219 results on '"Bibhuti B. Das"'
Search Results
2. A Review of Contemporary and Future Pharmacotherapy for Chronic Heart Failure in Children
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Bibhuti B. Das
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chronic heart failure ,heart failure in children ,HF with reduced ejection fraction (HFrEF) ,HF with preserved ejection fraction (HFpEF) ,pharmacotherapy for HFrEF and HFpEF in children ,Pediatrics ,RJ1-570 - Abstract
This review delves into the most recent therapeutic approaches for pediatric chronic heart failure (HF) as proposed by the International Society for Heart and Lung Transplantation (ISHLT), which are not yet publicly available. The guideline proposes an exhaustive overview of the evolving pharmacological strategies that are transforming the management of HF in the pediatric population. The ISHLT guidelines recognize the scarcity of randomized clinical trials in children, leading to a predominance of consensus-based recommendations, designated as Level C evidence. This review article aims to shed light on the significant paradigm shifts in the proposed 2024 ISHLT guidelines for pediatric HF and their clinical ramifications for pediatric cardiology practitioners. Noteworthy advancements in the updated proposed guidelines include the endorsement of angiotensin receptor-neprilysin inhibitors (ARNIs), sodium-glucose cotransporter 2 inhibitors (SGLT2is), and soluble guanylate cyclase (sGC) stimulators for treating chronic HF with reduced ejection fraction (HFrEF) in children. These cutting-edge treatments show potential for enhancing outcomes in pediatric HFrEF. Nonetheless, the challenge persists in validating the efficacy of therapies proven in adult HFrEF for the pediatric cohort. Furthermore, the proposed ISHLT guidelines address the pharmacological management of chronic HF with preserved ejection fraction (HFpEF) in children, marking a significant step forward in pediatric HF care. This review also discusses the future HF drugs in the pipeline, their mechanism of actions, potential uses, and side effects.
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- 2024
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3. Multimodality Imaging to Detect Rejection, and Cardiac Allograft Vasculopathy in Pediatric Heart Transplant Recipients—An Illustrative Review
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Bibhuti B. Das, Shriprasad Deshpande, and Tarique Hussain
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multimodality imaging ,heart transplantation ,children ,adolescent ,cardiac allograft rejection ,cardiac allograft vasculopathy ,Surgery ,RD1-811 - Abstract
The three most common modalities of graft surveillance in pediatric heart transplant (HT) recipients include echocardiography, coronary angiography, and endomyocardial biopsy (EMB). The survival outcomes after HT in children have improved considerably in recent years. However, allograft rejection and cardiac allograft vasculopathy remain the leading cause of death or re-transplantation. The routine surveillance by EMB and coronary angiography are invasive and risky. Newer noninvasive echocardiographic techniques, including tissue Doppler imaging (TDI), 2-D speckle tracking echocardiography, CT coronary angiography (CTCA), cardiovascular magnetic resonance (CMR), single-photon emission computed tomography (SPECT), and positron emission tomography (PET) and invasive techniques such as intravascular ultrasound (IVUS), functional flow reserve (CFR) of coronary arteries, optical coherence tomography (OCT), have emerged as powerful tools which may help early recognition of sub-clinical rejection, response to treatment, early detection, and progression of CAV. The multimodality imaging approach, including noninvasive and invasive tests, is the future for the transplanted heart to detect dysfunction, rejections, and early CAV. This review illustrates noninvasive and invasive imaging techniques currently used or could be considered for clinical use in detecting heart transplant rejection, dysfunction, and CAV in children.
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- 2022
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4. A Systematic Approach for the Interpretation of Cardiopulmonary Exercise Testing in Children with Focus on Cardiovascular Diseases
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Bibhuti B. Das
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exercise testing ,exercise physiology ,cardiopulmonary exercise testing ,children ,Diseases of the circulatory (Cardiovascular) system ,RC666-701 - Abstract
Cardiopulmonary exercise testing (CPET) is the clinical standard for children with congenital heart disease (CHD), heart failure (HF) being assessed for transplantation candidacy, and subjects with unexplained dyspnea on exertion. Heart, lung, skeletal muscle, peripheral vasculature, and cellular metabolism impairment frequently lead to circulatory, ventilatory, and gas exchange abnormalities during exercise. An integrated analysis of the multi-system response to exercise can be beneficial for differential diagnosis of exercise intolerance. The CPET combines standard graded cardiovascular stress testing with simultaneous ventilatory respired gas analysis. This review addresses the interpretation and clinical significance of CPET results with specific reference to cardiovascular diseases. The diagnostic values of commonly obtained CPET variables are discussed using an easy-to-use algorithm for physicians and trained nonphysician personnel in clinical practice.
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- 2023
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5. Presentation of SARS-CoV-2 in a Pediatric Heart Transplant Recipient with Multiple Underlying Comorbidities
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Bibhuti B. Das
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SARS-CoV-2 ,COVID-19 ,myocarditis ,pediatric heart transplantation ,Surgery ,RD1-811 - Abstract
A six-year-old heart transplant recipient with additional significant co-morbidities, including severe hypoxic-ischemic injury, gastrostomy, tracheostomy, and mechanical ventilation dependency, encountered SARS-CoV-2 infection. The patient received tacrolimus and mycophenolate to prevent graft rejection, presented initially with SARS-CoV-2 positive and presumed pseudomonas aeruginosa pneumonia. Twenty-three days later, the patient presented with fever recurrence with evidence for systemic inflammation, which resolved rapidly with high-dose methylprednisolone. Interestingly, while IgM to SARS-CoV-2 was present, IgG was not detected even three months after his first positive test for SARS-CoV-2. The author discusses potential immune mechanisms that might have affected the course of multi-system inflammatory syndrome children (MIS-C) in this patient.
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- 2021
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6. Reports of 2 Rare Associations of Hypoplastic Left Heart Syndrome
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K. Anitha Jayakumar, MD, Gouri Tilak, MD, and Bibhuti B. Das, MD
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congenital heart disease ,hypoplastic left heart syndrome ,pulmonary valve dysplasia ,levoatriocardinal veins ,palliative care ,Diseases of the circulatory (Cardiovascular) system ,RC666-701 - Abstract
This report describes 2 contrasting yet rare associations of hypoplastic left heart syndrome, 1 in a patient with pulmonary valve stenosis that was successfully surgically palliated and the other in a patient with an intact atrial septum and stenotic bilateral levoatriocardinal veins who was offered comfort care. These cases underscore the point that although both infants were born with hypoplastic left heart syndrome, the outcomes can dramatically differ as a result of anatomic and physiological variables. (Level of Difficulty: Advanced.)
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- 2019
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7. Senning Procedure for Physiological Atrial Inversion With Left Atrial Isomerism
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Bibhuti B. Das, MD, K. Anitha Jayakumar, MD, Ming-Lon Young, MD, and Kak-Chen Chan, MD
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atrial inversion ,left atrial isomerism ,left ventricular noncompaction ,Senning procedure ,Diseases of the circulatory (Cardiovascular) system ,RC666-701 - Abstract
We present the case of an infant with left atrial isomerism with complex pulmonary and systemic venous connections that resulted in physiological parallel circulation in the setting of ventriculoarterial concordance who was surgically treated using the Senning procedure. This case highlights a rare cause for cyanosis due to poor mixing from a parallel circulation and an issue with nomenclature. (Level of Difficulty: Advanced.)
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- 2019
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8. Successful Pulmonary Endarterectomy after Acute Pulmonary Embolism and Reversal of Acute Cor Pulmonale in an 11-Year-Old Boy with Nephrotic Syndrome
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Onyekachukwue Osakwe and Bibhuti B. Das
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acute pulmonary embolism ,child ,nephrotic syndrome ,pulmonary endarterectomy ,Pediatrics ,RJ1-570 - Abstract
Patients with nephrotic syndrome (NS) are at an increased risk for thromboembolic events, such as deep venous and arterial thrombosis and pulmonary embolism (PE). In general, PE in children differs from adults in incidence, predisposition, pathophysiology, presenting symptoms, and management strategies. There is a lack of treatment guidelines for PE in children, and the management strategies are mostly extrapolated from adult data. This case report highlights the presentation of acute cor pulmonale due to massive PE associated with NS and a successful pulmonary endarterectomy that reversed the child’s pulmonary hypertension and normalized right ventricular function.
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- 2022
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9. Compound Heterozygous Missense Variants in RPL3L Genes Associated with Severe Forms of Dilated Cardiomyopathy: A Case Report and Literature Review
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Bibhuti B. Das, Viswanath Gajula, Sandeep Arya, and Mary B. Taylor
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familial dilated cardiomyopathy ,RPL3L ,pediatric heart failure ,Pediatrics ,RJ1-570 - Abstract
Whole exome sequencing has identified an infant girl with fulminant dilated cardiomyopathy (DCM), leading to severe acute heart failure associated with ribosomal protein large 3-like (RPL3L) gene pathologic variants. Other genetic tests for mitochondrial disorders by sequence analysis and deletion testing of the mitochondrial genome were negative. Secondary causes for DCM due to metabolic and infectious etiologies were ruled out. She required a Berlin-Excor left ventricular assist device due to worsening of her heart failure as a bridge to orthotopic heart transplantation. At three months follow-up after heart transplantation, she has been doing well. We reviewed the literature on published RPL3L-related DCM cases and their outcomes. Bi-allelic variants in RPL3L have been reported in only seven patients from four unrelated families in the literature. RPL3L is a newer and likely pathogenic gene associated with a severe form of early-onset dilated cardiomyopathy with poor prognosis necessitating heart transplantation.
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- 2022
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10. Parametric Mapping Cardiac Magnetic Resonance Imaging for the Diagnosis of Myocarditis in Children in the Era of COVID-19 and MIS-C
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Bibhuti B. Das, Jyothsna Akam-Venkata, Mubeena Abdulkarim, and Tarique Hussain
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myocarditis ,children ,COVID-19 ,multisystem inflammatory syndrome in children ,mRNA COVID-19 vaccine ,cardiac MRI ,Pediatrics ,RJ1-570 - Abstract
Myocarditis comprises many clinical presentations ranging from asymptomatic to sudden cardiac death. The history, physical examination, cardiac biomarkers, inflammatory markers, and electrocardiogram are usually helpful in the initial assessment of suspected acute myocarditis. Echocardiography is the primary tool to detect ventricular wall motion abnormalities, pericardial effusion, valvular regurgitation, and impaired function. The advancement of cardiac magnetic resonance (CMR) imaging has been helpful in clinical practice for diagnosing myocarditis. A recent Scientific Statement by the American Heart Association suggested CMR as a confirmatory test to diagnose acute myocarditis in children. However, standard CMR parametric mapping parameters for diagnosing myocarditis are unavailable in pediatric patients for consistency and reliability in the interpretation. The present review highlights the unmet clinical needs for standard CMR parametric criteria for diagnosing acute and chronic myocarditis in children and differentiating dilated chronic myocarditis phenotype from idiopathic dilated cardiomyopathy. Of particular relevance to today’s practice, we also assess the potential and limitations of CMR to diagnose acute myocarditis in children exposed to severe acute respiratory syndrome coronavirus-2 infections. The latter section will discuss the multi-inflammatory syndrome in children (MIS-C) and mRNA coronavirus disease 19 vaccine-associated myocarditis.
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- 2022
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11. Novel Loss of Function in the AGK Gene
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Bibhuti B. Das, MD, Lazaro E. Hernandez, MD, Parul Jayakar, MD, Kathryn C. Chatfield, MD, PhD, and Maryanne Chrisant, MD
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acylglycerol kinase ,AGK ,mitochondrial cardiomyopathy ,Sengers syndrome ,Diseases of the circulatory (Cardiovascular) system ,RC666-701 - Abstract
The authors present a case of mitochondrial cardiomyopathy due to a novel mutation of AGK gene that led to progressive heart failure. The cardiac magnetic resonance image findings of diffusely elevated relaxation time and increase in extracellular volume in the myocardium without early or late gadolinium enhancement may suggest mitochondrial cardiomyopathy. The authors emphasized the multidisciplinary team approach in the care of patients with mitochondrial cardiomyopathies. (Level of Difficulty: Advanced.)
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- 2019
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12. Further Evidence of Autosomal Recessive Inheritance of RPL3L Pathogenic Variants with Rapidly Progressive Neonatal Dilated Cardiomyopathy
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Hemanth Nannapaneni, Stephanie Ghaleb, Sandeep Arya, Viswanath Gajula, Mary B. Taylor, and Bibhuti B. Das
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neonatal dilated cardiomyopathy ,familial dilated cardiomyopathy ,RPL3L gene ,gene mutation ,neonatal heart failure ,Diseases of the circulatory (Cardiovascular) system ,RC666-701 - Abstract
Neonatal dilated cardiomyopathy (DCM) is rare with high etiologic heterogeneity. Recently, biallelic, autosomal recessive, pathogenic variants in RPL3L (ribosomal protein L3-like) have been reported in the literature with severe early-onset DCM. In the present brief report, we identified two pathogenic RPL3L variants, each harbored in unaffected heterozygous parents: mother (RPL3L c.1076_1080delCCGTG (p.Ala359Glyfs*4)) and father (RPL3L c.80G > A (p.Gly27Asp)). Pathogenic variants were segregated as autosomal recessive to two offspring born with compound heterozygous RPL3L variants and affected by neonatal DCM. This is the second report in the literature to the best of our knowledge and our findings support the pathogenicity of biallelic RPL3L pathologic variants associated with rapidly progressive neonatal DCM and heart failure with a poor prognosis.
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- 2022
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13. Myocarditis and Pericarditis Following mRNA COVID-19 Vaccination: What Do We Know So Far?
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Bibhuti B. Das, William B. Moskowitz, Mary B. Taylor, and April Palmer
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COVID-19 vaccine ,myocarditis ,pericarditis ,Pediatrics ,RJ1-570 - Abstract
This is a cross-sectional study of 29 published cases of acute myopericarditis following COVID-19 mRNA vaccination. The most common presentation was chest pain within 1–5 days after the second dose of mRNA COVID-19 vaccination. All patients had an elevated troponin. Cardiac magnetic resonance imaging revealed late gadolinium enhancement consistent with myocarditis in 69% of cases. All patients recovered clinically rapidly within 1–3 weeks. Most patients were treated with non-steroidal anti-inflammatory drugs for symptomatic relief, and 4 received intravenous immune globulin and corticosteroids. We speculate a possible causal relationship between vaccine administration and myocarditis. The data from our analysis confirms that all myocarditis and pericarditis cases are mild and resolve within a few days to few weeks. The bottom line is that the risk of cardiac complications among children and adults due to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection far exceeds the minimal and rare risks of vaccination-related transient myocardial or pericardial inflammation.
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- 2021
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14. SARS-CoV-2 Myocarditis in a High School Athlete after COVID-19 and Its Implications for Clearance for Sports
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Bibhuti B. Das
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SARS-CoV-2 ,sports clearance after COVID-19 ,SARS-CoV-2 myocarditis ,CMR imaging ,Pediatrics ,RJ1-570 - Abstract
This case report describes a high school athlete with palpitation, myalgia, fatigue, and dyspnea on exertion after SARS-CoV-2 infection with evidence of myocarditis by cardiac magnetic resonance (CMR), but echocardiography and troponin were normal. This case is unusual as the standard cardiac tests recommended by the American Heart Association for sports clearance, including ECG, echocardiography, and cardiac biomarkers, were normal. Still, she continued to be symptomatic after mild COVID-19. The CMR was performed to evaluate her unexplained palpitation and showed patchy myocardial edema two months after her initial SARS-CoV-2 infection. In this case, the diagnosis of myocardial involvement would be missed by normal echocardiograms and cardiac bio-markers without CMR. Because acute myocarditis is a risk factor for sudden death in competitive athletes, pediatric cardiologists should consider performing additional tests such as cardiac MRI in symptomatic COVID-19 patients, even if cardiac biomarkers and echocardiograms are normal.
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- 2021
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15. Current and Future Drug and Device Therapies for Pediatric Heart Failure Patients: Potential Lessons from Adult Trials
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Bibhuti B. Das, William B. Moskowitz, and Javed Butler
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acute heart failure syndrome ,pediatric heart failure ,pharmacotherapy for heart failure ,device therapy for chronic heart failure ,Pediatrics ,RJ1-570 - Abstract
This review discusses the potential drug and device therapies for pediatric heart failure (HF) due to reduced systolic function. It is important to realize that most drugs that are used in pediatric HF are extrapolated from adult cardiology practices or consensus guidelines based on expert opinion rather than on evidence from controlled clinical trials. It is difficult to conclude whether the drugs that are well established in adult HF trials are also beneficial for children because of tremendous heterogeneity in the mechanism of HF in children and variations in the pharmacokinetics and pharmacodynamics of drugs from birth to adolescence. The lessons learned from adult trials can guide pediatric cardiologists to design clinical trials of the newer drugs that are in the pipeline to study their efficacy and safety in children with HF. This paper’s focus is that the reader should specifically think through the pathophysiological mechanism of HF and the mode of action of drugs for the selection of appropriate pharmacotherapy. We review the drug and device trials in adults with HF to highlight the knowledge gap that exists in the pediatric HF population.
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- 2021
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16. Interventional Cardiovascular Magnetic Resonance Imaging (iCMR) in an Adolescent with Pulmonary Hypertension
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Surendranath R. Veeram Reddy, Yousef Arar, Tarique Hussain, Gerald Greil, Luis Zabala, and Bibhuti B. Das
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interventional cardiac MRI ,pediatric pulmonary hypertension ,Medicine (General) ,R5-920 - Abstract
The interventional cardiac magnetic resonance imaging (iCMR) catheterization procedure is feasible and safe for children and adults with pulmonary hypertension and congenital heart defects (CHD). With iCMR, the calculation of pulmonary vascular resistance (PVR) in children with complex CHD with multilevel shunt lesions is accurate. In this paper, we describe the role of the MRI-guided right-sided cardiac catheterization procedure to accurately estimate PVR in the setting of multiple shunt lesions (ventricular septal defect and patent ductus arteriosus) and to address the clinical question of operability in an adolescent with trisomy 21 and severe pulmonary hypertension.
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- 2020
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17. Segmental Pulmonary Hypertension in Children with Congenital Heart Disease
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Bibhuti B. Das, Benjamin Frank, and Dunbar Ivy
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pulmonary hypertension in children ,congenital heart disease ,segmental pulmonary hypertension ,digital subtraction angiography ,Medicine (General) ,R5-920 - Abstract
Segmental pulmonary hypertension is a complex condition in children that encompasses many congenital heart diseases including pulmonary atresia with ventricular septal defect, hemitruncus/truncus arteriosus with branch pulmonary artery stenosis, unilateral absent pulmonary artery, and several post-tricuspid shunt lesions. Multimodality imaging is required to confirm and assess pulmonary vascular disease in subjects with major aorto-pulmonary collaterals. We describe 3 children with complex congenital heart defects who have a variable degree of segmental pulmonary hypertension and discuss management strategies and the role of interventional and/or pulmonary hypertension targeted therapies.
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- 2020
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18. Patent Foramen Ovale in Fetal Life, Infancy and Childhood
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Bibhuti B. Das
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foramen ovale ,patent foramen ovale (PFO) ,Medicine - Abstract
A patent foramen ovale (PFO) is a common, incidental echocardiographic finding in otherwise healthy and asymptomatic infants and children. However, a variety of clinical conditions have been ascribed to the presence of a PFO in childhood, such as cryptogenic stroke, platypnea-orthodeoxia syndrome, decompression sickness and migraine, although the data on these are controversial and sometimes contradictory. This review discusses embryology and correlation with post-natal anatomy, anatomical variations of the atrial septum, diagnostic modalities in special circumstances of PFO associated clinical syndromes, and the role of PFO in congenital heart disease, pulmonary hypertension, dilated cardiomyopathy and heart failure in children who require an extracorporeal membrane oxygenator or ventricular assist device as life support.
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- 2020
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19. Pulmonary Vascular Underperfusion Score in Premature Infants with Bronchopulmonary Dysplasia and Pulmonary Hypertension
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Bibhuti B. Das, Michelle-Marie Jadotte, and Kak-Chen Chan
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premature infant ,bronchopulmonary dysplasia ,pulmonary hypertension ,digital subtraction pulmonary angiography ,Medicine (General) ,R5-920 - Abstract
Pulmonary hypertension (PH) is a complication of bronchopulmonary dysplasia (BPD). The underlying pathophysiology of BPD-associated PH is complex and poorly understood. Echocardiogram may underestimate the severity of pulmonary hypertensive vascular disease in severe BPD. Digital subtraction pulmonary angiography (DSPA) is a potentially useful imaging modality for evaluating changes in the pulmonary vasculature of BPD-associated PH. In this study, we objectively quantified the pulmonary hypertensive vascular changes demonstrated by DSPA using a novel pulmonary vascular underperfusion score (PVUS) and correlated the scoring system with echocardiography parameters and cardiac hemodynamics by right heart catheterization.
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- 2019
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20. Current State of Pediatric Heart Failure
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Bibhuti B. Das
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pediatric heart failure ,advanced heart failure ,ventricular assist device ,mechanical circulatory support ,Pediatrics ,RJ1-570 - Abstract
Pediatric heart failure (HF) represents an important cause of morbidity and mortality in childhood. There is an overlapping relationship of HF, congenital heart disease, and cardiomyopathy. The goal of treatment of HF in children is to maintain stability, prevent progression, and provide a reasonable milieu to allow somatic growth and optimal development. Current management and therapy for HF in children are extrapolated from treatment approaches in adults. There are significant barriers in applying adult data to children because of developmental factors, age variation from birth to adolescence, and differences in the genetic expression profile and β-adrenergic signaling. At the same time, there are significant challenges in performing well-designed drug trials in children with HF because of heterogeneity of diagnoses identifying a clinically relevant outcome with a high event rate, and a difficulty in achieving sufficient enrollment. A judicious balance between extrapolation from adult HF guidelines and the development of child-specific data on treatment represent a wise approach to optimize pediatric HF management. This approach is helpful as reflected by the increasing role of ventricular assist devices in the management of advanced HF in children. This review discusses the causes, epidemiology, pathophysiology, clinical manifestations, conventional medical treatment, clinical trials, and the role of device therapy in pediatric HF.
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- 2018
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21. Therapeutic Approaches in Heart Failure with Preserved Ejection Fraction (HFpEF) in Children: Present and Future
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Bibhuti B. Das
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Pediatrics, Perinatology and Child Health ,Pharmacology (medical) - Published
- 2022
22. Trends in Contemporary Use of Ventricular Assist Devices in Children Awaiting Heart Transplantation and Their Outcomes by Race/Ethnicity
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Jaimin, Trivedi, Mark S, Slaughter, William B, Moskowitz, Stephanie, Ghaleb, and Bibhuti B, Das
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Biomaterials ,Biomedical Engineering ,Biophysics ,Bioengineering ,General Medicine - Abstract
This retrospective study included children aged ≤18 years who had durable ventricular assist devices (VADs) as a bridge to transplantation from the United Network Organ Sharing (UNOS) database between 2011 and 2020. We evaluated 90-day waitlist mortality and 1-year posttransplant mortality after VAD implantation in children stratified by race/ethnicity: Black, White, and Others. The VAD was used in a higher proportion of Black children listed for heart transplantation (HT) (26%) versus Other (25%) versus White (22%); p0.01. Black children had Medicaid health insurance coverage (67%) predominantly at the time of listing for HT. There was no significant overall difference in waitlist survival among the three groups supported with VAD at the time of listing (log-rank p = 0.4). On the other hand, the 90-day waitlist mortality after the VAD implantation at listing and while listed was the lowest among Black (6%) compared with White (13%) and Other (14%) (p0.01). The multivariate regression analysis showed that Other race (hazard ratio [HR], 2.29; p0.01), Black race (HR, 2.13; p0.01), use of mechanical ventilation (HR, 1.72; p = 0.01), and Medicaid insurance (HR, 1.54; p = 0.04) were independently associated with increased 1-year posttransplant mortality. In conclusion, Black children had more access to durable VAD support than White children. The 90-day waitlist mortality was significantly lower in Black children compared with White and Other after VAD implantation. However, Black and Other racial/ethnic children with VAD at transplant had higher 1-year posttransplant mortality than White children. Future studies to elucidate the reasons for these disparities are needed.
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- 2022
23. Anesthetic Considerations in Pediatric Patients With Acute Decompensated Heart Failure
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Roby Sebastian, Bibhuti B Das, Luis M. Zabala, Pablo Motta, and Sana Ullah
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Heart Defects, Congenital ,Heart Failure ,medicine.medical_specialty ,Acute decompensated heart failure ,Heart disease ,business.industry ,Cardiomyopathy ,medicine.disease ,Pediatrics ,Anesthesiology and Pain Medicine ,Heart failure ,Internal medicine ,Acute Disease ,Anesthetic ,medicine ,Etiology ,Cardiology ,Humans ,Anesthesia ,Child ,Cardiology and Cardiovascular Medicine ,business ,Anesthetics ,medicine.drug - Abstract
Acute decompensated heart failure (ADHF) in pediatrics is a significant cause for morbidity and mortality in children. Congenital heart disease and cardiomyopathy are the leading etiologies of ADHF. It is common for these children to undergo diagnostic, therapeutic, or surgical procedure under anesthesia, which may be associated with significant morbidity and mortality. The importance of preanesthetic multidisciplinary planning with all involved teams, including anesthesia, cardiology, intensive care, perfusion, and cardiac surgery, cannot be emphasized enough. In order to safely manage these patients, it is imperative for the anesthesiologist to understand the complex pathophysiological interactions between cardiopulmonary systems and anesthesia during these procedures. This review discusses the etiology, pathophysiology, clinical manifestations, and perioperative management of these patients.
- Published
- 2021
24. SARS-CoV-2 infection induced thyroid storm and heart failure in an adolescent girl
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Michael D Weiland, Jyothsna Akam-Venkata, William B. Moskowitz, Obiageli Obi, Divya Shakti, and Bibhuti B Das
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Cardiomyopathy, Dilated ,Pediatrics ,medicine.medical_specialty ,Acute decompensated heart failure ,Adolescent ,Graves' disease ,Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ,media_common.quotation_subject ,thyroid storm ,General Cardiology ,medicine ,Thyroid storm ,Humans ,Girl ,Child ,media_common ,Heart Failure ,business.industry ,SARS-CoV-2 ,Thyroid disease ,Brief Report ,fungi ,COVID-19 ,Dilated cardiomyopathy ,General Medicine ,medicine.disease ,graves disease ,Systemic Inflammatory Response Syndrome ,SARS-CoV-2 infection in children ,dilated cardiomyopathy ,Thyrotoxicosis ,Heart failure ,Pediatrics, Perinatology and Child Health ,Female ,Thyroid Crisis ,Cardiology and Cardiovascular Medicine ,business - Abstract
We report a case of thyroid storm precipitated by SARS-CoV-2 infection in an adolescent girl with a history of Graves disease and dilated cardiomyopathy. This case highlights that SARS-CoV-2 infection can potentially trigger a thyrotoxicosis crisis and acute decompensated heart failure in a patient with underlying thyroid disease and myocardial dysfunction even in the absence of multi-system inflammatory syndrome in children. We systematically reviewed the thyrotoxicosis cases with SARS-CoV-2 infection and described its impact on pre-existing dilated cardiomyopathy.
- Published
- 2021
25. Impact of induction therapy on cytomegalovirus infection and post‐transplant outcomes in pediatric heart transplant recipients receiving routine antiviral prophylaxis
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Suhua Zang, Xin Zhang, Jianli Niu, and Bibhuti B. Das
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Transplantation - Abstract
Induction therapy has been increasingly used in pediatric heart transplantation. This study evaluated the impact of anti-thymocyte globulin (ATG) versus basiliximab as induction therapy on post-transplant cytomegalovirus (CMV) infection, rejection at 1 year, coronary allograft vasculopathy (CAV), and mortality in pediatric heart transplant recipients receiving antiviral prophylaxis.Of the 96 patients (age 18 years) analyzed, 46 (47.9%) patients received basiliximab, and 50 (52.1%) received ATG. Median follow-up was 3.0 (IQR, 1.7-4.9) years with 32.3% reporting CMV infection. The ATG group, as compared with the basiliximab group, had similar incidences of CMV infection (36% vs. 28.3%, p = .418), CMV viremia (22% vs. 19.6%, p = .769), and CMV-positive tissue biopsy (30% vs. 22%, p = .486). The ATG group had lower incidences of rejection at 1 year (16% vs. 36.9%, p = .022) and CAV (4% vs. 23.9%, p = .006) with no difference in mortality (8% vs. 15.2%, p = .343), compared with the basiliximab group. Multivariate analysis showed that induction with ATG was associated with a lower risk of rejection at 1 year (OR, .31; 95% CI, .09-.94; p = .039) with no impact on the incidences of CMV infection (HR, 2.06; 95% CI, .54-7.89; p = .292), CAV (HR, .30; 95% CI, .04-2.58; p = .275), and mortality (HR, .39; 95% CI, .09-1.82; p = .233) compared to basiliximab induction.In conclusion, induction with ATG was associated with reduction in risk of rejection at 1 year with no effects on CMV infection, CAV, and mortality in pediatric heart transplant recipients with universal antiviral prophylaxis compared with basiliximab induction therapy.
- Published
- 2022
26. Impact of 2016 UNOS pediatric heart allocation policy changes on VAD utilization, waitlist, and post-transplant survival outcomes in children with CHD versus Non-CHD
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Bibhuti B. Das, Chad T. Blackshear, Seth T. Lirette, Mark S. Slaughter, Stephanie Ghaleb, William Moskowitz, Mohammad Ghanamah, and Phillip T. Burch
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Transplantation ,Article - Abstract
AIMS: We analyzed the impact of the revised pediatric heart allocation policy on types of ventricular assist device (VAD) utilization, and waitlist (WL) and post-heart transplant (HT) survival outcomes in congenital heart disease (CHD) versus non-CHD patients before (Era-1) and after (Era-2) pediatric heart allocation policy implementation. METHODS: We retrospectively reviewed the UNOS database from December 16, 2011, through March 31, 2021, for patients < 18 years old and listed for primary HT. We compared the differences observed between Era-1 and Era-2. RESULTS: 5551 patients were listed for HT, of whom 2447(44%) were in Era-1 and 3104(56%) were in Era-2. CHD patients were listed as status 1A unchanged, but the number of patients listed as status 1B decreased in Era-2, whereas the number of non-CHD patients listed as status 1A decreased, but status 1B increased. In Era-2 compared to Era-1, both temporary (1% to 4%, p < .001) and durable VAD (13.6% to 17.8%, p < .001) utilization increased, and the transplantation rate per 100-patient years increased in both groups. The median WL period for CHD patients increased marginally from 70 to 71 days (p = .06), whereas for non-CHD patients it decreased from 61 to 54 days (p < .001). Adjusted 90-day WL survival increased from 84% to 88%, p = .016 in CHD, but there was no significant change in non-CHD patients (p = .57). There was no significant difference in 1-year post-HT survival in CHD and non-CHD patients between Era-1 and Era-2. CONCLUSIONS: In summary, after the revised heart allocation policy implementation, temporary and durable VAD support increased, HT rate increased, waitlist duration marginally increased in the CHD cohort and decreased in the non-CHD cohort, and 90-day WL survival probability improved in children with CHD without significant change in 1-year post-HT outcomes. Future studies are needed to identify changes to the policy that may further improve the listing criteria to improve WL duration and post-HT survival.
- Published
- 2022
27. Racial and Ethnic Disparity in Multisystem Inflammatory Syndrome in Children Associated With SARS-CoV-2 in Mississippi, USA
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Bibhuti B. Das, Divya Shakti, Stephanie Ghaleb, Jyothsna Akam-Venkata, William B. Moskowitz, Onyekachukwu Osakwe, Michael D. Weiland, Sandeep Arya, Viswanath Gajula, and Mary B. Taylor
- Subjects
Male ,Mississippi ,SARS-CoV-2 ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Ethnicity ,Humans ,COVID-19 ,Female ,Child - Abstract
We aimed to study the disparity in the clinical profile and outcomes of hospitalized Multisystem Inflammatory Syndrome in Children (MIS-C) patients at our center. The second goal was to examine the temporal association with preceding SARS-CoV-2 infection by race/ethnicity in our community in Mississippi. We found the racial disparity in the prevalence of MIS-C exceeded its temporal association with SARS-CoV-2 infections. We included 51 consecutive MIS-C patients hospitalized, whose median age was 9 (interquartile range [IQR] 5-12) years, 58% were male, 71% were black, 25% were white, and 4% belonged to other groups. We found a delay between onset of symptoms and hospitalization in black patients compared with white patients with a median of 2 (IQR 0-7) vs median of 0 (0-5) urgent care visits ( P = .022), respectively. Black patients were hospitalized longer (median 8, IQR 2-39 days) than whites (median 5, IQR 3-14 days), P = .047. A total of 38.9% of blacks and 23.1% of whites were admitted to intensive care unit ( P = .498); 36.1% of blacks had severe cardiac involvement vs 23.1% of white patients, P = .531. Future studies of MIS-C are required to improve health equity for children.
- Published
- 2022
28. Diagnosis and Management of Cancer Treatment-Related Cardiac Dysfunction and Heart Failure in Children
- Author
-
Mohamed Hegazy, Stephanie Ghaleb, and Bibhuti B Das
- Subjects
Pediatrics, Perinatology and Child Health - Abstract
It is disheartening for parents to discover that their children have long-term cardiac dysfunction after being cured of life-threatening childhood cancers. As the number of childhood cancer survivors increases, early and late oncology-therapy-related cardiovascular complications continues to rise. It is essential to understand that cardiotoxicity in childhood cancer survivors is persistent and progressive. A child’s cancer experience extends throughout his lifetime, and ongoing care for long-term survivors is recognized as an essential part of the cancer care continuum. Initially, there was a lack of recognition of late cardiotoxicities related to cancer therapy. About 38 years ago, in 1984, pioneers like Dr. Lipshultz and others published anecdotal case reports of late cardiotoxicities in children and adolescents exposed to chemotherapy, including some who ended up with heart transplantation. At that time, cardiac tests for cancer survivors were denied by insurance companies because they did not meet appropriate use criteria. Since then, cardio-oncology has been an emerging field of cardiology that focuses on the early detection of cancer therapy-related cardiac dysfunction occurring during and after oncological treatment. The passionate pursuit of many healthcare professionals to make life better for childhood cancer survivors led to more than 10,000 peer-reviewed publications in the last 40 years. We synthesized the existing evidence-based practice and described our experiences in this review to share our current method of surveillance and management of cardiac dysfunction related to cancer therapy. This review aims to discuss the pathological basis of cancer therapy-related cardiac dysfunction and heart failure, how to stratify patients prone to cardiotoxicity by identifying modifiable risk factors, early detection of cardiac dysfunction, and prevention and management of heart failure during and after cancer therapy in children. We emphasize serial longitudinal follow-ups of childhood cancer survivors and targeted intervention for high-risk patients. We describe our experience with the new paradigm of cardio-oncology care, and collaboration between cardiologist and oncologist is needed to maximize cancer survival while minimizing late cardiotoxicity.
- Published
- 2023
29. Maximal versus sub-maximal effort during cardiopulmonary exercise testing in adults with congenital heart disease: outcome analysis of short-term cardiac-related events
- Author
-
Talya Kadish, Aliana Godoy, Bibhuti B. Das, and Jianli Niu
- Subjects
Adult ,Heart Defects, Congenital ,medicine.medical_specialty ,Heart disease ,Outcome analysis ,chemistry.chemical_element ,030204 cardiovascular system & hematology ,Oxygen ,03 medical and health sciences ,Oxygen Consumption ,0302 clinical medicine ,Heart Rate ,Internal medicine ,Exercise performance ,Heart rate ,medicine ,Humans ,Respiratory exchange ratio ,Heart Failure ,Exercise Tolerance ,business.industry ,Cardiopulmonary exercise testing ,030229 sport sciences ,General Medicine ,Prognosis ,medicine.disease ,chemistry ,Pediatrics, Perinatology and Child Health ,Exercise Test ,Cardiology ,Cardiology and Cardiovascular Medicine ,Ventilatory threshold ,business - Abstract
Peak respiratory exchange ratio is an objective marker of patient effort during cardiopulmonary exercise testing. We evaluated exercise variables in 175 adult congenital heart disease patients and the impact of respiratory exchange ratio on the prognostic value of exercise variables for short-term cardiac-related events. Of 175 patients, 110 completed the exercise test with a peak respiratory exchange ratio of ≥1.10 and the remaining 65 had a peak respiratory exchange ratio of
- Published
- 2020
30. Recent Era Outcomes of Mechanical Circulatory Support in Children With Congenital Heart Disease as a Bridge to Heart Transplantation
- Author
-
Bahaaldin Alsoufi, Shriprasad R. Deshpande, Bibhuti B. Das, Mark S. Slaughter, and Jaimin R. Trivedi
- Subjects
Heart Defects, Congenital ,medicine.medical_specialty ,Heart disease ,medicine.medical_treatment ,Biomedical Engineering ,Biophysics ,Bioengineering ,Bridge (interpersonal) ,Biomaterials ,Extracorporeal Membrane Oxygenation ,Internal medicine ,Medicine ,Humans ,Child ,Retrospective Studies ,Heart transplantation ,Heart Failure ,business.industry ,General Medicine ,medicine.disease ,Treatment Outcome ,Circulatory system ,Cardiology ,Heart Transplantation ,Heart-Assist Devices ,business - Abstract
The objective of the study is to compare the clinical characteristics, risk factors, and overall survival (waitlist and posttransplant) outcomes in children with congenital heart disease (CHD) bridged to transplantation with either a ventricular assist device (VAD) versus extracorporeal membrane oxygenation (ECMO) versus no mechanical circulatory support (MCS) in the recent era. The study included 2,899 primary heart transplantations in patients18 years with CHD between 2010 and 2019 from the United Network Organ Sharing database. Patients who had ECMO or VAD at listing or while listed were included, and their waitlist and posttransplant outcomes were compared with CHD patients who did not require MCS. Of all, 464 (16%) had ECMO and 200 (7%) VAD at the time of or during the listing. The VAD utilization increased over the last decade (4% in 2010 to 10% in 2019, p0.01). The 90 days post-MCS survival was better with VAD than ECMO (67 vs. 49%, p0.01). The transplantability rate at 90 days was decreased with younger age (odds ratio [OR], 0.91; 95% CI, 0.86-0.95), lower body mass index (BMI) (OR, 0.93; 95% CI, 0.89-0.98) and lower albumin3g/dl (OR, 0.6; 95% CI, 0.53-0.7). The multivariate model predicted that lower BMI (OR, 1.12; 95% CI, 1.06-1.18), pretransplant ECMO (OR, 2.19; 95% CI, 1.39-3.45), and higher bilirubin (OR, 1.15; 95% CI, 0.97-1.36) decreased 1-year posttransplant survival. Patients transplanted with VAD had better 1-year survival than ECMO (88 vs. 70%, p = 0.01). Waiting list survival of children with CHD supported by VAD is better compared to ECMO. The 1-year posttransplantation outcome of CHD patients supported by VAD is similar to the no MCS patients and better than ECMO-supported patients. There is no significant difference in post-HT survival between patients transitioned from ECMO to VAD while listed and those with VAD-first.
- Published
- 2022
31. Influence of Geopolymerization Factors on Sustainable Production of Pelletized Fly Ash Based Aggregates Admixed with Bentonite, Lime and Ggbs
- Author
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B. P. Sharath, Snehal K, and Bibhuti B. Das
- Published
- 2022
32. Use of selexipag in a child with pulmonary hypertension associated with sickle cell disease
- Author
-
Kak-Chen Chan, Bibhuti B Das, and Michelle Marie Jadotte
- Subjects
Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,Anemia ,Hypertension, Pulmonary ,Cell ,MEDLINE ,Anemia, Sickle Cell ,Disease ,Selexipag ,chemistry.chemical_compound ,Text mining ,Internal medicine ,Acetamides ,medicine ,Humans ,Prodrugs ,Pulmonary Wedge Pressure ,Child ,Antihypertensive Agents ,Transplantation ,business.industry ,medicine.disease ,Pulmonary hypertension ,medicine.anatomical_structure ,chemistry ,Pyrazines ,Female ,Surgery ,Cardiology and Cardiovascular Medicine ,business - Published
- 2020
33. Detection of parvovirus B19 and human herpesvirus 6 in pediatric dilated cardiomyopathy: Impact after heart transplantation
- Author
-
Jane Kuypers, Bhupesh K. Prusty, Bibhuti B Das, Keith R. Jerome, Jianli Niu, Eva Eliassen, Meei-Li Huang, and Haiying Zhu
- Subjects
medicine.medical_specialty ,lcsh:Diseases of the circulatory (Cardiovascular) system ,Myocarditis ,medicine.medical_treatment ,viruses ,polymerase chain reaction ,lcsh:Medicine ,Autopsy ,030204 cardiovascular system & hematology ,medicine.disease_cause ,Gastroenterology ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,medicine ,Digital polymerase chain reaction ,pediatric heart transplantation ,immunofluorescent assay ,Heart transplantation ,Cardiotropic viruses ,biology ,Parvovirus ,business.industry ,lcsh:R ,lcsh:RJ1-570 ,Dilated cardiomyopathy ,lcsh:Pediatrics ,medicine.disease ,biology.organism_classification ,dilated cardiomyopathy ,030228 respiratory system ,lcsh:RC666-701 ,Pediatrics, Perinatology and Child Health ,Enterovirus ,Human herpesvirus 6 ,Original Article ,coronary vasculopathy ,Cardiology and Cardiovascular Medicine ,business - Abstract
Objectives: The aim of this study is to evaluate HHV-6 and PVB19 infection using polymerase chain reaction (PCR) and immunofluorescent assay (IFA) in the myocardium of pediatric patients with dilated cardiomyopathy (DCM) and the impact of viral persistence in the cardiac allograft after heart transplantation (HT). Methods: Multiplex droplet digital PCR was used to analyze the prevalence of viral sequences in myocardial samples from 48 pediatric DCM patients and 10 control subjects. Of the 48 DCM patients, 44 underwent HT. After HT, consecutive endomyocardial biopsy (EMB) samples were analyzed for the presence of PVB19 and HHV-6 antigens using IFA and the patients were evaluated for rejections, coronary vasculopathy, and graft loss. Results: Of the 48 DCM patients, 14 had positive viral PCR results in explanted/autopsy hearts. Among them, PVB19 was found in 8/48, HHV6 in 4/48, both PVB19 and HHV6 in 1/48, and enterovirus in one, but no adenovirus was found. The EMB samples obtained after HT were positive for PVB19 and HHV-6 in 7/44 and 3/44 cases, respectively. Viral presence in both the explanted heart and the cardiac allograft was demonstrated in 4 patients, 3 of whom were positive for PVB19, and one of whom was positive for HHV-6 pretransplant. Coronary vasculopathy and graft loss were more common in patients with PVB19-positive myocardial tissues versus those who were PVB19-negative. Conclusions: There is an association between PVB19 and HHV-6 infection and DCM in children. The study suggests the persistence of PVB19 and HHV-6 in the host can lead to subsequent viral reactivation in the transplanted heart, even in those recipients who do not have active myocarditis. PVB19 in the cardiac allograft tended toward higher adverse post-HT events.
- Published
- 2020
34. Syncope in a Child with Pulmonary Hypertension and Positive Gene Tests for Hereditary Hemorrhagic Telangiectasia and Long QT Syndrome
- Author
-
Kak-Chen Chan and Bibhuti B Das
- Subjects
Pharmacology ,medicine.medical_specialty ,Ambrisentan ,business.industry ,Sildenafil ,Long QT syndrome ,Hematology ,medicine.disease ,Pulmonary hypertension ,chemistry.chemical_compound ,chemistry ,Nadolol ,Internal medicine ,medicine ,Pulmonary angiography ,Cardiology ,Family history ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,Telangiectasia ,medicine.drug - Abstract
We present a 10-year-old boy with syncope who was found to have long-QT syndrome and severe Pulmonary Hypertension (PH) both in the absence of a secondary cause; to our knowledge, this is the first report with this unusual coexistence. His genetic tests were positive for hereditary hemorrhagic telangiectasia and Long QT Syndrome (LQTS) without any family history of PH or LQTS. We demonstrated that digital subtraction pulmonary angiography was more useful compared to CT angiogram to demonstrate pulmonary vascular changes which correlated with a noresponse to acute vasoreactivity testing during right heart catheterization. He has been stable for the last 2 years on Ambrisentan, Sildenafil, and Nadolol without recurrence of symptoms.
- Published
- 2020
35. Recurrent pericarditis in an adolescent with Crohn's colitis
- Author
-
Bibhuti B. Das, Morgan Dodson, and Angel Guzman
- Subjects
lcsh:Diseases of the circulatory (Cardiovascular) system ,medicine.medical_specialty ,lcsh:Medicine ,Case Report ,030204 cardiovascular system & hematology ,Chest pain ,Inflammatory bowel disease ,Pericardial effusion ,pericarditis ,Vedolizumab ,03 medical and health sciences ,Pericarditis ,0302 clinical medicine ,inflammatory bowel disease ,Internal medicine ,medicine ,Adalimumab ,Crohn's disease ,business.industry ,lcsh:R ,lcsh:RJ1-570 ,lcsh:Pediatrics ,medicine.disease ,Infliximab ,digestive system diseases ,pericardial effusion ,030228 respiratory system ,lcsh:RC666-701 ,Pediatrics, Perinatology and Child Health ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,medicine.drug - Abstract
Extraintestinal manifestations are common complications of inflammatory bowel disease (IBD), whereas the recurrent pericarditis during remission of Crohn's disease is rarely reported. Chest pain developed in a 13-year-old adolescent male who had a history of Crohn's colitis since 9 years of age and was in remission for 4 years after treatment with infliximab, adalimumab, and vedolizumab. Physicians should be aware of the pericardial involvement in patients with a history of IBD. The literature on pericardial involvement in Crohn's disease is reviewed with emphasis on the management of recurrent pericardial effusion in the pediatric age group.
- Published
- 2020
36. ISHLT consensus statement on donor organ acceptability and management in pediatric heart transplantation
- Author
-
Renata Shih, Karen Lord, Manuela Camino, Jonathan Smith, Angie Scales, Josef Thul, Dimpna C. Albert, Sanjeev Kumar Khulbey, László Ablonczy, Anna Joong, Sharon Chen, Jacqueline M. Smits, Steven J. Kindel, Oliver Miera, Zdenka Reinhardt, Jens Böhmer, Robert G. Weintraub, Matthew Fenton, Jennifer Conway, Anne I. Dipchand, Michael A. McCulloch, Mariska Kemna, Kenneth R. Knecht, Ryan R. Davies, Javier Castro, Richard Kirk, Melanie D. Everitt, Claire Irving, Jonathan N. Johnson, Deipanjan Nandi, Lara Danziger-Isakov, Peta M. A. Alexander, Maryanne R.K. Chrisant, Dipankar Gupta, Luis Garcia-Guereta, Ashwin K. Lal, Gary Beasley, Gretchen B. Chapman, Janet Scheel, Justin Godown, Steve Zangwill, Susan W. Denfield, Antonio Amodeo, Warren A. Zuckerman, Shahnawaz Amdani, Jeffrey G. Gossett, Estela Azeka, Brian Feingold, David N. Rosenthal, Urs Christen, Iki Adachi, Oliver Niesse, Thomas Möller, Jean A Ballweg, Alicia Pérez-Blanco, Martin Schweiger, Ann Punnoose, Bibhuti B. Das, David M. Peng, Daniel Zimpfer, Alison Butler, and Kimberly Y. Lin
- Subjects
Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,Consensus ,Tissue and Organ Procurement ,Scoring system ,Waiting Lists ,Statement (logic) ,medicine.medical_treatment ,030204 cardiovascular system & hematology ,030230 surgery ,Risk Assessment ,Donor Selection ,03 medical and health sciences ,0302 clinical medicine ,medicine ,Humans ,Cardiopulmonary resuscitation ,Primary graft failure ,Child ,Intensive care medicine ,Transplantation ,business.industry ,Graft Survival ,Tissue Donors ,Donor heart ,Heart Transplantation ,Surgery ,Pediatric heart transplantation ,Waitlist mortality ,Cardiology and Cardiovascular Medicine ,business - Abstract
The number of potential pediatric heart transplant recipients continues to exceed the number of donors, and consequently the waitlist mortality remains significant. Despite this, around 40% of all donated organs are not used and are discarded. This document (62 authors from 53 institutions in 17 countries) evaluates factors responsible for discarding donor hearts and makes recommendations regarding donor heart acceptance. The aim of this statement is to ensure that no usable donor heart is discarded, waitlist mortality is reduced, and post-transplant survival is not adversely impacted.
- Published
- 2020
37. Use of the terminal complement inhibitor eculizumab in paediatric heart transplant recipients
- Author
-
Kimberly M. Molina, Bibhuti B. Das, K. Gambetta, Yuk M. Law, Deipanjan Nandi, and Kevin P. Daly
- Subjects
Graft Rejection ,Male ,medicine.medical_specialty ,Adolescent ,medicine.medical_treatment ,030204 cardiovascular system & hematology ,030230 surgery ,Antibodies, Monoclonal, Humanized ,03 medical and health sciences ,Complement inhibitor ,0302 clinical medicine ,Humans ,Medicine ,Child ,Intensive care medicine ,Heart transplantation ,business.industry ,Infant ,General Medicine ,Limiting ,Eculizumab ,Complement Inactivating Agents ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Heart Transplantation ,Female ,Graft survival ,Cardiology and Cardiovascular Medicine ,business ,Complement membrane attack complex ,Solid organ transplantation ,Medical therapy ,medicine.drug - Abstract
Antibody-mediated rejection is a major clinical challenge that limits graft survival. Various modalities of treatment have been reported in small studies in paediatric heart recipients. A novel approach is to use complement-inhibiting agents, such as eculizumab, which inhibits cleavage of C5 to C5a thereby limiting the formation of membrane attack complex and terminal complement-mediated injury of tissue-bound antibodies. This medical modality of treatment has theoretical advantages but the collective experience in its use in the solid organ transplant community remains small. We add to this experience by combining 14 cases from 6 paediatric heart centres in this descriptive study.
- Published
- 2019
38. Senning Procedure for Physiological Atrial Inversion With Left Atrial Isomerism
- Author
-
K. Anitha Jayakumar, Bibhuti B Das, Ming-Lon Young, and Kak-Chen Chan
- Subjects
medicine.medical_specialty ,CDH - Congenital diaphragmatic hernia ,Congenital Mini-Focus Issue ,TGA, transposition of the great arteries ,business.industry ,CDH, congenital diaphragmatic hernia ,SVC, superior vena cava ,left atrial isomerism ,Inversion (linguistics) ,Internal medicine ,RC666-701 ,Left atrial isomerism ,Cardiology ,medicine ,LA, left atrial ,Left ventricular noncompaction ,atrial inversion ,Diseases of the circulatory (Cardiovascular) system ,Senning Procedure ,left ventricular noncompaction ,Case Report: Clinical Case ,Senning procedure ,Cardiology and Cardiovascular Medicine ,business - Abstract
We present the case of an infant with left atrial isomerism with complex pulmonary and systemic venous connections that resulted in physiological parallel circulation in the setting of ventriculoarterial concordance who was surgically treated using the Senning procedure. This case highlights a rare cause for cyanosis due to poor mixing from a parallel circulation and an issue with nomenclature. (Level of Difficulty: Advanced.), Graphical abstract, The authors present the case of an infant with left atrial isomerism with complex pulmonary and systemic venous connections that resulted in…
- Published
- 2019
39. Successful recovery after blade atrial septostomy in a child with pulmonary hypertensive crisis and cardiac arrest requiring extracorporeal cardiopulmonary resuscitation
- Author
-
Onyekachukwu Osakwe and Bibhuti B. Das
- Subjects
Pediatrics, Perinatology and Child Health ,Cardiology and Cardiovascular Medicine - Published
- 2022
40. Contemporary outcomes of durable ventricular assist devices in adults with congenital heart disease as a bridge to heart transplantation
- Author
-
Mark S. Slaughter, Bibhuti B Das, Jaimin R. Trivedi, Shriprasad R. Deshpande, and Brian Kogon
- Subjects
Adult ,Heart Defects, Congenital ,Male ,medicine.medical_specialty ,Heart disease ,Adolescent ,medicine.medical_treatment ,Biomedical Engineering ,Medicine (miscellaneous) ,Bioengineering ,Biomaterials ,chemistry.chemical_compound ,Internal medicine ,medicine.artery ,parasitic diseases ,Medicine ,Humans ,cardiovascular diseases ,Retrospective Studies ,Heart transplantation ,Heart Failure ,Creatinine ,business.industry ,Mortality rate ,General Medicine ,medicine.disease ,Transplantation ,Bridge (graph theory) ,Treatment Outcome ,chemistry ,Ventricular assist device ,Pulmonary artery ,Cardiology ,Heart Transplantation ,Female ,Heart-Assist Devices ,biological phenomena, cell phenomena, and immunity ,business - Abstract
This study aimed to compare the clinical characteristics, risk factors, and overall survival outcomes in adults with congenital heart disease (ACHD) bridged to transplantation with a ventricular assist device (VAD) versus no-VAD.The study included 894 ACHD patients aged ≥18 years listed for primary heart transplantation between 2010 and 2019 from the United Network for Organ Sharing database. Primary outcomes were waitlist and 1-year post-transplant mortality between VAD and no-VAD ACHD patients.Of 894 ACHD patients included in the study, 91(10.1%) had VAD support at the time of listing. Patients who needed VAD support were mostly males, heavier, and had higher pulmonary artery pressure than the no-VAD group at the listing. The overall waitlist mortality was 38% in the VAD group than 17% in the no-VAD group (p 0.01). ECMO use was associated with significantly higher mortality than either group. There was no significant difference in 1-year post-transplant mortality between VAD versus no-VAD at the time of transplant (15% vs. 17%; p = 0.66). Multivariate regression analysis found that BMI20 kg/mThe one-year post-transplant mortality rate was no different for ACHD patients that received VAD versus no-VAD. These findings suggest that a VAD should be considered an option to support ACHD patients as a bridge to heart transplantation.
- Published
- 2021
41. A Review of the Cardiac and Cardiovascular Effects of COVID-19 in Adults and Children
- Author
-
Shriprasad R. Deshpande, Lara S. Shekerdemian, S Kristen Sexon Tejtel, and Bibhuti B Das
- Subjects
2019-20 coronavirus outbreak ,medicine.medical_specialty ,Myocarditis ,Coronavirus disease 2019 (COVID-19) ,business.industry ,Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ,Disease progression ,food and beverages ,Review ,030204 cardiovascular system & hematology ,medicine.disease ,Comorbidity ,03 medical and health sciences ,0302 clinical medicine ,medicine ,030212 general & internal medicine ,Cardiology and Cardiovascular Medicine ,Intensive care medicine ,business - Abstract
Symptomatic coronavirus disease 2019 (COVID-19) typically affects the respiratory system but can involve the cardiovascular system. Cardiac complications of COVID-19 can result directly from myocarditis or indirectly from numerous other mechanisms. Differentiating between primary and secondary cardiovascular involvement—our focus in this review—may help to identify the long-term effects of COVID-19 on the heart in adults and children.
- Published
- 2021
42. Myocarditis and Pericarditis Following mRNA COVID-19 Vaccination: What Do We Know So Far?
- Author
-
April Palmer, Bibhuti B Das, William B. Moskowitz, and Mary B. Taylor
- Subjects
medicine.medical_specialty ,Myocarditis ,Inflammation ,Review ,030204 cardiovascular system & hematology ,Chest pain ,Gastroenterology ,pericarditis ,Pediatrics ,RJ1-570 ,03 medical and health sciences ,Pericarditis ,0302 clinical medicine ,Cardiac magnetic resonance imaging ,Internal medicine ,medicine ,030212 general & internal medicine ,medicine.diagnostic_test ,biology ,business.industry ,medicine.disease ,Symptomatic relief ,Troponin ,Vaccination ,Pediatrics, Perinatology and Child Health ,biology.protein ,medicine.symptom ,myocarditis ,business ,COVID-19 vaccine - Abstract
This is a cross-sectional study of 29 published cases of acute myopericarditis following COVID-19 mRNA vaccination. The most common presentation was chest pain within 1–5 days after the second dose of mRNA COVID-19 vaccination. All patients had an elevated troponin. Cardiac magnetic resonance imaging revealed late gadolinium enhancement consistent with myocarditis in 69% of cases. All patients recovered clinically rapidly within 1–3 weeks. Most patients were treated with non-steroidal anti-inflammatory drugs for symptomatic relief, and 4 received intravenous immune globulin and corticosteroids. We speculate a possible causal relationship between vaccine administration and myocarditis. The data from our analysis confirms that all myocarditis and pericarditis cases are mild and resolve within a few days to few weeks. The bottom line is that the risk of cardiac complications among children and adults due to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection far exceeds the minimal and rare risks of vaccination-related transient myocardial or pericardial inflammation.
- Published
- 2021
43. Relation Between New York Heart Association Functional Class and Objective Measures of Cardiopulmonary Exercise in Adults With Congenital Heart Disease
- Author
-
Jianli Niu, Kak Chen Chan, Todd Roth, Bibhuti B. Das, Ming-Lon Young, and Laura E. Mendoza
- Subjects
Adult ,Heart Defects, Congenital ,Male ,medicine.medical_specialty ,Heart disease ,030204 cardiovascular system & hematology ,New york heart association ,Young Adult ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,Heart rate ,medicine ,Humans ,cardiovascular diseases ,Young adult ,Retrospective Studies ,business.industry ,Medical record ,Cardiopulmonary exercise ,Retrospective cohort study ,medicine.disease ,humanities ,Blood pressure ,030228 respiratory system ,Exercise Test ,Cardiology ,Female ,Cardiology and Cardiovascular Medicine ,business - Abstract
We aimed to compare New York Heart Association (NYHA) functional class in adult congenital heart disease (ACHD) patients with objectively measured cardiopulmonary exercise testing (CPET) parameters. This study included retrospective review of ACHD patients who underwent a CPET between August 2014 and April 2018 at our center. Patients were grouped according to severity of CHD, and NYHA class as recorded in their medical record or estimated from the clinical narrative. A total of 175 ACHD patients (mean age 30 ± 11 years) with NYHA class I-III enrolled in the study. The NYHA functional class was II or III in most complex CHD. There was a strong inverse relation between NYHA class and peak oxygen consumption, oxygen uptake efficiency slope, and the double product at peak exercise (product of heart rate and systolic blood pressure) (p0.0001). There was no relation between NYHA class and ventilation efficiency slope (p = 0.37). In conclusion, NYHA functional class correlates with objective measures of CPET, however there is wide variability in measured exercise capacity in each NYHA classification. Therefore, whereas NYHA class of patients is a simple measure for assessment of functional status, CPET is an important tool to identify the source of exercise limitation in ACHD patients.
- Published
- 2019
44. Novel Loss of Function in the AGK Gene
- Author
-
Parul Jayakar, Kathryn C. Chatfield, Lazaro E. Hernandez, Maryanne Chrisant, and Bibhuti B. Das
- Subjects
medicine.medical_specialty ,SENGERS SYNDROME ,business.industry ,Case Report ,mitochondrial cardiomyopathy ,Sengers syndrome ,medicine.disease ,Mitochondrial cardiomyopathy ,Clinical Case ,Heart failure ,Internal medicine ,RC666-701 ,acylglycerol kinase ,medicine ,Cardiology ,Diseases of the circulatory (Cardiovascular) system ,AGK ,End stage heart failure ,Cardiology and Cardiovascular Medicine ,business ,Novel mutation ,Gene ,Acylglycerol kinase ,Loss function - Abstract
The authors present a case of mitochondrial cardiomyopathy due to a novel mutation of AGK gene that led to progressive heart failure. The cardiac…, The authors present a case of mitochondrial cardiomyopathy due to a novel mutation of AGK gene that led to progressive heart failure. The cardiac magnetic resonance image findings of diffusely elevated relaxation time and increase in extracellular volume in the myocardium without early or late gadolinium enhancement may suggest mitochondrial cardiomyopathy. The authors emphasized the multidisciplinary team approach in the care of patients with mitochondrial cardiomyopathies. (Level of Difficulty: Advanced.), Graphical abstract
- Published
- 2019
45. Improvement of cardiopulmonary function after minimally invasive surgical repair of pectus excavatum (Nuss procedure) in children
- Author
-
Thomas Yeh, Bibhuti B. Das, and Michael R Recto
- Subjects
lcsh:Diseases of the circulatory (Cardiovascular) system ,pectus excavatum ,Oxygen pulse ,lcsh:Medicine ,030204 cardiovascular system & hematology ,Nuss procedure ,03 medical and health sciences ,0302 clinical medicine ,Pectus excavatum ,Medicine ,Treadmill ,Cardiopulmonary exercise stress test ,business.industry ,lcsh:R ,lcsh:RJ1-570 ,VO2 max ,lcsh:Pediatrics ,Stroke volume ,medicine.disease ,030228 respiratory system ,lcsh:RC666-701 ,Anesthesia ,Pediatrics, Perinatology and Child Health ,Haller index ,Original Article ,Cardiology and Cardiovascular Medicine ,business ,Respiratory minute volume - Abstract
Background: Severe pectus excavatum in children may result in cardiorespiratory functional impairment; therefore, we evaluated cardiopulmonary response to exercise before and after the Nuss procedure. Methods: Twenty-four physically active pediatric patients aged 9–18 years with severe pectus excavatum (Haller index >3.25) were included in the study. Cardiopulmonary exercise testing using treadmill and modified Bruce protocol was performed before and after the Nuss procedure. Results: Maximal oxygen uptake and oxygen pulse improved by 40.6% (32 ± 13–45 ± 10 ml/kg/min; P = 0.0001) and 44.4% (9 ± 4–13 ± 5 ml/beat; P = 0.03), respectively, after surgical correction of pectus excavatum by Nuss procedure. Significant improvement in maximum voluntary ventilation and minute ventilation after Nuss procedure was also noted. Conclusions: We found that, after repair of pectus excavatum by Nuss procedure, the exercise capacity as measured by maximal oxygen consumption improved significantly primarily due to increase in oxygen pulse, an indirect measurement of stroke volume.
- Published
- 2019
46. COVID-19 and Pulmonary Hypertension in Children: What Do We Know So Far?
- Author
-
Bibhuti B Das
- Subjects
medicine.medical_specialty ,Pediatrics ,Medicine (General) ,Coronavirus disease 2019 (COVID-19) ,Hypertension, Pulmonary ,Review ,R5-920 ,Pandemic ,Epidemiology ,medicine ,Humans ,Child ,Pandemics ,Potential risk ,business.industry ,SARS-CoV-2 ,Incidence (epidemiology) ,Incidence ,COVID-19 ,General Medicine ,medicine.disease ,Pulmonary hypertension ,pediatric pulmonary hypertension ,Health education ,business ,Rare disease - Abstract
The interplay between coronavirus disease 2019 (COVID-19) and pulmonary hypertension (PH) in children is unknown. Adults with PH are at potential risk for severe complications and high mortality due to associated comorbidities. It is difficult to extrapolate the outcomes of COVID-19 in adults to pediatric PH patients. Overall, a small number of COVID-19 cases is reported in patients with preexisting PH. Several factors may be responsible for the low incidence of COVID-19 in children with PH. Pulmonary hypertension is a rare disease, testing is not universal, and patients may have followed more rigorously the Center for Disease Control’s guidelines recommended for personal protection with mask-wearing, social distancing, and hand sanitization through ongoing health education. The small number of COVID-19 cases in patients with preexisting PH does not support that PH is protective for COVID-19. However, medications used to treat PH may have some protection against COVID-19. This review discusses the pathophysiology of PH occurring with COVID-19, differences between children and adults with COVID-19, strategies for management of preexisting PH in children during the ongoing pandemic, and its impact within the field of PH.
- Published
- 2020
47. Impact of peak respiratory exchange ratio on the prognostic power of symptoms-limited exercise testing using Bruce protocol in patients with Fontan physiology
- Author
-
Bibhuti B Das, Jianli Niu, Aliana Godoy, and Talya Kadish
- Subjects
Adult ,medicine.medical_specialty ,030204 cardiovascular system & hematology ,Incremental exercise ,03 medical and health sciences ,0302 clinical medicine ,Bruce protocol ,Oxygen Consumption ,Internal medicine ,Heart rate ,medicine ,Humans ,In patient ,030212 general & internal medicine ,Respiratory exchange ratio ,Retrospective Studies ,Heart Failure ,business.industry ,General Medicine ,Odds ratio ,Fontan physiology ,Middle Aged ,Prognosis ,Respiratory Function Tests ,Pediatrics, Perinatology and Child Health ,Cardiology ,Exercise Test ,Cardiology and Cardiovascular Medicine ,Ventilatory threshold ,business - Abstract
Objectives:We evaluated the impact of peak respiratory exchange ratio on the prognostic values of cardiopulmonary exercise variables during symptoms-limited incremental exercise tests in patients with Fontan physiology.Methods:Retrospective single-centre chart review study of Fontan patients who underwent exercise testing using the Bruce protocol between 2014 and 2018 and follow-up.Results:A total of 34 patients (age > 18 years) had a Borg score of ≥7 on the Borg 10-point scale, but only 50% of patients achieved a peak respiratory exchange ratio of ≥ 1.10 (maximal test). Peak oxygen consumption, percent-predicted peak oxygen consumption, and peak oxygen consumption at the ventilatory threshold was reduced significantly in patients with a peak respiratory exchange ratio of < 1.10. Peak oxygen consumption and percent-predicted peak oxygen consumption was positively correlated with peak respiratory exchange ratio values (r = 0.356, p = 0.039). After a median follow-up of 21 months, cardiac-related events occurred in 16 (47%) patients, with no proportional differences in patients due to their respiratory exchange ratio (odds ratio, 0.62; 95% CI: 0.18–2.58; p = 0.492). Multivariate Cox proportional hazard analysis showed percent-predicted peak oxygen consumption, peak heart rate, and the oxygen uptake efficient slope were highly related to the occurrence of events in patients only with a peak respiratory exchange ratio of ≥ 1.10.Conclusions:The value of peak cardiopulmonary exercise variables is limited for the determination of prognosis and assessment of interventions in Fontan patients with sub-maximal effort. Our findings deserve further research and clinical application.
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- 2020
48. Abstract 14835: Corona Virus Disease 2019 (covid-19) Myocarditis: Where and What is the Evidence?
- Author
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Bibhuti B. Das and Shriprasad R. Deshpande
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Myocarditis ,Coronavirus disease 2019 (COVID-19) ,business.industry ,Cardiovascular health ,Inflammatory response ,medicine.medical_treatment ,Virus diseases ,medicine.disease ,Corona (optical phenomenon) ,Cytokine ,Physiology (medical) ,Immunology ,medicine ,Cardiology and Cardiovascular Medicine ,business - Abstract
Background and Objectives: Cardiac complications due to COVID-19 can occur either directly (myopericaridal involvement) or indirectly by the profound systemic inflammatory response and cytokine storm leading to cardiac dysfunction. No virus RNA has been detected in cardiac myocytes in patients with COVID-19 from China. Our objective is to analyze the COVID-19 myocarditis cases reported in literature to-date. Methods: We used terms COVID-19, myocarditis, and cardiac involvement in COVID-19 to perform a search for myocarditis cases due to COVID-19 in PUBMED. We found a total of 8 cases with a diagnosis of COVID-19 myocarditis and analyzed their clinical data as summarized in Table. Results: Age ranged from 21-69 years and 75% were male. Of eight COVID-19 myocarditis patients, 3/8(38%) had pre-existing cardiac issues, 5/7(71%) had abnormal electrocardiograms and decreased function by echocardiograms. Troponin and BNP levels were elevated in all cases. CMR was performed in 5 cases and showed myocardial edema in T2 mapping and late gadolinium enhancement suggesting scar/fibrosis in 100% cases. No endomyocardial biopsy or autopsy was performed except one case, who was dead on arrival and autopsy study showed eosinophilic myocarditis, not a typical finding of viral myocarditis. Overall outcomes were 2 deaths and 6 patients recovered. Conclusions: COVID-19 can cause direct myocardial injury but the evidence is extremely limited at this stage. Although serum markers and imaging suggest cardiac involvement, the pathogenesis and long term impact of COVID-19 on cardiovascular system is unknown. Evaluation for myocarditis by endomyocardial biopsies may be appropriate in selected cases when a treatable type of myocarditis is suspected (e.g. giant cell myocarditis) or unexplained severe bi-ventircular dysfunction or arhrhythmia with normal coronaries to confirm a definitive diagnosis of myocarditis and identify its cause to enable possible etiology-directed therapy.
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- 2020
49. Outcomes of ECMO versus VAD in Children with Congenital Heart Disease as a Bridge to Transplant in Recent Era: Analysis from United Network for Organ Sharing Database
- Author
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Shriprasad R. Deshpande, Bahaaldin Alsoufi, Jaimin R. Trivedi, Mark S. Slaughter, and Bibhuti B Das
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Pulmonary and Respiratory Medicine ,United Network for Organ Sharing ,Pediatrics ,medicine.medical_specialty ,Heart disease ,030204 cardiovascular system & hematology ,03 medical and health sciences ,0302 clinical medicine ,parasitic diseases ,0502 economics and business ,medicine ,Overall survival ,cardiovascular diseases ,Transplantation ,Bridge to transplant ,business.industry ,05 social sciences ,medicine.disease ,surgical procedures, operative ,Waiting list ,050211 marketing ,Surgery ,Bridge to transplantation ,biological phenomena, cell phenomena, and immunity ,Cardiology and Cardiovascular Medicine ,business - Abstract
Purpose To compare the clinical characteristics, risk factors, and overall survival (waitlist and post-transplant) outcomes in children with congenital heart disease (CHD) bridged to transplantation with either a VAD vs. ECMO vs. no-mechanical circulatory support (MCS). Methods The study included 2899 primary heart transplantations in children Results Of all, 466(16%) had ECMO and 200(7%) VAD at the time of listing or while listed (Figure-1 A-B). There was an increasing VAD utilization trend during the last decade over ECMO (p Conclusion Waiting list survival of children with CHD supported by durable VAD is better compared to ECMO support. The long-term posttransplantation outcomes of CHD patients supported by VAD is similar to non-MCS patients and better than ECMO supported patients. The transition from ECMO to VAD while listed results in similar outcomes to VAD-first, so every effort should be made to select CHD patients eligible for VAD as a bridge to transplantation.
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- 2021
50. Cytomegalovirus infection and allograft rejection among pediatric heart transplant recipients in the era of valganciclovir prophylaxis
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Jianli Niu, Paul K. Sue, Bibhuti B. Das, and Bhupesh K. Prusty
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Graft Rejection ,Male ,medicine.medical_specialty ,030232 urology & nephrology ,Congenital cytomegalovirus infection ,030230 surgery ,Graft loss ,Antiviral Agents ,Gastroenterology ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,medicine ,Humans ,Valganciclovir ,Child ,Retrospective Studies ,CMV status ,Transplantation ,business.industry ,Infant ,virus diseases ,Cmv dnaemia ,Antibiotic Prophylaxis ,medicine.disease ,Cytomegalovirus infection ,Allograft rejection ,Median time ,Child, Preschool ,Cytomegalovirus Infections ,Pediatrics, Perinatology and Child Health ,Heart Transplantation ,Female ,business ,medicine.drug - Abstract
CMV infection remains a significant cause of morbidity among pediatric HTx recipients We explored the implications of CMV infection on post-transplant outcomes among CMV risk-stratified pediatric HTx recipients receiving VGC prophylaxis. Children who underwent HTx between January 2010 and October 2016 were stratified according to CMV risk at time of transplant and evaluated for evidence of post-transplant CMV infection, rejection, CAV, and graft loss. Among 97 children, 41 (42%) were considered HR or IR risk for CMV infection and received VGC prophylaxis. CMV DNAemia was observed in 34% of children, including 71% HR, 40% IR, and 18% LR individuals. Median time to CMV DNAemia following VGC prophylaxis was 32D among HR vs 277D in IR subjects (P = .042). No difference in overall graft loss was noted among groups, but CMV HR children had decreased rejection-free survival (3.5 years) compared to IR (6 years, P = .015) and LR children (8 years, P = .0003). CMV was noted on EMB in 13% of children but was not associated with increased CAV, rejection or graft loss. High-risk CMV status was associated with decreased time to CMV infection despite VGC prophylaxis, compared to IR, and decreased rejection-free survival times compared to both IR and LR recipients. Detection of CMV on EMB was not associated with increased rejection, CAV or graft loss. Additional studies are needed to explore the impact of CMV infection on rejection-free survival in HTx recipients.
- Published
- 2020
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