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7. Myosin Heavy Chain Converter Domain Mutations Drive Early-Stage Changes in Extracellular Matrix Dynamics in Hypertrophic Cardiomyopathy

Author

Hsieh, Jeanne, primary, Becklin, Kelsie L., additional, Givens, Sophie, additional, Komosa, Elizabeth R., additional, Lloréns, Juan E. Abrahante, additional, Kamdar, Forum, additional, Moriarity, Branden S., additional, Webber, Beau R., additional, Singh, Bhairab N., additional, and Ogle, Brenda M., additional

Published
2022
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14. Stem Cell–Derived Cardiomyocytes and Beta-Adrenergic Receptor Blockade in Duchenne Muscular Dystrophy Cardiomyopathy

Author

Kamdar, Forum, primary, Das, Satyabrata, additional, Gong, Wuming, additional, Klaassen Kamdar, André, additional, Meyers, Tatyana A., additional, Shah, Pruthvi, additional, Ervasti, James M., additional, Townsend, DeWayne, additional, Kamp, Timothy J., additional, Wu, Joseph C., additional, Garry, Mary G., additional, Zhang, Jianyi, additional, and Garry, Daniel J., additional

Published
2020
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15. rare case report of severe cardiomyopathy associated with myotonic dystrophy type 2.

Author

Touma, Anja M, Nijjar, Prabhjot S, Manousakis, Georgios E, and Kamdar, Forum

Subjects
MYOTONIA atrophica, CARDIAC magnetic resonance imaging, CARDIOMYOPATHIES, MUSCLE weakness, CARDIOLOGICAL manifestations of general diseases, CORNEAL dystrophies
Abstract

Background Myotonic dystrophies (DM) are multi-systemic diseases characterized by muscle weakness and myotonia. Despite a growing appreciation for the cardiovascular manifestations in myotonic dystrophy type 1 (DM1), cardiac involvement in myotonic dystrophy type 2 (DM2) has been less well characterized. In patients with DM2, cardiomyopathy has rarely been described. Case summary This case report describes a rare case of DM2 associated cardiomyopathy. A 56-year-old male with DM2 who presented with palpitations and fatigue. Cardiac magnetic resonance (CMR) imaging confirmed a severely enlarged left ventricular cavity with a left ventricular ejection fraction of 28% consistent with severely reduced global systolic function. The lateral wall epicardium exhibited late gadolinium enhancement in a pattern seen in myotonic dystrophy-related cardiomyopathy. Discussion This case highlights the potential for significant cardiovascular involvement in DM2, as well as the importance of screening, including CMR imaging, and therapy in the myotonic dystrophy patient population. [ABSTRACT FROM AUTHOR]

Published
2022
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20. Abstract 19236: Effect of Continuous Flow Left Ventricular Assist Device on Diastolic Pulmonary Artery Pressure-to-Pulmonary Capillary Wedge Pressure Gradient in End Stage Heart Failure Patients with Pulmonary Hypertension

Author

Thenappan, Thenappan, primary, Cogswell, Rebecca, additional, Kamdar, Forum, additional, Holley, Christopher, additional, Harvey, Laura, additional, Colvin-Adams, Monica, additional, Eckman, Peter, additional, Adatya, Sirtaz, additional, John, Ranjit, additional, Liao, Ken, additional, and Pritzker, Marc R, additional

Published
2014
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24. Stenting a Moving Target.

Author

KAMDAR, FORUM, PERA, VIJAY, and VALETI, UMA

Subjects
CARDIOPULMONARY resuscitation, CARDIOGENIC shock, TREATMENT of acute coronary syndrome, CARDIAC arrest, THERAPEUTICS, CATHETERIZATION
Abstract

Despite significant advances in the science of resuscitation, survival to discharge after an in-hospital cardiac arrest in the catheterization laboratory remains poor. Clinicians face the challenges of performing CPR during procedures to address the cause of the arrest and the limitations of prolonged manual CPR. In this article we describe the first case of a patient presenting in cardiogenic shock caused by acute coronary syndrome secondary to bypass graft failure who developed cardiac arrest and survived 80 minutes of resuscitation in the catheterization lab, allowing for revascularization of a vein graft. The patient experienced complete neurological and hemodynamic recovery. This case demonstrates the importance of prompt high-quality, uninterrupted CPR using an automated chest compression device to facilitate early emergent revascularization of a vein graft. [ABSTRACT FROM AUTHOR]

Published
2015

25. Abstract 17175: Cardioprotective Effects of Beta Blockers in Duchenne Muscular Dystophy

Author

Das, Satyabrata, Kamdar, Forum, Gong, Wuming, Klaassen Kamdar, Andre, Meyers, Tatyana, Townsend, Dewayne, Ervasti, James M, Kamp, Timothy J, Wu, Joseph C, Zhang, Jianyi, and Garry, Daniel J

Abstract

Background:Cardiomyopathy has emerged as a leading cause of death in DMD although limited therapies have been developed for dystrophic heart failure.Methods:We used DMD patient-specific hiPSC-derived cardiomyocytes as a model to assess physiological responses to adrenergic agonist and antagonist treatments. These agents were further examined in vivousing wildtype and mdxmouse models.Results:DMD hiPSC-derived cardiomyocytes displayed a significant increase in arrhythmic calcium traces, both at baseline and in response to adrenergic stimulation, when compared to isogenic controls. These arrhythmias were significantly decreased with the addition of the beta-blocker propranolol. Using telemetric monitoring, we observed that mdxmice experienced arrhythmic death in response to isoproterenol stimulation and that these lethal arrhythmias were rescued, in part, by propranolol pretreatment. Using single cell and bulk RNA-Seq in DMD derived hiPSCs and mdx mice, we have identified important pathways that are perturbed under baseline and evoked conditions and normalized following the delivery of propranolol. We further determined that relatively few DMD patients see a cardiovascular specialist or receive ?-blocker therapy.Conclusions:In these studies, we have identified important mechanisms and potential therapeutic targets in the dystrophic heart. Importantly, these results may serve as a prelude for clinical studies that examine the impact of ?-blocker therapy in patients with dystrophinopathies.

Published
2019
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26. A rare case report of severe cardiomyopathy associated with myotonic dystrophy type 2.

Author

Touma AM, Nijjar PS, Manousakis GE, and Kamdar F

Abstract

Background: Myotonic dystrophies (DM) are multi-systemic diseases characterized by muscle weakness and myotonia. Despite a growing appreciation for the cardiovascular manifestations in myotonic dystrophy type 1 (DM1), cardiac involvement in myotonic dystrophy type 2 (DM2) has been less well characterized. In patients with DM2, cardiomyopathy has rarely been described., Case Summary: This case report describes a rare case of DM2 associated cardiomyopathy. A 56-year-old male with DM2 who presented with palpitations and fatigue. Cardiac magnetic resonance (CMR) imaging confirmed a severely enlarged left ventricular cavity with a left ventricular ejection fraction of 28% consistent with severely reduced global systolic function. The lateral wall epicardium exhibited late gadolinium enhancement in a pattern seen in myotonic dystrophy-related cardiomyopathy., Discussion: This case highlights the potential for significant cardiovascular involvement in DM2, as well as the importance of screening, including CMR imaging, and therapy in the myotonic dystrophy patient population., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Published
2022
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