Extracardiac Sarcoidosis Research Articles

Myocardial damage in patients with sarcoidosis and preserved left ventricular systolic function: an observational study

Late gadolinium enhanced cardiovascular magnetic resonance (LGE-CMR) is a valuable test to detect myocardial damage in patients with sarcoidosis; however, the clinical significance of LGE in sarcoidosis patients with preserved left ventricular ejection fraction (LVEF) is not defined. We aim to characterize the prevalence of LGE, its associated cardiac findings, and its clinical implications in sarcoidosis patients with preserved LVEF. One hundred and fifty-two patients with biopsy proven extra-cardiac sarcoidosis, no known cardiac sarcoidosis, and LVEF ≥ 50% referred for LGE-CMR were included in this observational study. The presence of LGE in the left ventricular myocardium was considered diagnostic for cardiac sarcoidosis. The cohort was divided into two groups based on the presence or absence of LGE. Twenty-nine patients (19%) had LGE involving 11 ± 9% of the left ventricle. The modified Japanese Ministry of Health and Welfare (JMHW) criteria for diagnosing cardiac sarcoidosis only had a sensitivity of 52% and specificity of 83% for identifying myocardial LGE in these patients. Compared with those patients without LGE, those with LGE had a higher heart rate (84 ± 19 vs. 76 ± 18 b.p.m., P= 0.002), greater prevalence of an abnormal electrocardiogram (76 vs. 31%, P< 0.001), diastolic dysfunction (67 vs. 33%, P= 0.05), reduced right ventricular ejection fraction (49 ± 8 vs. 55 ± 6%, P= 0.012), and evidence of non-sustained ventricular tachycardia (33 vs. 6%). In patients with sarcoidosis and preserved systolic function, myocardial damage is commonly present and may increase the risk of ventricular tachy-arrhythmias. The JMHW Criteria were neither sensitive nor specific for predicting the presence of myocardial LGE.

Abstract P360: Temporal Variability in the ECG Manifestations of Cardiac Sarcoidosis

BACKGROUND: Temporal variability in ECG manifestations of cardiac sarcoidosis in patients with documented extra-cardiac sarcoidosis has not been previously reported. METHODS: Six or more 12-lead ECGs were reviewed for each of 8 patients (6 females, 6 African Americans, 38-63 years old) with documented extra-cardiac sarcoidosis. Analyzed ECGs were obtained at intervals no longer than 12 months apart. PR and QT intervals and QRS duration were measured in each ECG lead and averaged for three consecutive beats per lead. The presence of PVCs, AV block, RBBB, and/or QT prolongation was recorded. Between ECG variability was analyzed with ANOVA for continuous data and chi-square analysis for categorical data. RESULTS: Significant variability in ECG indicators of sarcoidosis activity, concurrent with clinical impression of sarcoidosis exacerbation, was detected in seven of eight patients. Most common were new QT prolongation (94%, p=0.031) and PR interval increase (87.5% of the analyzed ECGs, p=0.029). Concordant variability was noted for both in between visit and in between lead comparisons of PR duration, QT/QTc intervals, and QRS duration (p&lt;0.0001 in 5 patients; p&lt;0.001 in 2 patients). No PVCs were documented on the analyzed ECGs. CONCLUSION: ECG abnormalities diagnostic of cardiac sarcoidosis have high temporal variability which parallels clinical impression of extra-cardiac disease activity. A 12-lead ECG is warranted at each visit in patients with documented extra-cardiac sarcoidosis. At the same time, 12-lead ECGs are probably not sufficiently sensitive to detect arrhythmias and/or PVCs in patients with documented extra-cardiac sarcoidosis.

Can late gadolinium enhancement on cardiovascular magnetic resonance describe cardiac involvement in patients with systemic sarcoidosis and/or suspect of cardiac involvement of sarcoidosis with cardiac symptoms?

Background Cardiac involvement of sarcoidosis (CIS) is clinically evident in 5% of patients with systemic sarcoidosis, but CIS are reported to be found at autopsy in 30% to 50%. Some of CIS represents remission and recurrence in clinical setting, and it is difficult to diagnose CIS in cured condition. Purpose We assess whether LGE on CMR reflect CIS in patients with extracardiac sarcoidosis or suspect of CIS due to cardiac symptoms. Methods Forty-three patients (male/female = 12/31, age 30-78 years) with rule out or suspect of CIS with extracardiac sarcoidosis and suspect of CIS without extracardiac sarcoidosis underwent CMR examination. We diagnosed CIS using Diagnostic Standard and Guideline for Sarcoidosis 2006 of the Japanese Society of Sarcoidosis and Other Granulomatous Disorders (2006 criteria) as a gold standard, and subjects were divided into CIS which including suspect of CIS (s/o CIS) and without CIS. We defined CIS as fully satisfied of 2006 criteria, and s/o CIS as follows; satisfied with 1 major and 1 minor of 2006 criteria of CIS with systemic sarcoidosis findings and satisfied with clinical diagnostic criteria of CIS but lack of systemic sarcoidosis findings. We performed visual segment analysis using standard AHA 17-segments model. The LGE were assessed in each segment, localization (5 patterns: endocardial, midwall, epicardial, transmural, and multiple), distribution (3 types: patchy, linear as within 50% of myocardial thickness and band-like as greater than 50% of myocardial thickness), and signal intensity (2 types; lightly, definitively).

Dilated Cardiomyopathy With Conduction Disease and Arrhythmia

case presentation: A 48-year-old woman presents with exertional dyspnea and recurrent syncope. One year earlier, a permanent pacemaker was placed after she complained of fatigue and was found to have high-grade atrioventricular block. Now, she has echocardiographic evidence of moderate to severe left ventricular (LV) systolic dysfunction with regional wall-motion abnormalities. Nuclear imaging is notable for heterogeneous myocardial uptake of technetium Tc99m sestamibi, and coronary angiography reveals widely patent epicardial vessels. Multiple episodes of nonsustained ventricular tachycardia (VT) are documented on continuous ECG monitoring. What are the diagnostic considerations for this patient, and what further evaluations are indicated? This patient presents with dilated cardiomyopathy (DCM) with electric instability (DCM+E), which we define as conduction disease and arrhythmia out of proportion to the severity of LV systolic dysfunction. Diverse causes can result in DCM+E and fall into general categories of inflammatory, infectious, hereditary, and infiltrative processes. Cardiac presentation associated with these conditions is distinct from more common causes of DCM such as ischemic heart disease, viral myocarditis, valvular dysfunction, pregnancy, or substance abuse. Clinical features that are suggestive of DCM+E include supraventricular arrhythmias or conduction disease that precedes cardiomyopathy, multiple VT morphologies, and features suggestive of ischemic heart disease (Q waves, regional wall-motion abnormalities, perfusion defects, ventricular aneurysm) in the absence of epicardial stenoses. In this Clinician Update, we focus on the diagnostic approach to patients with DCM+E. Emphasis is placed on diagnoses that are relatively common or for which the clinical management would be impacted significantly by recognition of the underlying cause. Ischemic heart disease may present with conduction disease and a high burden of arrhythmia, especially in the setting of acute myocardial ischemia/infarction. The exclusion of obstructive coronary artery disease is strongly recommended in patients with DCM+E because atherosclerosis is so prevalent, evidence-based treatment is readily available, and the …

Sarcoid Myocarditis With Ventricular Tachycardia Mimicking ARVD/C

Cardiac sarcoidosis (CS) is a multisystem granulomatous disorder of unknown etiology with frequent cardiac involvement. We describe a patient presenting with a ventricular tachycardia, presumably originating in the right ventricle (RV). This patient had a malignant clinical course with initial diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C); however, at postmortem histopathology revealed epithelioid granulomas with fibrosis localized in the interventricular septum, typical for sarcoidosis, without signs of extracardiac sarcoidosis. In conclusion, sarcoid myocarditis may present with signs and symptoms of ARVD/C and only histopathology can differentiate the 2 diseases. In the cases of atypical clinical presentation or when histopathological proof of ARVD is absent, a close follow-up is advisable to identify other potentially treatable disorders.

Detection of Myocardial Damage in Patients With Sarcoidosis

In patients with sarcoidosis, sudden death is a leading cause of mortality, which may represent unrecognized cardiac involvement. Delayed-enhancement cardiovascular magnetic resonance (DE-CMR) can detect minute amounts of myocardial damage. We sought to compare DE-CMR with standard clinical evaluation for the identification of cardiac involvement. Eighty-one consecutive patients with biopsy-proven extracardiac sarcoidosis were prospectively recruited for a parallel and masked comparison of cardiac involvement between (1) DE-CMR and (2) standard clinical evaluation with the use of consensus criteria (modified Japanese Ministry of Health [JMH] guidelines). Standard evaluation included 12-lead ECG and at least 1 dedicated non-CMR cardiac study (echocardiography, radionuclide scintigraphy, or cardiac catheterization). Patients were followed for 21+/-8 months for major adverse events (death, defibrillator shock, or pacemaker requirement). Patients were predominantly middle-aged (46+/-11 years), female (62%), and black (73%) and had chronic sarcoidosis (median, 7 years) and preserved left ventricular ejection fraction (median, 56%). DE-CMR identified cardiac involvement in 21 patients (26%) and JMH criteria in 10 (12%, 8 overlapping), a >2-fold higher rate for DE-CMR (P=0.005). All patients with myocardial damage on DE-CMR had coronary disease excluded by x-ray angiography. Pathology evaluation in 15 patients (19%) identified 4 with cardiac sarcoidosis; all 4 were positive by DE-CMR, whereas 2 were JMH positive. On follow-up, 8 had adverse events, including 5 cardiac deaths. Patients with myocardial damage on DE-CMR had a 9-fold higher rate of adverse events and an 11.5-fold higher rate of cardiac death than patients without damage. In patients with sarcoidosis, DE-CMR is more than twice as sensitive for cardiac involvement as current consensus criteria. Myocardial damage detected by DE-CMR appears to be associated with future adverse events including cardiac death, but events were few, and this needs confirmation in a larger cohort.

The Work‐Up and Management of Patients with Apparent or Subclinical Cardiac Sarcoidosis: With Emphasis on the Associated Heart Rhythm Abnormalities

In patients with newly diagnosed AV block and/or ventricular tachycardia, cardiac sarcoidosis should always be considered in the differential diagnosis. In addition to the pacemaker implant, cardiac resynchronization therapy (CRT) should be selected for severe heart failure patients who have class III or IV heart failure, LVEF <or=35%, and a complete LBBB pattern. If the disease activity is high, corticosteroid therapy is recommended. Patients with extracardiac sarcoidosis need to be closely followed for potential cardiac involvement, as the mortality in sarcoidosis depends on cardiac involvement. Early diagnosis and treatment of cardiac sarcoidosis is essential. Positron emission tomography (PET) and cardiac magnetic resonance imaging (MRI) are considered to have high sensitivity for cardiac involvement, and are the preferred imaging modalities. However, even in the era of new technology, such as PET and cardiac MRI, early diagnosis of cardiac sarcoidosis is still difficult.