Outcome of Septal Myectomy in Patients With Fabry's Disease

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Outcome of Septal Myectomy in Patients With Fabry's Disease Meghana R. Kunkala, MD, Marie Christine Aubry, MD, Steve R. Ommen, MD, Bernard J. Gersh, MB, ChB, Hartzell V. Schaff, MD The Annals of Thoracic Surgery Volume 95, Issue 1, Pages 335-337 (January 2013) DOI: 10.1016/j.athoracsur.2012.05.087 Copyright © 2013 The Society of Thoracic Surgeons Terms and Conditions

Fig 1 Patient 1. (A, B) Preoperative echocardiograms demonstrating systolic anterior motion and hypertrophy of the left ventricle. (C, D) Echocardiograms after patient was weaned from bypass demonstrating myectomy changes and resolution of systolic anterior motion. The Annals of Thoracic Surgery 2013 95, 335-337DOI: (10.1016/j.athoracsur.2012.05.087) Copyright © 2013 The Society of Thoracic Surgeons Terms and Conditions

Fig 2 Patient 1. (A) Intraoperative photograph of excised specimen demonstrating endocardial scar. (B) High-power photomicrograph from a subaortic resection specimen shows diffusely enlarged myocytes with prominent central vacuolization of the cytoplasm (hematoxylin-eosin, original magnification ×400). The Annals of Thoracic Surgery 2013 95, 335-337DOI: (10.1016/j.athoracsur.2012.05.087) Copyright © 2013 The Society of Thoracic Surgeons Terms and Conditions

Fig 3 Patient 2. (A, B) Preoperative cardiac magnetic resonance image demonstrating extent of hypertrophy of the ventricle. (C) Transmission electron microscopy shows that the sarcoplasmic vacuoles contain myeloid figures (arrow) characterized by concentric laminated electron-dense material (original magnification ×11,000). The Annals of Thoracic Surgery 2013 95, 335-337DOI: (10.1016/j.athoracsur.2012.05.087) Copyright © 2013 The Society of Thoracic Surgeons Terms and Conditions