Publications by authors named "Shyam S Kothari"

124 Publications

Outcome of COVID-19-positive children with heart disease and grown-ups with congenital heart disease: A multicentric study from India.

Ann Pediatr Cardiol 2021 Jul-Sep;14(3):269-277. Epub 2021 Aug 26.

Children's Heart Center, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra, India.

Background: Outcome data of children with heart disease who acquired COVID-19 infection are limited.

Aims: We sought to analyze outcome data and identify risk factors associated with mortality in children with heart disease and grown-ups with congenital heart disease (GUCH) who had a laboratory-confirmed COVID-19 infection.

Settings And Design: This is a retrospective, multicentric, observational study.

Materials And Methods: The study included children with heart disease and GUCH population, who presented with either symptomatic or asymptomatic COVID-19 infection to any of the participating centers. COVID-19-negative patients admitted to these centers constituted the control group.

Results: From 24 pediatric cardiac centers across India, we included 94 patients with a median age of 12.5 (interquartile range 3-96) months and 49 (52.1%) patients were males. Majority (83 patients, 88.3%) were children. One-third of the patients ( = 31, 33.0%) had acyanotic congenital heart disease, and 41.5% ( = 39) were cyanotic, with > 80% of the patients being unoperated. Only 30 (31.9%) patients were symptomatic for COVID-19 infection, while the rest were incidentally detected positive on screening. A total of 13 patients died (case fatality rate: 13.8%). The in-hospital mortality rate among hospitalized patients was significantly higher among COVID-19-positive cases (13 of 48; 27.1%) as compared to COVID-negative admissions (9.2%) during the study period ( < 0.001). On multivariate analysis, the independent predictors of mortality among COVID-19-positive cases were severity of illness at admission (odds ratio [OR]: 535.7, 95% confidence interval [CI]: 6.9-41,605, = 0.005) and lower socioeconomic class (OR: 29.5, 95% CI: 1.1-814.7, = 0.046).

Conclusions: Children with heart disease are at a higher risk of death when they acquire COVID-19 infection. Systematic preventive measures and management strategies are needed for improving the outcomes.
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http://dx.doi.org/10.4103/apc.apc_134_21DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8457291PMC
August 2021

Non-specific aortoarteritis (NSAA) in children: a prospective observational study.

BMJ Paediatr Open 2021 9;5(1):e001106. Epub 2021 Aug 9.

Cardiology, All India Institute of Medical Sciences, New Delhi, Delhi, India.

Objective: Prospective data on clinical profile, natural history and outcomes of NSAA (non-specific aortoarteritis) in children is limited. We initiated this prospective study to evaluate the short-term and medium-term outcomes of NSAA in children.

Design: Prospective observational study.

Setting: Tertiary care hospital in India.

Patients: We included 28 consecutive children (<15 years) with a diagnosis of NSAA.

Main Outcome Measures: Clinical profile, symptoms, left ventricular ejection fraction (LVEF), the pattern of vessel involvement, complications and markers of disease activity were assessed at the time of diagnosis. All the patients underwent treatment and interventions as per the current guidelines and were followed up for a mean duration of 13.5±6.7 months for disease activity and outcomes.

Results: The mean age of the cohort was 10±2.9 years (14 boys). Twenty (71%) patients had hypertension. Half of the children presented with acute decompensated heart failure (ADHF). Only 21 patients (75%) met Sharma modified Ishikawa criteria for the diagnosis. The children with active disease (36%) were managed with immunosuppressive drugs. Percutaneous and surgical interventions were performed in 26 (93%) children. New York Heart Association functional class, LVEF and control of hypertension improved in most children on follow-up. Four children developed vascular restenosis requiring reinterventions. There was no death during mid-term follow-up.

Conclusions: Children with NSAA, unlike adults seldom present with classical features of the disease. ADHF and ventricular dysfunction are strikingly common in children. Appropriate immunosuppressive therapy for active disease and timely intervention improves clinical outcomes over a medium term follow-up. Future studies assessing long-term outcome are needed.
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http://dx.doi.org/10.1136/bmjpo-2021-001106DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8354261PMC
August 2021

Clarifying the anatomy of the superior sinus venosus defect.

Heart 2021 Aug 20. Epub 2021 Aug 20.

Biosciences Institute, University of Newcastle, Newcastle-upon Tyne, UK.

Objectives: We sought to clarify the variations in the anatomy of the superior cavoatrial junction and anomalously connected pulmonary veins in patients with superior sinus venosus defects using computed tomographic (CT) angiography.

Methods: CT angiograms of 96 consecutive patients known to have superior sinus venosus defects were analysed.

Results: The median age of the patients was 34.5 years. In seven (7%) patients, the defect showed significant caudal extension, having a supero-inferior dimension greater than 25 mm. All patients had anomalous connection of the right superior pulmonary vein. The right middle and right inferior pulmonary vein were also connected anomalously in 88 (92%) and 17 (18%) patients, respectively. Anomalous connection of the right inferior pulmonary vein was more common in those with significant caudal extension of the defect (57% vs 15%, p=0.005). Among anomalously connected pulmonary veins, the right superior, middle, and inferior pulmonary veins were committed to the left atrium in 6, 17, and 11 patients, respectively. The superior caval vein over-rode the interatrial septum in 67 (70%) patients, with greater than 50% over-ride in 3 patients.

Conclusion: Anomalous connection of the right-sided pulmonary veins is universal, but is not limited to the right upper lobe. Not all individuals have over-riding of superior caval vein. In a minority of patients, the defect has significant caudal extension, and anomalously connected pulmonary veins are committed to the left atrium. These findings have significant clinical and therapeutic implications.
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http://dx.doi.org/10.1136/heartjnl-2021-319334DOI Listing
August 2021

Delivery sheath tip invagination - An unusual cause of failure to retrieve duct occluder.

Ann Pediatr Cardiol 2021 Apr-Jun;14(2):242-243. Epub 2021 Apr 10.

Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India.

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 them effectively.
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http://dx.doi.org/10.4103/apc.APC_165_20DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8174637PMC
April 2021

A rare pediatric case of bilateral bronchopulmonary vascular malformations and right isomerism.

J Card Surg 2021 Aug 24;36(8):2937-2938. Epub 2021 Apr 24.

Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India.

We present an unusual case of an 18-day-old term neonate with coexistent bilateral bronchopulmonary vascular malformations and right isomerism. This case highlights the importance of computed tomography angiography in depicting such complex anomalies and classifying them according to components involved providing a systematic approach for evaluation of the disease process.
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http://dx.doi.org/10.1111/jocs.15573DOI Listing
August 2021

Retroesophageal left brachiocephalic vein in tetralogy of fallot: an anomalous course depicted on computed tomography angiography.

Acta Cardiol 2021 Apr 8:1-2. Epub 2021 Apr 8.

Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India.

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http://dx.doi.org/10.1080/00015385.2021.1909277DOI Listing
April 2021

Ivabradine Versus Amiodarone in the Management of Postoperative Junctional Ectopic Tachycardia: A Randomized, Open-Label, Noninferiority Study.

JACC Clin Electrophysiol 2021 08 31;7(8):1052-1060. Epub 2021 Mar 31.

Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, New Delhi, India.

Objectives: This study sought to compare the efficacy of ivabradine and amiodarone in the management of postoperative junctional ectopic tachycardia (JET) after cardiac surgery in children.

Background: JET is a serious arrhythmia occurring in children after cardiac surgery and requires aggressive management. Amiodarone has been conventionally used in its treatment. Recent studies have reported the utility of ivabradine in this regard.

Methods: In this open-label randomized controlled trial, 94 children (age ≤18 years) who developed postoperative JET were allocated to receive either amiodarone or ivabradine. The primary endpoint was restoration of normal sinus rhythm.

Results: Sinus rhythm was achieved in 43 out of the 46 patients (93.5%) in the amiodarone group and 46 out of the 48 patients (95.8%) in the ivabradine group (mean difference of treatment effect: 2.3%; 95% confidence interval: -6.7% to 11.5%). The median (interquartile range) time taken to achieve sinus rhythm conversion was similar in both the groups: 21.5 (17-30.2) hours versus 22 (13.4-38.5) hours (p = 0.36)]. The time taken to rate control of JET was significantly less in the amiodarone group: median 7.0 (5.5-9.5) hours versus 8.0 (5.8-10.8) hours (p = 0.02)]. No drug-related adverse events were observed in the ivabradine group.

Conclusions: Oral ivabradine is not inferior to intravenous amiodarone in converting postoperative JET to sinus rhythm. There was no difference in time taken to sinus rhythm conversion between the groups, although the rate control was earlier in patients who received amiodarone. Monotherapy with ivabradine may be considered as an alternative to amiodarone in the management of postoperative JET. (Comparison of Two Drugs, Ivabradine and Amiodarone, in the Management of Junctional Ectopic Tachycardia, an Abnormality in Cardiac Rhythm in Patients Under 18 years Who Undergo Cardiac Surgery: CTRI/2018/08/015182).
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http://dx.doi.org/10.1016/j.jacep.2021.01.020DOI Listing
August 2021

Changing Pattern of Congenital Heart Disease Care During COVID-19 Pandemic.

Indian J Pediatr 2021 Sep 23;88(9):899-904. Epub 2021 Mar 23.

Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India.

Objective: To study the impact of coronavirus disease 2019 (COVID-19) pandemic on the utilization of pediatric cardiac care services and to determine the role of teleconsultation services in delivering healthcare in this subset of population.

Methods: It was a retrospective, observational study. All children who attended pediatric cardiology outpatient/teleconsultation services or were admitted to pediatric cardiology ward between April 1, 2019 to July 31, 2019 and April 1, 2020 to July 31, 2020, were recruited in the study. Data for patients who underwent surgery or catheter intervention for congenital heart disease were also recorded and analyzed. Comparisons were drawn between the statistics during the two time-periods.

Results: Physical outpatient services were discontinued and were replaced by teleconsultations from April 2020. Inpatient admissions during COVID-19 pandemic (n = 66) decreased by two-thirds as compared to the admissions during similar period in 2019 (n = 189). Similarly, the percentage decrease during these 4 mo of pandemic were 84% for catheter interventions, 90% for total congenital heart disease (CHD) surgeries, and 40% for emergency CHD surgeries. The number of patients availing successful teleconsultation was 1079, which was only 15% of the total number of patients attending physical outpatient services (n = 7176) during the corresponding period in the year 2019. During the pandemic, systematic teleconsultation and local evaluation and investigations aided in better management of patients with CHD.

Conclusions: The utilization of cardiovascular services for CHD has reduced significantly during COVID-19 pandemic, for both out- and inpatient care. Teleconsultation services have streamlined the follow-up care to some extent and have helped in noncontact triaging of these patients for further care.
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http://dx.doi.org/10.1007/s12098-021-03702-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7985229PMC
September 2021

An evidence-based scoring system to diagnose acute rheumatic fever with carditis in children.

Int J Cardiol 2021 06 2;333:146-151. Epub 2021 Mar 2.

Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India.

Backgound: Acute rheumatic fever (ARF) with carditis and chronic rheumatic heart disease (RHD) may be difficult to differentiate and may lead to missed diagnosis of carditis. We aimed to determine differences between these groups and to develop a new scoring system for this purpose.

Methods: Children (N = 514; mean age 11.6 ± 2.8, range 5-18 years; 65% males) enrolled in a RHD registry at a tertiary care centre were studied. Clinical, laboratory and echocardiographic features of acute rheumatic carditis group (N = 126) were compared with chronic RHD group (N = 388). We performed multiple regression analysis and then developed a new scoring system using independent predictors. Accuracy of this scoring system was assessed using receiver operating characteristic (ROC) curve analysis.

Results: Patients with ARF and carditis were younger, more commonly had history of fever, arthritis/arthralgias and had worse NYHA class. On echocardiography, severity of mitral regurgitation, presence of mitral leaflet nodules, mitral valve prolapse and pericardial effusion were more common in ARF group. On multiple regression analysis, following features were found to be independently predictive of ARF with carditis: age -negative association, NYHA class, severity of mitral regurgitation, mitral leaflet nodules, erythrocyte sedimentation rate and antistreptolysin titer. Based on these 6 variables, a new scoring system (0-13) was developed. A score of 5 or more was found to be best threshold for diagnosis of carditis (ROC AUC 0.87, sensitivity 76%, specificity 79%).

Conclusion: A new scoring system based on independent statistical associations appears promising for differentiating ARF with carditis from chronic RHD.
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http://dx.doi.org/10.1016/j.ijcard.2021.02.084DOI Listing
June 2021

Reversible complete heart block due to hypercalcaemia.

BMJ Case Rep 2021 Jan 18;14(1). Epub 2021 Jan 18.

Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India.

A 65-year-old woman presented to the emergency room with a syncope. An ECG done revealed complete heart block with a narrow QRS escape rhythm and a normal QT interval. Further investigation revealed severe hypercalcaemia and elevated parathormone levels. Her heart block disappeared on correction of the hypercalcaemia. A right inferior parathyroid adenoma was found and surgically removed. Thus, hypercalcaemia may lead to reversible complete heart block without QT interval shortening.
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http://dx.doi.org/10.1136/bcr-2020-238537DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7813424PMC
January 2021

Effect of acute lower respiratory tract infection on pulmonary artery pressure in children with post-tricuspid left-to-right shunt.

Cardiol Young 2021 May 12;31(5):812-816. Epub 2021 Jan 12.

Department of Cardiology, Professor and Head of Cardiology, All India Institute of Medical Sciences, New Delhi 110029, India.

We sought to examine the influence of clinically severe lower respiratory tract infection on pulmonary artery pressure in children having CHD with post-tricuspid left-to-right shunt, as it may have physiological and clinical implications. In a prospective single-centre observational study, 45 children with post-tricuspid left-to-right shunt and clinically severe lower respiratory tract infection were evaluated during the illness and 2 weeks after its resolution. Pulmonary artery systolic pressure was estimated non-invasively using shunt gradient by echocardiography and systolic blood pressure measured non-invasively.Median pulmonary artery systolic pressure during lower respiratory tract infection was only mildly (although statistically significantly) elevated during lower respiratory tract infection [60 (42-74) versus 53 (40-73) mmHg, (p < 0.0001)]. However, clinically significant change in pulmonary artery systolic pressure defined as the increase of >10 mmHg was present in only 9 (20%) patients. In the absence of hypoxia or acidosis, only a small minority (9%, n = 4) showed significant pulmonary artery systolic pressure rise >10 mmHg. In the absence of hypoxia or acidosis, severe lower respiratory tract infection in patients with acyanotic CHD results in only mild elevation of pulmonary artery systolic pressure in most of the patients.
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http://dx.doi.org/10.1017/S1047951120004734DOI Listing
May 2021

Hemangioma - A pointer to Abernethy syndrome?

Authors:
Shyam S Kothari

Ann Pediatr Cardiol 2020 Jul-Sep;13(3):269-271. Epub 2020 Jul 2.

Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India.

Diffuse pulmonary arteriovenous malformations or pulmonary arterial hypertension (PAH) may result from congenital portosystemic venous shunts. Hemangioma as a physical sign of congenital portosystemic shunts (like Abernethy syndrome) has not been described. I report two children (one with severe cyanosis from pulmonary arteriovenous malformations and the other with severe PAH) with cutaneous hemangioma and Abernethy syndrome. Hemangioma may be a clinical pointer to portosystemic shunts even in the absence of obvious liver disease.
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http://dx.doi.org/10.4103/apc.APC_91_20DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7437616PMC
July 2020

Systolic excursion of the leaflets of the truncal valve: An unusual mechanism for pulmonary stenosis in common arterial trunk.

Ann Pediatr Cardiol 2020 Jul-Sep;13(3):194-198. Epub 2020 Jun 29.

Institute of Genetic Medicine, University of Newcastle, Newcastle upon Tyne, UK.

Background: Pulmonary stenosis in patients with common arterial trunk protects the pulmonary vasculature. In our recently published prospective study of common arterial trunk, some patients with sinusal origin of the pulmonary arterial segment had pulmonary stenosis induced by systolic excursion of a truncal valvar leaflet. We aimed to determine the detailed morphologic characteristics of this unusual finding.

Methods And Results: All 70 patients underwent echocardiography and computed tomographic angiography as per predefined study protocol. In selected cases, we also performed cardiac catheterization. Among 27 patients with aortic dominance, we found sinusal origin of the pulmonary arterial segment. In 5 of these patients, pulmonary stenosis was induced by systolic excursion of a truncal valvar leaflet. In all these patients, the truncal valve was trisinusate, albeit with asymmetric sinuses. The pulmonary arterial segment arose from the largest left sinus with its relatively large leaflet obstructing the pulmonary orifice during systole. In the remaining 22 patients, without pulmonary stenosis but with sinusal origin of pulmonary arterial segment, the truncal valve was quadrisinusate in 7, bisinusate in 2, and trisinusate in 13. None of the patients with quadrisinusate and bisinusate truncal valves had pulmonary stenosis. Among the 13 patients with trisinusate valves, the sinuses of the truncal valve were symmetrical in 6, while in 7, the pulmonary orifice originated from a smaller asymmetric sinus.

Conclusion: Pulmonary stenosis, produced by a relatively large leaflet of an asymmetric truncal sinus, may prevent early development of pulmonary vascular disease. Timely recognition of this unusual mechanism of pulmonary stenosis is important for optimal management.
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http://dx.doi.org/10.4103/apc.APC_22_20DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7437627PMC
June 2020

Separate arch origin of the left external carotid artery with common trunk giving rise to the left internal carotid artery and left subclavian artery.

J Card Surg 2020 Nov 13;35(11):3136-3137. Epub 2020 Aug 13.

Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India.

We present a case of a 3-year-old boy with tetralogy of Fallot having the direct origin of the left external carotid artery from the aorta with a common trunk giving rise to the left internal carotid artery and left subclavian artery, in a right-sided aortic arch. We also highlight the potential implications in management.
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http://dx.doi.org/10.1111/jocs.14931DOI Listing
November 2020

Bail-out wire stenting of restrictive atrial septal defect in cardiac total anomalous pulmonary venous connection.

Ann Pediatr Cardiol 2020 Apr-Jun;13(2):180-181. Epub 2020 Apr 1.

Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India. E-mail:

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http://dx.doi.org/10.4103/apc.APC_197_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7331830PMC
April 2020

Characteristics of Children with Acute Rheumatic Carditis from a High-Incidence Region: Importance of Unexplained Worsening of Functional Class.

Cardiology 2020 11;145(8):522-528. Epub 2020 Jun 11.

Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India.

Background: Acute rheumatic fever (ARF) and acute rheumatic carditis (ARC) continue to be a major public health problem in developing countries.

Objective: To study the characteristics of children with ARC being treated at a tertiary centre.

Methods And Results: We studied 126 children (mean age 10.4 ± 2.3 years, range 5-15 years, 60% males) diagnosed with ARC by treating cardiologists. Most had lower socio-economic status. Fifty of 126 (40%) presented with a first episode of ARC. Joint symptoms were present in 29% and fever in 25%. Only 2.4% had subcutaneous nodules and none had erythema marginatum or chorea. Fifty-one percent presented in NYHA class II and 29% in NYHA class III or IV. Tachycardia and heart failure were present in 53% and 21%, respectively. Recent worsening of NYHA class (dyspnoea) was the commonest feature (48%). Laboratory investigations showed raised antistreptolysin O titres (>333 units) in only 36.7% of patients. Raised C-reactive protein (CRP) was present in 70%, while raised erythrocyte sedimentation rate was found in only 37% of patients. On the basis of above findings, the modified Jones criteria (2015) for the diagnosis of ARF were satisfied only in 46% of children. Echocardiography showed mitral valve thickening in 77% and small nodules on the tip of the leaflets in 43% (27 and 8%, respectively for aortic valve). Left ventricular ejection fraction was <50% in only 3 patients. The dominant valve lesion was mitral regurgitation (MR) (present in 95% of patients; severe in 78%, moderate in 15%), while aortic regurgitation was present in 44% (severe in 14%).

Conclusions: The criteria are often not satisfied by patients being treated for ARC. Recent unexplained worsening of dyspnoea, young age, significant MR, echocardiographic nodules, and elevated CRP are important indicators.
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http://dx.doi.org/10.1159/000508035DOI Listing
April 2021

Herculean mistake: mephentermine associated cardiomyopathy.

Phys Sportsmed 2021 02 13;49(1):116-122. Epub 2020 May 13.

Department of Cardiology, All India Institute of Medical Sciences (AIIMS) , New Delhi, India.

: A 32-year-old professional bodybuilder presented with acute decompensated heart failure. He gave a history of anabolic androgenic steroids (AAS) use for >2 years and mephentermine use for the preceding 3 months. : Transthoracic echocardiography showed severe left ventricular (LV) dysfunction with a large pedunculated, mobile thrombus attached to the ventricular apex. The patient had an embolic stroke during the hospital stay, with complete neurological recovery. Following the cessation of mephentermine use, there was a steady improvement in LV function over a follow-up of 2 months. However, at 3 months, his ventricular function showed deterioration, which coincided with mephentermine reuse. : Though AAS abuse by athletes leading to such a presentation has been documented, to the best of our knowledge, a similar role of mephentermine has not been reported.
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http://dx.doi.org/10.1080/00913847.2020.1763146DOI Listing
February 2021

"Treat and repair" strategy for shunt lesions: a critical review.

Pulm Circ 2020 Apr-Jun;10(2):2045894020917885. Epub 2020 Apr 9.

Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India.

The issue of operability in patients with shunt lesions and raised pulmonary vascular resistance is contentious. Several reports suggest that patients traditionally considered inoperable may be operated after treatment with targeted drug therapy for pulmonary arterial hypertension. We reviewed all the published literature of "treat and repair" approach to gain more insights into the utility of this approach. A critical appraisal of the published literature suggests that this approach is less established for patients with post tricuspid shunts, and for patients with pre-tricuspid shunts with modestly elevated indexed pulmonary vascular resistance (possibly greater than 11 WU.m). Targeted drug therapy may be able to extend the therapeutic window in carefully selected patients, but its use as a routine in this setting seems unwarranted.
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http://dx.doi.org/10.1177/2045894020917885DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7153197PMC
April 2020

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. Abridged Secondary Publication.

Indian Pediatr 2020 02;57(2):143-157

4Fortis Escorts Heart Institute, New Delhi, India.

Justification: A number of guidelines are available for management of congenital heart diseases 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 congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition.

Process: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on 10th and 11th of August 2018 at the All India Institute of Medical Sciences, New Delhi. The meeting was supported by Children's HeartLink, a non-governmental organization based in Minnesota, USA.

Objectives: To frame evidence based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases.

Recommendations: Evidence based recommendations are provided for indications and timing of intervention in common congenital heart diseases, 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 congenital heart diseases (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein anomaly and others). In addition, protocols for follow-up of post surgical patients are also described, disease wise.
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February 2020

Circumflex retroesophageal left aortic arch with right ductus arteriosus: A rare complete vascular ring diagnosed on computed tomography angiography.

Ann Pediatr Cardiol 2020 Jan-Mar;13(1):98-99. Epub 2019 Nov 7.

Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India.

A circumflex retroesophageal left aortic arch with a right-sided ductus is an extremely rare cause of a complete vascular ring, which may result in severe tracheobronchial compression, leading to respiratory compromise, especially in children. We present a case of a 6-month-old female child with stridor and feeding difficulties since birth with interspersed self-resolving episodes of cyanosis and apnea, secondary to the presence of the above-mentioned vascular ring.
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http://dx.doi.org/10.4103/apc.APC_128_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6979015PMC
November 2019

Myocardial perfusion abnormalities in Eisenmenger syndrome.

Nucl Med Commun 2020 Mar;41(3):206-211

Departments of Cardiology.

Background: Compared to primary pulmonary hypertension (PPH), the right ventricular (RV) contractile function is preserved for a long time in patients with Eisenmenger syndrome and is likely the most important determinant of relatively higher survival. The differences in myocardial perfusion have been purported to explain this discrepancy. The exact prevalence of myocardial perfusion abnormalities in Eisenmenger syndrome is not known. We sought to examine the prevalence of myocardial perfusion abnormalities in patients with Eisenmenger syndrome.

Methods: In this prospective study, 20 consecutive adult patients with Eisenmenger syndrome were subjected to clinical assessment, six-minute walk test and echocardiography. Myocardial perfusion was assessed using one day stress-rest Gated Technetium-99 m Sestamibi single-photon emission computed tomography.

Results: Nineteen (95%) patients were in New York Heart Association functional class I or II. All patients had RV hypertrophy. Five (25%) patients had RV systolic dysfunction. Left ventricular systolic function was normal in all except in three patients. Two (10%) patients had perfusion defects in the RV and 4 (20%) patients had perfusion defects in the left ventricle (LV).

Conclusion: Myocardial perfusion defects, both in RV and LV, occur even in asymptomatic or mildly symptomatic patients with Eisenmenger syndrome.
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http://dx.doi.org/10.1097/MNM.0000000000001147DOI Listing
March 2020

Prevalence of hypertension among Indian adults: Results from the great India blood pressure survey.

Indian Heart J 2019 Jul - Aug;71(4):309-313. Epub 2019 Sep 18.

Objective: Hypertension is the most important risk factor for cardiovascular morbidity and mortality. There is limited data on hypertension prevalence in India. This study was conducted to estimate the prevalence of hypertension among Indian adults.

Methods: A national level survey was conducted with fixed one-day blood pressure measurement camps across 24 states and union territories of India. Hypertension was defined as systolic blood pressure (BP) ≥140 mmHg or a diastolic BP ≥90 mmHg or on treatment for hypertension. The prevalence was age- and gender-standardized according to the 2011 census population of India.

Results: Blood pressure was recorded for 180,335 participants (33.2% women; mean age 40.6 ± 14.9 years). Among them, 8,898 (4.9%), 99,791 (55.3%), 35,694 (11.9%), 23,084 (12.8%), 9,989 (5.5%), and 2,878 (1.6%) participants were of the age group 18-19, 20-44, 45-54, 55-64, 65-74, and ≥ 75 years, respectively. Overall prevalence of hypertension was 30.7% (95% confidence interval [CI]: 30.5, 30.9) and the prevalence among women was 23.7% (95% CI: 23.3, 24). Prevalence adjusted for 2011 census population and the WHO reference population was 29.7% and 32.8%, respectively.

Conclusion: There is a high prevalence of hypertension, with almost one in every three Indian adult affected.
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http://dx.doi.org/10.1016/j.ihj.2019.09.012DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6890959PMC
May 2020

Clarifying the anatomy of common arterial trunk: a clinical study of 70 patients.

Eur Heart J Cardiovasc Imaging 2020 08;21(8):914-922

Institute of Genetic Medicine, University of Newcastle, Newcastle upon Tyne NE1 7RU, UK.

Aims: Anatomic variations in hearts with common arterial trunk are well-known, although there is no large study of living patients. Detailed knowledge of the origins of the pulmonary and coronary arteries is vital for surgical management. We sought to clarify the variations using computed tomography.

Methods And Results: We prospectively studied 70 consecutive patients using echocardiography and computed tomography. In 63 (90%) patients, there was aortic dominance, while 7 (10%) had dominance of the pulmonary component. In 27 (43%) patients with aortic dominance, part of the pulmonary segment arose from a truncal valvar sinus. A long confluent pulmonary channel was more common in patients with sinusal origin compared to those with non-sinusal origin of the pulmonary segment (19 vs. 0; P = 0.0005). Close proximity between the orifices of the coronary arteries and the pulmonary component was also more frequent with sinusal origin (21 vs. 6; P < 0.001) with 5 (19%) patients having pulmonary flow obstructed by a truncal valvar leaflet.

Conclusion: Sinusal origin of the pulmonary component is common with aortic dominance, frequently in association with a long confluent pulmonary segment, which may be in close proximity to the origin of a coronary artery. One-fifth of patients with sinusal origin of pulmonary component have a truncal valvar leaflet obstructing the pulmonary orifice. These morpho-anatomic findings have important implications for management.
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http://dx.doi.org/10.1093/ehjci/jez255DOI Listing
August 2020

Guidelines for the management of common congenital heart diseases in India: A consensus statement on indications and timing of intervention.

Indian Heart J 2019 May - Jun;71(3):207-223. Epub 2019 Aug 12.

Fortis Escorts Heart Institute, New Delhi, India.

Introduction: A number of guidelines are available for management of congenital heart diseases 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 congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition.

Process: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on the 10th and 11th of August, 2018 at the All India Institute of Medical Sciences.

Objectives: The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases and (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases.

Recommendations: Evidence-based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts, obstructive lesions, and cyanotic congenital heart diseases. In addition, protocols for follow-up of postsurgical patients are also described.
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http://dx.doi.org/10.1016/j.ihj.2019.07.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6796629PMC
February 2020

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.

Ann Pediatr Cardiol 2019 Sep-Dec;12(3):254-286

Fortis Escorts Heart Institute, New Delhi, India.

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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http://dx.doi.org/10.4103/apc.APC_32_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6716301PMC
September 2019

Operable ventricular septal defect despite severe pulmonary hypertension and cyanosis!

Cardiol Young 2019 Jul 23;29(7):986-988. Epub 2019 Jul 23.

Departments of Cardiology, and Cardiothoracic surgery, All India Institute of Medical Sciences, New Delhi, India.

Patients with a significant left-to-right shunt at ventricular level may become inoperable at an early age due to irreversible pulmonary vascular disease. On the other hand, even suprasystemic pulmonary hypertension due to mitral stenosis remains treatable. We report a 24-year-old patient with large ventricular septal defect, severe mitral stenosis and cyanosis who improved after surgical correction of both the lesions. This emphasises the importance of additional post-capillary pulmonary hypertension in Eisenmenger syndrome.
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http://dx.doi.org/10.1017/S1047951119001057DOI Listing
July 2019

Ivabradine for congenital junctional ectopic tachycardia in siblings.

Ann Pediatr Cardiol 2018 May-Aug;11(2):226-228

Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India E-mail:

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http://dx.doi.org/10.4103/apc.APC_25_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5963246PMC
June 2018
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