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.

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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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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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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.

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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.

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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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Background: There is a known association between congenital heart disease (CHD) and other congenital anomalies (CA). These associations have been altered by changes in prenatal factors in recent time. We reviewed the largest database of inpatient hospitalization information and analyzed the current association between common CHD diagnoses and other congenital anomalies. Materials and Methods: Case-control study design. We reviewed the Nationwide Inpatient Sample (NIS) database from 1998 to 2008 and identified all live births with CHD diagnosis (case) and live births without CHD diagnosis (control). We compared prevalence of associated congenital anomalies between the case and control groups. Results: Our cohort consisted of 97,154 and 12,078,482 subjects in the case and control groups, respectively. In the CHD population, prevalence of non-syndromic congenital anomaly (NSCA), genetic syndrome (GS), and overall extra-cardiac congenital anomaly (CA) were 11.4, 2.2, and 13.6%, respectively. In the control group, prevalence of NSCA, GS, and CA were 6.7, 0.3, and 7.0%, respectively. NSCA (odds ratio (OR): 1.88, confidence interval (CI): 1.73-1.94), GS (OR 2.52, CI 2.44-2.61), and overall CA (OR: 2.01, CI: 1.97-2.14) were strongly associated with CHD. Prevalence of GS and multiple organ-system CA decreased significantly over the study period. Conclusions: This is the largest and most comprehensive population-based study evaluating association between CHD and extra-cardiac malformation (ECM) in newborns. There was significant decrease in prevalence of GS and multiple CA over the study period.

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Management of complex congenital heart disease in patients with Heterotaxy syndrome (HS) has steadily improved. However, there is an insufficient appreciation of various non-cardiac issues that might impact the overall status of these patients. This article briefly reviews the implications of gastrointestinal, immunologic, genitourinary, respiratory, and central nervous system involvement in HS patients with a view to aid in their comprehensive clinical management.

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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.

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Sudden death in the young is rare. About 25% of cases occur during sports. Most young people with sudden cardiac death (SCD) have underlying heart disease, with hypertrophic cardiomyopathy and coronary artery anomalies being commonest in most series. Arrhythmogenic right ventricular dysplasia and long QT syndrome are the most common primary arrhythmic causes of SCD. It is estimated that early cardiopulmonary resuscitation and widespread availability of automatic external defibrillators could prevent about a quarter of pediatric sudden deaths.

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Background : Selecting the device size using a sizing balloon could oversize the ostium secundum atrial septal defect (OSASD) with floppy margins and at times may lead to complications. Identifying the firm margins using trans-esophageal echocardiography (TEE) and selecting appropriate-sized device optimizes ASD device closure. This retrospective study was undertaken to document the safety and feasibility of device closure without balloon sizing the defect. Methods : Sixty-one consecutive patients who underwent trans-catheter closure of OSASD guided by balloon sizing of the defect and intra procedural fluoroscopy (group I) and 67 consecutive patients in whom TEE was used for defect sizing and as intraprocedural imaging during device deployment (group II) were compared. The procedural success rate, device characteristics, and complications were compared between the two groups. Results : The procedure was successful in 79.7 % patients. The success rate in group II (60 of 67, 89.6%) was significantly higher than in group I (41 of 61, 67.2 %) (P = 0.002). Mean upsizing of ASD device was significantly lower in group II (P < 0.001). TEE also provided better success rate with smaller device in subjects with large ASD (>25 mm) and in those who were younger than 14 years of age. There were four cases of device embolization (two in each group); of which one died in group II despite successful surgical retrieval. Conclusion : Balloon sizing may not be essential for successful ASD device closure. TEE-guided sizing of ASD and device deployment provides better success rate with relatively smaller sized device.

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Introduction: Three-dimensional. (3D) printing is an innovative manufacturing process that allows computer.assisted conversion of 3D imaging data into physical “printouts” Healthcare applications are currently in evolution. Objective: The objective of this study was to explore the feasibility and impact of using patient-specific 3D-printed cardiac prototypes derived from high.resolution medical imaging data. (cardiac magnetic resonance imaging/computed tomography. [MRI/CT]) on surgical decision-making and preoperative planning in selected cases of complex congenital heart diseases. (CHDs). Materials and Methods: Five patients with complex CHD with previously unresolved management decisions were chosen. These included two patients with complex double.outlet right ventricle, two patients with criss-cross atrioventricular connections, and one patient with congenitally corrected transposition of great arteries with pulmonary atresia. Cardiac MRI was done for all patients, cardiac CT for one; specific surgical challenges were identified. Volumetric data were used to generate patient-specific 3D models. All cases were reviewed along with their 3D models, and the impact on surgical decision-making and preoperative planning was assessed. Results: Accurate life-sized 3D cardiac prototypes were successfully created for all patients. The models enabled radically improved 3D understanding of anatomy, identification of specific technical challenges, and precise surgical planning. Augmentation of existing clinical and imaging data by 3D prototypes allowed successful execution of complex surgeries for all five patients, in accordance with the preoperative planning. Conclusions: 3D-printed cardiac prototypes can radically assist decision-making, planning, and safe execution of complex congenital heart surgery by improving understanding of 3D anatomy and allowing anticipation of technical challenges.

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Rheumatic heart disease (RHD) is a disease of poverty, is almost entirely preventable, and is the most common cardiovascular disease worldwide in those under 25 years. RHD is caused by acute rheumatic fever (ARF) which typically results in cumulative valvular lesions that may present clinically after a number of years of subclinical disease. Therapeutic interventions, therefore, typically focus on preventing subsequent ARF episodes (with penicillin prophylaxis). However, not all patients with ARF develop symptoms and not all symptomatic cases present to a physician or are correctly diagnosed. Therefore, if we hope to control ARF and RHD at the population level, we need a more reliable discriminator of subclinical disease. Recent studies have examined the utility of echocardiographic screening, which is far superior to auscultation at detecting RHD. However, there are many concerns surrounding this approach. Despite the introduction of the World Heart Federation diagnostic criteria in 2012, we still do not really know what constitutes the most subtle changes of RHD by echocardiography. This poses serious problems regarding whom to treat and what to do with the rest, both important decisions with widespread implications for already stretched health-care systems. In addition, issues ranging from improving the uptake of penicillin prophylaxis in ARF/RHD-positive patients, improving portable echocardiographic equipment, understanding the natural history of subclinical RHD and how it might respond to penicillin, and developing simplified diagnostic criteria that can be applied by nonexperts, all need to be effectively tackled before routine widespread screening for RHD can be endorsed.

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Objectives: To study the blood pressure of school going children in a rural area and its relationship with the anthropometric indices. Methods : A prospective, cross-sectional study was carried out from November 2006 to December 2007 in the school going children between the ages of 6-17 years from eight different schools in the rural areas of Wardha district. The height, weight, systolic blood pressure (SBP), and diastolic blood pressure (DBP) were recorded in both sexes followed by complete clinical examination with special emphasis on cardiovascular system. Hypertension (HT) was defined as SBP or DBP exceeding the 95th percentile for age, gender, and height on at least three separate occasions, 1-3 weeks apart. SPSS software was used to analyze the data. Coefficient correlation tests were employed to assess the relation between BP and anthropometric variables. Results :Of 2643 school children, 1227 were boys and 1416 girls with a male to female ratio of 1:1.16. In boys, SBP and DBP increased with age except a marginal decline in SBP at the age of 17 years (-0.09) and decrease in the DBP (-1.29) at 16 years of age. In girls, SBP and DBP also increased with age except at 11 years, wherein there was a mild decrease in SBP (-0.09) as well as the DBP (-0.24). Correlation coefficient analysis showed highly significant positive correlation of height with SBP and DBP. There was a significant correlation of SBP and DBP with the weight, and body mass index (BMI). The prevalence of HT was 5.75% (i.e., 3.25% for systolic HT and 2.49% for diastolic HT). Conclusion : We recommend that there is a need for checking BP to detect HT in children, so that remedial measures can be initiated as early as possible.

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Objective: To assess the birth prevalence and pattern of congenital heart disease (CHD) using echocardiography in babies born in a community hospital of North India. Methods: A cross-sectional observational study conducted over a period of 3 years. Newborns born over a specific 8-h period of the day were recruited in the study. They underwent routine clinical examination and pulse oximetry, followed by screening echocardiography for diagnosing a CHD. Results: A total of 20,307 newborns were screened, among which 874 had abnormal echocardiograms; 687 had insignificant CHDs, 164 had significant CHDs, and 24 had other abnormal cardiac findings. The birth prevalence of significant CHDs was 8.07 per 1000 live births; 131 newborns had an acyanotic CHD (79.9%) and 33 a cyanotic CHD (20.1%). Ventricular septal defect (VSD) was the most common acyanotic CHD, present in 116 newborns, giving a prevalence of 5.7/1000 live births. Among the cyanotic CHD, transposition of great arteries was most common (prevalence 0.34/1000 live births). Conclusion: The CHD birth prevalence in our study is similar to the reported worldwide birth prevalence. Acyanotic CHD (mostly VSD) is seen in about three-fourths of babies born with CHD. The more sinister cyanotic CHD is present in remaining 25%.

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Introduction: Congenital heart defects (CHDs) are an important cause of mortality and morbidity in children representing a major global health burden. It is thus important to determine their prevalence and spectrum and identify risk factors associated with the development of heart defects. Materials and Methods: A case-control study was carried out in the Department of Pediatrics and Center of Cardiology, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India, from February 2014 to August 2015. All patients referred with complaints or clinical examination suggestive of CHDs were further evaluated with echocardiography. On Echocardiography, patients having CHDs were included as cases and those having a normal echocardiographic study were included as controls. Healthy controls were also included. 400 cases and 400 controls were thus identified; preterms having patent ductus arteriosus and patent foramen ovale and those with acquired heart defects were excluded. Risk factors among cases and controls were further studied. Results: Acyanotic heart defects were 290 (72.50%) of the total heart defects, whereas the contribution of cyanotic heart defects was 110 (27.50%). Out of all CHDs, ventricular septal defect was the most common lesion with contribution of 152 (38%) cases, whereas among the cyanotic heart defects, Tetralogy of Fallot was the most common lesion (18% of total cases). Out of the total 400 cases, 261 were males (65.25%). On univariate analysis, paternal age (odds ratio, OR, 2.01), bad obstetric history (OR, 2.65), antenatal febrile illness (OR, 4.12), and advanced maternal age (OR, 3.28) were found to increase the risk of CHD whereas intake of multivitamin (OR, 3.02) was found to be protective. The risk factors were further analyzed with multivariate logistic regression analysis and all the above factors were found to be significantly associated. Conclusion: We noted that the profile of CHD in our population was similar to the published literature although many were missed during infancy and detected later in life. Several antenatal factors were found to be associated with the incidence of congenital heart disease emphasizing the need to prioritize antenatal care and counseling to pregnant mothers along with good maternal nutrition and folic acid supplementation.

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Familial hypercholesterolemia (FH) is a genetic disorder of lipoprotein metabolism resulting in elevated serum low-density lipoprotein (LDL) cholesterol levels leading to increased risk for premature cardiovascular diseases (CVDs). The diagnosis of this condition is based on clinical features, family history, and elevated LDL-cholesterol levels aided more recently by genetic testing. As the atherosclerotic burden is dependent on the degree and duration of exposure to raised LDL-cholesterol levels, early diagnosis and initiation of treatment is paramount. Statins are presently the mainstay in the management of these patients, although newer drugs, LDL apheresis, and other investigational therapies may play a role in certain subsets of FH, which are challenging to treat. Together these novel treatments have notably improved the prognosis of FH, especially that of the heterozygous patients. Despite these achievements, a majority of children fail to attain targeted lipid goals owing to persistent shortcomings in diagnosis, monitoring, and treatment. This review aims to highlight the screening, diagnosis, goals of therapy, and management options in patients with FH.

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Hypocalcemia is a less known but treatable cause for dilated cardiomyopathy, leading to severe heart failure in children. Cardiogenic shock related to hypocalcemic cardiomyopathy is a rare event. We describe 5 infants presenting with cardiogenic shock over 3 years, who were found to have severe hypocalcemia as a sole cause of myocardial dysfunction. The patients responded to calcium and vitamin D supplementation promptly and left ventricular systolic function normalized within months of treatment. In any case of cardiogenic shock, hypocalcemia should be included in the differential diagnosis and must be investigated.

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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.

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Medicine in the 21 ^st century is increasingly dependent on technology. Unlike in many other areas, the cost of medical technology is not declining and its increasing use contributes to the spiraling healthcare costs. Many medical professionals equate progress in medicine to increasing use of sophisticated technology that is often expensive and beyond the reach of the average citizen. Pediatric heart care is very technology-intensive and therefore very expensive and beyond the reach of the vast majority of children in the developing world. There is an urgent need to address this situation through development and use of appropriate technology in accordance with the needs and priorities of the society. A number of simple and inexpensive quality measures that have the potential of improving outcomes substantially without the need for expensive equipment should be instituted before embracing high-end technology. Innovations to reduce costs that are commonly used in limited resource environments should be tested systematically.

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In a retrospective analysis of echocardiograms, the incidence of retroaortic innominate vein was found to be 0.55% amongst children with congenital heart disease. It was most commonly associated with tetralogy of Fallot and right aortic arch.

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Mitral valve disease is quite variable and can occur as an isolated defect or in association with other complex left sided lesions. These lesions are often best described with detailed pre-operative imaging studies to define the valve anatomy and to access associated left heart disease. Depending on the type of mitral valve disease, various surgical repair techniques have led to improved survival in the recent era. We describe lesion specific approach to mitral valve repair and results.

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Objective: To determine the delay in diagnosis of various types of congenital heart defects in children and factors associated with such delay. Patients and Methods: For this observational study, 354 patients having congenital heart disease (CHD) presenting for the first time to the Department of Cardiology, Children's Hospital, Lahore, Pakistan, between January 1, 2015 and June 30, 2015, were enrolled after obtaining informed verbal consent from the guardian of each child. Demographical profile and various factors under observation were recorded. Results: Among the 354 enrolled children (M: F 1.7:1) with age ranging from 1 to 176 months (median 24 months), 301 (85.1%) had delayed diagnosis of CHD (mainly acyanotic 65.3%), with median delay (8 months). Main factors for delay were delayed first consultation to a doctor (37.2%) and delayed diagnosis by a health professional (22.5%). Other factors included delayed referral to a tertiary care hospital (13.3%), social taboos (13.0%), and financial constraints (12.3%). Most children were delivered outside hospital settings (88.7%). Children with siblings less than two (40%) were less delayed than those having two or more siblings (60%, P < 0.001). Conclusion: Diagnosis of congenital heart defect was delayed in majority of patients. Multiple factors such as lack of adequately trained health system and socioeconomic constraints were responsible for the delay. There is a need to develop an efficient referral system and improve public awareness in developing countries for early diagnosis and management of such children.

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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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