Annals of Pediatric Cardiology
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   Table of Contents - Current issue
May-August 2017
Volume 10 | Issue 2
Page Nos. 109-222

Online since Tuesday, April 25, 2017

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A roadmap for the aspiring interventional pediatric cardiologist p. 109
Raman Krishna Kumar
DOI:10.4103/apc.APC_52_17  PMID:28566816
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Three-dimensional-printed cardiac prototypes in complex congenital cardiac defects: New technology with exciting possibilities p. 114
Sreekanthan Sundararaghavan
DOI:10.4103/apc.APC_48_17  PMID:28566817
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Three-dimensional-printed cardiac prototypes aid surgical decision-making and preoperative planning in selected cases of complex congenital heart diseases: Early experience and proof of concept in a resource-limited environment p. 117
Mahesh Kappanayil, Nageshwara Rao Koneti, Rajesh R Kannan, Brijesh P Kottayil, Krishna Kumar
DOI:10.4103/apc.APC_149_16  PMID:28566818
Introduction: Three-dimensional. (3D) printing is an innovative manufacturing process that allows computer.assisted conversion of 3D imaging data into physical “printouts” Healthcare applications are currently in evolution. Objective: The objective of this study was to explore the feasibility and impact of using patient-specific 3D-printed cardiac prototypes derived from high.resolution medical imaging data. (cardiac magnetic resonance imaging/computed tomography. [MRI/CT]) on surgical decision-making and preoperative planning in selected cases of complex congenital heart diseases. (CHDs). Materials and Methods: Five patients with complex CHD with previously unresolved management decisions were chosen. These included two patients with complex double.outlet right ventricle, two patients with criss-cross atrioventricular connections, and one patient with congenitally corrected transposition of great arteries with pulmonary atresia. Cardiac MRI was done for all patients, cardiac CT for one; specific surgical challenges were identified. Volumetric data were used to generate patient-specific 3D models. All cases were reviewed along with their 3D models, and the impact on surgical decision-making and preoperative planning was assessed. Results: Accurate life-sized 3D cardiac prototypes were successfully created for all patients. The models enabled radically improved 3D understanding of anatomy, identification of specific technical challenges, and precise surgical planning. Augmentation of existing clinical and imaging data by 3D prototypes allowed successful execution of complex surgeries for all five patients, in accordance with the preoperative planning. Conclusions: 3D-printed cardiac prototypes can radically assist decision-making, planning, and safe execution of complex congenital heart surgery by improving understanding of 3D anatomy and allowing anticipation of technical challenges.
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Neonates with critical congenital heart defects: Impact of fetal diagnosis on immediate and short-term outcomes p. 126
Sylvia Michael Colaco, Tanuja Karande, Prashant Raviprakash Bobhate, Rashmi Jiyani, Suresh G Rao, Snehal Kulkarni
DOI:10.4103/apc.APC_125_16  PMID:28566819
Background: Fetal echocardiography is being increasingly used for prenatal diagnosis of congenital cardiac malformations, but its impact on the neonatal outcomes in low- and middle-income countries is still unknown. Aims: The objective of this study is to determine the impact of fetal echocardiography on immediate postnatal and short.term outcome in a tertiary pediatric cardiac center. Study Design: This is a prospective study. Materials and Methods: One hundred consecutive patients with critical congenital heart defects (CHD) requiring active medical or surgical interventions in the 1st month of life were included in the study. The detailed history, postnatal examination findings, and fetal echocardiogram report were recorded. They were divided into two groups as antenatally diagnosed and postnatally diagnosed. Pre- and post-procedural variables were compared between the two groups. Results: Twenty-nine neonates were diagnosed antenatally while 71 were diagnosed postnatally. Totally, 10 babies (34.5%) among the antenatally diagnosed group were delivered in a tertiary health-care setup. The mean age at presentation was 0. day in the antenatally diagnosed group while 10 days. (0–30 days) in the postnatally diagnosed group. (P = 0.01). A total of 17 (58.6%) patients in the antenatal group had duct dependent CHD, and 15 (88.2%) of these patients were transported on prostaglandin E1. In comparison, 19/34 (55.9%) patients in the postnatal group were transported on prostaglandin. The pH on admission in the antenatal group was 7.32 ± 0.05 as compared to 7.28 ± 0.05 in the postnatal group. (P = 0.0004). There were 4 (5.6%) deaths in the postnatal group during transfer. There was no significant difference in the postoperative variables in both groups. Conclusions: Fetal echocardiography identifies patients with complex CHD resulting in better parental counseling, thus facilitating delivery at a tertiary care center and preoperative stabilization. This results in improved preoperative mortality and better stabilization.
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Patent ductus arteriosus closure using Occlutech® Duct Occluder, experience in Port Elizabeth, South Africa p. 131
Lungile Pepeta, Adele Greyling, Mahlubandile Fintan Nxele, Zongezile Masonwabe Makrexeni
DOI:10.4103/0974-2069.205144  PMID:28566820
Background: Percutaneous closure of patent ductus arteriosus (PDA) has become standard therapy. Experience with the Occlutech® Duct Occluder is limited. Methods: Data regarding ductal closure using Occlutech® Duct Occluder were reviewed and prospectively collected. Demographics, hemodynamic and angiographic characteristics, complications, and outcomes were documented. Results: From March 2013 to June 2016, 65 patients (43 females and 22 males) underwent percutaneous closure of the PDA using Occlutech® Duct Occluder. The median age of the patients was 11 months (range, 1–454 months) and the median weight was 8.5 kg (range 2.5–78 kg). The mean pulmonary artery median pressure was 27 mmHg (range, 12–100 mmHg) and the QP: Qs ratio median was 1.8 (range, 1–7.5), with a pulmonary vascular resistance mean of 2.7 WU (standard deviation [SD] ±2.1). Thirty-two patients had Krichenko Type A duct (49%); 7, Type C (11%); 4, Type D (6%); and 22, Type E (34%). The ductal size (narrowest diameter at the pulmonic end) mean was 3.5 mm (SD ± 1.9 mm). The screening time mean was 17.3 min (SD ± 11.6). Out of 63 patients with successful closure of the PDA using Occlutech® Duct Occluder, there were 15 patients with small PDAs; 25 with moderate PDAs, and 23 with large PDAs. In one patient, the device dislodged to the descending aorta, and in two patients, to the right pulmonary artery immediately following deployment, with successful percutaneous (two) and surgical (one) retrieval. Complete ductal occlusion was achieved in all 63 patients on day one. Conclusion: The Occlutech® Duct Occluder is a safe and effective device for closure of ducts in appropriately selected patients.
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Mid-term outcomes of surgical repair for anomalous origin of the left coronary artery from the pulmonary artery: In infants, children and adults p. 137
Maziar Gholampour Dehaki, Alwaleed Al-Dairy, Yousef Rezaei, Alireza Alizadeh Ghavidel, Gholamreza Omrani, Nader Givtaj, Reza Sadat Afjehi, Hassan Tatari, Amir Hossein Jalali, Mohammad Mahdavi
DOI:10.4103/0974-2069.205140  PMID:28566821
Background: Anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital malformation. We sought to evaluate in-hospital and mid-term outcomes of patients with a diagnosis of ALCAPA who underwent surgical repair. Objectives: The objective of this study is to evaluate the mid-term outcomes of surgical repair of ALCAPA at our center and to analyze the surgical techniques used. Materials and Methods: In a retrospective study, we analyzed early and mid-term clinical and echocardiographic data to determine the outcomes of patients who underwent surgical repair of ALCAPA in our institution between 2005 and 2015. Results: Twenty-one patients underwent surgical repair for ALCAPA using aortic reimplantation (n = 10, 47.6%), ostial closure. (n = 8, 38.1%), or ligation. (n = 3, 14.3%). The median age of patients was 24. months. (range 22 days to 51 years). There were 2 (9.5%) in-hospital mortalities in infants undergoing the reimplantation technique. All patients were followed up for a median of 21 months. (range 1–60 months). No patients required reoperation, and there was no mortality from discharge to mid-term follow-up. Severe early postoperative mitral regurgitation. (MR) was associated with composite end-point, defined as a combination of mortality after surgery, moderate to severe MR, and moderate to severe left ventricular dysfunction at late follow-up. (P = 0.019) while mitral valve repair was not. (P = 0.469). Conclusion: The surgical management of ALCAPA can be associated with good in-hospital and mid-term outcomes regardless of the age, at which the patient has been operated.
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Cost-effectiveness analysis of different devices used for the closure of small-to-medium-sized patent ductus arteriosus in pediatric patients p. 144
Sonia A El-Saiedi, Amal M El Sisi, Rodina Sobhy Mandour, Doaa M Abdel-Aziz, Wael A Attia
DOI:10.4103/0974-2069.205138  PMID:28566822
Aims: In this study, we examined the differences in cost and effectiveness of various devices used for the closure of small to medium sized patent ductus arteriosus (PDA). Setting and Design: We retrospectively studied 116 patients who underwent closure of small PDAs between January 2010 and January 2015. Subjects and Methods: Three types of devices were used: the Amplatzer duct occluder (ADO) II, the cook detachable coil and the Nit Occlud coil (NOC). Immediate and late complications were recorded and patients were followed up for 3 months after the procedure. Statistical Methods: All statistical calculations were performed using Statistical Package for the Social Science software. P <0.05 were considered significant. Results: We successfully deployed ADO II devices in 33 out of 35 cases, cook detachable coils in 36 out of 40 cases and NOCs in 38 out of 41 cases. In the remaining nine cases, the first device was unsuitable or embolized and required retrieval and replacement with another device. Eleven patients (9.5%) developed vascular complications and required anticoagulation therapy. Patients who had hemolysis or vascular complications remained longer in the intensive care unit, with consequently higher total cost (P = 0.016). Also, the need for a second device increased the cost per patient. Conclusions: The cook detachable coil is the most cost.effective device for closure of medium.sized PDAs. Calculations of the incremental cost.effectiveness. (ICE) revealed that the Cook detachable coil had less ICE than the ADO II and NOC. The NOC was more effective with fewer complications.
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Correlation of electrocardiogram parameters and hemodynamic outcomes in patients with isolated secundum atrial septal defects p. 152
Mohammad Refaei, Sunjidatul Islam, Andrew S Mackie, Joseph Atallah
DOI:10.4103/0974-2069.205139  PMID:28566823
Objective: The characteristic rSR' pattern in lead V1 on electrocardiogram (ECG) has been described in association with atrial septal defect (ASD) and right ventricular dilation. We aimed to determine if temporal ECG changes can guide a more discriminate and cost-effective screening during follow-up of isolated secundum ASD. Methods: Our study population included all pediatric patients followed at the Stollery Children's Hospital with a secundum ASD, not associated with other significant heart disease, between 2004 and 2010. We collected clinical as well as serial echocardiographic and ECG data. Results: We identified 141 patients with ASD, 95% were asymptomatic and 88% referred for a murmur. Moderate-to-large (>5 mm) ASDs were present in 52%. The prevalence of an rSR' pattern was 26% in the overall cohort and 54% in the large ASD group. During median follow-up of 28.7 months, 37 patients underwent surgical or transcatheter closure. Among patients with rSR' on ECG, 78% had moderate-to-large ASD size. In that group, the presence versus the absence of rSR' correlated with lower positive predictive value (PPV) for spontaneous closure (7% vs. 36%; P = 0.01) and higher PPV for device or surgical closure (71% vs. 38%; P = 0.02). Conclusion: We observed a lower prevalence of rSR' pattern in patients with isolated ASD than previously reported. However, an rSR' pattern had incremental value in predicting the need for surgical or device intervention for closure in moderate.large groups. This can be used to tailor patient echocardiographic screening and caregiver counseling.
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Use of strain, strain rate, tissue velocity imaging, and endothelial function for early detection of cardiovascular involvement in patients with beta-thalassemia p. 158
Abhinav Gupta, Aditya Kapoor, Shubha Phadke, Archana Sinha, Shridhar Kashyap, Roopali Khanna, Sudeep Kumar, Naveen Garg, Satyendra Tewari, Pravin Goel
DOI:10.4103/apc.APC_132_16  PMID:28566824
Background: Global ventricular function often remains normal in patients with beta-thalassemia major. (β-TM) until late. Tissue Doppler and strain imaging may be useful to assess regional myocardial function abnormalities in these patients. Methods: Systolic (Sm), early diastolic (Em), and late diastolic (Am) (Em/Am) myocardial velocities at basal lateral and septal left ventricular (LV) segments, strain (S), and strain rate (SR) in basal and mid LV, right ventricular (RV) and septum were measured in 30 patients (β-TM, 12.4 ± 5.2 years, serum ferritin 2603.1 μg/L) and twenty controls (12.5 ± 5.2 years). Flow-mediated dilatation (FMD) vasodilatation as a measure of endothelial function was also assessed. Results: Patients had significantly higher LV mass index (169.45 ± 61.14 vs. 104.66 ± 24.42; P = 0.009) while global LV Sm and diastolic function was similar to controls. Patients had significantly lower lateral Em velocity, Em (10.12 ± 1.16 vs. 17.9 ± 2.11; P = 0.002), Em/Am ratio (0.811 ± 0.192 vs. 2.06 ± 0.62; P = 0.001) at the basal lateral LV, lower strain values at the basal lateral LV (19.5 ± 4.17 vs. 24.196 ± 1.81; P = 0.002), mid lateral LV (19.07 ± 3.98 vs. 25.56 ± 2.62; P = 0.042), basal septum (17.04 ± 3.44 vs. 25.43 ± 2.53; P = 0.001), and mid septum (20.49 ± 5.34 vs. 24.45 ± 2.20; P = 0.001) as compared to controls. SR at the basal and mid segment of the lateral LV wall and at the basal and mid septum was also significantly lower in patients. SR in basal and mid RV although lower was not significantly different from controls. Patients also had significantly lower FMD (7.57 ± 3.16 vs. 18.08 ± 1.9, P = 0.018) implying endothelial dysfunction. Conclusions: Tissue Doppler, strain and SR imaging are useful to quantify regional myocardial function in asymptomatic α-TM patients with preserved global Sm and diastolic function.
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Catheter hemodynamic assessment of the univentricular circulation p. 167
Oliver Stumper, Gemma Penford
DOI:10.4103/apc.APC_160_16  PMID:28566825
Children with very complex congenital heart disease not amenable to biventricular repair are increasingly being considered for a palliative univentricular care pathway. This involves a staged surgical approach culminating in the Fontan circulation with passive pulmonary blood flow and added resistances. The catheter based hemodynamic assessment at all three stages of this palliation is described in detail. Frequent pitfalls, inherent limitations and potential errors are discussed and clinical examples are illustrated.
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Isomerism in the setting of the so-called “heterotaxy”: The usefulness of computed tomographic analysis p. 175
Shumpei Mori, Robert H Anderson, Tatsuya Nishii, Kensuke Matsumoto, Rohit S Loomba
DOI:10.4103/apc.APC_171_16  PMID:28566826
The most complex combinations of congenital cardiac malformations are found in the setting of bodily isomerism. The question remains, however, as to whether evidence of cardiac isomerism is always to be found in the setting of bodily isomerism, also known as “heterotaxy.” We have previously shown that, when assessed on the basis of the extent of the pectinate muscles relative to the atrioventricular junctions, there is always isomerism of the atrial appendages in this setting. Doubt has been remained, however, as to whether these cardiac features can accurately be recognized during life. We have now encountered two patients showing features of the left and right bodily isomerism. Examinations of these patients made using computed tomography show that all features of isomerism, no matter how complex, can now be visualized during life. The images currently presented show, furthermore, that the features of the so-called “heterotaxy” can be seen during life, not only within the heart but also in all the thoracic and abdominal organs, albeit that the isomeric features are confined to the thoracic organs. Based on the images presented, we argue that if each system of organs is analyzed and described in independent fashion; then it is possible for clinicians to exclude any suggestion of ambiguity and to provide accurate descriptions of the overall arrangement. We further discuss the appropriate terminology to describe the entity we prefer to call isomerism, along with the indications and usefulness of computed tomography in revealing the anatomic features of the congenitally malformed heart.
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A North African perspective on pediatric cardiac services: A focused interview with Dr. Sulafa Ali p. 187
Sulafa Ali, Sangeetha Viswanathan
DOI:10.4103/apc.APC_153_16  PMID:28566827
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Aberrant right subclavian artery presenting as tracheoesophagial fistula in a 50-year-old lady: Case report of a rare presentation of a common arch anomaly p. 190
Sayyed Ehtesham Hussain Naqvi, Mohammed Hanif Beg, Shyam Kumar Singh Thingam, Eram Ali
DOI:10.4103/apc.APC_158_16  PMID:28566828
A 50-year-old, woman with a 2-year history of progressive dysphagia and 2-month history of chronic cough was referred to our center in a state of generalized sepsis. Provisional diagnosis of carcinoma esophagus with tracheoesophagial fistula was made. Evaluation of the patient revealed an aberrant right subclavian artery with retroesophageal course with compression of the esophagus and trachea with fistulous communication in between. The patient was managed with medical stabilization and with feeding jejunostomy, but she succumbed to underlying severe sepsis. This presentation of aberrant subclavian artery at this advanced age rare and is therefore reported.
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Pitfalls of stenting coarctation of an angulated right circumflex aortic arch in Goldenhar syndrome p. 194
Elaheh Malakan Rad, Hojjat Mortezaeian, Hamid Reza Pouraliakbar, Ziyad M Hijazi
DOI:10.4103/apc.APC_134_16  PMID:28566829
We report stenting of coarctation of an angulated right circumflex aortic arch (RCAA) using four Cheatham Platinum stents in a child with Goldenhar syndrome. Difficulties in measuring the accurate length of the curved and narrowed transverse aortic arch marked discrepancy between the luminal diameters of the long narrow transverse arch and wide descending thoracic aorta, increased displacement force caused by the 90° bend between the two parts resulted in repeated stent migrations. We discuss the tips to avoid distal stent migration in the setting of an angulated RCAA.
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Modified transjugular approach for percutaneous atrial septal defect closure p. 197
Rishi Ashokkumar Bhargava, Ankur Phatarpekar, Charan P Lanjewar, Prafulla G Kerkar
DOI:10.4103/apc.APC_167_16  PMID:28566830
Femoral venous route is routinely used for percutaneous closure of atrial septal defects (ASDs). However, a situation may arise where transfemoral approach is not feasible. We describe a successful transjugular closure of a moderate-sized ASD in a 49-year-old symptomatic man with interrupted inferior vena cava, using a novel deployment technique, which helped in overcoming difficulties such as maintaining stable sheath position and minimizing risk of air embolism.
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Repair of anomalous mitral arcade in a child p. 200
Siddharthan Deepti, Velayoudam Devagourou, Shyam Sunder Kothari
DOI:10.4103/apc.APC_141_16  PMID:28566831
A 13-year-old girl presented with exertional dyspnea and congestive heart failure. Echocardiography revealed severe congenital mitral stenosis due to anomalous mitral arcade with severe pulmonary hypertension. She underwent successful mitral valve repair. The case is reported for its rarity.
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Recanalization of an occluded left pulmonary artery: A case report and review of the literature p. 203
Ali Ibrahim Elarabi, Ming Chern Leong, Mazeni Alwi
DOI:10.4103/0974-2069.205157  PMID:28566832
We report an 8-year-old male child with tetralogy of Fallot (TOF), who developed left pulmonary artery (LPA) atresia, following surgical repair of TOF and left pulmonary arterioplasty at the age of 6 years. He underwent successful radiofrequency recanalization and stenting of the LPA. The LPA exhibited satisfactory growth for 3 months, following recanalization and stenting.
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Stent migration after right ventricular outflow tract stenting in the severe cyanotic Tetralogy of Fallot case p. 206
Tamaki Hayashi, Saleem Akhtar, Mazeni Alwi
DOI:10.4103/0974-2069.205143  PMID:28566833
We report our experience with a stent migration after right ventricle outflow tract stenting and converted to patent ductus arteriosus stenting in Tetralogy of Fallot (TOF) with severe infundibular stenosis. Finally, the patient achieved to TOF repair, and the migrated stent was removed without any complication.
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The utility of computed tomographic angiography in a neonate on extracorporeal membrane oxygenation with extreme cyanosis after Blalock–Taussig shunt p. 209
Jess D Rames, Minoo N Kavarana, U Joseph Schoepf, Anthony Marcus Hlavacek
DOI:10.4103/0974-2069.205137  PMID:28566834
A modified Blalock–Taussig shunt (mBTS) is often employed to provide pulmonary blood flow in neonates that are born with cyanotic congenital heart defects. However, acute shunt thrombosis can occur in the postoperative period, resulting in profound cyanosis. In this case report, we describe the utility of computed tomographic angiography (CTA) in the management of a neonate with extreme cyanosis after placement of a mBTS while on extracorporeal membrane oxygenation. Using CTA, several small clots were identified in the shunt as well as stenosis of the left pulmonary artery; neither of which were identified with echocardiography. The CTA allowed for quick identification of the disorder and helped direct prompt surgical intervention.
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Anomalous origin of right coronary artery causing myocardial ischemia in a young patient p. 212
Anil Kumar Singhi, Ejaz Ahmad Bari, Sunip Banerjee
DOI:10.4103/apc.APC_151_16  PMID:28566835
Anomalous right coronary artery from left coronary sinus can have dynamic narrowing and kinking causing symptoms of myocardial ischemia and sudden cardiac death. Surgical repair of the anomaly is required in the symptomatic patient because of risk of ischemia or ventricular arrhythmia. Asymptomatic incidentally diagnosed low-risk patients can be closely followed up with exercise restriction as per present guideline.
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Apparent normal arrangement pattern of three-vessel view in a fetus with transposition of great arteries and L-malposed aorta p. 215
Sudeep Verma, Shanthi Chidambaratanu, Raja Vijaylakshmi, Latha Srinivasan, Indrani Suresh
DOI:10.4103/apc.APC_10_17  PMID:28566836
Transposition of great arteries (TGA) is more commonly associated with D-malposition of great arteries where anterior aorta produces characteristic “I” sign in the three-vessel view (3VV) in fetal heart imaging. We describe two cases with TGA and L-malposition of aorta where 3VV imaging showed an apparently normal arrangement of vessels while outflow tract imaging proved vital in diagnosing transposition anatomy. Apparently, normal 3VV in the presence of disproportionate vessel caliber and inability to produce normal outflow images should raise the suspicion. Attempts should be made to produce views to show great arteries originating from respective ventricles to rule out ventriculoarterial discordance and to complete segmental analysis.
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Massive biventricular rhabdomyoma in a neonate p. 218
Manish Pandey, Rimlee Dutta, Shyam S Kothari
DOI:10.4103/apc.APC_164_16  PMID:28566837
Rhabdomyoma is a well characterised entity in a neonate. Herein, we report a massive biventricular rhabdomyoma in a neonate presenting with cyanosis and congestive heart failure which was confirmed on autopsy. The report is for documentation of an unusually large tumour.
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Birth prevalence of congenital heart disease: A cross-sectional observational study from North India p. 220
Mahmood Dhahir Al-Mendalawi
DOI:10.4103/apc.APC_124_16  PMID:28566838
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Author's Response p. 220
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Spontaneous pneumoperitoneum: A rare entity p. 221
Himanshu Pratap, Neeraj Awasthy, KS Dagar
DOI:10.4103/0974-2069.205159  PMID:28566840
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