Annals of Pediatric Cardiology
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September-December 2019
Volume 12 | Issue 3
Page Nos. 191-353

Online since Wednesday, August 21, 2019

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INVITED EDITORIAL  

Artificial intelligence in pediatric cardiology and cardiac surgery: Irrational hype or paradigm shift? Highly accessed article p. 191
Anthony C Chang
DOI:10.4103/apc.APC_55_19  PMID:31516273
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ORIGINAL ARTICLES Top

Comparison of antistreptolysin O and anti-deoxyribonucleic B titers in healthy children to those with acute pharyngitis, acute rheumatic fever, and rheumatic heart disease aged 5–15 years Highly accessed article p. 195
Navjot Saini, Dinesh Kumar, Swarnim Swarnim, Dheeraj Bhatt, Sunil Kishore
DOI:10.4103/apc.APC_60_18  PMID:31516274
Background: Acute rheumatic fever (ARF) affects millions of children in the third world countries like India. The diagnosis of rheumatic fever is based on the Jones criteria with serological titers, antistreptolysin O titer (ASO), and anti-deoxyribonucleic B (ADB), taken as evidence of recent streptococci infection. There is a lack of recent data available on ASO and ADB titers in children from the Delhi/NCR and thus adequate geographical area-specific cutoffs for the region are not available. Aims and Objectives: The aim of this study is to determine and compare the ASO and ADB antibody titers in children with acute pharyngitis, ARF, rheumatic heart disease (RHD), and in healthy children of the Delhi/NCR region. Materials and Methods: Twenty-six cases of ARF, 51 cases of RHD, 50 cases of acute pharyngitis, and 84 healthy normal children were included in the study. A single ASO and ADB titer measurement was done in these children. Results: The ASO titers was raised in acute pharyngitis – 303 IU/ml (interquartile range [IQR], 142–520 IU/ml) and ARF – 347.5 IU/ml (IQR, 125–686 IU/ml) children in comparison to healthy controls – 163.5 IU/ml (IQR, 133–246.5 IU/ml) and RHD patients – 163 IU/ml (IQR, 98.250–324.500). The ADB titers were highest in ARF patients – 570.5 IU/ml (IQR, 276–922 IU/ml) followed with RHD – 205 IU/ml (IQR, 113.6–456.5), healthy controls – 78.25 IU/ml (IQR, 53.39–128.15 IU/ml), and acute pharyngitis – 75.12 IU/ml (IQR, 64.5–136 IU/ml). The upper limit of normal (ULN) values of ASO and ADB computed from normal healthy children were 262.4 IU/ml and 134.44 IU/ml, respectively, and these can be used as cutoff values for recent streptococcal infection in this geographical area. Conclusions: The median ASO titers in acute pharyngitis group and ARF were significantly raised compared to that of the control group. The ADB titers were raised in ARF and RHD patients albeit the levels were higher in ARF patients. The derived ULN values can be used as cutoff reference.
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Different habitus but similar electrocardiogram: Cardiac repolarization parameters in children – Comparison of elite athletes to obese children p. 201
Christian Paech, Janina Moser, Ingo Dähnert, Franziska Wagner, Roman Antonin Gebauer, Toralf Kirsten, Mandy Vogel, Wieland Kiess, Antje Körner, Bernd Wolfarth, Jan Wüstenfeld
DOI:10.4103/apc.APC_90_18  PMID:31516275
Introduction: The standard 12-lead electrocardiogram (ECG) remains a widely used tool in the basic cardiac evaluation of children and adolescents. With the emergence of inherited arrhythmia syndromes, the period of cardiac repolarization has been the focus of attention. So far, data on cardiac repolarization and its normal variants in healthy children are scarce. This may cause uncertainties in the differentiation between pathologies and normal variants. As abnormal autonomic regulation seems to be a major influencing factor on cardiac repolarization, this study aimed to evaluate the parameters of cardiac repolarization of children in extremely good physical shape to obese children to improve knowledge about cardiac repolarization in these subgroups of pediatric patients that are vastly affected by the alterations of autonomic regulation. Methods: A total of 426 pediatric volunteers (84 lean, healthy controls; 130 obese healthy pediatric volunteers; and 212 elite athletes) were enrolled in the study, and the parameters of cardiac repolarization were determined in 12-lead ECG. Results: Most importantly, there were no pathological findings, neither in the healthy controls nor in the obese or athletes. Athletes showed overall shorter corrected QT intervals than children from the other groups. This is also true if a correction of the QT interval is performed using the Hodges formula to avoid bias due to a tendency to lower heart rates in athletes. Athletes showed the shortest Tpeak-to-end ratios between the groups. The comparison of athletes from primarily strength and power sports versus those from endurance sports showed endurance-trained athletes to have significantly longer QT intervals. Conclusions: This study suggests that neither obesity nor extensive sports seems to result in pathological cardiac repolarization parameters in healthy children. Therefore, pathology has to be assumed if abnormal repolarization parameters are seen and might not be simply attributed to the child's habitus or an excellent level of fitness.
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Percutaneous closure of patent ductus arteriosus with the Nit-Occlud® patent ductus arteriosus device in 268 consecutive cases p. 206
Andrii V Maksymenko, Yulia L Kuzmenko, Arkadii A Dovhaliuk, Oleksandra O Motrechko, Florian E Herrmann, Nikolaus A Haas, Anja Lehner
DOI:10.4103/apc.APC_151_18  PMID:31516276
Background: The pfm Nit-Occlud® patent ductus arteriosus (PDA) device is well established for interventional closure of PDA. However, there are still limited data concerning its efficacy and follow-up in larger patient groups. Aims: This study aimed to evaluate the safety and efficacy of the Nit-Occlud® PDA device, implanted both through transpulmonary and transaortic approach, in a large cohort. Methods: From July 2008 to December 2015, 268 consecutive patients were admitted for transcatheter closure of a PDA and were treated with the Nit-Occlud® coil. Clinical, echocardiographic, and angiographic data were evaluated. Results: The median age was 5.2 years (range, 5 months to 62 years), and the median weight was 19.3 kg (range: 5.5–97 kg). Ten (3.7%) patients had weight <10 kg. The most common ductus types treated were Krichenko Type E and A (44.0% and 33.2%, respectively). Twelve (4.5%) patients were treated for residual shunting after surgical PDA closure. The median diameter at the narrowest point was 1.5 mm (range: 0.4–4 mm), the median size of the ampulla was 5 mm (range: 1–15 mm), and the median length was 9 mm (range: 2–25 mm). Device implantation could be successfully achieved in all cases. Closure rates documented immediately after the procedure, at 3–10 days, 1 month, and 6 months after intervention were 62%, 95.1%, 97.8%, and 98.5%, respectively. With the exception of one minor thromboembolic event, there were no procedure-related complications. Conclusion: Closure of PDA with various anatomic variations and sizes can be performed effectively and safely using the Nit-Occlud® coil.
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Long-term follow-up and outcomes of discrete subaortic stenosis resection in children p. 212
Lulu Abushaban, Babu Uthaman, John Puthur Selvan, Mustafa Al Qbandi, Prem N Sharma, Thinakar Vel Mariappa
DOI:10.4103/apc.APC_120_18  PMID:31516277
Background: Studies of long-term outcomes of discrete subaortic stenosis (DSS) are rare. Therefore, we reviewed the long-term outcomes of subaortic membrane resection in children with isolated DSS over 16 years from a single institution. Materials and Methods: We retrospectively reviewed the records of patients (n = 27) who underwent resection of DSS between 2000 and 2017. Patients with major concomitant intracardiac anomalies were excluded. Indications for surgery were mean left ventricular outflow tract (LVOT), Doppler gradient >30 mmHg, and/or progressive aortic insufficiency. Results: The mean age at diagnosis was 3.77 ± 3.49 years (range, 0.25–13 years) and the mean age at surgery was 6.36 ± 3.69 years (range, 1–13 years). All patients underwent resection of subaortic membrane. The mean LVOT Doppler gradient decreased from 40.52 ± 11.41 mmHg preoperatively to 8.48 ± 5.06 mmHg postoperatively (P < 0.001). The peak instantaneous LVOT Doppler gradient decreased from 75.41 ± 15.22 mmHg preoperatively to 18.11 ± 11.44 mmHg postoperatively (P < 0.001). At the latest follow-up, the peak gradient was 17.63 ± 8.93 mmHg. The mean follow-up was 7.47 ± 3.53 years (median 6.33 years; range 2.67–16 years). There was no operative mortality or late mortality. Recurrence of subaortic membrane occurred in 7 (25.92%, 7/27) patients who underwent primary DSS operation. Four (14.81%, 4/27) patients required reoperation for DSS recurrence at a median time of 4.8 years (3.1–9.1 years) after the initial repair. Risk factors for reoperation were age <6 years at initial repair. Eighteen (66.66%, 18/27) patients had AI preoperatively and progression of AI occurred in 70.37% (19/27). This included 4 (22.22%, 4/18) patients who had worsening of their preoperative AI. Short valve-to-membrane distance was found to be prognostically unfavorable. One (3.7%, 1/27) patient had an iatrogenic ventricular septal defect, and 2 (7.4%, 2/27) patients had complete AV block following membrane resection. Conclusions: Resection of subaortic membrane in children is associated with low mortality. Higher LVOT gradient, younger age at initial repair, and shorter valve-to-membrane distance were found to be associated with adverse outcome. Recurrence and reoperation rates are high, and progression of aortic insufficiency following subaortic membrane resection is common. Therefore, these patients warrant close follow-up into adult life.
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Atrial and ventricular ejection force of the fetal heart: Which of the four chambers is the dominant? p. 220
Elaheh Malakan Rad, Toktam Sheykhian, Ali Akbar Zeinaloo
DOI:10.4103/apc.APC_146_18  PMID:31516278
Background/Aim: This study aimed to measure and compare the ejection force of the cardiac chambers in healthy singleton fetuses and to investigate the relationship of ejection force of cardiac chambers with gestational age, fetal sex, and fetal heart rate. Patients and Methods: A prospective study was performed on 68 singleton fetuses with a gestational age of 17–34 weeks. Atrial and ventricular ejection force was measured. Measurements were repeated in 18 of the fetuses to assess intraobserver reliability. Results: The right atrium had the highest ejection force of all the cardiac chambers. Ejection force of both atria and ventricles increased with gestational age. Conclusion: The right atrium is the dominant chamber of the fetal heart in 17–34 weeks of gestation. Comparison of our values with previous studies indicates that left atrial ejection force almost doubles in the 1st month after birth. This study highlights the crucial role of the right atrium in the fetal cardiac function during 17–34 weeks of gestation.
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Options for coronary translocation and other considerations in aortic root translocation (Bex-Nikaidoh procedure) p. 228
Swaminathan Vaidyanathan, Marathe Supreet, Marathe Shilpa, Alphonso Nelson, Agarwal Vijay
DOI:10.4103/apc.APC_183_18  PMID:31516279
Introduction: The surgical options for patients with transposition of the great arteries (TGA), ventricular septal defect (VSD), and left ventricular outflow tract obstruction include intracardiac baffling with the right ventricle to pulmonary artery (PA) conduit (Rastelli procedure), “reparation a l'etage ventriculaire” or aortic root translocation (Bex-Nikaidoh procedure). The Bex-Nikaidoh procedure allows a more normal, anatomically aligned left ventricular outflow tract. However, the operation is technically demanding, and coronary translocation remains one of the major challenges for successful root translocation. Methods: All patients who underwent aortic root translocation in a single institute over a period of 2 years from January 2015 to December 2017 were included in the study. Surgical technique and early outcomes are described with specific focus on the different observed coronary artery patterns and surgical strategies for translocation. Results: Fourteen patients underwent aortic root translocation. The coronary artery patterns observed could be categorized into four different patterns based on the size of the pulmonary annulus and the relative position of the PA relative to the aorta. Successful translocation of the coronary arteries was achieved in every patient. Mean follow-up was 18.42 ± 9.22 months. There was no mortality and no reoperation during the follow-up period. Conclusion: The Bex-Nikaidoh procedure is a promising surgical option for TGA, VSD, and pulmonary stenosis. Good outcomes are achievable despite wide variations in anatomy using a tailored approach for coronary translocation.
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REVIEW ARTICLES Top

Functional cardiac measurements performed by two-dimensional Doppler echocardiography in normal fetuses: Determination of Z-scores and future prospects p. 233
Luciane Alves Rocha, Nathalie Jeanne Bravo-Valenzuela, Liliam Cristine Rolo, Edward Araujo Junior
DOI:10.4103/apc.APC_173_18  PMID:31516280
Two-dimensional (2D) echocardiogram with the aid of color Doppler and pulsed Doppler allows one to record blood flow waveforms in several structures of the heart. The determination of normal values of these flows in the fetus can help understand cardiac hemodynamics. Given this importance, numerous surveys have been conducted with various existing echocardiographic techniques in order to improve the functional evaluation and consequently, planning of delivery. The aim of this review was to discuss the findings of the reference values of blood flows obtained by 2D echocardiography with Doppler, the current trend of the determination of Z-scores in the functional measurements, and their future prospects.
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Abernethy malformation: Our experience from a tertiary cardiac care center and review of literature p. 240
Sushil Azad, Adhi Arya, Radhakrishnan Sitaraman, Amit Garg
DOI:10.4103/apc.APC_185_18  PMID:31516281
Abernethy malformation, also called as congenital extrahepatic portosystemic venous shunt, is a rare anomaly involving the portal venous system. Although rare, it is increasingly being reported and is important to diagnose given the adverse clinical consequences in untreated patients. It has myriad of clinical presentations, from being completely asymptomatic to causing hepatic carcinoma, hepatic encephalopathy, severe pulmonary hypertension, and diffuse pulmonary arteriovenous malformation. We describe our experience with five cases in a tertiary pediatric cardiac care center with Abernethy malformation, with review of literature and also discuss possible therapeutic implications.
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STATE OF THE ART Top

Anomalous aortic origin of the pulmonary arteries: Case series and literature review p. 248
Salvatore Agati, Carlos Guerra Sousa, Felice Davide Calvaruso, Rosanna Zanai, Ivana Campanella, Daniela Poli, Alfredo Di Pino, Luca Borro, Fiore Salvatore Iorio, Massimiliano Raponi, Robert H Anderson, Simone Reali, Andrea De Zorzi, Aurelio Secinaro
DOI:10.4103/apc.APC_89_18  PMID:31516282
Anomalous origin of the pulmonary arteries from the ascending aorta is a rare, but severe clinical entity necessitating a scrupulous evaluation. Either the right or the left pulmonary arteries can arise directly from the ascending aorta while the other pulmonary artery retains its origin from the right ventricular outflow tract. Such a finding can be isolated or can coexist with several congenital heart lesions. Direct intrapericardial aortic origin, however, must be distinguished with origin through a persistently patent arterial duct. In the current era, clinical manifestations usually become evident in the newborn rather than during infancy, as used to be the case. They include respiratory distress or congestive heart failure due to increased pulmonary flow and poor feeding. The rate of survival has now increased due to early diagnosis and prompt surgical repair, should now be expected to be at least 95%. We have treated four neonates with this lesion over the past 7 years, all of whom survived surgical repair. Right ventricular systolic pressure was significantly decreased at follow-up. Our choice of treatment was to translocate the anomalous pulmonary artery in end-to-side fashion to the pulmonary trunk. Our aim in this report is to update an Italian experience in the diagnosis and treatment of anomalous direct origin of one pulmonary artery from the aorta, adding considerations on the lessons learned from our most recent review of the salient literature.
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SPECIAL ARTICLE Top

Indian guidelines for indications and timing of intervention for common congenital heart diseases: Revised and updated consensus statement of the Working group on management of congenital heart diseases Highly accessed article p. 254
Anita Saxena, Jay Relan, Ravi Agarwal, Neeraj Awasthy, Sushil Azad, Manisha Chakrabarty, Kulbhushan S Dagar, Velayoudam Devagourou, Baiju S Dharan, Saurabh K Gupta, Krishna S Iyer, M Jayranganath, Raja Joshi, BR J Kannan, Ashish Katewa, Vikas Kohli, Shyam S Kothari, KM Krishnamoorthy, Snehal Kulkarni, R Manoj Kumar, R Krishna Kumar, Sunita Maheshwari, Krishna Manohar, Ashutosh Marwah, Smita Mishra, Smruti R Mohanty, K Samba Murthy, K Nageswara Rao, PV Suresh, S Radhakrishnan, Palleti Rajashekar, S Ramakrishnan, Nitin Rao, Suresh G Rao, HM Chinnaswamy Reddy, Rajesh Sharma, Krishnanaik Shivaprakash, Raghavan Subramanyan, R Suresh Kumar, Sachin Talwar, Munesh Tomar, Sudeep Verma, R Vijaykumar
DOI:10.4103/apc.APC_32_19  PMID:31516283
A number of guidelines are available for the management of congenital heart diseases (CHD) from infancy to adult life. However, these guidelines are for patients living in high-income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for CHD, as often these patients present late in the course of the disease and may have coexisting morbidities and malnutrition. Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on August 10 and 11, 2018, at the All India Institute of Medical Sciences. The meeting was supported by Children's HeartLink, a nongovernmental organization based in Minnesota, USA. The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common CHD; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for CHD; and (iii) indications for use of pacemakers in children. Evidence-based recommendations are provided for indications and timing of intervention in common CHD, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus, and others), obstructive lesions (pulmonary stenosis, aortic stenosis, and coarctation of aorta), and cyanotic CHD (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein's anomaly, and others). In addition, protocols for follow-up of postsurgical patients are also described, disease wise. Guidelines are also given on indications for implantation of permanent pacemakers in children.
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BRIEF COMMUNICATION Top

Surgical repair for common arterial trunk with pulmonary dominance, hypoplasia of ascending aorta, and interrupted aortic arch p. 287
Sachin Talwar, Bharat Siddharth, Saurabh Kumar Gupta, Amolkumar Bhoje, Shiv Kumar Choudhary
DOI:10.4103/apc.APC_147_18  PMID:31516284
The arrangement of aortic and pulmonary pathways is extremely variable in the hearts with a common arterial trunk. Almost always, interruption of the aortic arch is seen in the setting of hypoplasia of the ascending aorta and dominance of the pulmonary circulation. This subset poses substantial challenges in surgical repair and portends poor outcomes. In this report, we briefly describe the technique of ascending aorta reconstruction and other aspects of the surgical repair of this rare malformation.
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CASE REPORTS Top

Exercise-induced syncope and Brugada syndrome p. 292
Anjan S Batra, Rachel Watson, Anthony C McCanta
DOI:10.4103/apc.APC_131_18  PMID:31516285
Brugada syndrome (BrS) is a hereditary condition that is characterized by ST elevation, ventricular tachycardia or fibrillation, and sudden cardiac death in otherwise healthy patients. Life-threatening arrhythmias generally occur, while at rest, with fever or during vagotonic states. Exercise is generally not considered a trigger for ventricular arrhythmias or syncope in patients with BrS. We describe a patient who presented with exercise-induced syncope, ventricular tachycardia during an exercise test, and was found to be both genotypically and phenotypically positive for BrS. This case highlights a potentially important role of exercise testing in diagnosing and risk stratifying certain patients with BrS.
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Virtual three-dimensional model for preoperative planning in a complex case of a double outlet right ventricle p. 295
Swati Garekar, Alpa Bharati, Firoza Kothari, Sachin Patil, Shyam Dhake, Shivaji Mali, Amit Mhatre, Dilip Bind, Ashwini Joshi, Bharat Soni, Dhananjay Malankar
DOI:10.4103/apc.APC_141_18  PMID:31516286
A 3-year-old child with a complex variant of double outlet right ventricle achieved a successful biventricular repair after detailed delineation of the intracardiac anatomy on multimodality imaging. A virtual three-dimensional (3D) model based on computed tomography was used successfully in the absence of an actual 3D-printed model. This case report seeks to highlight and hence increase the utilization of the virtual 3D model in resource-limited settings.
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Noonan syndrome associated with anomalous left coronary artery from the pulmonary artery in a patient with the rare RAF1 mutation: A case report and review of literature p. 298
Richard U Garcia, Raya Safa, Chelsea Evans, Dominic Alessio, Ralph Delius, Bahig Shehata
DOI:10.4103/apc.APC_144_18  PMID:31516287
We present the case of a 7-week-old male infant diagnosed with anomalous left coronary artery from the pulmonary artery (ALCAPA) who underwent repair by left coronary artery reimplantation, followed by an eventful postoperative period including need for venous arterial extracorporeal membrane oxygenation and mitral valve replacement due to mitral calcification and severe insufficiency. He also required heart transplant due to severe rapidly progressive biventricular hypertrophy. The pathology examination of the explanted heart showed massive cardiomegaly. Subsequently, the infant's cardiomyopathy panel was positive for RAF1 mutation, consistent with diagnosis of a rare form of Noonan syndrome. To our knowledge, this autosomal dominant condition in association with ALCAPA has not been previously reported in the literature.
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Complete transposition of great arteries associated with total anomalous pulmonary venous connection: An unusual cause for early left ventricular myocardial mass regression p. 302
Neeraj Aggarwal, Reena K Joshi, Nabil Paktin, Mridul Agarwal, Raja Joshi
DOI:10.4103/apc.APC_102_18  PMID:31516288
A 24-day-old apparently asymptomatic neonate was found to have complete transposition of great arteries with small patent ductus arteriosus and restrictive patent foramen ovale. The neonate was found to have relatively high saturations (saturations = 88%) despite inadequate mixing communications. Echo findings were suggestive of significant dilatation of right atrium and right ventricle and left ventricular (LV) mass regression. Further echo interrogation revealed coexisting total anomalous pulmonary venous connection (TAPVC) as the cause of relatively high saturations and early LV mass regression. The patient was planned for follow-up and underwent successful Senning repair at the age of 8 months. Hemodynamics and echo findings of this association of TGA with TAPVC have been described in this case report.
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Severe thrombocytopenia in tetralogy of Fallot patients: A contraindication for corrective surgery? p. 305
Suraj Patil, Jay Relan, Milind Hote, Shyam Sunder Kothari
DOI:10.4103/apc.APC_71_18  PMID:31516289
A 3-year-old boy with tetralogy of Fallot and recurrent cyanotic spells was found to have severe thrombocytopenia with platelet counts in the range of 11–30,000/mm[3]. There was a hesitation to operate in view of the high bleeding risk due to profound thrombocytopenia. However, the total correction was done after excluding other causes of thrombocytopenia. His platelet count dramatically improved after the operation.
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Temporary bronchial stenting for airway compression in the interstage palliation of functional single ventricle p. 308
Jason Hawes Barnes, Richard Paul Boesch, Karthik Balakrishnan, Sameh M Said, Charlotte S Van Dorn
DOI:10.4103/apc.APC_94_18  PMID:31516290
The Norwood procedure is the first of three palliative surgical procedures offered for hypoplastic left heart syndrome (HLHS). Due to the small size of the thorax and proximity of airway and vascular structures, compression of the airway is possible following the Norwood procedure. We describe the management of an infant with HLHS following Stage I surgical palliation who developed refractory respiratory failure secondary to severe left bronchial compression.
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Retrograde transcatheter closure of anterior mitral valve leaflet perforation p. 312
Berke Sengun, Işıl Yildirim, Ömer Yildiz, Alpay Celiker
DOI:10.4103/apc.APC_162_18  PMID:31516291
Transcatheter closure of mitral valve leaflet perforation is a very rarely performed and a difficult procedure for repairing the defect. Herein, we are the first to report on both the safety and feasibility of percutaneous retrograde transcatheter closure of anterior mitral valve leaflet perforation with an AMPLATZER™ Duct Occluder II (6 mm × 6 mm, ADO II; Abbott Vascular, IL, USA) device in a 19-year-old patient with a severe mitral valve regurgitation following cardiac surgery.
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Thoracic electrical impedance tomography to minimize right heart strain following cardiac arrest p. 315
Gregory Hansen, Tanya Holt, Jeffrey Dmytrowich
DOI:10.4103/apc.APC_189_18  PMID:31516292
Titrating ventilator settings to minimize pulmonary arterial pressures and optimize both ventilation and oxygen delivery can be challenging following cardiac arrest. Erroneous ventilator adjustments can lead to unnecessary strain on the right ventricle that may be particularly vulnerable during the acute recovery. We report a child with fulminant myocarditis who was mechanically ventilated using thoracic electrical impedance tomography to optimize regional lung inflation and possibly curtail right ventricular afterload following cardiac arrest.
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First report of successfully palliating a hypoplastic left heart syndrome patient with anomalous left coronary artery from the pulmonary artery beyond fontan p. 318
Kyle W Riggs, Nina M Price, Nicholas Szugye, Bryan H Goldstein, David L S Morales
DOI:10.4103/apc.APC_152_18  PMID:31516293
We report a case of hypoplastic left heart syndrome with an anomalous left coronary artery from the pulmonary artery (ALCAPA) identified intraoperatively during the Stage-II palliation. Due to recurring ventricular fibrillation on sternotomy, a hybrid Stage-I palliation was performed. During comprehensive Stage-II, the ALCAPA was reimplanted in the neoaorta and measures, including a nontraditional Damus connection/arch reconstruction and classic bilateral Glenn procedures, were taken to avoid compression of the coronary artery. After a successful Fontan procedure, he continues to do well at 5 years old, becoming the first patient reported in the literature to survive all the three stages of single-ventricle palliation.
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Closure of right pulmonary artery to left atrium fistula by duct occluder device p. 321
Ijaz Hussain, Roomana KhawajaKhail, Kamran Khattak, Muhammad Irfan, Adnan Mehmood Gul
DOI:10.4103/apc.APC_155_18  PMID:31516294
We report the successful transcatheter closure of the right pulmonary artery fistula to the left atrium in a 4-year-old boy, who had presented with cyanosis and easy fatigability, identified after two-dimensional echocardiogram with bubble contrast study, computed tomography (CT) angiography, and cardiac catheterization. The fistula was successfully closed by a transcatheter approach using an 18/16 duct occluder device. There was marked improvement clinically and no complication on 2-months follow-up.
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“Idiopathic” pulmonary arterial hypertension in early infancy: Excluding NFU1 deficiency p. 325
Paquay Stephanie, Barrea Catherine, Sluysmans Thierry, Vachiery Jean-Luc, Loeckx Isabelle, Seneca Sara, Vô Christophe, Nassogne Marie-Cecile
DOI:10.4103/apc.APC_136_18  PMID:31516295
NFU1 deficiency is a rare metabolic disorder affecting iron–sulfur cluster synthesis, an essential pathway for lipoic acid-dependent enzymatic activities and mitochondrial respiratory chain complexes. It is a little-known cause of pulmonary arterial hypertension (PAH), while PAH is a prominent feature of the disease. We herein report on a female infant diagnosed as having idiopathic PAH since 1 month of age, who did not respond to bosentan plus sildenafil. NFU1 deficiency was only suggested and confirmed at 10 months of age when she demonstrated neurological deterioration along with high glycine levels in body fluids. Unexplained PAH in early infancy should prompt clinicians to perform amino acid chromatography searching for high glycine levels. Early recognition will avoid further invasive procedures and enable appropriate genetic counseling to be offered. No effective treatment is currently able to prevent the fatal course of this metabolic condition.
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Transcatheter closure of a rare coronary artery fistula using a modified mother–child technique p. 329
Catarina Perez-Brandao, António Fiarresga, Lídia Sousa, José D Martins
DOI:10.4103/apc.APC_175_18  PMID:31516296
Coronary artery fistulas (CAFs) are rare abnormal communications between a normal coronary artery and a cardiac chamber or great vessel, such as the pulmonary artery, bypassing the myocardial capillary network. We report the case of a 17-year-old male with a medical history of pulmonary valve stenosis, who presented with progressive dyspnea and fatigue. Transthoracic Doppler echocardiography showed multiple continuous flows both on the apical interventricular septum and entering the left atrium. A tortuous CAF arising from the left main coronary artery to the left atrium was revealed by coronary angiography. The lesion was successfully closed percutaneously using an off-label Amplatzer™ Duct Occluder II Additional Sizes with a backup support of a modified “mother–child” system. This case highlights the effort of both pediatric and adult cardiology teams to come up with new potential strategies and combined techniques to overcome the difficulties of managing complicated CAFs, such as the use of percutaneous coronary intervention techniques and the selection of the most adequate occlusion devices, even when used off-label.
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Corkscrew aortic arch in PHACES syndrome: Multimodal imaging of an unusual morphology of tortuous aortic arch in a rare but well-defined syndrome p. 333
Ronak Sheth, Arvind Sahay Singh, Sreeja Pavithran, Kothandam Sivakumar
DOI:10.4103/apc.APC_188_18  PMID:31516297
PHACES syndrome, a diffuse aortocraniocerebral vasculopathy, is a neural tube migration disorder, characterized by aortic coarctation and aberrant arch branches. Clinical diagnosis, echocardiography, and surgical management of coarctation in this syndrome are challenging due to peculiar morphological differences. Corkscrew aortic arch, an extreme tortuosity of the aortic arch described in arterial tortuosity syndrome, is not reported in PHACES syndrome so far. Multimodal imaging of this unusual corkscrew aortic arch in two patients with PHACES syndrome is presented.
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Modified underlying cardiac disease severity in twin-twin transfusion syndrome p. 336
Hirotaka Ishido, Satoshi Masutani, Yukiko Mikami, Kazunori Baba, Michitaka Fuse, Keiko Mizuta, Risa Tanaka, Kenji Sugamoto, Yoichi Iwamoto, Hideaki Senzaki
DOI:10.4103/apc.APC_112_18  PMID:31516298
Twin-twin transfusion syndrome or related conditions affect fetal loading. We report monochorionic-diamniotic twins. Twin 1 had Ebstein anomaly with mild tricuspid regurgitation (TR) and slightly thickened tricuspid valve leaflets with plastering. Twin 2 had tricuspid valve dysplasia (with abnormal thickening but without plastering) with moderate TR and mild right atrial dilatation. After birth, the severity of TR was greatly reduced in the recipient but increased in the donor. Therefore, intravascular volume change which was due to twin-twin transfusion syndrome seemed to affect the severity of the valvar disease in fetuses. This case suggests that the intrinsic severity of fetal tricuspid valvular disease may be overestimated in the recipient and underestimated in the donor twin. These factors need to be taken into consideration in clinical decision-making.
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IMAGES Top

Utility of three-dimensional echocardiography and magnetic resonance imaging in the diagnosis of double-orifice tricuspid valve p. 339
Sandeep Mohanty, Sreeja Pavithran, Ravi Agarwal, Kothandam Sivakumar
DOI:10.4103/apc.APC_179_18  PMID:31516299
Duplication of atrioventricular valves involves the mitral valve more often than the tricuspid valve and is often associated with other cardiac defects. Double-orifice tricuspid valve (DOTV) is often identified in surgery or autopsy and missed on echocardiography, as the two orifices are orthogonal to the imaging plane. If suspected on echocardiography, it masquerades as mild tricuspid hypoplasia. Three-dimensional echocardiography and magnetic resonance imaging of a DOTV are presented.
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Hammock effect and complete cusp prolapse: Rare mechanisms of Melody valve failure demonstrated by intracardiac echocardiography p. 342
Simone Jhaveri, Lourdes Prieto, Patcharapong Suntharos
DOI:10.4103/apc.APC_143_18  PMID:31516300
Transcatheter pulmonary valve replacement using Melody valve (Medtronic, Minneapolis MN) has significantly increased in the recent decades. Melody valve failures, although rare, can be problematic and require re-intervention. Through intracardiac echocardiography, we present two patients who each had a rare etiology for dysfunction of their Melody valve. Hammock effect, wherein the valve does not oppose the stent and complete cusp failure causing severe regurgitation have not been previously described as causes of Melody valve failure in the absence of endocarditis. Awareness and knowledge of these mechanisms is pivotal in the management of this patient population.
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Case of anomalous origin of right coronary artery from pulmonary artery associated with interrupted aortic arch type A, diagnosed by multidetector computed tomography angiography p. 345
Neetu Soni, Sunil Kumar Jain, Anil Kumar, Renu Kadian, Shou Li
DOI:10.4103/apc.APC_69_18  PMID:31516301
Anomalous origin of the right coronary artery from pulmonary artery (ARCAPA) is a rare congenital anomaly of the coronary circulation, which can be easily missed by echocardiography. Interrupted aortic arch (IAA) is another rare congenital cardiac abnormality that typically presents in the first few weeks of life. We present a case of ARCAPA associated with IAA diagnosed with the help of multidetector computed tomography angiography, in a 7-year-old boy.
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LETTERS TO EDITOR Top

Comment: Prevalence of hypertension and prehypertension in schoolchildren from central India p. 348
Mahmood Dhahir Al-Mendalawi
DOI:10.4103/apc.APC_64_19  PMID:31516302
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Reply: Prevalence of hypertension and prehypertension in schoolchildren from Central India p. 349
Ashish Patel, Anil Bharani, Meenakshi Sharma, Anuradha Bhagwat, Neepa Ganguli, Dharampal Singh Chouhan
DOI:10.4103/apc.APC_90_19  PMID:31516303
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Persistent femoral lymphatic leak following Fontan surgery: Role of fluorescein dye p. 350
Debabrata Gohain, Sabarinath Menon, Sudip Dutta Baruah, Sowmya V Ramanan, Baiju S Dharan, K Jayakumar
DOI:10.4103/apc.APC_157_18  PMID:31516304
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Preoperative adrenal insufficiency in a neonate with congenital heart disease surgery p. 351
Shankar Vithalrao Kadam, Kamlesh B Tailor, Vinay H K Joshi, Suresh G Rao
DOI:10.4103/apc.APC_108_18  PMID:31516305
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