Annals of Pediatric Cardiology
About us | Current Issue | Archives | Ahead of Print | Instructions | Submission | Subscribe | Advertise | Contact | Reader Login
  My preferences 


Export selected to
Reference Manager
Medlars Format
RefWorks Format
BibTex Format
  Access statistics : Table of Contents
   2021| January-March  | Volume 14 | Issue 1  
    Online since January 16, 2021

  Archives   Previous Issue   Next Issue   Most popular articles   Most cited articles
Hide all abstracts  Show selected abstracts  Export selected to
  Viewed PDF Cited
Pulmonary arteriovenous malformations in children after the Kawashima procedure: Risk factors and midterm outcome
Ibrahim J Alibrahim, Mohammed H A Mohammed, Mohamad S Kabbani, Abdulraouf M Z. Jijeh, Omar R Tamimi, Abdullah A Alghamdi, Fahad Alhabshan
January-March 2021, 14(1):10-17
Background : Pulmonary arteriovenous malformations (PAVMs) are the major cause of progressive cyanosis in patients palliated with bidirectional cavopulmonary connection (BCPC). The aim of our study is to analyze the occurrence of PAVMs in patients after Kawashima procedure, to study the effect of total cavopulmonary connection (TCPC) on PAVMs, to evaluate the effect of axillary arteriovenous fistula (AAVF) creation on PAVMs, and to study the risk factors for PAVMs. Methods : In this retrospective cohort study, all patients with left isomerism and azygous continuation of an interrupted inferior vena cava who underwent Kawashima procedure from July 2001 to December 2017 were included. Results : Twenty.six patients after Kawashima procedure were included in our study. PAVMs were diagnosed in 12 patients (46%). Five of these 12 patients underwent TCPC with complete resolution of hypoxemia. Three patients underwent AAVF creation, 2 had complete resolution, while 1 had partial resolution of hypoxemia. Fourteen patients (54%) did not develop PAVMs. Nakata index below 267 mm2/m2 and McGoon ratio below 1.9 predicted the development of PAVMs with high sensitivity and specificity. Conclusions : PAVMs represent a serious complication in patients who undergo Kawashima procedure. Small size of pulmonary arteries is an important risk factor for the development of PAVMs. Resolution of hypoxemia after TCPC completion supports the hepatic factor hypothesis. Early TCPC completion in these patients may help to avoid the development of PAVMs by restoring the hepatic factor. Resolution of hypoxemia after AAVF creation may support the lack of pulsatile flow hypothesis.
  2,015 107 -
Melody valve to replace the mitral valve in small children: Lessons learned
Vilius Dranseika, Rene Pretre, Oliver Kretschmar, Hitendu Dave
January-March 2021, 14(1):35-41
Objective: Infants requiring mitral valve replacement have few viable options. Recently, stented bovine jugular vein graft (Melody) has been surgically implanted in such cases. Herein, we report our experience, elaborating on evolution of implantation technique, pitfalls, as well as long-term outcome (including late dilatability). Methods: Seven Melody valves were implanted (2013–2019). The median patient age and weight were 6.7 (1.8–30.5) months and 5.8 (4.6–9.5) kg, respectively. The indications for implantation were mitral stenosis and/or regurgitation postatrioventricular septal defect (AVSD) repair (5), congenital mitral valve dysplasia (1), and Shone's complex (1). Operative technique involved shortening the valve and creating a neo-sewing ring at 2/3 (atrial)–1/3 (ventricular) junction. Implantation was followed by intraoperative balloon dilatation. Results: Five out of seven patients survived the perioperative period (one death due to technical failure and the other due to acute respiratory distress syndrome postcardiopulmonary bypass). Two out of five medium-term survivors got transplanted (1) or died due to acute myeloid leukemia (1). No valves were replaced. The mean echo gradient at discharge was a median 4 (2–6) mmHg. None of the patients showed left ventricular outflow tract or pulmonary venous obstruction. Two Melody valves were dilated late (5 months and 3 years postoperatively), resulting in decreasing mean gradients from 6 to 1 and from 17 to 4 mmHg. At last follow-up, surviving Melody had a mean gradient of 4 (1–9) mmHg. Conclusions: Mitral valve replacement with a Melody valve is feasible in infants, is reproducible, shows good immediate results, and offers the possibility of later dilatation. This technique offers a better solution compared to the existing alternatives for infants requiring a prosthetic mitral valve.
  1,739 100 -
Comparison of right ventricular outflow tract gradient under anesthesia with post-operative gradient in patients undergoing tetralogy of Fallot repair
Dheemta Toshkhani, Virendra Kumar Arya, Kamal Kajal, Shyam K S Thingnam, Sandeep Singh Rana
January-March 2021, 14(1):18-25
Background : Intra-cardiac repair for tetralogy of Fallot has some degree of residual right ventricular outflow tract (RVOT) obstruction. However, the measurement of this gradient intra-operatively might get affected by the depth of anesthesia which is important for the long-term outcome. Aims : The primary aim was to compare intraoperative RVOT gradient post repair under two different anesthetic depths of 1% and 2% end-tidal sevoflurane. The secondary objective was to follow up the changes in RVOT gradient till 1 month postoperatively. Design : Observational study. Setting : Advanced Cardiac Centre of PGIMER, Chandigarh. Methods : Following intracardiac repair, RVOT gradient was measured directly by placing needle into the right ventricle and pulmonary artery at sevoflurane 1%, and subsequently, at 2% end.tidal concentration while maintaining hemodynamic stability. These gradients were also measured using transesophageal echocardiography (TEE) ( NCT03234582). Results : Twenty-one patients were included in this study that had intra-cardiac repair, of which pulmonary annulus was preserved for 15 cases. Mean RVOT gradients measured invasively and by TEE at end-tidal sevoflurane concentration of 1% and 2% were not significantly different (6.67 ± 4.16 mmHg vs. 6.76 ± 3.82 mmHg, P > 0.05 invasively and 13.01 ± 7.40 mmHg vs. 12.53 ± 7.11 mmHg, P > 0.05 by TEE, respectively). RVOT gradient measured by trans-thoracic echocardiography (TTE) postoperatively at the time of extubation and during follow-up at 1 month showed significant reduction (11.37 ± 6.00 mmHg, P < 0.05 and 9.23 ± 4.92 mmHg, P < 0.01 respectively). Six patients who underwent repair with transannular patch had significant pulmonary regurgitation (PR) following surgery, with no significant change in PR severity or RVOT gradient on increasing anesthetic depth. Conclusions : Postoperative RVOT gradient was not altered by changing depth of anesthesia provided systemic blood pressure was maintained. One month postrepair RVOT gradients were significantly reduced as compared to the intraoperative values.
  1,659 107 -
Utility of late pulmonary artery banding in single-ventricle physiology: A mid-term follow-up
Aritra Mukherji, Sanjiban Ghosh, Nihar Pathak, Jayita Nandi Das, Nilanjan Dutta, Debasis Das, Amitabha Chattopadhyay
January-March 2021, 14(1):26-34
Background: The standard first stage palliation for univentricular heart with unrestricted pulmonary blood flow (PBF) is surgical pulmonary artery (PA) banding for which the ideal age is within the first 8 weeks of life. This study aimed to look for the utility of PA band done beyond 3 months of age for patients presenting beyond the stipulated period. Materials and Methods: This is a retrospective analysis of the outcome of twenty patients with single ventricle (SV) physiology with unrestricted PBF who presented late and were selected on the basis of clinical, radiological, and echocardiographic parameters for PA banding. Results: The median age of the patients was 5.5 months (3.5–96 months), and the median body weight was 4.7 kg (3.2–22.0 kg). The patients were divided into three groups as follows: ten patients between 3 and 6 months of age (Group A), seven patients between 6 months to 1 year of age (Group B), and three patients > 1 year of age with additional features of pulmonary venous hypertension (Group C). The mean reduction of PA pressures following PA band was 60.9%, 48.8%, and 58.3% and the mean fall in oxygen saturation was 10.4%, 8.0%, and 6.6% in the three groups, respectively. The postoperative mortality rate was 10%. The mean follow up duration was 13.5 months (7–23 months). There was a statistically significant improvement in weight for age Z scores following PA band (P = 0.0001). On follow up cardiac catheterization, the mean PA pressures were 16.6 (±3.6), 22.7 (±5.7), and 33.3 (±12.4) mmHg, respectively, in the three groups, and the mean pulmonary vascular resistance index was 1.86 (±0.5), 2.45 (±0.7), and 3.5 (±1.6) WU.m2, respectively. Subsequently, seven patients in Group A, three patients in Group B, and one patient from Group C underwent successful bidirectional Glenn (BDG) surgery. Conclusions: Late PA band in selected patients with SV physiology can have definite benefit in terms of correction of heart failure symptoms and subsequent conversion to BDG and can potentially change the natural history of disease both in terms of survival and quality of life.
  1,648 117 -
Use of selexipag in a teenage patient with pulmonary arterial hypertension
Nathalie Jeanne Magioli Bravo-Valenzuela, Flavia Navarro, Socrates Pereira Silva
January-March 2021, 14(1):75-78
Pulmonary arterial hypertension (PAH) is a chronic, progressive, multifactorial disease. Currently, combination therapy is an attractive option for PAH management because three pathways (endothelin, nitric oxide, and prostacyclin) are involved in this disease. Selexipag is a novel oral prostacyclin pathway drug and is a highly selective IP prostacyclin receptor agonist with vasodilatory and antiproliferative effects. We report the case of a teenage patient with idiopathic PAH who presented in World Health Organization functional Class IV and showed no clinical improvement with dual therapy. We added oral selexipag to the treatment regimen and observed substantial improvement in her quality of life at the short-time follow-up. Despite the lack of childhood data regarding the use of selexipag in pediatric patients with PAH, the use of this drug in the current teenage patient improved her quality of life and exercise capacity.
  1,345 77 -
Defining transposition: What have we learnt?
Max E Roderick, Nikita Maria, Adrian Crucean, John Stickley, David J Barron, Robert H Anderson
January-March 2021, 14(1):63-66
Understanding transposition is important for all who hope to effectively treat patients with the condition. The variants of the condition are frequently debated in the literature. We describe an unusual variant of transposition, in which despite the arterial roots being supported by morphologically inappropriate ventricles, the roots themselves were normally related, with the intrapericardial arterial trunks spiraling as they extended into the mediastinum. The specimen was identified following the re-categorization of our archive, and we subsequently conducted a detailed analysis of the underlying morphology. Using the principles of sequential segmental analysis, we compared the morphology with standard examples previously described. We show how it was the recognition of such hearts that promoted that concept that the combination of connections across the atrioventricular and ventriculo-arterial junctions was the essence of transposition. In the most common variant, the arrangements are concordant at the atrioventricular junctions, but discordant at the ventriculo-arterial junctions. We suggest that the overall arrangement of discordant ventriculo-arterial connections is best described simply as “transposition.” When the discordant ventriculo-arterial connections are combined with similarly discordant connections at the atrioventricular junctions, the transposition is congenitally corrected. We point out that the use of “d” and “l” as prefixes does not distinguish between transposition and its congenitally corrected variant. For those using segmental notations, the correct description for the rare variant found in the setting of a posteriorly located aortic root with the usual atrial arrangement is transposition (S, D, NR).
  1,321 71 -
Tetralogy of fallot with coronary crossing the right ventricular outflow tract: A tale of a bridge and the artery
Sachin Talwar, Sanjoy Sengupta, Supreet Marathe, Pradeep Vaideeswar, Balram Airan, Shiv Kumar Choudhary
January-March 2021, 14(1):53-62
A coronary artery crossing the right ventricular outflow tract is a subset of a larger pathomorphological cohort known as an anomalous coronary artery (ACA) in the tetralogy of Fallot (TOF). The best possible outcome in a patient with TOF and ACA is decided by judicious selection of optimum preoperative investigative information, the timing of surgery, astute assessment of preoperative surgical findings, and appropriate surgical technique from a wide array of choices. In most instances, the choice of surgical technique is determined by the size of the pulmonary annulus and the anatomical relation of ACA to the pulmonary annulus. In the present era, complete, accurate preoperative diagnosis and primary repair is a routine procedure with strategies to avoid a right ventricle-to-pulmonary artery conduit.
  1,212 144 -
Use of strain, strain rate, tissue velocity imaging, and endothelial function for early detection of cardiovascular involvement in young diabetics
Atul Kaushik, Aditya Kapoor, Preeti Dabadghao, Roopali Khanna, Sudeep Kumar, Naveen Garg, Satyendra Tewari, Pravin K Goel, Archana Sinha
January-March 2021, 14(1):1-9
Background: Subtle structural and functional changes may precede the onset of overt global left ventricular (LV) dysfunction. Data pertaining to tissue velocity imaging (TVI)and strain imaging to assess regional myocardial function and flow mediated vasodilatation are limited in young patients with diabetes. Materials: Conventional echocardiography, TVI parameters along with strain (S), and strain rate (SR) were measured in 50 young diabetics (15.16 ± 2.95 years, mean HBA1c 8.15 ± 1.37 g %) and 25 controls (15.60 ± 2.51 years). Flow-mediated dilation (FMD), nitrate--mediated dilatation (NMD), and carotid intima–media thickness were also assessed. Results: Conventional echocardiography parameters were similar in patients and controls; however, deceleration time of the mitral inflow velocity (early deceleration time) was significantly shorter in patients when compared with controls (149.06 ± 31.66 vs. 184.56 ± 19.27 ms, P =0.001). Patients had lower strain values at the basal lateral LV (21.39 ± 4.12 vs. 23.78 ± 2.02; P =0.001), mid-lateral LV (21.43 ± 4.27 vs. 23.17 ± 1.92 P =0.02), basal septum (20.59 ± 5.28 vs. 22.91 ± 2.00; P = 0.01), and midseptum (22.06 ± 4.75 vs. 24.10 ± 1.99; P = 0.01) as compared to controls. SR at the basal and midsegments of the lateral LV wall and at the basal septum was also significantly lower in diabetic patients. Diabetic children also had endothelial dysfunction with significantly lower FMD (8.36 ± 4.27 vs. 10.57 ± 4.12, P = 0.04). Conclusions: LV strain indices and flow--mediated dilatation are impaired in asymptomatic children and adolescents with type 1 diabetes mellitus despite absence of overt heart failure and normal ejection fraction. Early detection of subclinical regional myocardial dysfunction by deformation analysis including strain and strain rate may be useful in the asymptomatic diabetic population.
  1,240 109 -
Re-entrant ventricular tachycardia in a postoperative case of tetralogy of Fallot – Ablated successfully under the three-dimensional mapping system
Suresh Kumar Paidi, Krishna Kumar Mohanan Nair, Narayanan Namboodiri, Sridhar Balaguru, Ajitkumar Valaparambil
January-March 2021, 14(1):107-112
A 47-year-old female underwent cardiac repair for tetralogy of Fallot at the age of 12 years. Subsequently, she was asymptomatic on follow-up. Recently, she presented elsewhere with palpitations and presyncope with documented ventricular tachycardia (VT) having left bundle branch block morphology with inferior QRS axis and late precordial transition. She was reported to have cardioverted and referred to our center for electrophysiology study (EP). She underwent EP study which induced clinical VT which was hemodynamically stable and the mechanism of VT was confirmed as re-entry. With the help of three-dimensional mapping system, VT circuit was identified in the posterior right ventricular outflow tract region between the pulmonary valve and upper end of ventricular septal defect patch. Delivery of radiofrequency energy during VT terminated the tachycardia with no further inducible VT despite aggressive pacing protocols.
  1,228 57 -
Massive ductal aneurysm in an asymptomatic child with Loeys-Dietz syndrome
Saira Siddiqui, Antonio Polanco, Michael P DiLorenzo, Amee Shah, Michael Snyder, Harry Dietz, Emile Bacha, Kanwal Majeed Farooqi
January-March 2021, 14(1):113-115
An asymptomatic 3-year-old with Loeys-Dietz Syndrome (LDS) followed for a small patent ductus arteriosus and dilated aorta was found to have a massive ductal aneurysm on routine surveillance cardiac magnetic resonance. The aneurysm was successfully resected. Serial advanced imaging tools are useful in surveillance, diagnosis, and management in patients with LDS.
  1,080 59 -
Virtual modeling and interactive virtual reality display of unusual high-riding cervical aortic arch
Sarv Priya, Prashant Nagpal
January-March 2021, 14(1):122-124
A cervical aortic arch is a rare vascular malformation that is characterized as a high positioned aortic arch, above the clavicle. The knowledge of its branching pattern is essential to characterize the entity further accurately. Noninvasive cross-sectional imaging, including computed tomography angiography or magnetic resonance angiography, is the imaging methods of choice. Due to highly complex anatomy, three-dimensional (3D) images help in providing improved anatomical visualization. Virtual reality is a relatively new computer-generated simulation technique that allows the interactive display of high-resolution models using a wearable headset and interactive controllers. We describe a rare form of a cervical arch and briefly discuss the latest methods of improved visualization using 3D virtual reality displays and smartphones.
  1,094 45 -
Persistent atypical atrial flutter after device closure of the atrial septal defect in a young man
Keerthika Ravella, Shrikant Bhoskar, Neeta Bachani, Yash Lokhandwala, Bharat Dalvi
January-March 2021, 14(1):79-81
Atrial flutter is uncommon in young patients with uncorrected atrial septal defect (ASD). Although rare, it has been reported in the younger population following device closure of ASD/patent foramen ovale. We describe a case of persistent atypical atrial flutter following device closure of ASD in a young man and discuss the management strategy given the various underlying dilemmas.
  1,048 79 -
Video-assisted thoracoscopic pacemaker lead placement in children with atrioventricular block
Sergey Termosesov, Ekaterina Kulbachinskaya, Ekaterina Polyakova, Dmitriy Khaspekov, Ivan Grishin, Vera Bereznitskaya, Maria Shkolnikova
January-March 2021, 14(1):67-71
Background: The pacemaker lead placement is presented as one of the most appropriate procedures in children with a complete atrioventricular block (AVB). Despite the fact that video-assisted thoracic surgery (VATS) for epicardial lead placement has demonstrated positive results as to the feasibility, safety, and efficacy in adults, its role in pacemaker implantation in children remains unclear. Aim: This study sought to assess the intermediate-term outcomes of video-assisted thoracoscopic pacemaker lead placement in children with complete AVB Materials and Methods: From May 2017 to November 2019, five children with complete AVB underwent minimally invasive left ventricular (LV) lead placements via thoracoscopic video assistance approach. The procedure was performed under complex intratracheal anesthesia with single-lung ventilation, all pacing parameters were evaluated in perioperative and follow-up periods. Results: The median age of children at implantation was 3 years (range: 2 to 4 years), the median weight was 13 kg (range: 12–15 kg). All procedures were completed successfully, pacing thresholds for the active lead measured 0.3-1.1V, with R-wave amplitude of 8-18 mV and impedance of 560-1478 Ohm. Conclusion: Thoracoscopic pacemaker lead placement may provide a potential alternative to the transthoracic approach of epicardial lead placement in children with AVB.
  1,014 81 -
Balloon pulmonary valvotomy in severe dysplastic pulmonary valve restenosis: A novel maneuver for stabilizing balloon positioning
Palanivel Rajan, Pruthvi C Revaiah, Parag Barwad
January-March 2021, 14(1):116-118
Balloon pulmonary valvuloplasty (BPV) is an interventional procedure that has practically replaced the need of open-heart surgery in patients with severe valvular pulmonary stenosis. However, BPV in dysplastic pulmonary valve (PV) still remains a challenge in the current milieu of advanced technologies. We encountered a unique situation of severe restenosis of PV wherein the attempt to balloon dilate the valve was futile because no balloon remained stable at the level of the valve. This situation was overcome using a PDA device delivery sheath to keep the balloon positioned at the desired location.
  1,024 57 -
Very late trans-catheter recruitment of congenitally “absent” pulmonary artery
Alessandra Pizzuto, Giovanbattista Calabri, Magdalena Cuman, Giuseppe Santoro
January-March 2021, 14(1):130-131
  1,025 52 -
Congenital long QT syndrome and patent ductus arteriosus: A rare surgical scenario
Javid Raja, Sabarinath Menon, Devarakonda Bhargava Venkata, KP Unnikrishnan, Narayanan Namboodiri
January-March 2021, 14(1):85-87
Congenital long QT syndrome (LQTS) is a rare cardiac condition characterized by abnormality of either sodium or potassium ion channels resulting in prolongation of QT interval and thereby predisposing to life-threatening arrhythmia. Once the syndrome is diagnosed, measures should be taken to avoid sudden cardiac death. We present a rare case of LQTS associated with patent ductus arteriosus in a child, and a unique approach was used in managing both conditions.
  1,008 64 -
Radiofrequency ablation for fascicular ventricular tachycardia causing tachycardiomyopathy and brief literature review
Sanjeev S Mukharjee, Soumya Kanti Mohapatra, Aritra Mukherji, Arnab De, Anil Kumar Singhi
January-March 2021, 14(1):99-104
A 10-years-old boy presented with a history of effort intolerance and palpitations for 4 months. His electrocardiogram showed wide complex tachycardia suggestive of fascicular ventricular tachycardia (VT). The echocardiogram showed moderate-to-severe left ventricular systolic dysfunction without any structural lesion. The tachycardia was unresponsive to adenosine and direct current cardioversion. It responded to oral verapamil. The electrophysiology study confirmed the tachycardia as left posterior fascicular VT. The tachycardia was successfully ablated guided by Purkinje potential on three-dimensional mappings. He showed improvement in ventricular functions before discharge. He is doing well on short-term follow-up.
  980 76 -
Clinical profiles and risk factors for early and medium-term mortality following heart transplantation in a pediatric population: A single-center experience
Komarakshi R Balakrishnan, Kemundel Genny Suresh Rao, Ganapathy Krishnan Subramaniam, Murali Krishna Tanguturu, Ajay Arvind, Veena Ramanan, Jagdish Dhushyanthan, K Ramasubramanian, K Suresh Kumaran, Gunalan Sellamuthu, Mohan Rajam, Senthil Mettur, Pradeep Gnansekharan, Ravikumar Ratnagiri
January-March 2021, 14(1):42-52
Aims and Objectives: There is a paucity of data regarding the outcomes of Heart transplantation in children from the Indian subcontinent. The data of patients under the age of 18 undergoing an isolated heart transplantation was analyzed for patient clinical profiles and risk factors for early and medium-term mortality. Hospital mortality was defined as death within 90 days of transplantation and medium-term survival as follow up of up to 6 years. Materials and Methods: A total of 97 patients operated between March 2014 and October 2019 were included in this study .Data was collected about their INTERMACS status, pulmonary vascular resistance, donor heart ischemic times, donor age, donor to recipient weight ratio and creatinine levels. Results: The age range was from 1 to 18 with a mean of 10.6 ± 4.6 years. 67 % patients were in INTERMACS category 3 or less.12 children were on mechanical circulatory support at the time of transplant. The 90 day survival was 89 %.The risk factors for hospital mortality was lower INTERMACS category (odd’s ratio 0.2143, P = 0.026), elevated creatinine (odd’s ratio 5.42, P = 0.076) and elevated right atrial pressure ( odd’s ratio 1.19, P = 0.015).Ischemic time, pulmonary vascular resistance (PVR) and PVR index (PVRI) had no effect on 90 day survival. Kaplan Meier estimates for 5 year survival was 73 %.The medium term survival was affected by INTERMACS category (Hazard ratio 0.7, P = .078) , donor age > 25 ( Hazard ratio 1.6, P = 0.26) and raised serum creatinine values.(Hazard ratio 2.7, P = 0.012). All the survivors are in good functional class. Conclusions: Excellent outcomes are possible after heart transplantation in a pediatric population even in a resource constrained environment of a developing economy. More efforts are needed to promote pediatric organ donation and patients need to be referred in better INTERMACS category for optimal outcomes.
  948 86 -
Iatrogenic pericardial defect
Brett J Murray, Jacob B Diamond, Robert F English
January-March 2021, 14(1):105-106
Pericardial defects are uncommon, usually congenital, and frequently involve a complete absence of the pericardium. Partial pericardial defects are more likely to result in complications. Iatrogenic pericardial defects are usually partial defects and may present with cardiac strangulation. We present the case of an iatrogenic pericardial defect in an asymptomatic 20-year-old female.
  981 49 -
Pericardial agenesis
Paula Concejo Iglesias, Elisa Aguirre Pascual, Leticia Albert de la Torre, David Coca Robinot, Belén Toral Vázquez, Miguel A Granados Ruiz
January-March 2021, 14(1):119-121
Congenital pericardial defect is a rare and usually asymptomatic condition which is classified incomplete or partial. Up to 70% of cases consist of complete absence of left pericardium. The diagnosis may be challenging due to its low frequency and absence of correlation with any specific finding on the clinical examination. Cardiac magnetic resonance imaging is the gold standard imaging technique for the diagnosis confirming the absence of pericardium, although other indirect signs may be seen. In partial defects, surgery is the treatment option. We present an incidental finding of total agenesis of the left pericardium in an asymptomatic 16-year-old male diagnosed in a preoperative assessment of a bone fracture.
  950 45 -
Staged interventional solution for a diagnostic dilemma caused by hypoplastic left ventricle with severe aortic arch hypoplasia
Santosh Wadile, Kothandam Sivakumar
January-March 2021, 14(1):95-98
Duct dependent aortic arch obstruction with borderline left ventricular hypoplasia presents a diagnostic dilemma. If the left ventricle (LV) is adequate to sustain systemic cardiac output without a patent duct, arch obstruction is relieved surgically and duct is divided. Inadequate LVs do not tolerate duct division, and these patients need more complex Norwood type surgeries. However, catheter-based interventions for arch obstruction can retain ductal patency. The progressive changes in anatomy and physiology of a neonate who presented with hypoplastic left heart structures and patent duct with advancing age and modified by serial catheter-based interventions are presented to highlight the use of interventions in this therapeutic dilemma.
  931 53 -
DiGeorge syndrome and anomalous right aortic arch with arch-on-arch and figure-of-eight configurations: Aortic sac maldevelopment and left brachiocephalic artery abnormal remodeling
Elaheh Malakan Rad, Sara Momtazmanesh
January-March 2021, 14(1):125-127
We report a 6-month-old female infant with deletion of chromosome 22q11.2 (DiGeorge/VFS TUPLE 1), normal atrial arrangement with concordant atrioventricular connection, pulmonary atresia, large subaortic ventricular septal defect, diminutive native pulmonary arteries, a characteristic weird-shape right aortic arch with arch-on-arch appearance and figure of 8 configuration. We presented the cardiac computed tomographic angiographic and cardiac angiographic features. Using Autodesk 3ds Max 2018 software, we explained and illustrated the speculative embryologic etiology of this bizarre aortic archanomaly with the extensive abnormal remodeling of the left brachiocephalic artery, based on a “five-embryonic aortic arches” concept. As to the best of the authors' knowledge, this is the first report of a genetically confirmed case of DiGeorge syndrome and an exceedingly rare type of right aortic arch anomaly with embryologic explanation according to the “five-embryonic-aortic-arches” concept. It seems that the constellation of pulmonary atresia, bizarreshaped right aortic arch due to abnormal development of the aortic sac, and abnormal remodeling of the left brachiocephalic artery may be strongly suggestive of DiGeorge syndrome.
  922 57 -
Catheter closure of a patent vertical vein after repair of an unusual total anomalous variant of pulmonary venous drainage
Paolo Ferrero, Isabelle Piazza, Nageswara Rao Koneti, Matteo Ciuffreda
January-March 2021, 14(1):82-84
Total anomalous pulmonary venous drainage (TAPVD) encompasses a wide spectrum of anatomical variants. The infradiaphragmatic type is almost always obstructive owing to the interposition of intrahepatic resistances; since almost invariable, the vertical vein inserts on the portal venous system. On correction of this variant, the vertical vein might not be ligated to avoid postoperative pulmonary hypertension. We hereby describe an unusual case of infradiaphragmatic TAPVD, with a vertical vein connected to ductus venosus. Since vertical vein was not ligated, it realized an unrestrictive pathway between the left atrium and the suprahepatic veins which resulted in persistent chylous peritoneal drainage. The patient successfully underwent catheter occlusion of the vertical vein which led to complete resolution of the clinical picture.
  891 54 -
Transcatheter treatment of an unusual coronary arteriovenous malformation with a fistulous sac in the interventricular septum
Anupama Nair, Kshitij Sheth, Rahul Sheth, Bharat Dalvi
January-March 2021, 14(1):91-94
Coronary artery fistula (CAF) is a rare congenital anomaly with a reported incidence of 0.2%–0.6%. There is a wide variation in the clinical presentation depending on the size and the site of the fistula. Transcatheter closure is currently the treatment of choice in these patients. We report a case of CAF with an unusually large fistulous sac within the interventricular septum. The fistula had connections with all the three major coronary arteries, namely, left anterior descending (LAD), left circumflex , and right coronary arteries but did not have any exit resulting in to and fro movement of blood within the sac and the feeding vessels. The patient was managed successfully by transcatheter coil embolization.
  880 58 -
Overdrive suppression of postoperative sustained ventricular tachycardia by atrial pacing and its hemodynamic effect
Mohammed Sameer, Sudip Dutta Baruah, Sabarinath Menon, Baiju Sasi Dharan, Sowmya V Ramanan, Javid S Raja, Narayanan Namboodiri
January-March 2021, 14(1):88-90
Sustained ventricular tachycardia (VT) in the early postoperative period following intracardiac repair for tetralogy of Fallot is rare. In stable VT, amiodarone forms the mainstay of management. However, where amiodarone and other antiarrhythmic drugs are contraindicated, suppressive overdrive atrial pacing can be used as a safe and efficient alternative to maintain cardiac output. We present a case of 1-year 5-month-old child who developed VT with low cardiac output syndrome with deranged hepatic function, who was managed efficiently using suppressive atrial pacing to ameliorate the effects of sustained VT.
  866 71 -
Myocardial infarction due to thrombosis of native aorta late after Fontan procedure for hypoplastic left heart syndrome
Bhavi Patel, Tacy Downing, Lourdes Prieto, Darline Santana-Acosta
January-March 2021, 14(1):72-74
Patients with single ventricle physiology who have undergone the Fontan procedure are at risk for long-term complications such as thrombus formation. Thrombus formation in the native aortic root (NAR) can be life-threatening if retrograde filling of the coronary arteries (CAs) is impaired. We present three cases of NAR thrombus with embolization to the CA, resulting in myocardial infarction. Thromboembolic events are well-documented complications of Fontan physiology, and optimal prevention and treatment strategies are poorly defined.
  828 70 -
The slow-flat-slow sequence in malignant vasovagal syncope
Guy Vaksmann, Ivan Bouzguenda, Marie-Dominique Lamblin
January-March 2021, 14(1):128-129
We present the electroencephalographic and electrocardiographic tracing obtained in an 8-year old boy who experienced malignant vasovagal syncope during the recording. This tracing illustrates the highly specific “slow-flat-slow” sequence described in cases of syncope induced by severe cerebral hypoperfusion.
  816 46 -
A shining star of congenital heart surgery – A tribute to Dr. William I Norwood Jr (1941–2020)
Smruti Ranjan Mohanty
January-March 2021, 14(1):132-133
  394 51 -