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Characterization Of Cardiomyopathy In A Mouse Model Of Duchenne Muscular Dystrophy (Dmd) Using Echocardiography, Dce-Ct, And Pet-Fdg, Seyed Hamed Moazami

Electronic Thesis and Dissertation Repository

Duchenne muscular dystrophy (DMD) is an X-linked recessive neuromuscular disease that is the result of a loss of functional dystrophin, which causes cardiomyocyte fibrosis and death, leading to cardiomyopathy. In this thesis, I have utilized dynamic contrast-enhanced computed tomography (DCE-CT), positron emission tomography-fluorodeoxyglucose (PET-FDG), echocardiography, and traditional histology to longitudinally assess disease progression and degree of cardiomyopathy in a murine model of DMD (mdx:utrn-/-). No significant changes were observed in the blood flow, blood volume, or cardiac volume measured via DCE-CT, nor in standard uptake value (SUV) of glucose as measured by PET-FDG in the left myocardium between and within …

Coronary Artery Remodeling In A Model Of Left Ventricular Pressure Overload Is Influenced By Platelets And Inflammatory Cells, Fanmuyi Yang, Anping Dong, Paul Mueller, Jessica Caicedo, Alyssa Moore Sutton, Juliana Odetunde, Cordelia J. Barrick, Yuri M. Klyachkin, Ahmed Abdel-Latif, Susan S. Smyth

Internal Medicine Faculty Publications

Left ventricular hypertrophy (LVH) is usually accompanied by intensive interstitial and perivascular fibrosis, which may contribute to arrhythmogenic sudden cardiac death. The mechanisms underlying the development of cardiac fibrosis are incompletely understood. To investigate the role of perivascular inflammation in coronary artery remodeling and cardiac fibrosis during hypertrophic ventricular remodeling, we used a well-established mouse model of LVH (transverse aortic constriction [TAC]). Three days after pressure overload, macrophages and T lymphocytes accumulated around and along left coronary arteries in association with luminal platelet deposition. Consistent with these histological findings, cardiac expression of IL-10 was upregulated and in the systemic circulation, …

The Ventricular Volume Variability Study Of The Pediatric Heart Network: Study Design And Impact Of Beat Averaging And Variable Type On The Reproducibility Of Echocardiographic Measurements In Children With Chronic Dilated Cardiomyopathy., Steven D. Colan, Girish S. Shirali, Renee Margossian, Dianne Gallagher, Karen Altmann, Charles Canter, Shan Chen, Fraser Golding, Elizabeth Radojewski, Michael Camitta, Michael Carboni, Jack Rychik, Mario Stylianou, Lloyd Y. Tani, Elif Seda Selamet Tierney, Yanli Wang, Lynn A. Sleeper, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Clinical trials often rely on echocardiographic measures of left ventricular size and function as surrogate end points. However, the quantitative impact of factors that affect the reproducibility of these measures is unknown. To address this issue, the National Heart, Lung, and Blood Institute-funded Pediatric Heart Network designed a longitudinal observational study of children with known or suspected dilated cardiomyopathy aged 0 to 22 years from eight pediatric clinical centers.

METHODS: Clinical data were collected together with 150 echocardiographic indices of left ventricular size and function. Separate observers performed duplicate echocardiographic imaging. Multiple observers performed measurements from three cardiac cycles …

Does Initial Shunt Type For The Norwood Procedure Affect Echocardiographic Measures Of Cardiac Size And Function During Infancy?: The Single Vventricle Reconstruction Trial., Peter C. Frommelt, Lin T. Guey, L Luann Minich, Majeed Bhat, Tim J. Bradley, Steve D. Colan, Greg Ensing, Jessica Gorentz, Haleh Heydarian, J Blaine John, Wyman W. Lai, Jami C. Levine, William T. Mahle, Stephen G. Miller, Richard G. Ohye, Gail D. Pearson, Girish S. Shirali, Pierre C. Wong, Meryl S. Cohen, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The Pediatric Heart Network trial comparing outcomes in 549 infants with single right ventricle undergoing a Norwood procedure randomized to modified Blalock-Taussig shunt or right ventricle-pulmonary artery shunt (RVPAS) found better 1-year transplant-free survival in those who received RVPAS. We sought to compare the impact of shunt type on echocardiographic indices of cardiac size and function up to 14 months of age.

METHODS AND RESULTS: A core laboratory measured indices of cardiac size and function from protocol exams: early after Norwood procedure (age 22.5 ± 13.4 days), before stage II procedure (age 4.8 ± 1.8 months), and at 14 …

Thickened Ascending Aortic Wall Mimicking Intramural Hematoma., Kentaro Yamane, Hitoshi Hirose, Benjamin A. Youdelman, James Diehl

Department of Surgery Faculty Papers

A 45-year-old Hispanic woman presented with a 3-day history of ‘‘burning’’ chest pain. A computed tomo- graphic angiogram of the chest revealed the ascending aorta had a maximum diameter of 40 mm with marked thickening of the aortic wall (Figure 1), which we con- cluded was an intramural hematoma. On entering the pericardium, a milky-white plaque-like area on the ascending aorta was encountered (Figure 2). The ascending aorta was firm to palpation. Intraoperative transesophageal echocardiography and epiaortic ultra- sound showed a hyperechoic aortic wall with no find- ings compatible with aortic dissection. The ascending aorta had an irregular surface contour, …

Effect Of Obstructive Sleep Apnea On Mitral Valve Tenting., Gregg S. Pressman, Vincent M. Figueredo, Abel Romero-Corral, Ganesan Murali, Morris N Kotler

Division of Cardiology Faculty Papers

Obstructive apneas produce high negative intrathoracic pressure which imposes an afterload burden on the left ventricle. Such episodes might produce structural changes in the left ventricle over time. Doppler echocardiograms were obtained within 2 months of attended polysomnography. Patients were grouped according to apnea-hypopnea index (AHI): mild/no OSA (AHI < 15) and mod/severe OSA (AHI ≥ 15). Mitral valve tenting height and area, left ventricular (LV) long and short axis, and LV end-diastolic volume (LVEDV), were measured along with tissue Doppler parameters. Comparisons of measurements at baseline and follow up between and within groups were obtained; correlations between absolute changes (deltas) in echocardiographic parameters were also performed. After a mean follow up of 240 days mitral valve tenting height increased significantly (1.17 ± 0.12 cm to 1.28 ± 0.17 cm, p=0.001) in mod/severe OSA as did tenting area (2.30 ± 0.41 cm² to 2.66 ± 0.60 cm², p=0.0002); delta tenting height correlated with delta LVEDV (rho 0.43, p=0.01) and delta tenting area (rho 0.35, p=0.04). In mild/no OSA patients there was no significant change in tenting height; there was a borderline significant increase in tenting area (2.20 ± 0.44 cm …

Left Atrial Volume: Comparison Of 2d And 3d Transthoracic Echocardiography With Ecg-Gated Ct Angiography., Anish R Koka, Stuart D Gould, Alyson N Owen, Ethan J. Halpern

Department of Radiology Faculty Papers

RATIONALE AND OBJECTIVES: Left atrial volume (LAV) measurement by conventional two-dimensional (2D) transthoracic echocardiography (TTE) may be limited by the geometric model, by suboptimal definition of left atrial endocardium, or by chamber foreshortening. Three-dimensional (3D) TTE is posited to eliminate chamber foreshortening, and LAV measurement by 3D TTE should be more reflective of true LAV. The aim of this study was to compare conventional 2D TTE and newer 3D TTE for measurements of LAV to multidetector computed tomographic (MDCT) measurements using automated chamber reconstruction (ACR).

MATERIALS AND METHODS: Twenty-two subjects consented to undergo 2D TTE and 3D TTE immediately prior …

Coronary Arteriovenous Fistulae: The Complexity Of Coronary Artery-To-Coronary Sinus Connections, Todd L Kiefer, Anna Lisa Crowley, James Jaggers, J Kevin Harrison

The Texas Heart Institute Journal

Coronary artery fistulae are a rare cardiovascular anomaly. Even less common are multiple fistulae involving more than 1 coronary artery. Herein we report the case of a 47-year-old woman who had fistulae from both the right coronary and left circumflex coronary arteries draining into the coronary sinus. These lesions we attempted to close percutaneously but subsequently closed surgically. We discuss diagnostic imaging approaches, along with closure indications, closure options, and outcomes.

Cellular Retrograde Cardiomyoplasty And Relaxin Therapy For Postischemic Myocardial Repair In A Rat Model, Gabriella Di Lascio, Guy Harmelin, Mattia Targetti, Cristina Nanni, Giacomo Bianchi, Tommaso Gasbarri, Sandro Gelsomino, Daniele Bani, Sandra Zecchi Orlandini, Massimo Bonacchi

The Texas Heart Institute Journal

We sought to determine whether skeletal myoblasts, wild-type or engineered to express relaxin, might improve myocardial viability and performance in a rat model of chronic myocardial infarction. Our purpose was to investigate a potential new therapy for heart failure.

From October 2005 through September 2009, we surgically induced acute myocardial infarction in 80 male Wistar rats. Thirty days after surgery, the rats underwent reoperation for the retrograde coronary venous infusion of skeletal myoblasts, relaxin, or both. The animals were randomly assigned to 4 experimental groups: R1 (the control group, which underwent saline-solution infusion), R2 (systemic relaxin therapy), R3 (myoblast infusion), …

Intramyocardial Hematoma From Blunt Trauma Mimicking Apical Hypertrophic Cardiomyopathy, Meta Linda Mobula, Sammy Zakaria, Glenn A Hirsch

The Texas Heart Institute Journal

No abstract provided.

Contegra Valved Conduit In The Paediatric Population: An Exciting Prospect For Right Ventricle To Pulmonary Artery Reconstruction; Experience And Outcomes At Aga Khan University, Maria Tariq Siddiqui, Asif Hasan, Shazia Mohsin, Mohammad Hamid, Muneer Amanullah

Department of Anaesthesia

Objective: The focus of this study is to share the experience and outcomes of Contegra graft implantation in the paediatric and adult population in Pakistan. Methods: Between May 2007 and July 2011, 16 patients, underwent implantation of a Contegra valved conduit. All operations were performed through a median sternotomy with cardiopulmonary bypass. Indications included: Pulmonary atresia with ventricular septal defect (n=11), Tetralogy of Fallot with absent Pulmonary Valve (PV) syndrome (n=2), double outlet right ventricle, transposition of great arteries and pulmonary stenosis (n=1), isolated aortic valve disease (n=1) and a pseudo-aneurysm with infective endocarditis (n=1).Conduit sizes varied between 16-22 mm. …