Right ventricular sarcoidosis: is it time for updated diagnostic criteria?

A 55-year-old woman with a history of complete heart block, atrial flutter, and progressive right ventricular failure was referred to our tertiary care center to be evaluated for cardiac transplantation. The patient's clinical course included worsening right ventricular dysfunction for 3 years...

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Veröffentlicht in:Texas Heart Institute journal 2014-04, Vol.41 (2), p.203-207
Hauptverfasser: Vakil, Kairav, Minami, Elina, Fishbein, Daniel P
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Minami, Elina
Fishbein, Daniel P
description A 55-year-old woman with a history of complete heart block, atrial flutter, and progressive right ventricular failure was referred to our tertiary care center to be evaluated for cardiac transplantation. The patient's clinical course included worsening right ventricular dysfunction for 3 years before the current evaluation. Our clinical findings raised concerns about arrhythmogenic right ventricular cardiomyopathy. Noninvasive imaging, including a positron emission tomographic scan, did not reveal obvious myocardial pathologic conditions. Given the end-stage nature of the patient's right ventricular failure and her dependence on inotropic agents, she underwent urgent listing and subsequent heart transplantation. Pathologic examination of the explanted heart revealed isolated right ventricular sarcoidosis with replacement fibrosis. Biopsy samples of the cardiac allograft 6 months after transplantation showed no recurrence of sarcoidosis. This atypical presentation of isolated cardiac sarcoidosis posed a considerable diagnostic challenge. In addition to discussing the patient's case, we review the relevant medical literature and discuss the need for updated differential diagnostic criteria for end-stage right ventricular failure that mimics arrhythmogenic right ventricular cardiomyopathy.
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The patient's clinical course included worsening right ventricular dysfunction for 3 years before the current evaluation. Our clinical findings raised concerns about arrhythmogenic right ventricular cardiomyopathy. Noninvasive imaging, including a positron emission tomographic scan, did not reveal obvious myocardial pathologic conditions. Given the end-stage nature of the patient's right ventricular failure and her dependence on inotropic agents, she underwent urgent listing and subsequent heart transplantation. Pathologic examination of the explanted heart revealed isolated right ventricular sarcoidosis with replacement fibrosis. Biopsy samples of the cardiac allograft 6 months after transplantation showed no recurrence of sarcoidosis. This atypical presentation of isolated cardiac sarcoidosis posed a considerable diagnostic challenge. 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The patient's clinical course included worsening right ventricular dysfunction for 3 years before the current evaluation. Our clinical findings raised concerns about arrhythmogenic right ventricular cardiomyopathy. Noninvasive imaging, including a positron emission tomographic scan, did not reveal obvious myocardial pathologic conditions. Given the end-stage nature of the patient's right ventricular failure and her dependence on inotropic agents, she underwent urgent listing and subsequent heart transplantation. Pathologic examination of the explanted heart revealed isolated right ventricular sarcoidosis with replacement fibrosis. Biopsy samples of the cardiac allograft 6 months after transplantation showed no recurrence of sarcoidosis. This atypical presentation of isolated cardiac sarcoidosis posed a considerable diagnostic challenge. 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subjects Arrhythmogenic Right Ventricular Dysplasia - diagnosis
Atrial Flutter - etiology
Atrioventricular Block - etiology
Cardiomyopathies - complications
Cardiomyopathies - diagnosis
Cardiomyopathies - physiopathology
Cardiomyopathies - surgery
Cardiotonic Agents - therapeutic use
Case Reports
Diagnosis, Differential
Disease Progression
Echocardiography - methods
Female
Heart Failure - etiology
Heart Transplantation - methods
Heart Ventricles - pathology
Humans
Middle Aged
Positron-Emission Tomography - methods
Sarcoidosis - complications
Sarcoidosis - diagnosis
Sarcoidosis - physiopathology
Sarcoidosis - surgery
Treatment Outcome
Ventricular Dysfunction, Right - diagnosis
Ventricular Dysfunction, Right - etiology
Ventricular Dysfunction, Right - physiopathology
Ventricular Dysfunction, Right - surgery
title Right ventricular sarcoidosis: is it time for updated diagnostic criteria?
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