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   Table of Contents - Current issue
Coverpage
January-April 2022
Volume 6 | Issue 1
Page Nos. 1-87

Online since Friday, April 29, 2022

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ORIGINAL RESEARCH  

A Study on the Cardiac Manifestations and Their Short-Term Outcome in Patients with Kawasaki Disease in an Indian Population Highly accessed article p. 1
Gopalan Nair Rajesh, S Prasanth, Haridasan Vellani, Jomy Vadasseril Jose
DOI:10.4103/jiae.jiae_75_20  
Objectives: This study aimed to assess the prevalence of cardiac manifestations and their short-term outcome in patients with Kawasaki disease (KD) in an Indian population. Methods: This single-center prospective study enrolled a total of 56 consecutive patients with classic and incomplete KD. Six months and 1-year clinical outcomes were analyzed. Results: Fifty-six consecutive patients were enrolled in the study and followed up for 1 year. About 87% completed 6 months follow-up. A diagnosis of complete KD was made in 41 (73%) patients and incomplete KD in 15 (27%). Cardiac manifestations were present in 25% of patients with KD. Coronary involvement was observed in 11 (20%) patients by either Japanese Ministry of Health Criteria or a z score of ≥2.5. Nonpurulent conjunctivitis was significantly higher among children with cardiac involvement (79% vs. 43% P = 0.04). The mean value of erythrocyte sedimentation rate (ESR) was significantly higher among patients with cardiac involvement (101.92 ± 22 vs. 74.26 ± 28.6; P = 0.002). An ESR value of >100 mm/hr was predictive of cardiac involvement (P = 0.03). The mean serum glutamic-pyruvic transaminase value was higher among those with cardiac involvement (P = 0.008). Coronary dilatation got resolved in 3 months in 73% of patients. Valvular regurgitations, aortic root involvement, and depressed left ventricular myocardial function were not observed in the population studied. Conclusions: Cardiac manifestations were present in 25% of patients with KD, 20% of children had coronary involvement in the form of an aneurysm, or ectasia. About 73% of patients with coronary dilatation got resolved in 3 months. Significant valvular heart diseases, aortic root involvement, and myocardial contractile dysfunction were not seen in the studied population with KD.
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Measurement of Caval Aorta Diameter Index by Transthoracic Echocardiogram in Normal Indian Population Highly accessed article p. 8
Shailendra Sompura, Venkatesan Sangareddi, N Swaminathan
DOI:10.4103/jiae.jiae_24_21  
Background: Bedside ultrasonography is a promising noninvasive and rapid technique to assess body fluid status. Early detection of hypovolemia and hypervolemia is crucial to guide management in critically ill patients. There is a considerable demographic variability in the normal range of inferior vena cava dimensions as seen in numerous studies and a steadfast formula to calculate caval diameter based on demographic parameters and body growth is not available till date. Caval aorta diameter index had a positive correlation with invasively measured central venous pressure as established by previous studies done in intensive care units. The simplicity of examination technique makes this index useful in every situation where body fluid status has therapeutic and prognostic implications. This study aims to identify standard normal measurement of caval aorta index by easily available two-dimensional echocardiography in subcostal view and by right coronal approach/transhepatic view in different age groups in Indian patients. Materials and Methods: One hundred and two patients referred for routine cardiac status evaluation who were asymptomatic and clinically normal without any major medical illness with normal echocardiogram were prospectively analyzed and caval aorta index assessed in both subxiphoid and transhepatic right coronal view. Results: Normal caval aorta index measured in subcostal view was 1.108 ± 0.076 and measured in transhepatic view was 1.101 ± 0.082 (mean ± 2 standard deviation). The difference of caval aorta index measured in both views was not significant statistically (P = 0.207). Conclusion: In this study, we provide a normal reference level for sonographic caval aorta index in Indian population measured in subxiphoid and transhepatic views. We also conclude that transhepatic right coronal view measurements for this index are comparable to subcostal view, so both views can be used alternatively when one window is suboptimal.
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A Pilot Project to Identify Simple Echocardiographic Tools as an Alternative to Cardiac Magnetic Resonance Imaging to Predict a Reduced Right Ventricular Ejection Fraction in Patients with Repaired Tetralogy of Fallot Highly accessed article p. 13
Asish Ranjan Mohakud, Kothandam Sivakumar, Arvind Sahay Singh, Pramod Sagar
DOI:10.4103/jiae.jiae_26_21  
Background: Right ventricular (RV) dysfunction is an important predictor of adverse events after tetralogy of Fallot (TOF) repair. A study comparing echocardiography with cardiac magnetic resonance (CMR) might provide simple tools for their serial inexpensive assessment. Methods: Echocardiographic RV functional parameters including fractional area change (FAC), rate of pressure rise (dP/dt), free wall strain, myocardial performance index (MPI), tricuspid annular plane systolic excursion (TAPSE), and tissue Doppler S' velocity in patients with repaired TOF were correlated with CMR-derived ejection fraction (EF) using receiver operator characteristic (ROC) curves. Bland–Altman plots analyzed agreement between the RV end-diastolic volumes (EDVs), end-systolic volumes (ESVs), and EF derived from three-dimensional echocardiography (3DE) and CMR. Results: Eighteen patients were included. The echocardiographic parameters that showed a good positive correlation with CMR-derived EF were FAC (r = 0.851, P = 0.001), dP/dt (r = 0.730, P = 0.001), and free wall strain (r = −0.660, P = 0.003). ROC curve analysis provided a cutoff value for FAC (<36.6), dP/dt (<370 mmHg/s), free wall strain (<−18.5) and MPI (>0.565) to predict EF <45%. TAPSE and S' velocity had poor correlation. Correlation was strong between 3DE and CMR-derived EDV (r = 0.938, P <0.001), ESV (r = 0.936, P <0.001) and EF (r = 0.916, P <0.001). 3DE underestimated volumes compared to CMR with a mean bias of − 31.78 ± 18.8 ml for EDV and −17.28 ± 11.6 ml for ESV, but EF was not affected (mean bias − 1% ± 3.7%). Conclusions: RV free wall strain, FAC and dP/dt were simple tools with good accuracy to predict RV EF <45% in patients with repaired TOF. TAPSE and S' velocity assessed basal longitudinal function without considering the dysfunctional outflow tract and showed poor correlation. EF assessment by 3DE was a good alternative to CMR. Underestimation of volumes by 3DE might restrict its use in timing surgery.
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An Observational Data Analytical Research on Pediatric Cardiomegaly as a Predictor of Structural or Functional Heart Diseases: Chest X-ray versus Echocardiography Comparison and Contemplation p. 21
Munesh Tomar, Fatima Afreen Ahmad, Maitri Chaudhuri, Vikas Agarwal
DOI:10.4103/jiae.jiae_51_21  
Background: Chest X-ray (CXR) is a routine, noninvasive test advised in a plethora of pediatric non-cardiac conditions such as bronchopneumonia, respiratory distress, failure to thrive, suspected tuberculosis, and for preoperative anesthesia clearance. Finding of cardiomegaly (CM) on CXR is a common cause of referral to a pediatric cardiologist. The question lies in its significance: whether CM in CXR corroborates with cardiac disease (s), structural or functional. Materials and Methods: The data of 229 children (median age: 6 months), referred to pediatric cardiology unit for echocardiography with CXR depicting CM over a period of 2 years (June 2019 to July 2021), were retrospectively reviewed. Clinical and laboratory findings, CXR, and echocardiography were analyzed for all children to determine the positive predictive value of CM in CXR to detect cardiac disease. Echocardiography was taken as gold standard to reach the cardiac diagnosis. Results: True CM was noted in 85% (Group A), whereas 15% had false CM (Group B). Group A comprised structural heart defect in 71.7% and ventricular dysfunction in 13.3%. Less common causes were severe anemia, hypertensive heart failure, arrhythmogenic cardiomyopathy, diphtheritic cardiomyopathy, multisystem inflammatory syndrome in children, pericardial effusion, thiamine deficiency, and severe idiopathic pulmonary arterial hypertension. In Group B, the most common reasons for false diagnosis were expiratory film (n = 18), thymic shadow (n = 12), and chest deformity and mediastinal mass (n = 2 each). Conclusion: CM in CXR strongly correlates with cardiac involvement and has a high positive predictive value. Combining clinical, laboratory, and CXR interpretation enables the pediatrician to achieve a rapid functional diagnosis. Medical stabilization can be initiated with this knowledge pending the availability of echocardiography in resource-limited areas. Detailed evaluation at a pediatric cardiac center should be completed to reach a final diagnosis in all such patients. In the absence of congenital heart disease, known acquired causes leading to cardiac compromise should be actively looked for.
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STATE-OF-THE-ART REVIEW Top

Reperfusion Injury-Related Intramyocardial Hemorrhage: Pivotal Role of Echocardiography and Magnetic Resonance Imaging in Diagnosis and Prognosis p. 32
Jagdish Chander Mohan, Madhu Shukla, Nitin Burkule
DOI:10.4103/jiae.jiae_3_22  
Myocardial reperfusion injury is defined as the death of cardiomyocytes as a direct result of one or more events initiated by reperfusion. These events could be inflammation, oxidative stress, calcium overload, neurohumoral activation, cytotoxicity of anaerobic metabolites, etc. Intramyocardial hemorrhage as a consequence of ischemia–reperfusion injury during acute myocardial infarction and subsequently is frequent and portends worse prognosis. Animal studies have demonstrated that intramyocardial hemorrhage does not occur with ST-elevation myocardial infarction unless myocardium is reperfused with blood. Magnetic resonance imaging is the technique of choice for its detection but has limited availability in emergencies and is expensive. Echocardiography can be used for indirect myocardial tissue characterization. Echocardiography coupled with myocardial contrast imaging is increasingly being used for detecting hemorrhage in infarcted segments. In the presence of wall motion abnormality, increased segmental echogenicity, significantly increased wall thickness underlying hypermobile endocardium, and neocavitations within the myocardium are the characteristic features. Occasionally, extensive wall splitting and formation of pseudotumor due to large hematoma are the striking features of intramyocardial hemorrhage. Intramyocardial hemorrhage in acute myocardial infarction can occur during early phase, following reperfusion and during remodeling process. There is no definite echocardiographic imaging method to assess reperfusion hemorrhage in vivo, but signal-void cavity-like or cystic appearance within the myocardium in the setting of myocardial infarction is highly suggestive. Detecting hypointense area of hemorrhage could be complicated by low-intensity echoes emanating from the normal myocardium. Echocardiography should be performed in every patient before and after reperfusion therapy and serially till discharge. There are no studies comparing the diagnostic yield of echocardiography compared to magnetic resonance imaging. Those with obvious myocardial hematoma need special attention with regard to antiremodeling agents, dual antiplatelet therapy, and possibly surgery. A majority of patients with significant intramural hemorrhage end up having reduced left ventricular function, adverse remodeling, true or pseudo-aneurysms, and even heart failure although spontaneous resorption has also been reported.
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REVIEW ARTICLE Top

Risk Stratification in Acute Normotensive Pulmonary Embolism– Role of Echocardiography Imaging and Biomarkers p. 45
Rohit Tandon, Adesh Kumar Singh, Bishav Mohan
DOI:10.4103/jiae.jiae_41_21  
Acute pulmonary embolism (PE) is an important cause of mortality. It requires alertness to facilitate early diagnosis which becomes a benchmark for further risk stratification and optimal management. Although pulmonary artery imaging by computed tomography scan has become the gold standard in diagnosis of acute PE, echocardiography also plays a complementary role as an imaging modality in deciding about the treatment and for prognostication. Combining echocardiography with cardiac-specific biomarker assays further enhances the required diagnostic yield in the emergency setting. In this chapter, we mainly focus on the role of echocardiography along with specific biomarker assays in prognostication of acute PE patients who are normotensive at presentation.
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INTERESTING CASE REPORTS Top

An Unusual Case: Intravascular Journey of a Benign Uterine Tumor to the Heart p. 53
Deepak Ramchand Nenwani, N Swaminathan, S Venkatesan, C Elangovan, K Tamil Selvan
DOI:10.4103/jiae.jiae_10_20  
Intravenous leiomyomatosis (IVL) is a rare neoplasm characterized by histologically benign-looking smooth muscle cell tumor mass which grows within the intrauterine and extrauterine venous system. In this report, we present an unusual case of IVL which had originated from uterus and extended throughout the right cardiac chambers. A 36-year-old female patient came with a chief complaint of palpitation for 1-month duration. She also had a history of amenorrhea for 7 months. She had abdominal distension and doughy feeling of the abdomen. Seven months prior, ultrasound abdomen done during gynecological evaluation showed fibroid uterus. Echocardiography done showed multiple masses occupying almost all of the right atrium and moving in and out of the right ventricle. Inferior vena cava (IVC) was dilated and 90% of the IVC was filled with the mass. Cardiothoracic surgery and surgical oncology references were done. The patient was planned for simultaneous abdominal and cardiothoracic surgery. Large abdominal mass along with cardiac mass was resected along with mesentery, omentum, and abdominal lymph nodes. Histopathological and microscopic report was suggestive of leiomyoma and leiomyomatosis. Lymph node microscopy showed reactive hyperplasia.
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Quadrivalvular Involvement in Rheumatic Heart Disease: A Rare Case Report p. 56
CM Satvic, Arnab Ghosh Chaudhury, Lachikarathman Devegowda, Prabhavathi Bhat, CN Manjunath
DOI:10.4103/jiae.jiae_56_20  
Four-valve involvement (mitral, aortic, tricuspid, and pulmonary) in rheumatic heart disease (RHD) is an exceedingly rare entity. We report the case of a 24-year-old male, a known case of RHD, who presented with dyspnea and presyncope for 2 months. On examination, the patient had severe mitral stenosis, moderate mitral regurgitation, severe aortic stenosis, severe aortic regurgitation, and severe tricuspid regurgitation. Echocardiography revealed severe tricuspid stenosis with moderate pulmonary stenosis in addition to other clinically detectable valve lesions. The patient underwent aortic and mitral valve replacement, tricuspid valve repair with pulmonary valvotomy successfully.
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A Case of Bioprosthetic Valve Thrombosis: Is it Time to Revise Our Thinking? p. 59
Ramkumar Rajappan, PR Vydianathan, R Shanmuga Sundaram
DOI:10.4103/jiae.jiae_81_20  
Surgical valve replacement is a time-tested modality of treatment in patients with significant valvular heart disease. Bioprosthetic valves (BPVs) are preferred among certain patients because of less thrombogenicity which warrants only a short duration of anticoagulation as recommended by guidelines. Subclinical thrombosis as well as symptomatic BPV thrombosis is being increasingly recognized nowadays, more because of improvement in diagnostic modalities and increasing awareness. Our case report highlights the occurrence of BPV thrombosis in a patient who underwent surgical BPV implantation and was on anticoagulation for guideline recommended duration. This report also highlights the need for regular periodic echocardiographic screening of these patients to detect BPV thrombosis at an early stage.
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Drowning in the Effusion: Cardiac Tamponade as Presenting Feature of Lymphoma in a Young Boy p. 63
Anil Kumar Singhi, AP Bharat, Roy Varghese, Kothandam Sivakumar
DOI:10.4103/jiae.jiae_14_21  
Cardiac tamponade in children has multiple etiological factors such as infection, post-inflammatory, malignancy, or blunt trauma. Detailed evaluation of the accompanying clinical features along with laboratory investigations and imaging help in appropriate diagnosis and management. We describe a case where cardiac tamponade manifested as the first presentation of childhood lymphoma but was mistaken as post-traumatic hemopericardium and tamponade.
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Ebstein Anomaly of the Tricuspid Valve with Intact Inter-atrial Septum and Cor Triatriatum Dexter in an Asymptomatic Adult: The Tri-chambered Right Atrium p. 66
Madhu Shukla, Jagdish Chander Mohan
DOI:10.4103/jiae.jiae_21_21  
Ebstein anomaly of the tricuspid valve is characterized by apical displacement of septal and posterior leaflets of the tricuspid valve leading to the atrialization of right ventricular cavity and severe low-pressure tricuspid regurgitation. Cor triatriatum dexter is a rare congenital cardiac anomaly in which a membranous structure divides the right atrium into two chambers. This combination is rare and very few cases have been described in the literature. We herein report an asymptomatic young female who was incidentally detected to have these anomalies with intact atrial septum. Echocardiographic examination showed tri-chambered right atrium consisting of atrialized right ventricle, anterior right atrial chamber, and the posterior right atrial sinus portion.
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A patient with type IV unroofed coronary sinus presenting with wide QRS Tachycardia p. 69
Ashish Kumar, Rohit Tandon, Abhishek Goyal, Gurpreet Singh Wander
DOI:10.4103/jiae.jiae_25_21  
Unroofed coronary sinus (UCS) is a congenital heart disease characterized by defects in the formation of the coronary sinus. UCS usually manifests as dyspnea on effort, fatigue, or palpitation. However, here, we present a case of a 53-year-old female whose presentation was wide QRS tachycardia. In this interesting case, we describe the role of various echocardiographic modalities used to reach its elusive diagnosis.
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Mega Coronary Arteries with Obstructive Coronary Artery Disease in Hypertrophic Obstructive Cardiomyopathy: A Case Report and Literature Review p. 72
Debasish Das, Debasis Acharya, Tutan Das, Subhas Pramanik
DOI:10.4103/jiae.jiae_44_21  
We report a rare case of hypertrophic obstructive cardiomyopathy (HOCM) presenting with mega coronary arteries with obstructive coronary artery disease. Interestingly, the patient had large septals, large diagonals, large obtuse marginal with a large left atrial branch, and a large conus almost mimicking a duplicate right coronary artery. Because of massive left ventricular hypertrophy, to overcome the supply-demand mismatch, coronary arteries may become large or with more arborization in hypertrophic cardiomyopathy but the same is rarely encountered in routine clinical practice. Our case is a rare illustration of the simultaneous presence of mega coronary arteries and critical obstructive coronary artery disease in HOCM in a young male of 38 years of age.
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Dextroversion and D-Transposition of the Great Arteries, Left-Handed Ventricular Topology Related to Multiple Ventricular Septal Defects, and Left Juxtaposition of Atrial Appendage in a 1-Year-Old Child p. 76
Elio Caruso, Silvia Farruggio, Maria Vittoria Raciti, Salvatore Agati
DOI:10.4103/jiae.jiae_50_21  
We present a 1-year-old female infant case in natural history, affected by D-transposition of the great arteries with multiple ventricular septal defects in dextroversion, left juxtaposition of atrial appendages, and persistent left superior caval vein draining into coronary sinus. This is an uncommon case, and its particularity is due to clinical and anatomical findings diagnosed late in a setting of a complex cardiac malformation. After the diagnosis, the patient underwent palliative arterial switch operation without complications.
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Unraveling the Mystery of Young Stroke: A Rare Cardiac Manifestation of Systemic Disease p. 80
Siddharthan Deepti, A Shaheer Ahmed
DOI:10.4103/jiae.jiae_15_21  
A 38-year-old male presented with a history of acute-onset hemiparesis 6 months back. On evaluation, he was found to have eosinophilia. Echocardiography was done which revealed a thrombus in the left atrium attached to the posterior mitral leaflet. The rest of the cardiac chambers were normal. Diagnosis of idiopathic hypereosinophilic syndrome was made after extensive evaluation. The patient was started on steroids and oral anticoagulation, following which eosinophil count and size of thrombus reduced.
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INTERESTING IMAGES Top

A Humongous Atrial Septal Defect p. 82
Tarun Bansal, Antara Banerji, Ajay Bahadur, Anupam Mehrotra, Pradyot Tiwari
DOI:10.4103/jiae.jiae_4_21  
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Low-Velocity Flow Doppler Enhancement for the Study of Spontaneous Echo Contrasts Highly accessed article p. 84
George Thomas
DOI:10.4103/jiae.jiae_47_21  
Detection of spontaneous echo contrasts (SEC) is important in assessing the risk of stroke and thromboembolism. Transthoracic echocardiography is the most widely used primary imaging for left ventricular SEC. Although harmonic imaging improves visualization to an extent, further improvement in the detection of the phenomenon is desirable. We are presenting images of two patients acquired with a novel method using Doppler to enhance the detection of SEC.
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LETTER TO THE EDITOR Top

Three-Dimensional Transesophageal Echocardiography Workflow in the Operating Room and Intervention Suits p. 86
Rahul Shabadi
DOI:10.4103/jiae.jiae_10_22  
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