Prospective Study of Cardiac Sarcoid Mimicking Arrhythmogenic Right Ventricular Dysplasia

Vasaiwala, Smit; Finn, R N Cynthia; Delpriore, R N Jeanne; Leya, Fred; Gagermeier, James; Akar, Joseph G.; Santucci, Peter; Dajani, Khaled A.; Bova, Davide; Picken, Maria M.; Basso, Cristina; Marcus, Frank I.; Wilber, David J.

doi:10.1111/j.1540-8167.2008.01351.x

Cited by 127 publications

(80 citation statements)

References 30 publications

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“…Clinical features and electro- cardiographic findings are also similar to those in ARVC. Vasaiwala et al (45) showed that 15% of a group of 20 patients who were initially diagnosed with ARVC had sarcoidosis at endomyocardial biopsy. None of these patients had radiographic evidence of sarcoidosis.…”

Section: Pathologic Conditions That Mimic Findings In Arvc Sarcoidosismentioning

confidence: 99%

Cardiac MR Findings and Potential Diagnostic Pitfalls in Patients Evaluated for Arrhythmogenic Right Ventricular Cardiomyopathy

Rastegar¹,

Burt

Corona‐Villalobos

et al. 2014

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Section: Pathologic Conditions That Mimic Findings In Arvc Sarcoidosismentioning

confidence: 99%

Cardiac MR Findings and Potential Diagnostic Pitfalls in Patients Evaluated for Arrhythmogenic Right Ventricular Cardiomyopathy

Rastegar¹,

Burt

Corona‐Villalobos

et al. 2014

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“…Nearly all patients have predominant LV disease. However, since 2003, 16 cases of RV sarcoidosis mimicking ARVC have been described (Table I), [1][2][3][4][5][6][7][8][9][10][11][12] and only 2 patients had isolated RV involvement. 1,6 Our patient had a similar uncommon presentation of cardiac sarcoidosis with isolated RV involvement and LV-sparing that clinically mimicked ARVC.…”

Section: Discussionmentioning

confidence: 99%

Right Ventricular Sarcoidosis: Is It Time for Updated Diagnostic Criteria?

Vakil

Minami

Fishbein

2014

Texas Heart Institute Journal

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C ardiac involvement in systemic sarcoidosis has been reported frequently. Nearly all patients with cardiac sarcoidosis have left ventricular (LV) involvement, and most have predominant LV disease. Few prior reports have highlighted the atypical presentation of cardiac sarcoidosis when it clinically presents as arrhythmogenic right ventricular cardiomyopathy (ARVC). Isolated right ventricular (RV) involvement in cardiac sarcoidosis has rarely been reported. We report the case of a patient who presented with end-stage RV failure from isolated RV sarcoidosis and eventually needed cardiac transplantation. Given the inaccuracy of the currently available invasive and noninvasive tests in detecting RV sarcoidosis, such atypical presentations pose a considerable diagnostic challenge. In this report, we review the medical literature in regard to patients who have presented with predominant RV sarcoidosis, and also summarize the diagnostic approach that could be undertaken for earlier detection of this disease. Case ReportA 55-year-old white woman was referred for cardiac-transplant evaluation because of end-stage RV failure. Three years earlier, she had presented with syncope and complete heart block. A 2-dimensional (2D) echocardiogram at that time revealed mildly reduced RV systolic function and borderline LV systolic function (ejection fraction, 0.50-0.55). An electrocardiogram showed complete heart block with atrioventricular (AV) dissociation and a ventricular escape rhythm of 30 beats/min. Coronary angiograms revealed normal coronary arteries. No apparent metabolic or infectious causes explained her signs and symptoms. The diagnosis was idiopathic high-grade AV block in the presence of nonischemic cardiomyopathy, and a permanent pacemaker was implanted.The patient's only other medical issue was controlled hypertension, treated with lisinopril. Her family medical history was not pertinent. Because of the new diagnosis of borderline low LV ejection fraction, carvedilol therapy was initiated. She did well for 2 years with New York Heart Association (NYHA) functional class II symptoms and then presented with atrial flutter. She underwent successful ablation with an uneventful postprocedural course. During the next 8 months, she exhibited substantial clinical

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“…Diagnosis is confirmed by EMB and thoracic CT scans. 151 DCM is particularly difficult to distinguish from non-classic forms of ACM, and these two entities can significantly overlap and harbour similar mutations. Palpitations, (pre)syncope and ventricular arrhythmias are present at an early stage in ACM, often in the absence of gross structural abnormalities, which is the opposite in DCM.…”

Section: Differential Diagnosismentioning

confidence: 99%

“…In a study of patients with suspected ARVC/D also being evaluated by EMB, a high incidence (15 %) of cardiac sarcoid was identified. 151 Cardiac sarcoid should be considered if respiratory and systemic symptoms, high-grade atrioventricular conduction block and septal involvement are present, and familial disease is absent. Diagnosis is confirmed by EMB and thoracic CT scans.…”

Section: Differential Diagnosismentioning

confidence: 99%

Arrhythmogenic Cardiomyopathy: Electrical and Structural Phenotypes

Akdiş

Brunckhorst

Duru

et al. 2016

Arrhythmia &Amp; Electrophysiology Review

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This overview gives an update on the molecular mechanisms, clinical manifestations, diagnosis and therapy of arrhythmogenic cardiomyopathy (ACM). ACM is mostly hereditary and associated with mutations in genes encoding proteins of the intercalated disc. Three subtypes have been proposed: the classical right-dominant subtype generally referred to as ARVC/D, biventricular forms with early biventricular involvement and left-dominant subtypes with predominant LV involvement. Typical symptoms include palpitations, arrhythmic (pre)syncope and sudden cardiac arrest due to ventricular arrhythmias, which typically occur in athletes. At later stages, heart failure may occur. Diagnosis is established with the 2010 Task Force Criteria (TFC). Modern imaging tools are crucial for ACM diagnosis, including both echocardiography and cardiac magnetic resonance imaging for detecting functional and structural alternations. Of note, structural findings often become visible after electrical alterations, such as premature ventricular beats, ventricular fibrillation (VF) and ventricular tachycardia (VT). 12-lead ECG is important to assess for depolarisation and repolarisation abnormalities, including T-wave inversions as the most common ECG abnormality. Family history and the detection of causative mutations, mostly affecting the desmosome, have been incorporated in the TFC, and stress the importance of cascade family screening. Differential diagnoses include idiopathic right ventricular outflow tract (RVOT) VT, sarcoidosis, congenital heart disease, myocarditis, dilated cardiomyopathy, athlete’s heart, Brugada syndrome and RV infarction. Therapeutic strategies include restriction from endurance and competitive sports, β-blockers, antiarrhythmic drugs, heart failure medication, implantable cardioverter-defibrillators and endocardial/epicardial catheter ablation.

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Prospective Study of Cardiac Sarcoid Mimicking Arrhythmogenic Right Ventricular Dysplasia

Cited by 127 publications

References 30 publications

Cardiac MR Findings and Potential Diagnostic Pitfalls in Patients Evaluated for Arrhythmogenic Right Ventricular Cardiomyopathy

Cardiac MR Findings and Potential Diagnostic Pitfalls in Patients Evaluated for Arrhythmogenic Right Ventricular Cardiomyopathy

Right Ventricular Sarcoidosis: Is It Time for Updated Diagnostic Criteria?

Arrhythmogenic Cardiomyopathy: Electrical and Structural Phenotypes

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