Annals of Pediatric Cardiology
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   Table of Contents - Current issue
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January-April 2017
Volume 10 | Issue 1
Page Nos. 1-106

Online since Thursday, December 29, 2016

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EDITORIAL  

Should we close small ventricular septal defects? p. 1
Sangeetha Viswanathan, R Krishna Kumar
DOI:10.4103/0974-2069.197054  PMID:28163421
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ORIGINAL ARTICLES Top

Transcatheter pulmonary valve perforation using chronic total occlusion wire in pulmonary atresia with intact ventricular septum p. 5
Shweta Bakhru, Shilpa Marathe, Manish Saxena, Sudeep Verma, Rajan Saileela, Tapan K Dash, Nageswara Rao Koneti
DOI:10.4103/0974-2069.197065  PMID:28163422
Background: Perforation of pulmonary valve using radiofrequency ablation in pulmonary atresia with intact ventricular septum (PA IVS) is a treatment of choice. However, significant cost of the equipment limits its utility, especially in the developing economies. Objective: To assess the feasibility, safety, and efficacy of perforation of pulmonary valve using chronic total occlusion (CTO) wires in patients with PA IVS as an alternative to radiofrequency ablation. Methods: This is a single.center, nonrandomized, retrospective study conducted during June 2008 to September 2015. Twenty-four patients with PA IVS were selected for the procedure during the study period. The median age and weight of the study population were 8. days and 2.65 kg, respectively. Four patients were excluded after right ventricular angiogram as they showed right ventricular-dependent coronary circulation. The pulmonary valve perforation was attempted using various types of CTO wires based on the tip load with variable penetrating characteristics. Results: The procedure was successful in 16 of twenty patients using CTO wires: Shinobi in nine, Miracle in four, CROSS-IT in two, and Conquest Pro in one. Two patients had perforation of right ventricular outflow tract (RVOT). Pericardiocentesis was required in one patient to relieve cardiac tamponade. Later, the same patient underwent successful hybrid pulmonary valvotomy. The other patient underwent ductus arteriosus. (DA) stenting. Balloon atrial septostomy was needed in three cases with systemic venous congestion. Desaturation was persistent in five cases necessitating DA or RVOT stenting to augment pulmonary blood flow. There were two early and two late deaths. The mean follow-up was 22.66. ± 16 months. Three patients underwent one and half ventricle repair and one Blalock-Taussig shunt during follow-up. Conclusion: Perforation of the pulmonary valve can be done successfully using CTO wires in selected cases of pulmonary atresia with intact ventricular septum.
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Mechanism of valve failure and efficacy of reintervention through catheterization in patients with bioprosthetic valves in the pulmonary position p. 11
Ryan Callahan, Lisa Bergersen, Christopher W Baird, Diego Porras, Jesse J Esch, James E Lock, Audrey C Marshall
DOI:10.4103/0974-2069.197049  PMID:28163423
Background: Surgical and transcatheter bioprosthetic valves (BPVs) in the pulmonary position in patients with congenital heart disease may ultimately fail and undergo transcatheter reintervention. Angiographic assessment of the mechanism of BPV failure has not been previously described. Aims: The aim of this study was to determine the mode of BPV failure (stenosis/regurgitation) requiring transcatheter reintervention and to describe the angiographic characteristics of the failed BPVs and report the types and efficacy of reinterventions. Materials and Methods: This is a retrospective single-center review of consecutive patients who previously underwent pulmonary BPV placement. (surgical or transcatheter) and subsequently underwent percutaneous reintervention from 2005 to 2014. Results: Fifty-five patients with surgical. (41) and transcutaneous pulmonary valve. (TPV) (14) implantation of BPVs underwent 66 catheter reinterventions. The surgically implanted valves underwent fifty reinterventions for indications including 16 for stenosis, seven for regurgitation, and 27 for both, predominantly associated with leaflet immobility, calcification, and thickening. Among TPVs, pulmonary stenosis. (PS) was the exclusive failure mode, mainly due to loss of stent integrity. (10) and endocarditis. (4). Following reintervention, there was a reduction of right ventricular outflow tract gradient from 43 ± 16 mmHg to 16 ± 10. mmHg (P < 0.001) and RVp/AO ratio from 0.8 ± 0.2 to 0.5 ± 0.2 (P < 0.001). Reintervention with TPV placement was performed in 45. (82%) patients. (34 surgical, 11 transcatheter) with no significant postintervention regurgitation or paravalvular leak. Conclusion: Failing surgically implanted BPVs demonstrate leaflet calcification, thickness, and immobility leading to PS and/or regurgitation while the mechanism of TPV failure in the short- to mid-term is stenosis, mainly from loss of stent integrity. This can be effectively treated with a catheter.based approach, predominantly with the valve-in-valve technique.
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Cardiac magnetic resonance feature tracking in Kawasaki disease convalescence p. 18
Konstantinos Bratis, Pauline Hachmann, Nicholas Child, Thomas Krasemann, Tarique Hussain, Sophie Mavrogeni, Rene Botnar, Reza Razavi, Gerald Greil
DOI:10.4103/0974-2069.197046  PMID:28163424
Objective: The objective of this study was to determine whether left ventricular (LV) myocardial deformation indices can detect subclinical abnormalities in Kawasaki disease convalescence. We hypothesized that subclinical myocardial abnormalities due to inflammation represent an early manifestation of the disease that persists in convalescence. Background: Myocardial inflammation has been described as a global finding in the acute phase of Kawasaki disease. Despite normal systolic function by routine functional measurements, reduced longitudinal strain and strain rate have been detected by echocardiography in the acute phase. Methods and Results: Peak systolic LV myocardial longitudinal, radial, and circumferential strain and strain rate were examined in 29 Kawasaki disease convalescent patients (15 males; mean [standard deviation] age: 11 [6.6] years; median interval from disease onset: 5.8 [5.4] years) and 10 healthy volunteers (5 males; mean age: 14 [3.8] years) with the use of cardiac magnetic resonance (CMR) feature tracking. Routine indices of LV systolic function were normal in both groups. Comparisons were made between normal controls and (i) the entire Kawasaki disease group, (ii) Kawasaki disease subgroup divided by coronary artery involvement. Average longitudinal and circumferential strain at all levels was lower in patients compared to normal controls. In subgroup analysis, both Kawasaki disease patients with and without a history of coronary involvement had similar longitudinal and circumferential strain at all levels and lower when compared to controls. There were lower circumferential and longitudinal values in Kawasaki disease patients with persisting coronary artery lesions when compared to those with regressed ones. Conclusion: In this CMR study in Kawasaki disease convalescent patients with preserved routine functional indices, we detected lower circumferential and longitudinal strain values compared to normal controls, irrespective of the coronary artery status.
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Elective nasal continuous positive airway pressure to support respiration after prolonged ventilation in infants after congenital cardiac surgery p. 26
Hemang Gandhi, Amit Mishra, Rajesh Thosani, Himanshu Acharya, Ritesh Shah, Jigar Surti, Alpesh Sarvaia
DOI:10.4103/0974-2069.197055  PMID:28163425
Background: We sought to compare the effectiveness of oxygen (O2) treatment administered by an O2 mask and nasal continuous positive airway pressure (NCPAP) in infants after congenital cardiac surgery. Methods: In this retrospective observational study, 54 infants undergoing corrective cardiac surgery were enrolled. According to the anesthesiologist's preference, the patients ventilated for more than 48 h were either put on NCPAP or O2 mask immediately after extubation. From pre-extubation to 24 h after treatment, arterial blood gas and hemodynamic data were measured. Results: After 24 h of NCPAP institution, the patients showed a significant improvement in oxygenation compared to O2 mask group. Respiratory rate (per minute) decreased from 31.67 ± 4.55 to 24.31 ± 3.69 (P < 0.0001), PO2 (mmHg) increased from 112.12 ± 22.83 to 185.74 ± 14.81 (P < 0.0001), and PCO2 (mmHg) decreased from 42.88 ± 5.01 to 37.00 ± 7.22 (P < 0.0076) in patients on NCPAP. In this group, mean pediatric cardiac surgical Intensive Care Unit (PCSICU) stay was 4.72 ± 1.60 days, with only 2 (11.11%) patients requiring re-intubation. Conclusion: NCPAP can be used safely and effectively in infants undergoing congenital cardiac surgery to improve oxygenation/ventilation. It also reduces the work of breathing, PCSICU stay, and may reduce the likelihood of re-intubation.
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A pediatric echocardiographic Z-score nomogram for a developing country: Indian pediatric echocardiography study – The Z-score p. 31
Rajendra Kumar Gokhroo, Avinash Anantharaj, Devendra Bisht, Kamal Kishor, Nishad Plakkal, Rajeswari Aghoram, Nivedita Mondal, Shashi K Pandey, Ramsagar Roy
DOI:10.4103/0974-2069.197053  PMID:28163426
Background: Almost all presently available pediatric echocardiography Z-score nomograms are based on Western data. They may not be a suitable reference standard for assessing the sizes of cardiac structures of children from developing countries. Objective: This study's objective was to collect normative data of 21 commonly measured cardiovascular structures using M-mode and two-dimensional echocardiography in Indian children aged between 4 and 15 years and to derive Z-score nomograms for each. Subjects and Methods: The study was conducted at two centers in India . Ajmer, Rajasthan, and Mohali, Punjab. We studied a community-based sample involving healthy school going children. After excluding children with cardiovascular abnormalities on the screening echocardiogram, 746 children were included in the final analysis. Echocardiographic assessment was performed using a Philips iE33 system. Results and Analysis: For each parameter measured, seven models were evaluated to assess the relationship of that parameter with the body surface area and the one with the best fit was used to plot the Z-score chart for that parameter. Z score charts were thus derived. Conclusions: The Z-score nomograms derived by this study may be better alternatives to the Western nomograms for use in India and other developing countries for preprocedural decision making in the pediatric population. However, they will require validation in large-scale studies before they can become clinically applicable.
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REVIEW ARTICLE Top

Rheumatic heart disease screening: Current concepts and challenges p. 39
Scott Dougherty, Maziar Khorsandi, Philip Herbst
DOI:10.4103/0974-2069.197051  PMID:28163427
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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REGIONAL PERSPECTIVES Top

Pediatric cardiovascular care in Uganda: Current status, challenges, and opportunities for the future p. 50
Twalib Olega Aliku, Sulaiman Lubega, Judith Namuyonga, Tom Mwambu, Michael Oketcho, John O Omagino, Craig Sable, Peter Lwabi
DOI:10.4103/0974-2069.197069  PMID:28163428
In many developing countries, concerted action against common childhood infectious diseases has resulted in remarkable reduction in infant and under-five mortality. As a result, pediatric cardiovascular diseases are emerging as a major contributor to childhood morbidity and mortality. Pediatric cardiac surgery and cardiac catheterization interventions are available in only a few of Sub-Saharan African countries. In Uganda, open heart surgeries (OHSs) and interventional procedures for pediatric cardiovascular disease are only possible at the Uganda Heart Institute (UHI), having been started with the help of expatriate teams from the years 2007 and 2012, respectively. Thereafter, independent OHS and cardiac catheterization have been possible by the local team at the UHI since the year 2009 and 2013, respectively. The number of OHSs independently performed by the UHI team has progressively increased from 10 in 2010 to 35 in 2015, with mortality rates ranging from 0% to 4.1% over the years. The UHI pediatric catheterization team has independently performed an increasing number of procedures each year from 3 in 2013 to 55 in 2015. We herein describe the evolution and current status of pediatric cardiovascular care in Uganda, highlighting the unique aspects of its establishment, existing constraints, and future plans.
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HOW I DO IT Top

Modified closed chamber sutureless technique for anomalous pulmonary venous connection p. 58
Sabarinath Menon, Thomas Mathew, Jayakumar Karunakaran, Baiju Sashidhar Dharan
DOI:10.4103/0974-2069.197066  PMID:28163429
Visibility continues to be a major problem during repair of obstructed total anomalous pulmonary venous connection (TAPVC) resulting in frequent use of deep hypothermia and low flow bypass. Sutureless technique for primary repair of anomalous pulmonary venous connection is fast becoming popular. In this described modification of sutureless technique through the lateral approach, the left atrium is marsupialized around the common pulmonary venous chamber, except on the right lateral aspect, providing a bloodless field with minimal retraction of heart facilitating the surgery at mild hypothermia. This technique can be particularly useful in small confluence obstructed TAPVC and in mixed TAPVC.
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CASE REPORTS Top

Surgical repair of tricuspid valve leaflet tear following percutaneous closure of perimembranous ventricular septal defect using Amplatzer duct occluder I: Report of two cases p. 61
Saatchi Mahesh Kuwelker, Devi Prasad Shetty, Bharat Dalvi
DOI:10.4103/0974-2069.197052  PMID:28163430
Tricuspid valve (TV) injury following transcatheter closure of perimembranous ventricular septal defect (PMVSD) with Amplatzer ductal occluder I (ADO I), requiring surgical repair, is rare. We report two cases of TV tear involving the anterior and septal leaflets following PMVSD closure using ADO I. In both the patients, the subvalvular apparatus remained unaffected. The patients presented with severe tricuspid regurgitation (TR) 6 weeks and 3 months following the device closure. They underwent surgical repair with patch augmentation of the TV leaflets. Postoperatively, both are asymptomatic with a mild residual TR.
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Removal of a retained intracardiac radiolucent guidewire fragment using an Atrieve™ vascular snare using combined fluoroscopy and transesophageal echocardiography guidance in an infant p. 65
Asif Padiyath, Eudice E Fontenot, Boban P Abraham
DOI:10.4103/0974-2069.197068  PMID:28163431
Retained intravascular foreign body is a well-known complication of central venous access placement in children as well as adults. Most of these foreign bodies are radio-opaque and hence are removed under fluoroscopy guidance. In our case, we describe the removal of an intracardiac radiolucent foreign body in an infant utilizing a combination technique - transesophageal echocardiogram and fluoroscopy.
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Early primary graft failure after a pediatric heart transplant and successful rescue with plasmapheresis, immunoglobulins, and alemtuzumab p. 69
Shashi Raj, Phillip Ruiz, Paolo Rusconi
DOI:10.4103/0974-2069.197063  PMID:28163432
Early primary graft failure after pediatric orthotopic heart transplantation (OHT) has a high mortality rate and can occur due to several causes including but not limited to prolonged graft ischemia time, suboptimal preimplant myocardial preservation, hyperacute rejection, and maladaptation of the graft to the host's hemodynamic status. Mechanical circulatory support with either extracorporeal membrane oxygenation (ECMO) or ventricular assist device has been used for the rescue of primary graft failure in pediatric patients after heart transplant. Cardiac arrest before ECMO initiation in these patients is associated with adverse neurologic outcome although those surviving to hospital discharge generally have excellent long-term outcome. We report a case of early primary graft failure after OHT who required ECMO support and successful rescue with plasmapheresis, immunoglobulins, and alemtuzumab.
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Congenital pseudoaneurysm of mitral-aortic intervalvular fibrosa masquerading as left atrial mass in fetal life p. 72
Shanthi Chidambarathanu, Vijayalakshmi Raja, Indrani Suresh
DOI:10.4103/0974-2069.197062  PMID:28163433
A 28-week-old fetus was detected to have a single left atrial mass in prenatal ultrasound. Postnatal echocardiography showed an aneurysm between the anterior mitral leaflet and aortic valve, to the left of atrioventricular junction and communicating with the left ventricle through a narrow mouth. It probably originated from the mitral-aortic intervalvular fibrous tissue and an inherent weakness at this site might be the cause. Reported cases of pseudoaneurysm of mitral-aortic intervalvular fibrosa and subvalvular ventricular aneurysms seen following infective endocarditis, surgery, or trauma seem to have a similar anatomical background. This case explains the possibility of congenital aneurysm in this location which needs to be considered a differential diagnosis in similar cases.
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Aortic and pulmonary artery calcification: An unusual manifestation of twin-to-twin transfusion syndrome p. 75
Sumitra Venkatesh, J Sanyukta, S Jain, SS Prabhu, S Kulkarni
DOI:10.4103/0974-2069.197059  PMID:28163434
Twin-to-twin transfusion syndrome (TTTS) at times complicates monochorionic twin gestations, resulting in conditions ranging from discordant sizes to fetal demise of one baby. Various types of cardiac defects have been described in the recipient twin of this syndrome. Isolated great artery calcification, i.e. aortic and pulmonary artery calcification is one such uncommon condition associated with TTTS. Calcification of the walls of great vessels may be due to chronic vascular injury sustained as a result of circulatory volume overload in the recipient twin. It may also cause severe systemic hypertension and cardiomyopathy. An accurate diagnosis is important for an optimal follow-up and appropriate genetic counseling. We report a case of aortic and pulmonary artery calcification in association with TTTS.
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Isolated left brachiocephalic artery with the right aortic arch: A rare differential of large patent ductus arteriosus p. 78
Gajendra Dubey, Saurabh Kumar Gupta, Shyam Sundar Kothari
DOI:10.4103/0974-2069.197067  PMID:28163435
We report a case of isolation of the left brachiocephalic artery with the right aortic arch in a 9-year-old male child masquerading as large patent ductus arteriosus with left-to-right shunt. We have emphasized the subtle clinical findings which served as clues to the diagnosis.
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IMAGES Top

Interventional therapy for partial anomalous pulmonary venous connection with dual drainage p. 82
Saurabh Kumar Gupta, Anita Saxena, Rajnish Juneja
DOI:10.4103/0974-2069.197072  PMID:28163436
A 6-year-old boy presented with dual drainage of left upper pulmonary vein, with connection to innominate vein inaddition to its normal connection to the left atrium. Despite relief of aortic stenosis at the age of 3 years, significant left to right shunt persisted. The dual drainage allowed successful percutaneous closure of the levoatriocardinal vein without obstruction to the pulmonary venous flow to the left atrium.
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Pseudoaneurysm of the left atrium following infective endocarditis p. 84
Devi A Manuel, Bino John Sahayo, Viji Samuel Thomson, Jacob Jose
DOI:10.4103/0974-2069.197048  PMID:28163437
Transthoracic echocardiogram of a 3-year-old child showed a hypoechoic cavity in the posterior wall of the left atrium communicating with the left ventricle through an orifice in the mitral annulus, suggestive of pseudoaneurysm (Ps), probably the result of infective endocarditis. Three-dimensional echocardiography was helpful to confirm the diagnosis and assess the anatomical relationship of the Ps.
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A rare case of cardiac tumor in a child p. 87
Mallar Mukharjee, Jigna N Bathia, Apurba Ghosh, Anil Kumar Singhi
DOI:10.4103/0974-2069.197070  PMID:28163438
Pediatric cardiac tumors are rare and usually benign. An infectious etiology like tuberculosis invading myocardium and presenting as infiltrative mass is extremely rare. We present a case of a 15 month old girl with clinical feature of cardiac failure who had infiltrative multiple myocardial masses in echocardiogram. Advanced cardiac imaging by Cardiac Magnetic resonance imaging (MRI ) helped in tissue delineation. Therapeutic trial of anti-tubercular drugs in view clinical suspicion of Tuberculosis resulted in complete remission of symptom and disappearance of the cardiac mass.
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LETTERS TO EDITOR Top

Impact of Abernathy malformation on pulmonary circulatory hemodynamics in a univentricular heart p. 90
Vinoth Doraiswamy, Kothandam Sivakumar
DOI:10.4103/0974-2069.197071  PMID:28163439
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Isolated facial nerve palsy after arterial switch operation: A rarity p. 92
Manoj Kumar Sahu, Intekhab Alam, Sarvesh Pal Singh, Ramesh Menon, Sachin Talwar
DOI:10.4103/0974-2069.197057  PMID:28163440
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Transcatheter closure of aortopulmonary window with Amplatzer duct occluder II p. 93
Hemant Kumar Nayak, Nurul Islam, Bhanu Kumar Bansal
DOI:10.4103/0974-2069.197060  PMID:28163441
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Transfemoral balloon angioplasty of severe coarctation of aorta in 1200 g newborn p. 95
Gaurav Garg, Naresh Goyal, Gaurav Mandhan, Poonam Sidana
DOI:10.4103/0974-2069.197047  PMID:28163442
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Spectrum of cyanotic congenital heart disease diagnosed by echocardiographic evaluation in patients attending a tertiary cardiac care center of South Rajasthan p. 97
Amit Kumar, Kapil Bhargava
DOI:10.4103/0974-2069.197050  PMID:28163443
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Left pulmonary artery sling without symptoms p. 98
Shashi Raj, Samir Chandra
DOI:10.4103/0974-2069.197061  PMID:28163444
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Massive pericardial effusion, yet no signs of tamponade! p. 100
Sunitha Vaidyanathan, Amol Gupta, Kothandam Sivakumar
DOI:10.4103/0974-2069.197064  PMID:28163445
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Delivery cable induced cardiac tamponade during transcatheter closure of atrial septal defect: A preventable complication p. 101
Ramachandra Barik
DOI:10.4103/0974-2069.197058  PMID:28163446
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A novel technique for percutaneous closure of an atrial septal defect in a patient with interrupted inferior vena cava using a “modified” short sheath from an internal jugular vein approach p. 102
Tharakanatha R Yarrabolu, Andrew Robinson, Athar M Qureshi
DOI:10.4103/0974-2069.197056  PMID:28163447
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“Hemodynamic vice” of the right-sided ascending vertical vein in the setting of supracardiac total anomalous pulmonary venous connection in a neonate: Anatomic-embryological correlation p. 104
Atul Achyut Kalantre, Bhavik Champaneri, Brijesh Kottayil, Balu Vaidyanathan
DOI:10.4103/0974-2069.187091  PMID:28163448
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