPs are reported. Results: A total of 39 AVPs were implanted in 31 patients. Thirteen (33%) were AVP type I and 23 (59%) were AVP type II. AVP type III were implanted in two patients and type IV in one patient. The major indications for their use included closure of pulmonary arteriovenous malformation (AVM) (n = 7), aortopulmonary collaterals (n = 7), closure of a patent Blalock-Taussig shunt (n = 5), systemic AVM (n = 5), coronary AVM (n = 4), patent ductus arteriosus (PDA) (n = 3), pulmonary artery aneurysms (n = 3), and venovenous collaterals (n = 2). Deployment of the AVP was done predominantly via the 5 - 7F Judkin's right coronary guide catheter. Overall 92% of the AVPs could be successfully deployed and resulted in occlusion of the target vessel in all cases, within 10 minutes. No procedure-related or access site complication occurred. Conclusions: AVPs are versatile, easy to use, and effective devices to occlude the vascular communications in a variety of settings. AVP II is especially useful in the closure of tubular structures with a high flow.
  7,851 532 5
HOW I DO IT
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
  7,509 794 2