Isolated Severe Right Ventricular Hypertrophic Cardiomyopathy

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Presentation transcript:

Isolated Severe Right Ventricular Hypertrophic Cardiomyopathy Meng Li, MD, Li Zhang, MD, PhD, Ziming Zhang, MD, Shuyuan Wang, MD, Nianguo Dong, MD, PhD, Guohua Wang, MD, PhD, Mingxing Xie, MD, PhD The Annals of Thoracic Surgery Volume 107, Issue 1, Pages e23-e25 (January 2019) DOI: 10.1016/j.athoracsur.2018.05.061 Copyright © 2019 The Society of Thoracic Surgeons Terms and Conditions

Fig 1 (A) Twelve-lead electrocardiography revealed sinus rhythm, suspicious right ventricular wall hypertrophy, abnormal Q waves (II, III, aVF, V6), and ST-T segment changes. (B) Posteroanterior and (C) laterolateral chest roentgenographic projections revealed that the border of the heart was greatly enlarged, and the esophagus was obviously pressed. The Annals of Thoracic Surgery 2019 107, e23-e25DOI: (10.1016/j.athoracsur.2018.05.061) Copyright © 2019 The Society of Thoracic Surgeons Terms and Conditions

Fig 2 (A) Transthoracic echocardiography revealed massive pericardial effusion (PCE) and cardiac tamponade. Transthoracic echocardiography 2 weeks after emergency pericardial puncture. (B) Apical four-chamber view, right ventricular (RV) wall asymmetric hypertrophy; the middle to apical two-thirds RV cavities were occluded. The whole occluded RV cavity was 4.0 cm at end-diastole (red double-arrowhead line). (C, D) Left-sided contrast echocardiography outlined the endocardium more clearly. (E, F) Cardiac magnetic resonance confirmed severe RV wall hypertrophy. (G) Cardiac magnetic resonance late gadolinium enhancement, in the four-chamber view, the hypertrophic RV wall showed central piebald delayed enhancement (white arrow), whereas the left ventricular wall showed no obvious abnormality. The whole occluded cavity thickness was approximately 4.3 cm at end-diastole (red double-arrowhead line). (AAo = ascending aorta; CS = coronary sinus; LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle.) The Annals of Thoracic Surgery 2019 107, e23-e25DOI: (10.1016/j.athoracsur.2018.05.061) Copyright © 2019 The Society of Thoracic Surgeons Terms and Conditions

Fig 3 (A) Right ventricular wall hypertrophy was visualized during the operation. (B) The left ventricle (LV) showed no obvious abnormality. (C, D) The pathologic diagnosis showed that the size of the nuclei was nonuniform, with many larger and deep-dyeing nuclei (arrow); the right ventricular myofibrils were arranged irregularly, and some of the myofibrils were hypertrophic. (RV = right ventricle.) The Annals of Thoracic Surgery 2019 107, e23-e25DOI: (10.1016/j.athoracsur.2018.05.061) Copyright © 2019 The Society of Thoracic Surgeons Terms and Conditions