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Surgical Correction Of Aorto-Pulmonary Window: A Rare And Lethal Cause Of Pulmonary Hypertension, Adeel Ahmed Shamim, Mubashir Zareen Khan, Mehnaz Ahmed Atiq, Mansoor Ahmed Khan, Muhammad Muneer Amanullah 2011 Aga Khan University

Surgical Correction Of Aorto-Pulmonary Window: A Rare And Lethal Cause Of Pulmonary Hypertension, Adeel Ahmed Shamim, Mubashir Zareen Khan, Mehnaz Ahmed Atiq, Mansoor Ahmed Khan, Muhammad Muneer Amanullah

Section of Cardiothoracic Surgery

Aorto-pulmonary window is a relatively rare congenital cardiac malformation with an overall incidence of 0.1%. Pulmonary hypertension develops quickly if the lesion is left untreated hence early surgical intervention is warranted after diagnosis. The surgery for correction of APW has evolved over years, currently an open repair on cardiopulmonary bypass (CPB) with a single patch technique yields good results. Mortality is affected by association of pulmonary hypertension and other cardiac malformations. We present a case of an infant with a large type II APW with a relatively low pulmonary vascular resistance. Hospital stay was complicated because of pulmonary arterial disease …


Ganglioneuroblastoma Of The Posterior Mediastinum: A Case Report., Saulat Fatimi, Samira A Bawany, Awais Ashfaq 2011 Aga Khan University

Ganglioneuroblastoma Of The Posterior Mediastinum: A Case Report., Saulat Fatimi, Samira A Bawany, Awais Ashfaq

Section of Cardiothoracic Surgery

Introduction:Ganglioneuroblastoma is a rare peripheral neuroblastic tumor that is derived from developing neuronal cells of the sympathetic nervous system, and is found mostly, but not exclusively, in babies and young children.

Case Presentation:

To the best of our knowledge, there have been no previously reported cases of ganglioneuroblastoma of the mediastinum from Pakistan. We present a case of ganglioneuroblastoma in an eight-year-old Pakistani Sindhi boy incidentally found to have a large posterior mediastinal mass that on biopsy initially looked like ganglioneuroma. He underwent successful excision of the mediastinal mass and remained stable post-operatively. Final pathology findings showed a ganglioneuroblastoma. …


Factors Associated With Serum Brain Natriuretic Peptide Levels After The Fontan Procedure., Andrew M. Atz, Victor Zak, Roger E. Breitbart, Steven D. Colan, Sara K. Pasquali, Daphne T. Hsu, Minmin Lu, Lynn Mahony, Stephen M. Paridon, Michael D. Puchalski, Tal Geva, Brian W. McCrindle, Pediatric Heart Network Investigators, Girish S. Shirali 2011 Children's Mercy Hospital

Factors Associated With Serum Brain Natriuretic Peptide Levels After The Fontan Procedure., Andrew M. Atz, Victor Zak, Roger E. Breitbart, Steven D. Colan, Sara K. Pasquali, Daphne T. Hsu, Minmin Lu, Lynn Mahony, Stephen M. Paridon, Michael D. Puchalski, Tal Geva, Brian W. Mccrindle, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: Although a useful marker of heart failure in adults, the utility of brain natriuretic peptide concentration (BNP) for children after the Fontan procedure is not well studied.

DESIGN: BNP was measured in 510 patients who were 6-18 years old in the Pediatric Heart Network Fontan cross-sectional study at a median of 8.2 years after Fontan. Patients underwent echocardiography, exercise testing, magnetic resonance imaging (MRI) and functional health status questionnaires. Associations of BNP with baseline patient characteristics, medical history and cross-sectional assessment were examined with multivariable linear regression analyses.

RESULTS: The distribution of BNP was highly skewed, median 13.0 pg/mL …


Bapcap2-1 Does Preoperative Aspirin Improve Outcomes In Cardiac Surgery Patients?, Jian-Zhong Sun, L. Cao, H. Liu, W. Sun, S Silvestry, J. Diehl 2011 Thomas Jefferson University

Bapcap2-1 Does Preoperative Aspirin Improve Outcomes In Cardiac Surgery Patients?, Jian-Zhong Sun, L. Cao, H. Liu, W. Sun, S Silvestry, J. Diehl

Department of Anesthesiology Faculty Papers

Best Abstracts - Runner-up Session 2 presented at Euroanaesthesia 2011 in the Netherlands.

Background and Goal of Study: The effects of preoperative aspirin on major cardiocerebral and renal outcomes and mortality remain uncertain.


Surgical Management Of Complete Atrioventricular Septal Defect: Associations With Surgical Technique, Age, And Trisomy 21., Andrew M. Atz, John A. Hawkins, Minmin Lu, Meryl S. Cohen, Steven D. Colan, James Jaggers, Ronald V. Lacro, Brian W. McCrindle, Renee Margossian, Ralph S. Mosca, Lynn A. Sleeper, L LuAnn Minich, Pediatric Heart Network Investigators, Girish S. Shirali 2011 Children's Mercy Hospital

Surgical Management Of Complete Atrioventricular Septal Defect: Associations With Surgical Technique, Age, And Trisomy 21., Andrew M. Atz, John A. Hawkins, Minmin Lu, Meryl S. Cohen, Steven D. Colan, James Jaggers, Ronald V. Lacro, Brian W. Mccrindle, Renee Margossian, Ralph S. Mosca, Lynn A. Sleeper, L Luann Minich, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: We sought to evaluate the contemporary results after repair of a complete atrioventricular septal defect and to determine the factors associated with suboptimal outcomes.

METHODS: The demographic, procedural, and outcome data were obtained within 1 and 6 months after repair of a complete atrioventricular septal defect in 120 children in a multicenter observational study from June 2004 to 2006.

RESULTS: The median age at surgery was 3.7 months (range, 9 days to 1.1 years). The type of surgical repair was a single patch (18%), double patch (72%), and a single atrial septal defect patch with primary ventricular septal defect …


Chemical Cardiomyopathies: The Negative Effects Of Medications And Nonprescribed Drugs On The Heart., Vincent M Figueredo 2011 Thomas Jefferson University

Chemical Cardiomyopathies: The Negative Effects Of Medications And Nonprescribed Drugs On The Heart., Vincent M Figueredo

Division of Cardiology Faculty Papers

The heart is a target of injury for many chemical compounds, both medically prescribed and not medically prescribed. Pathophysiologic mechanisms underlying the development of chemical-induced cardiomyopathies vary depending on the inciting agent, including direct toxic effects, neurohormonal activation, altered calcium homeostasis, and oxidative stress. Numerous chemicals and drugs are implicated in cardiomyopathy. This article discusses examples of medication and nonprescribed drug-induced cardiomyopathies and reviews their pathophysiologic mechanisms.


Renin-Angiotensin-Aldosterone Genotype Influences Ventricular Remodeling In Infants With Single Ventricle., Seema Mital, Wendy K. Chung, Steven D. Colan, Lynn A. Sleeper, Cedric Manlhiot, Cammon B. Arrington, James F. Cnota, Eric M. Graham, Michael E. Mitchell, Elizabeth Goldmuntz, Jennifer S. Li, Jami C. Levine, Teresa M. Lee, Renee Margossian, Daphne T. Hsu, Pediatric Heart Network Investigators, Girish S. Shirali 2011 Children's Mercy Hospital

Renin-Angiotensin-Aldosterone Genotype Influences Ventricular Remodeling In Infants With Single Ventricle., Seema Mital, Wendy K. Chung, Steven D. Colan, Lynn A. Sleeper, Cedric Manlhiot, Cammon B. Arrington, James F. Cnota, Eric M. Graham, Michael E. Mitchell, Elizabeth Goldmuntz, Jennifer S. Li, Jami C. Levine, Teresa M. Lee, Renee Margossian, Daphne T. Hsu, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

Background: We investigated the effect of polymorphisms in the renin-angiotensin-aldosterone system (RAAS) genes on ventricular remodeling, growth, renal function, and response to enalapril in infants with single ventricle.

Methods and results: Single ventricle infants enrolled in a randomized trial of enalapril were genotyped for polymorphisms in 5 genes: angiotensinogen, angiotensin-converting enzyme, angiotensin II type 1 receptor, aldosterone synthase, and chymase. Alleles associated with renin-angiotensin-aldosterone system upregulation were classified as risk alleles. Ventricular mass, volume, somatic growth, renal function using estimated glomerular filtration rate, and response to enalapril were compared between patients with ≥2 homozygous risk genotypes (high risk), and those …


Effects Of Walnut Consumption As Part Of Heart Healthy Diet On Atherogenic Lipoprotein Subclasses, Kimberly L. Dulaney, MD, John M. Morgan, MD, David M. Capuzzi, MD 2011 Thomas Jefferson University Hospital

Effects Of Walnut Consumption As Part Of Heart Healthy Diet On Atherogenic Lipoprotein Subclasses, Kimberly L. Dulaney, Md, John M. Morgan, Md, David M. Capuzzi, Md

The Medicine Forum

Nutritional therapy is fundamental in the treatment of
cardiovascular disease. Studies that utilized a low-fat diet
supplemented with 64 grams/day of walnuts have shown
a significant reduction in serum levels of total and LDL
cholesterol, and triglycerides. This study was designed to
see if lipoprotein subclasses varied in patients eating a
low-fat walnut supplemented diet.


Lipid Phosphate Phosphatase 3 Enables Efficient Thymic Egress, Béatrice Bréart, Willy D. Ramos-Perez, Alejandra Mendoza, Abdelghaffar K. Salous, Michael Gobert, Yong Huang, Ralf H. Adams, Juan J. Lafaille, Diana Escalante-Alcalde, Andrew J. Morris, Susan R. Schwab 2011 New York University

Lipid Phosphate Phosphatase 3 Enables Efficient Thymic Egress, Béatrice Bréart, Willy D. Ramos-Perez, Alejandra Mendoza, Abdelghaffar K. Salous, Michael Gobert, Yong Huang, Ralf H. Adams, Juan J. Lafaille, Diana Escalante-Alcalde, Andrew J. Morris, Susan R. Schwab

Gill Heart & Vascular Institute Faculty Publications

The signaling lipid sphingosine-1-phosphate (S1P) stabilizes the vasculature, directs lymphocyte egress from lymphoid organs, and shapes inflammatory responses. However, little is known about how S1P distribution is controlled in vivo, and it is not clear how a ubiquitously made lipid functions as a signal that requires precise spatial and temporal control. We have found that lipid phosphate phosphatase 3 (LPP3) enables efficient export of mature T cells from the thymus into circulation, and several lines of evidence suggest that LPP3 promotes exit by destroying thymic S1P. Although five additional S1P-degrading enzymes are expressed in the thymus, they cannot compensate for …


Post-Exercise Hypotension In Brief Exercise, Jeremiah G. Bush 2011 Western Kentucky University

Post-Exercise Hypotension In Brief Exercise, Jeremiah G. Bush

Masters Theses & Specialist Projects

The purpose of this investigation was to examine whether a single 10 minute bout of exercise, performed at multiple intervals throughout the day to equal 30 minutes, can effectively elicit post-exercise hypotension (PEH). Secondly, it is important to explore whether a light (40% VO2R) or moderate (70% VO2R) intensity is required to elicit PEH within 10 minutes. Subjects (N=11) completed a VO2max test utilizing the Bruce Treadmill protocol. Each subject returned within 3 – 5 days to complete two separate exercising trials. A counter balanced system was employed so that each subject did not perform the same intensity rotation (Counter …


Strategies To Reduce Disease Burden From Diabetes And Hypertension In Developing World, Waris Qidwai 2011 Aga Khan University

Strategies To Reduce Disease Burden From Diabetes And Hypertension In Developing World, Waris Qidwai

Department of Family Medicine

No abstract provided.


Evaluation Of Kawasaki Disease Risk-Scoring Systems For Intravenous Immunoglobulin Resistance., Lynn A. Sleeper, L Luann Minich, Brian M. McCrindle, Jennifer S. Li, Wilbert Mason, Steven D. Colan, Andrew M. Atz, Beth F. Printz, Annette Baker, Victoria L. Vetter, Jane W. Newburger, Pediatric Heart Network Investigators, Girish S. Shirali 2011 Children's Mercy Hospital

Evaluation Of Kawasaki Disease Risk-Scoring Systems For Intravenous Immunoglobulin Resistance., Lynn A. Sleeper, L Luann Minich, Brian M. Mccrindle, Jennifer S. Li, Wilbert Mason, Steven D. Colan, Andrew M. Atz, Beth F. Printz, Annette Baker, Victoria L. Vetter, Jane W. Newburger, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: To assess the performance of 3 risk scores from Japan that were developed to predict, in children with Kawasaki disease, resistance to intravenous immunoglobulin (IVIG) treatment.

STUDY DESIGN: We used data from a randomized trial of pulsed steroids for primary treatment of Kawasaki disease to assess operating characteristics of the 3 risk scores, and we examined whether steroid therapy lowers the risk of coronary artery abnormalities in patients prospectively classified as IVIG resistant.

RESULTS: For comparability with published cohorts, we analyzed the data of 99 patients who were not treated with steroids (16% IVIG-retreated) and identified male sex, lower …


Takotsubo Cardiomyopathy Vs Acute Myocardial Infarction: Diagnostic Utility Of Subtle Ecg Differences, Asma Saba Syed, Umair Khalid 2011 Aga Khan University

Takotsubo Cardiomyopathy Vs Acute Myocardial Infarction: Diagnostic Utility Of Subtle Ecg Differences, Asma Saba Syed, Umair Khalid

Section of Cardiology

The clinical findings of Takatsubo Cardiomyopathy and acute myocardial infarction can be very similar. While Takatsubo cardiomyopathy rarely leads to severe complications, acute myocardial infarction can be life threatening. Treatment of both these conditions is different and so it is imperative for clinicians to have a high index of suspicion for either. Several EKG differences between the two entities have been proposed. This article summarizes the EKG changes most likely seen in Takatsubo cardiomyopathy and compares them to those seen in Acute Myocardial infarction.


Oxidative Stress Accumulates In Adipose Tissue During Aging And Inhibits Adipogenesis, Hannes M. Findeisen, Kevin J. Pearson, Florence Gizard, Yue Zhao, Hua Qing, Karrie L Jones, Dianne Cohn, Elizabeth B. Heywood, Rafael de Cabo, Dennis Bruemmer 2011 University of Kentucky

Oxidative Stress Accumulates In Adipose Tissue During Aging And Inhibits Adipogenesis, Hannes M. Findeisen, Kevin J. Pearson, Florence Gizard, Yue Zhao, Hua Qing, Karrie L Jones, Dianne Cohn, Elizabeth B. Heywood, Rafael De Cabo, Dennis Bruemmer

Saha Cardiovascular Research Center Faculty Publications

Aging constitutes a major independent risk factor for the development of type 2 diabetes and is accompanied by insulin resistance and adipose tissue dysfunction. One of the most important factors implicitly linked to aging and age-related chronic diseases is the accumulation of oxidative stress. However, the effect of increased oxidative stress on adipose tissue biology remains elusive. In this study, we demonstrate that aging in mice results in a loss of fat mass and the accumulation of oxidative stress in adipose tissue. In vitro, increased oxidative stress through glutathione depletion inhibits preadipocyte differentiation. This inhibition of adipogenesis is at …


Role Of Antiarrhythmic Drugs: Frequent Implantable Cardioverter-Defibrillator Shocks, Risk Of Proarrhythmia, And New Drug Therapy., Christopher Droogan, Chinmay Patel, Gan-Xin Yan, Peter R Kowey 2011 Main Line Health Heart Center

Role Of Antiarrhythmic Drugs: Frequent Implantable Cardioverter-Defibrillator Shocks, Risk Of Proarrhythmia, And New Drug Therapy., Christopher Droogan, Chinmay Patel, Gan-Xin Yan, Peter R Kowey

Department of Medicine Faculty Papers

The implantable cardioverter-defibrillator (ICD) is the standard of care in patients with ischemic and nonischemic cardiomyopathy who are at high risk for arrhythmic events and sudden cardiac death. Although an ICD saves life, ICD shocks are emotionally and physically debilitating. Most patients receive adjuvant antiarrhythmic drug therapy to circumvent episodes of recurrent ventricular and supraventricular arrhythmias. Antiarrhythmic drugs including β-blockers, sotalol, amiodarone, and azimilide are effective at reducing the shock burden. This article describes data supporting the need for and potential risks and benefits of adjuvant antiarrhythmic drug therapy and examines the benefits and pitfalls of the same in ICD-implanted …


Partial Volume Correction Incorporating Rb-82 Positron Range For Quantitative Myocardial Perfusion Pet Based On Systolic-Diastolic Activity Ratios And Phantom Measurements, Nils P Johnson, Stefano Sdringola, K Lance Gould 2011 The Texas Medical Center Library

Partial Volume Correction Incorporating Rb-82 Positron Range For Quantitative Myocardial Perfusion Pet Based On Systolic-Diastolic Activity Ratios And Phantom Measurements, Nils P Johnson, Stefano Sdringola, K Lance Gould

Faculty, Staff and Student Publications

BACKGROUND: Quantitative myocardial PET perfusion imaging requires partial volume corrections.

METHODS: Patients underwent ECG-gated, rest-dipyridamole, myocardial perfusion PET using Rb-82 decay corrected in Bq/cc for diastolic, systolic, and combined whole cycle ungated images. Diastolic partial volume correction relative to systole was determined from the systolic/diastolic activity ratio, systolic partial volume correction from phantom dimensions comparable to systolic LV wall thicknesses and whole heart cycle partial volume correction for ungated images from fractional systolic-diastolic duration for systolic and diastolic partial volume corrections.

RESULTS: For 264 PET perfusion images from 159 patients (105 rest-stress image pairs, 54 individual rest or stress images), …


The Challenge Of Communicating Cardiovascular Risk Information To Our Patients., Samuel S Gidding 2011 Thomas Jefferson University

The Challenge Of Communicating Cardiovascular Risk Information To Our Patients., Samuel S Gidding

Department of Pediatrics Faculty Papers

Each of the major cardiovascular risk factors presents unique challenges to the clinician caring for children.  Dyslipidemia and hypertension are silent and require measurement; the challenge is first risk classification and then communicating strategies for treatment, with attendant cost, in the absence of perceptible disease morbidity.  Obesity and physical inactivity, while generally obvious to the patient, require change in lifestyle for successful management; the challenge is to convince the patient to make that change.  Diabetes mellitus is a significant disease requiring complex management strategies for the patient to execute; the challenge is to add cardiovascular risk reduction strategies to the …


Aneurysm Of Sinus Of Valsalva, Fateh Ali Tipoo Sultan, Nageeb Basir, Saulat H. Fatimi 2011 Aga Khan University

Aneurysm Of Sinus Of Valsalva, Fateh Ali Tipoo Sultan, Nageeb Basir, Saulat H. Fatimi

Section of Cardiology

Aneurysm of sinus of Valsalva is a rare cardiac abnormality with congenital origin in most of the cases. If it is located in the right coronary sinus, it usually ruptures into a right heart chamber and frequently a ventricular septal defect (VSD) coexists with this condition. Early diagnosis and immediate surgical treatment can save the patient’s life in most cases. All the 3 cases reported in this series had aneurysm of right sinus of Valsalva with associated VSD and mild degree of aortic regurgitation (AR). Two of the cases ruptured aneurysm into the right ventricle. Trans-esophageal echocardiography was used to …


Carotid Cavernous Fistula In A Patient With Coronary Artery Disease, Omer Ashraf, Syed Shahabuddin, Muhammad Ehsan Bari, Hasanat Sharif 2011 Aga Khan University

Carotid Cavernous Fistula In A Patient With Coronary Artery Disease, Omer Ashraf, Syed Shahabuddin, Muhammad Ehsan Bari, Hasanat Sharif

Section of Cardiology

Carotid cavernous fistulas are abnormal communications between the carotid and cavernous vasculature, with potential for serious neurological and ocular sequelae. There is considerable literature on the neuro-radiological management of carotico-cavernous fistula depending upon their flow status. The coronary artery bypass grafting (CABG) for coronary artery disease (CAD) is a well established method of revascularization. However, the association of carotid cavernous fistula in presence of CAD is an infrequent occurrence. We present a case of coronary artery disease scheduled for bypass surgery, developed spontaneous bilateral carotid cavernous fistulas, highlighting a unique aspect of association between coronary and carotid disease.


Cardiac Electrophysiology Studies And Ablations For Treatment Of Supraventricular Arrhythmias--An Initial Experience From Karachi, Azam Shafquat, Aamer Imdad, Salman Khalid, Syed Zahid Jamal 2011 National Institute of Cardiovascular Diseases

Cardiac Electrophysiology Studies And Ablations For Treatment Of Supraventricular Arrhythmias--An Initial Experience From Karachi, Azam Shafquat, Aamer Imdad, Salman Khalid, Syed Zahid Jamal

Section of Cardiology

OBJECTIVES: To review the clinical experience, complications and outcome of Cardiac Electrophysiology Studies and Catheter Radiofrequency Ablation in treatment of supraventricular tachycardias in Karachi.

METHODS: A retrospective review of records of all patients who underwent an electrophysiological study and radiofrequency ablation at National Institute of Cardiovascular Diseases and Aga Khan University Hospital from 2005 to 2007 was performed. Patient's demographics, disease and procedural data were collected and analyzed using SPSS.V.II.

RESULTS: We enrolled 168 patients, 150 (89%) from National Institute of Cardiovascular Diseases and 18 (11%) from Aga Khan University Hospital. Sample had 86 (51%) male participants. The mean age …


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