Annals of Pediatric Cardiology
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   2021| April-June  | Volume 14 | Issue 2  
    Online since May 3, 2021

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Evaluation of high-dose aspirin elimination in the treatment of Kawasaki disease in the incidence of coronary artery aneurysm
Farima Sanati, Mohammadmehdi Bagheri, Shahryar Eslami, Ali Khalooei
April-June 2021, 14(2):146-151
Background: Standard first-step therapy for Kawasaki disease consists of Intravenous immunoglobulin and high dose Aspirin (80-100 mg/kg/day). The standard dose of Intravenous immunoglobulin (2gr/kg) is strongly effective in reducing the risk of coronary arteries abnormalities. So, the proper dose and efficacy of Aspirin to decrease the risk of coronary arteries abnormalities is a controversial issue. In this study, it is tried to assess the result of eliminating high-dose Aspirin in the treatment of the acute phase of Kawasaki and observe the incidence rate of coronary arteries abnormalities when only Intravenous immunoglobulin was administered. Methods: This study is a prospective randomized, open-label, blinded end-points clinical trial performed in Afzalipour hospital in Kerman University of Medical Sciences from September 2017 to September 2018 in 62 patients with typical and atypical Kawasaki disease. The study group received Intravenous immunoglobulin (2 g/kg) and the control group get the same dose of Intravenous immunoglobulin plus Aspirin with the dose of 80-100 mg/Kg/day until they were afebrile for 48 hours. Afterward, both groups received a daily single dose (3-5 mg/kg) of Aspirin for six weeks. Echocardiography was done after two weeks, six weeks, and six months. Internal the diameter of the left and right main coronary arteries was measured and then the corresponding Z-score was calculated. Results: In the study group, coronary arteries abnormalities decreased from 38.7% in the 2nd week to 16.1% in the 6th month. In the control group, it declined from 54.8% to 22.6%. There was no statistically significant difference between the groups in term of frequency abnormal coronary arteries abnormality at the study period (P=0.151). Conclusions: We concluded that high dose Aspirin does not have a significant role in preventing coronary arteries abnormalities in Kawasaki disease and giving standard 2 gr/kg/day Intravenous immunoglobulin without high-dose Aspirin in acute-phases therapy does not increase the risk of coronary arteries abnormality.
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Annals of pediatric cardiology: A glorious journey and a vision for the future
Sivasubramanian Ramakrishnan
April-June 2021, 14(2):135-138
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Use of cribriform amplatzer septal occluder in the pediatric population: Feasibility, safety, and technical considerations
Priya Pradhan, Sneha Jain, Supratim Sen, Bharat Dalvi
April-June 2021, 14(2):159-164
Purpose : Fenestrated atrial septal defects (F-ASDs) in the pediatric population pose a challenge for transcatheter device closure since multiple devices are not preferred in small hearts. Oversizing the Amplatzer Septal Occluder (ASO) to cover the surrounding fenestrations usually distorts the central waist as well as the disc profile and often defeats the purpose. This is a retrospective observational study with an aim to assess the feasibility and safety of cribriform ASO in closing F-ASDs in small children. Methods : Sixteen children with F-ASD who underwent device closure with cribriform ASO were included in the study. The fenestrated septal length (FSL) and the total septal length (TSL) were measured on transesophageal echocardiogram. A device size which was 1.5–2 times the FSL but smaller than the TSL was selected. The defect was closed with a device passed through a relatively centrally placed smaller fenestration. Results : The median age of the cohort was 5 years (2.5–10.5). Majority (14/16) required 25 or 30 mm cribriform ASO. Aneurysmal interatrial septum was seen in most of our patients (11/15). All the patients had successful device implantation. Complete closure of the defect was seen in 11 patients while 5 patients had insignificant residual shunt at a median follow-up of 40 months (1–60 months). There were no other complications. Conclusions : Cribriform ASO can be used safely and effectively in closing F-ASDs in children. Deployment of the device through a small central hole allows covering maximum fenestrations and gives more stability to the device. Residual shunts, although not infrequent, are insignificant.
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Carvedilol improves left ventricular diastolic dysfunction in patients with transfusion-dependent thalassemia
Suchaya Silvilairat, Pimlak Charoenkwan, Suwit Saekho, Adisak Tantiworawit, Nipon Chattipakorn
April-June 2021, 14(2):152-158
Background: Iron overload cardiomyopathy is the most common cause of death in patients with transfusion-dependent thalassemia. Aim: The aim of this study was to determine the efficacy of carvedilol treatment in patients with transfusion-dependent thalassemia who had left ventricular diastolic dysfunction. Methods: Eighteen patients with transfusion-dependent thalassemia who had left ventricular diastolic dysfunction were enrolled. All patients had normal left ventricular systolic function and were given carvedilol with the target dose of 0.8 mg/kg/day. Ventricular function and the level of cardiac iron were assessed by echocardiography and magnetic resonance imaging at 0, 3, and 6 months. Results: The median age of the patients was 19 years (range 13–25 years). Four patients had severe left ventricular Grade III diastolic dysfunction and fourteen patients had Grade II diastolic dysfunction. The grade of left ventricular diastolic dysfunction was improved at 3 months after the carvedilol treatment. The Doppler parameters, including pulmonary vein atrial reversal velocity, pulmonary vein atrial reversal duration, and the difference of pulmonary vein atrial reversal and the mitral valve atrial contraction wave duration at 3 months after the carvedilol treatment, were significantly lower than these parameters before the treatment. Conclusions: Among patients with transfusion-dependent thalassemia who had left ventricular diastolic dysfunction without systolic dysfunction, treatment with carvedilol for 3 months was associated with improvement in Doppler parameters of left ventricular diastolic function. However, this finding and its clinical significance need to be confirmed in further double-blind controlled studies.
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Early diagnosis of diaphragm palsy after pediatric cardiac surgery and outcome after diaphragm plication - A single-center experience
Divyakant Parmar, Jigar Panchal, Neha Parmar, Pankaj Garg, Amit Mishra, Jigar Surti, Kartik Patel
April-June 2021, 14(2):178-186
Objective : The aims of our prospective observational study were to evaluate the (1) reliability of clinical signs in the early detection of diaphragm palsy (DP); (2) reliability of ultrasonography using echo machine as a bedside tool for the diagnosis of DP; and (3) does early diaphragm plication result in the improved outcome? We also sought to determine the incidence and predominant risk factors for DP and diaphragm plication at our center. Materials and Methods : This prospective observational study included patients with suspected DP from January 2015 to December 2018. Patients with suspected DP were initially evaluated by bedside ultrasonography using echo machine and confirmed by fluoroscopy. Diaphragm plication was considered for patients having respiratory distress, difficult weaning, or failed extubation attempt without any obvious cardiac or pulmonary etiology. Patients were followed for 3 months after discharge to assess diaphragm function. Results : A total of 87 patients were suspected of DP based on clinical signs. DP was diagnosed in 61 patients on fluoroscopy. The median time from index operation to diagnosis was 10 (1–59) days. Diaphragm plication was done among 52 patients and not done in nine patients. Bedside ultrasonography using echo machine was 96.7% sensitive and 96.15% specific in diagnosing DP. Early plication (<14 days) significantly reduced the need for nasal continuous positive airway pressure (65% vs. 96%, P = 0.02), duration of mechanical ventilation (12 vs. 25 days, P = 0.018), intensive care unit (ICU) stay (25 days vs. 39 days, P = 0.019), and hospital stay (30 days vs. 46 days, P = 0.036). Conclusion : Hoover's sign and raised hemidiaphragm on chest X-ray are the most specific clinical signs to suspect unilateral DP. Bedside ultrasonography using an echo machine is a good diagnostic investigation comparable to fluoroscopy. Early plication facilitates weaning from the ventilator and thereby decreases the ICU stay and hospital stay.
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A comprehensive study of congenital unilateral absence of branch pulmonary artery associated with other congenital heart defects and ipsilateral non-unifocalizable major aorto-pulmonary collateral arteries: A single-center retrospective study
H Ravi Ramamurthy, Varsha Walavalkar, Satheesh Siddaiah, Sunita Maheshwari
April-June 2021, 14(2):139-145
Introduction: Congenital unilateral absence of pulmonary artery (UAPA) is a rare congenital anomaly with the complete absence of intrapericardial segment of one of the branch pulmonary arteries. Sixty percent are associated with other congenital heart defects (CHD) that often need correction. Aim To analyze the data of patients with UAPA and ipsilateral non-unifocalizable major aortopulmonary collateral arteries (MAPCAs) associated with other CHD to identify the commonly associated CHD, their management strategies and outcomes. Materials and Methods: Retrospective data of patients admitted for congenital UAPA with other CHD was compiled from hospital records from 2002 to 2015. The associated CHD were categorized as group I with the decreased pulmonary flow and group II with increased pulmonary flow to the unaffected contralateral pulmonary artery. The determinants of their management were analyzed. Results: Sixty-five patients of UAPA and ipsilateral non-unifocalizable MAPCAs associated with other CHD were identified. Group I had 41 patients and Group II had 24. The most common CHD associated with UAPA was tetralogy of Fallot (TOF) in 31 patients (47.7.2%). Fifty-three patients underwent surgery, 48 (73.8%) underwent single lung corrective surgery, 5 (7.6%) palliative surgery and 12 (18.4) received no surgery. Four operated patients died in the immediate postoperative period. The lowest Mc Goon ratio and Nakata index to undergo corrective surgery were 1.0 and 87.4 mm2/m2. A follow-up of 21 patients was done, among which 11 patients who underwent single-stage corrective surgery, all are in NYHA class II and saturating above 95%. Conclusions: Congenital UAPA is a rare anomaly and associated with a variety of CHDs, TOF being the most common. Single lung corrective surgery in patients with ipsilateral non-unifocalizable MAPCAs has good immediate and long term survival.
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Aortic size in children: Systolic measurements are different from diastolic measurements
Mohammad F Al-mousily, Leo Lopez, Juan Carlos Muniz, Nao Sasaki, Irwin Seltzer, Joshua Gruber, Elizabeth Welch
April-June 2021, 14(2):165-169
Background : Current guidelines recommended aortic measurements during diastole in adults and during systole in children. Recent studies in adults have demonstrated noteworthy differences in aortic measurements during systole and diastole in the same subjects. In the present study, we aimed to characterize systolic and diastolic differences in aortic measurements in healthy children. Materials and Methods : This retrospective study included 272 children who had a complete echocardiogram and no heart disease. Aortic measurements at the annulus (ANN), aortic root (AOR), sinotubular junction (STJ), and ascending aorta (AAO) were performed. Systolic and diastolic values were compared by calculating the mean systolic to diastolic (SD) percent difference for each segment; if the SD difference was >5%, it was considered clinically important. Similar measurements were conducted by another observer in 18% of the subjects. Results : Systolic measurements were larger than diastolic measurements with mean SD percent differences >5% (P < 0.001) for the AOR (7.3% ± 5.5%), STJ (10.24% ± 7.1%), and AAO (9.8% ± 7.4%). There was no clinically significant SD difference for the ANN. There was an excellent intraclass correlation coefficient between observers (0.982–0.995). Conclusions : Systolic measurements for the AOR, STJ, and AAO were larger than diastolic measurements. Normal reference values are utilized to design treatment for patients with abnormal aortic sizes, and the timing in the cardiovascular cycle used to decide the reference values should be equivalent to the timing used to make measurements in clinical practice. This is particularly imperative as patients transition their care from a pediatric to an adult cardiologist.
  1,674 109 -
Aortopulmonary window: Clues to fetal diagnosis
Parag S Bhalgat, Fazal Nabi, Sudeshna Ray
April-June 2021, 14(2):247-248
Being a planar structure, fetal diagnosis of aorto-pulmonary window poses great challenge. A few echocardiographic signs can help to clinch the diagnosis.
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Profile of Kawasaki disease at a tertiary care center in India
Sagar Bhattad, Sandip Gupta, Neha Israni, Sweta Mohanty
April-June 2021, 14(2):187-191
Background : Kawasaki disease (KD) is the most common cause of acquired heart disease in developed countries. KD is increasingly being reported from India; however, studies involving the large number of patients are few. Methods : All children presenting to the center from January 2017 to December 2019, diagnosed to have KD, were retrospectively included in the study. Clinical and laboratory profiles, including echocardiograms, were reviewed. Factors contributing to intravenous immunoglobulin (IVIg) refractoriness and the development of coronary artery abnormalities (CAA) were assessed. Results : A total of 39 children with KD presented to the center during the study. While 32 received initial treatment at our center, seven were referred after the initial IVIg infusion. The age range was 2 months to 11 years (mean 42.15 ± 38.51 months). More than two-thirds of the cohort was male (n = 27/39). Mucosal involvement was the commonest clinical abnormality for the group, followed by rash. Hemoglobin was significantly lower in the group with coronary artery involvement (P = 0.001). CAA (61.5%), incomplete KD, and atypical features were much more common in infants compared to the rest. Refractoriness to treatment was significantly more common in infants (P = 0.029). Conclusions : A significant proportion of infants with KD had cardiac involvement. Infants were more likely to have IVIg-resistant disease.
  1,516 121 -
Primary cardiac fibroma in infants: A case report and review of cases of cardiac fibroma managed through orthotopic heart transplant
Moises Rodriguez-Gonzalez, Alvaro A Pérez-Reviriego, Elena Gomez.Guzman, María Ángeles Tejero.Hernandez, Alicia Zorrilla Sanz, Israel Valverde
April-June 2021, 14(2):224-227
Cardiac fibromas (CF) are the second most common cardiac tumors in children. They can be aggressive tumors despite their benign histopathologic nature, accounting for the highest mortality rate among primary cardiac tumors. CF usually presents a progressive growth and spontaneous regression is rare. Therefore, a complete surgical excision is the preferred therapeutic approach when patients become symptomatic or if mass-related life-threatening complications are anticipated, even in asymptomatic patients. However, some cases are not good candidates for surgical excision due to the impossibility of preserving a normal cardiac anatomy or function after the tumor resection. Orthotopic heart transplantation (OHT) can be an exceptional but adequate alternative for some giant unresectable CF in children. In this article, we report our experience with the case of a 7-month-old infant with a giant unresectable cardiac fibroma who was successfully managed through OHT.
  1,481 128 -
Left ventricular restrictive physiology in kids with atrial septal defects: Something unexpected!
Alessia Faccini, Matteo Casenghi, Mario Carminati, Gianfranco Butera
April-June 2021, 14(2):228-230
Atrial septal defect (ASD) associated with left ventricular (LV) diastolic dysfunction (DD) is rare in children. DD is common in elderly patients undergoing ASD closure. Restrictive physiology among children undergoing percutaneous ASD closure is something unexpected that has not been described before in the literature. Hence, we report on a case of a child referred to our center for ASD closure in which an LV DD has been unmasked with a balloon occlusion test and has been managed with pharmacological preconditioning and subsequent ASD closure with good outcome. Albeit rare, LV DD can occur on children undergoing ASD closure.
  1,473 118 -
A prospective randomized clinical study of perioperative oral thyroid hormone treatment for children undergoing surgery for congenital heart diseases
Alok Kumar, Nikhil Tiwari, H Ravi Ramamurthy, Vivek Kumar, Gaurav Kumar
April-June 2021, 14(2):170-177
Context : Thyroid hormone deficiency is known to occur after cardiac surgery and known as nonthyroid illness (NTI). The beneficial role perioperative thyroid hormone supplementation in children has been debatable more so with oral supplementation. Aims : The aim is to evaluate the role of pre-operative oral thyroid hormone therapy in preventing NTI. To assess its effect on post-operative thyroid hormone levels, hemodynamic parameters, and cardiac function of infants and small children undergoing pediatric cardiac surgery. Settings and Design : Prospective randomized, double-blinded controlled trial at a tertiary level pediatric cardiothoracic center. Materials and Methods : Sixty-five children aged under 18 months undergoing corrective surgeries on cardiopulmonary bypass were included. Patients were randomized into two equal groups: placebo group (given placebo) and thyroxine group (given thyroxine tablet 10 μg/kg) orally once a day starting on the preoperative evening till the fifth postoperative day. The postoperative hemodynamics, inotropic requirement, ventilatory requirement, and cardiac function on echocardiography were observed. Statistical Tests : Shapiro–Wilk test, Mann–Whitney/t-test, Chi-square test, ANOVA with Tukey correction were used. Results : Serum triiodothyronine and thyroxine levels postoperatively were significantly higher in the thyroxine group than in the placebo group. There was no significant difference in left ventricular ejection fraction, hemodynamic variables, extubation time, and length of intensive care unit (ICU) stay between the two groups. Conclusions : In infants and small children undergoing corrective cardiac surgery, perioperative oral thyroid hormone therapy reduces the severity of postoperative NTI. It increases the serum level of thyroid hormones but the therapy does not translate to better hemodynamics, reduced inotropic requirement, reduced ventilatory requirement, improved myocardial function or reduced ICU stay when compared to placebo.
  1,315 156 -
A systematic approach to epicardial echocardiography in pediatric cardiac surgery: An important but underutilized intraoperative tool
Neeraj Awasthy, Sumir Girotra, Nilanjan Dutta, Sushil Azad, Sitaraman Radhakrishnan, Krishna Subramony Iyer
April-June 2021, 14(2):192-200
Intraoperative echocardiography is an integral component of the peri-operative management of pediatric heart disease. It confirms the adequacy of surgery, identifies residual lesions, and can provide useful hemodynamic data. It, therefore, helps to decide on the need for revision of repair and guides the postoperative management strategy. Intraoperative echocardiography is done with the use of either an epicardial probe or a transesophageal probe. Epicardial echocardiography is a simple, useful modality and has the ability to perform imaging in cases where transesophageal echocardiography cannot be easily performed, for example, in low birth weight babies. We attempt to describe in detail the technique of epicardial echocardiography and the various views that we have found useful for a complete postsurgical evaluation. The limitations of the technique are also discussed in detail.
  1,276 143 -
Intracardiac atrial overdrive pacing as an alternative to extracorporeal membrane oxygenation in the treatment of cardiogenic shock due to drug refractory and incessant persistent junctional reciprocating tachycardia in a 7-month-old infant
Tomasz M Ksiazczyk, Radoslaw Pietrzak, Bozena Werner
April-June 2021, 14(2):231-234
In the case of prolonged, undiagnosed persistent junctional reciprocating tachycardia in infants, compensatory mechanisms are exhausted leading to heart failure. However, when cardioverted to sinus rhythm patients often deteriorate due to cardiac output dependency on the higher rates. Extracorporeal mechanical oxygenation (ECMO) is often used to stabilize their hemodynamic status. An 7-month-old female infant was admitted in cardiogenic shock due to drug refractory supraventricular tachycardia (SVT). Pharmacological cardioversion to sinus rhythm with heart rate (HR) of 90 bpm was achieved but resulted in hemodynamic deterioration and early recurrence of arrhythmia. Right atrial overdrive pacing (ODP) wire was introduced through femoral vein and allowed to override the tachycardia with 2:1 A:V block and HR of 160 bpm. ODP was continued for 24 h allowing to wean off the inotropic support. We postulate that ODP can be a safe and less invasive alternative to ECMO in stabilizing infants with cardiogenic shock due to intractable SVTs.
  1,228 121 -
Pulmonary sling in a patient with common arterial trunk
Mohamed Sobh, Patrick Langguth, Kolja Becker, Jens Scheewe, Anselm Uebing, Inga Voges
April-June 2021, 14(2):239-241
We report a rare association of common arterial trunk with left pulmonary artery sling and highlight the importance of cross-sectional imaging in complex congenital cardiac lesions. The patient was antenatally diagnosed with common arterial trunk and underwent surgical repair in the neonatal period. At the age of 20 months, the patient presented with respiratory symptoms and increased right ventricular pressure. Multislice computed tomography demonstrated a pulmonary sling with compression of the distal trachea. Surgical correction of the pulmonary sling and change of the right ventricular to pulmonary artery conduit to a bigger size was performed.
  1,230 89 -
Transposition of the great arteries with total anomalous pulmonary venous connection in a 1½year-old child: Pulmonary arterial hypertension - An advantage
Rose Okwunu Abah, Atul Prabhu, Ashish Katewa, Balswaroop Sahu
April-June 2021, 14(2):235-238
Transposition of the great arteries with total anomalous pulmonary venous connection is a rare anomaly with varied management approach depending on the time of presentation; the management of cases which present early is usually by anatomical surgical approach while late presentation is by physiologic approach. This is due to early left ventricular mass regression as a result of the absence of volume and pressure load to the left ventricle (LV). We report a late presentation (at 1½ years of age) that had a successful anatomic surgical correction because the LV was “prepared” by both pressure and volume load from pulmonary arterial hypertension and large ostium secundum atrial septal defect.
  1,197 107 -
Pulmonary artery thromboembolism in a critically ill neonate successfully treated using thrombolytic therapy
Gerson Valencia Villeda, Robert Spencer, Matthew A Crysta, Jeffrey D Dayton, Usha Krishnan
April-June 2021, 14(2):215-219
Pulmonary artery thromboembolism is a common and treatable cause of adult pulmonary hypertension. Although rare in children, if undiagnosed, it can result in significant morbidity and mortality. We report a case of a cyanotic neonate found to have bilateral pulmonary arterial thrombi who successfully underwent thrombolysis using tissue plasminogen activator with prompt resolution of right ventricular hypertension.
  1,200 101 -
Rare cause of neonatal pulmonary hypertension: Congenital intrahepatic portosystemic shunt through an aneurysm
Satish Kumar Avula, Sudeep Verma, Anantha Ram, Reena Lankala
April-June 2021, 14(2):220-223
Congenital portosystemic shunt is a rare congenital anomaly with abnormal communication between portal venous and systemic venous systems. It can be intrahepatic or extrahepatic. Typically, the intrahepatic shunts are managed conservatively as many of them close spontaneously. We present and discuss clinical, radiological findings of an intrahepatic shunt showing the early occurrence of pulmonary arterial hypertension in the neonatal period which required therapeutic intervention.
  1,128 96 -
Delivery sheath tip invagination – An unusual cause of failure to retrieve duct occluder
Anunay Gupta, Saurabh Kumar Gupta, Sivasubramanian Ramakrishnan, Shyam S Kothari
April-June 2021, 14(2):242-243
Transcatheter closure of patent ductus arteriosus is the standard of care. Retrieval of a duct occluder device is generally easy until it is detached from the delivery cable. We report two instances of failed retrieval of the device due to sheath tip invagination. The report highlights the importance of prompt identification of the mechanism of unforeseen complications in managing it effectively.
  1,092 88 -
Response from the authors to the letter to editor (MVR in infants)
Hitendu Dave
April-June 2021, 14(2):249-250
  1,106 56 -
Technical considerations of coarctation stenting in double-barreled aorta – A persistent fifth arch mimic
Shrusthi Walad, KN Harikrishnan, Arun Gopalakrishnan, Sivasankaran Sivasubramonian, Bijulal Sasidharan
April-June 2021, 14(2):201-203
The double-barreled aorta connecting the ascending aorta and descending aorta caudal to the normal fourth aortic arch has fascinated the interests of cardiac morphologists for over a century. This condition is commonly associated with coarctation. While the controversies surrounding the embryology of the double-barreled aorta have settled down, we present a case-based illustration of the technical aspects of coarctation stenting peculiar to this condition.
  1,006 95 -
Coarctation of aorta intervention: When covered stents should have been first choice?
Palanivel Rajan, Navjyot Kaur, Parag Barwad, Pruthvi C Revaiah, Manojkumar Rohit
April-June 2021, 14(2):204-207
Coarctation of aorta (CoA) is one of the common congenital heart diseases. The two approaches for intervention in CoA include surgical and transcatheter (TC). Out of the two TC interventions available, stenting has been proved better than balloon angioplasty. We have two types of stents; the conventional ones – balloon expandable and the covered stent grafts. The elective covered stent implantation in all CoA has not offered any advantage. However, there are peculiar situations, apart from acute aortic complications, when they should be considered the first choice. We describe our experience of three cases of coarctation stenting, in which covered stenting should have been the preferred choice. A 32-year-old female with Turner's syndrome and severe CoA developed dissection after balloon angioplasty which was successfully managed with a covered stent. A 27-year-old female with near atresia of aorta was managed with balloon expandable stent which remained underexpanded despite post dilatation. A 17-year-old girl with severe CoA and patent ductus arteriosus (PDA) was managed with balloon angioplasty for the CoA and Amplatzer Duct Occluder I for the PDA. However, she developed re-coarctation in 6 months which was managed with a covered stent. Not all CoA requires the covered stents, but there are certain “high risk” CoA which require covered stent as first choice.
  936 121 -
Catheter-directed thrombolysis for in situ pulmonary artery thrombosis in children
Evyatar Hubara, Sharon Borik, Gili Kenet, David Mishaly, Amir Vardi
April-June 2021, 14(2):211-214
In situ pulmonary artery thrombosis (ISPAT) is a unique form of pulmonary embolism characterized by local formation of thrombus in the pulmonary arteries. We present here a baby with hypoplastic left heart syndrome who developed ISPAT after Glenn surgery. The patient underwent catheter-directed thrombolysis, followed by systemic anticoagulation with excellent results.
  949 108 1
Rapid progression of aortic stenosis in a 3-month-old infant with bicuspid aortic valve and DeSanto-Shinawi syndrome
Daiji Takajo, Ghadir Katato, Sanjeev Aggarwal
April-June 2021, 14(2):208-210
A 3-month-old female was diagnosed at 1 month of age with DeSanto-Shinawi syndrome (DSS) and bicuspid aortic valve with trivial stenosis. The aortic valve stenosis progressed to severe within 2 months and required balloon aortic valvuloplasty. This is the first case of aortic stenosis (AS) associated with DSS, and the syndrome may be the reason for the rapid worsening of AS in this case.
  960 94 1
A case of partial anomalous systemic venous drainage and perioperative detection of cerebral arteriovenous malformations
Nilanjan Dutta, Debasis Das, Raghu Maruti Govindappa, Patralekha Das, Rangan Koley, Manish Kumar Sharma, Amitabha Chattopadhyay, Sanjiban Ghosh
April-June 2021, 14(2):244-246
We report a case of 8-year-old boy with unexplained desaturation and clubbing. Echocardiography showed anomalous drainage of right superior vena cava into left atrium. He did not have any neurological symptoms preoperatively. Some perioperative observations and subtle postoperative behavioral changes prompted us to investigate him further. He was found to have extensive cerebral arteriovenous malformations and hemiatrophy of brain.
  832 97 -
Mitral valve replacement in infants
Arkalgud Sampathkumar
April-June 2021, 14(2):249-249
  618 75 -
Anatomical substrate for biventricular repair in patients with left isomerism
Andrea Madrigali, Carolina Putotto, Bruno Marino, Paolo Versacci
April-June 2021, 14(2):250-251
  526 57 -