Annals of Pediatric Cardiology
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   2017| September-December  | Volume 10 | Issue 3  
    Online since August 21, 2017

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Brugada syndrome in children - Stepping into unchartered territory
Shashank P Behere, Steven N Weindling
September-December 2017, 10(3):248-258
DOI:10.4103/apc.APC_49_17  PMID:28928611
Brugada syndrome (BrS) is an autosomal dominant inherited channelopathy. It is associated with a typical pattern of ST-segment elevation in the precordial leads V1–V3 and potentially lethal ventricular arrhythmias in otherwise healthy patients. It is frequently seen in young Asian males, in whom it has previously been described as sudden unexplained nocturnal death syndrome. Although it typically presents in young adults, it is also known to present in children and infants, especially in the presence of fever. Our understanding of the genetic pathogenesis and management of BrS has grown substantially considering that it has only been 24 years since its first description as a unique clinical entity. However, there remains much to be learned, especially in the pediatric population. This review aims to discuss the epidemiology, genetics, and pathogenesis of BrS. We will also discuss established standards and new innovations in the diagnosis, prognostication, risk stratification, and management of BrS. Literature search was run on the National Center for Biotechnology Information's website, using the Medical Subject Headings (MeSH) database with the search term “Brugada Syndrome” (MeSH), and was run on the PubMed database using the age filter (birth–18 years), yielding 334 results. The abstracts of all these articles were studied, and the articles were categorized and organized. Articles of relevance were read in full. As and where applicable, relevant references and citations from the primary articles were further explored and read in full.
  6,975 429 13
Clarifying the anatomy and physiology of totally anomalous systemic venous connection
Saurabh Kumar Gupta, Rajnish Juneja, Robert H Anderson, Gurpreet S Gulati, Velayoudam Devagorou
September-December 2017, 10(3):269-277
DOI:10.4103/apc.APC_68_17  PMID:28928613
The description of totally anomalous systemic venous connection is limited to case reports. In this review, we seek to clarify anatomic, physiologic, and hemodynamic aspects of this extremely rare anomaly. We also present findings of two patients in whom connection of all the systemic veins was anomalous. In the first patient, with usual atrial arrangement, all systemic veins, including the coronary sinus, were connected anomalously to the morphologically left atrium. Limited left-to-right shunt across an atrial septal defect provided the only source of blood flow to the lungs. The diagnosis was established by saline contrast echocardiography and cardiac catheterization. Extreme hypoplasia of the right ventricle precluded corrective surgery, so we performed a bidirectional Glenn operation, along with atrial septectomy. The second patient had isomerism of the left atrial appendages, which creates problems in the definition in anatomic terms since the connection of the systemic veins can never be normal anatomically when both atriums possess a morphologically left appendage. Our patient, nonetheless, had all the systemic and pulmonary veins, connected to the left-sided atrial chamber which then connected to the left ventricle, thus producing hemodynamics of totally anomalous systemic venous connection. We propose an algorithm for evaluation of this hemodynamic combination and discuss management options. We also intend to clarify the potential differences between connection and drainage, with particular attention to the arrangement of atrial appendages. Even though the hemodynamics may be comparable, in anatomic terms, both systemic and pulmonary venoatrial connection will always be anomalous with isomeric atrial appendages.
  4,871 489 4
Human herpesvirus 6-induced inflammatory cardiomyopathy in immunocompetent children
Surabhi Reddy, Eva Eliassen, Gerhard R Krueger, Bibhuti B Das
September-December 2017, 10(3):259-268
DOI:10.4103/apc.APC_54_17  PMID:28928612
Over the last decade, human herpesvirus 6 (HHV-6) has been implicated in the etiology of pediatric myocarditis and subsequent dilated cardiomyopathy (DCM). This review provides an overview of recent literature investigating the pathophysiological relevance of HHV-6 in inflammatory cardiomyopathy. We examined 11 cases of previously published pediatric myocarditis and/or DCM associated with HHV-6 and also our experience of detection of virus particles in vascular endothelium of HHV-6 positive endomyocardial biopsy tissue by electron microscopy. The exact role of the presence of HHV-6 and its load remains controversial as the virus is also found in the heart of healthy controls. Therefore, the question remains open whether and how cardiac HHV-6 may be of pathogenetic importance. Quantitative polymerase chain reaction or mRNA testing allows differentiation between low-level latent virus found in asymptomatic myocardium and active HHV-6 infection. Although only a small number of pediatric cases have been reported in literature, HHV-6 should be considered as a causative agent of inflammatory cardiomyopathy, especially in children under three who might be experiencing a primary infection. Future studies are needed to establish a threshold for determining active infection in biopsy samples and the role of coinfections other cardiotropic viruses.
  4,874 238 8
Speckle tracking evaluation of right ventricular functions in children with sickle cell disease
Osama Abd Rab Elrasol Tolba, Mohamed Ramadan El-Shanshory, Mohamed Abd Elaziz El-Gamasy, Walid Ahmed El-Shehaby
September-December 2017, 10(3):230-233
DOI:10.4103/apc.APC_140_16  PMID:28928607
Background: Cardiac dysfunction is a risk factor for death in patients with sickle cell disease (SCD). Aim of the Work: Aim of the work is to evaluate the right ventricular systolic and diastolic functions by tissue Doppler and speckling tracking imaging in children with SCD. Subjects and Methods: Thirty children with SCD and thirty controls were subjected to clinical, laboratory evaluations, and echocardiographic study using GE Vivid 7 (GE Medical System, Horten, Norway with a 3.5-MHz multifrequency transducer) including; Two-dimensional and tissue Doppler echocardiographic study (lateral tricuspid valve annulus peak E' velocity, lateral tricuspid valve annulus peak A' velocity, E'/A' ratio, isovolumetric relaxation time, lateral tricuspid valve annulus S' and septal S' waves and peak longitudinal systolic strain [PLSS] and time to PLSS) were done in six right ventricular segments. Results: There was a significant decrease in right ventricular systolic and diastolic function in patients group when compared to controls. Conclusions: Children with SCD have impaired right ventricular systolic and diastolic functions when compared to healthy children with early evaluation of the systolic dysfunction by speckle tracking imaging technique.
  4,256 219 2
Quantification of ventricular unloading by 3D echocardiography in single ventricle of left ventricular morphology following superior cavo-pulmonary anastomosis and Fontan completion – a feasibility study
Deepa Sasikumar, Bijulal Sasidharan, Baiju S Dharan, Arun Gopalakrishnan, Kavasseri M Krishnamoorthy, Sivasubramanian Sivasankaran
September-December 2017, 10(3):224-229
DOI:10.4103/apc.APC_12_17  PMID:28928606
Background: Three-dimensional echocardiography. (3DE) is comparable to cardiac magnetic resonance imaging for estimating ventricular volume in congenital heart diseases. However, there are limited data on estimation of ventricular volumes by 3DE in univentricular heart and change in ventricular volumes after surgical creation of cavopulmonary connection. We sought to quantify the unloading of the single ventricle of left ventricular. (LV) morphology by 3DE after superior cavopulmonary anastomosis. (SCPA) or Fontan operation over a period of 3 months and thereby derive a preliminary 3DE data set on this patient subset. Patients and Methods: Eighteen patients with functional single ventricle of LV morphology, who underwent SCPA or completion of Fontan circulation, were included in the study. Volume of the ventricle was estimated by 3DE before surgery and after surgery. (in the early postoperative phase and 3 months after surgery), and indexed end-diastolic volume. (EDV), end.systolic volume. (ESV), and ejection fraction. (EF) were derived. Results: Twelve patients underwent SCPA and six patients underwent staged completion of Fontan circulation. Before surgery, EDV was similar in both groups. There was a significant fall in EDV immediately after SCPA (from 48.3 ± 14.9 ml/m2 to 39.5 ± 12.3 ml/m2). However, EDV increased at 3 months' follow-up to 41.3 ± 10.5 ml/m2. There was no significant fall in EDV immediately after Fontan operation (47.2 ± 10.1 ml/m2−46.6 ± 14.2 ml/m2), but EDV continued to fall at 3 months of follow-up (44.7 ± 10. ml/m2). There was no significant change in ESV in either group, but EF fell significantly after SCPA. Conclusions: We provide preliminary information on 3DE volume data of single ventricle of LV morphology and the pattern of unloading after SCPA and Fontan operation. Immediate significant volume unloading occurred after SCPA which tended to catch-up after 3 months, whereas continued fall in ventricular volume with time was noted after Fontan.
  4,120 260 1
Initial experience with the 3.3 Fr Mongoose® pigtail catheter for aortic angiography during patent ductus arteriosus closure in small patients
Zachary Hena, Nicole J Sutton, Gregory J Gates, Benjamin H Taragin, Robert H Pass
September-December 2017, 10(3):240-244
DOI:10.4103/apc.APC_9_17  PMID:28928609
Background: Smaller femoral arterial sheaths may be associated with fewer vascular complications. The 3.3 Fr Mongoose® Pediavascular pigtail catheter is a catheter that allows higher flow rates, potentially resulting in improved angiographic quality. We reviewed our experience with this small catheter during patent ductus arteriosus (PDA) closure. Materials and Methods: Review of patients ≤20 kg in whom the Mongoose® catheter was used during PDA closure from 12/13 to 4/15. Angiographic efficacy and procedural details were compared to ten 4 Fr catheter cases. Comparisons were performed using Mann–Whitney U-test; P < 0.05 was statistically significant. Results: Twelve (9 female) patients were catheterized with a 3.3 Fr Mongoose®. Median weight 10.5 kg (range 6.4–18.2), height 81 cm (range 37–111), and body surface area (BSA) 0.47 m2 (range 0.33–0.75) were similar to ten patients (3 females) in the 4 Fr control group (P = NS); median weight 9.9 kg (range 6–16.8), height 80 cm (range 64–102), and BSA 0.46 m2 (range 0.31–0.74). Angiographic quality was subjectively adequate with both with no difference in the median pixel density between the two techniques (3.3 Fr: 76.7 [range 33.5–90] and 4 Fr: [70; 38–102]; P = NS). Contrast used was similar between the groups (3.3 Fr: median 4.2 ml/kg and 4 Fr: 4.9 ml/kg; P = NS). Median radiation dose was similar in the two groups (3.3 Fr: 28.1 mGy [range 17.2–38] and 4 Fr: 38 mGy [range 20.4–58.5]; P = NS). All ducts were closed at latest follow-up ( P = NS). No complications were encountered. Conclusions: The 3.3 Fr Mongoose® allowed similar angiography to the 4 Fr pigtail catheter, allowing safe and effective transcatheter PDA closure in small children.
  4,142 177 -
Intraocular pressure in children after congenital heart surgery: A single-center study
Sunali Goyal, Paul H Phillips, Lamonda A Corder, Michael J Robertson, Xiomara Garcia, Michael L Schmitz, Punkaj Gupta
September-December 2017, 10(3):234-239
DOI:10.4103/apc.APC_41_17  PMID:28928608
Background: The impact of varied cardiac physiologies on intraocular pressure (IOP) among children undergoing heart operations is unknown. Aim: The aim of this study was to determine the IOP among children with varying cardiovascular physiologies and varying hemodynamics after their heart operation. Setting and Design: This was a prospective, observational study. Materials and Methods: Patients ≤18 years undergoing congenital heart surgery were included in this study. IOP measurement was performed by Icare® tonometer between 3 and 14 days after heart operation. Statistical Analysis: Summary statistics were estimated for all demographic, anthropometric, and clinical data. Results: A total of 116 eyes from 58 children were included. The mean and standard deviation age was 28.4 (45.8) months. Single-ventricle anatomy was present in 26 patients (45%). Despite similar heart rate and blood pressure, the mean IOP among the patients with single-ventricle anatomy was significantly elevated as compared to patients with two-ventricle anatomy (18 mm Hg vs. 12 mm Hg, P < 0.001). There was no difference in IOP measurements based on the complexity of operation performed. We noted that patients undergoing surgical palliation with central shunt (21 mm Hg), Fontan operation (19 mm Hg), bidirectional Glenn operation (19 mm Hg), Norwood operation (19 mm Hg), or definitive repairs such as tetralogy of Fallot repair (17 mm Hg), and atrioventricular canal repair (19 mm Hg) were associated with the highest IOPs in the study cohort. Conclusions: This study demonstrates that IOPs vary with varying cardiovascular physiology after pediatric cardiac surgery.
  3,913 162 -
Chronic antepartum maternal hyperoxygenation in a case of severe fetal Ebstein's anomaly with circular shunt physiology
Alisa Arunamata, David M Axelrod, Katherine Bianco, Sowmya Balasubramanian, Amy Quirin, Theresa A Tacy
September-December 2017, 10(3):284-287
DOI:10.4103/apc.APC_20_17  PMID:28928616
Perinatal mortality remains high among fetuses diagnosed with Ebstein's anomaly of the tricuspid valve. The subgroup of patients with pulmonary valve regurgitation is at particularly high risk. In the setting of pulmonary valve regurgitation, early constriction of the ductus arteriosus may be a novel perinatal management strategy to reduce systemic steal resulting from circular shunt physiology. We report the use of chronic antepartum maternal oxygen therapy for constriction of the fetal ductus arteriosus and modulation of fetal pulmonary vascular resistance in a late presentation of Ebstein's anomaly with severe tricuspid valve regurgitation, reversal of flow in the ductus arteriosus, and continuous pulmonary valve regurgitation.
  3,901 164 4
Flecainide toxicity in a preterm neonate with permanent junctional reciprocating tachycardia
Balaganesh Karmegaraj, Danish Menon, Mukund A Prabhu, Balu Vaidyanathan
September-December 2017, 10(3):288-292
DOI:10.4103/apc.APC_31_17  PMID:28928617
We report a case of flecainide toxicity in a premature neonate with permanent junctional reciprocating tachycardia which was managed successfully by reversal of the sodium blockade with intravenous sodium bicarbonate and supportive care. This report highlights the importance of strict supervision and monitoring while administering antiarrhythmic drugs in neonates and prompt institution of appropriate remedial action for treatment when toxicity is suspected.
  3,607 194 2
Stenting of right ventricular outflow tract in Tetralogy of Fallot with subarterial ventricular septal defect: A word of caution
Jonathan Lee, Sivakumar Sivalingam, Mazeni Alwi
September-December 2017, 10(3):281-283
DOI:10.4103/apc.APC_168_16  PMID:28928615
We report a case of Tetralogy of Fallot with severe cyanosis who underwent a successful right ventricular outflow tract stenting. Follow-up echocardiography revealed moderate aortic regurgitation due to the impingement of the stent on the aortic valve. The patient underwent successful surgical correction at which time the stent was removed completely with a resolution of the aortic regurgitation.
  3,432 314 3
Change of guard: Introducing the new editor of the Annals of Pediatric Cardiology, Dr. KS Iyer
Raman Krishna Kumar
September-December 2017, 10(3):223-223
DOI:10.4103/apc.APC_114_17  PMID:28928605
  3,546 182 -
Does heart-type fatty acid-binding protein predict clinical outcomes after pediatric cardiac surgery?
Egmond S Evers, Varsha Walavalkar, Suresh Pujar, Latha Balasubramanian, Frits W Prinzen, Tammo Delhaas, Ward Y Vanagt, Shreesha Maiya
September-December 2017, 10(3):245-247
DOI:10.4103/apc.APC_33_17  PMID:28928610
Introduction: The early identification of vulnerable pediatric cardiac surgery patients can help clinicians provide them with timely support. Heart-type fatty acid-binding protein. (H-FABP) is an early biomarker of myocardial injury in acute myocardial infarction in adults. In this study, we evaluated the correlations between postoperative H-FABP, creatine kinase-myocardial band (CK-MB), troponin-I, total bypass time, and clinical outcomes. Methods: In 32 pediatric patients that underwent ventricular septal defect. closure we measured H-FABP, troponin-I and CK-MB preoperatively and 1, 3, and 6 h after aortic declamping. Spearman's Rho correlations were calculated between laboratory and clinical parameters including inotropic support duration, aortic cross-clamp time, total bypass time, ventilation.weaning.time, and total Intensive Care Unit stay. Results: H-FABP, CK-MB, troponin-I, and total bypass time have a similarly weak to moderate correlation with clinical outcome measures. Conclusions: The predictive value of H-FABP for clinical outcome is not stronger than that of CK-MB, Troponin-I, or bypass times.
  3,158 143 -
Central perforation of atretic pulmonary valve using coronary microcatheter
Saurabh Kumar Gupta, Rajnish Juneja, Anita Saxena
September-December 2017, 10(3):304-305
DOI:10.4103/apc.APC_72_17  PMID:28928622
Percutaneous perforation of pulmonary valve, using 0.014” guidewires meant for coronary artery chronic total occlusion (CTO), is increasingly being performed for select cases of pulmonary atresia with intact ventricular septum (PA-IVS). Despite growing experience, procedural failures and complications are not uncommon. Even in infants treated successfully, the orifice created in the atretic pulmonary valve is eccentric. In this report, we present usefulness of coronary microcatheter in alignment of perforating coronary guidewire to the center of atretic pulmonary valve resulting in central perforation.
  3,090 168 1
Hypoplastic left heart syndrome with coronary-cameral fistulas: Echocardiographic demonstration of coronary artery steal and successful interventional treatment
Arshid Mir, Mary Niu, Merick Yamada, Ed Overholt
September-December 2017, 10(3):306-309
DOI:10.4103/apc.APC_22_17  PMID:28928623
We report a case of a patient with hypoplastic left heart syndrome with significant coronary-cameral fistulas and exertional symptoms from coronary steal. Symptoms resolved following successful coil occlusion of his left ventricle.
  3,105 148 -
Anomalous origin of right pulmonary artery from innominate artery: Repair using pulmonary artery pedicled flap plasty
Roy Varghese, Jai Ganesh, Jeeva Nandam, Sujathaa Ravikumar, Sivakumar Kothandam
September-December 2017, 10(3):278-280
DOI:10.4103/apc.APC_144_16  PMID:28928614
Origin of the right pulmonary artery from innominate artery is an exceedingly rare anomaly. We report two cases with this anomaly that underwent surgical repair. The surgical technique described achieves tissue-to-tissue anastomosis using a pedicled flap from the main pulmonary artery.
  3,052 147 1
Congenital left atrial appendage aneurysm: Atypical presentation
Mehdi Bamous, Mahdi Aithoussa, Ayoub Abetti, Abdelatif Boulahya
September-December 2017, 10(3):293-294
DOI:10.4103/apc.APC_4_17  PMID:28928618
Congenital left atrial appendage aneurysm is a rare condition caused by dysplasia of the atrial muscles. We report a case of a 14-year-old boy, with a 5-month history of cough and in sinus rhythm. Transthoracic echocardiography and computerized tomographic angiography confirmed the aneurysm of the left atrial appendage which was resected through median sternotomy on cardiopulmonary bypass. This case is presented not only for its rarity but also for its atypical clinical presentation.
  3,016 157 2
Anomalous origin of the left brachiocephalic artery in the right aortic arch: Is there a method to the madness?
Mani Ram Krishna, Ganesh Kumar Gnanappa, Rachel Fitzpatrick, Julian Ayer, David Winlaw
September-December 2017, 10(3):301-303
DOI:10.4103/apc.APC_16_17  PMID:28928621
The anomalous origin of the left brachiocephalic artery in a right sided aortic arch is a rare vascular ring which might lead to esophageal compression. The exact embryological origin of this anomaly is still widely debated. We present an infant who presented with esophageal compression symptoms and review the various hypotheses about the embryological origin of this anomaly.
  2,974 147 -
Ductal aneurysm with postsubclavian coarctation of aorta in an adult
Dibya Ranjan Behera, Krishna Kumar Mohanan Nair, Bijulal Sasidharan
September-December 2017, 10(3):310-311
DOI:10.4103/apc.APC_165_16  PMID:28928624
We describe a case of ductal aneurysm in an adult patient with post subclavian coarctation of aorta, which is a very rare association.
  2,952 110 -
Staphylococcal endocarditis after chicken pox in a child with structurally normal heart
Dheeraj Deo Bhatt, Nihit Kharkwal, Dinesh Kumar Yadav
September-December 2017, 10(3):312-313
DOI:10.4103/apc.APC_17_17  PMID:28928625
  2,879 142 1
Consideration of pyloric stenosis as a cause of feeding dysfunction in children with cyanotic heart disease
Nayan T Srivastava, John J Parent, Marcus S Schamberger
September-December 2017, 10(3):298-300
DOI:10.4103/apc.APC_51_17  PMID:28928620
Feeding difficulty has been reported at a higher incidence in infants with cyanotic heart disease and single ventricle physiology necessitating specialized feeding strategies. However, structural causes of feed intolerance in this subset of patients should not be ignored. This case series highlights three recent cases of pyloric stenosis in infants with left-sided obstructive lesions at our institution. In all three cases, the initial presumed diagnosis was feeding intolerance related to heart disease, and there was significant clinical improvement following identification and correction of pyloric stenosis.
  2,873 135 -
Percutaneous management of complex acquired aortic coarctation in an adult with tetralogy of Fallot and pulmonary atresia
Michael D Seckeler, Emily Lawson, Brent J Barber, Scott E Klewer
September-December 2017, 10(3):295-297
DOI:10.4103/apc.APC_19_17  PMID:28928619
We present the case of a female adult with complex cyanotic congenital heart disease who had long-standing thoracic aortic obstruction due to scarring from earlier surgical procedures. She was symptomatic but felt to be too high risk for surgical intervention. With careful planning, she was able to undergo successful stenting of her aorta with subsequent clinical improvement. This case highlights some of the complexities of caring for adults with congenital heart disease and the importance of a thorough understanding of their anatomy and physiology and prior interventions before undertaking interventions.
  2,788 119 1
A pediatric echocardiographic Z-score nomogram for a developing country: Indian pediatric echocardiography Study – The Z-score
Mahmood Dhahir Al-Mendalawi
September-December 2017, 10(3):314-315
DOI:10.4103/apc.APC_23_17  PMID:28928626
  2,372 182 -