Systemic Sarcoidosis and Electrocardiographic Conduction Abnormalities: Electrophysiologic Evaluation of Two Patients

doi:10.1378/chest.78.4.601

Chest

Volume 78, Issue 4, October 1980, Pages 601-604

https://doi.org/10.1378/chest.78.4.601 Get rights and content

Two patients with long histories of sarcoidosis had progression of conduction abnormalities to heart block and severe bradycardia. Conduction system involvement was trifascicular in both patients, though evidence for left ventricular functional impairment was otherwise lacking. Sudden death is seen more commonly in patients with sarcoidosis who have diffuse myocardial involvement, while conduction abnormalities can occur with relatively localized disease.

Section snippets

Case 1

A 46-year-old black man was noted to have paratracheal adenopathy on chest radiograph in 1964. A scalene node biopsy revealed noncaseating granulomas consistent with sarcoidosis. Results of culture for tuberculosis and fungi were negative. Repeated electrocardiograms were normal. The patient remained asymptomatic on no medications until March, 1977, when he noted a 14-pound weight loss. An electrocardiogram revealed a prolonged P-R interval, left anterior fascicular block and right bundle

Discussion

It is difficult to predict which patients with sarcoidosis will develop rhythm disturbances. The two patients reported were followed-up since 1964 and 1973 with gradual progression of conduction block. Neither patient had syncope, and both patients were only mildly symptomatic. Neither patient had clinical evidence of ischemic heart disease. The rather benign clinical course may have been due to the good left ventricular function in both patients. Even with profound bradycardia, their only

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Cardiac sarcoid: A clinicopathologic study of 84 unselected patients with systemic sarcoidosis
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Cited by (12)

Electrocardiographic abnormalities and risk of developing cardiac events in extracardiac sarcoidosis
2015, International Journal of Cardiology
Citation Excerpt :
However, it is difficult to use these imaging modalities for primary routine screening in patients with extracardiac sarcoidosis because of their limited availability and cost. In electrocardiography, which is frequently used in routine screening [13], abnormalities have been found in as many as 50% of patients with sarcoidosis and may occur without clinical evidence of cardiac involvement [14–16]. Therefore, this study aimed to evaluate the association of electrocardiographic abnormalities with the risk of developing cardiac events in patients with extracardiac sarcoidosis.
Cardiac involvement is a leading cause of death from sarcoidosis. Because the efficacy of corticosteroid treatment is limited in patients with cardiac manifestation, early diagnosis is important. However, cardiac involvement is difficult to identify at early stages and is often underdiagnosed. Therefore, this study aimed to identify electrocardiographic risk factors for cardiac events in patients with extracardiac sarcoidosis.
This prospective observational cohort study included 227 patients with extracardiac sarcoidosis who did not have any cardiac manifestation (age, 49 ± 17 years; women, 63%). We studied the association of electrocardiographic abnormalities with developing cardiac manifestations.
During a follow-up of 6.3 ± 3.7 years, 11 patients developed cardiac events, including advanced atrioventricular block (4 patients), ventricular tachycardia (4 patients), and systolic dysfunction (3 patients). All patients had electrocardiographic abnormalities prior to the development of cardiac events. In multivariate analyses, the baseline heart rate and PR interval were associated with increased risk of developing cardiac events. The QRS duration and corrected QT interval were not associated with cardiac manifestations. The multivariate analyses also revealed that baseline conduction disorder, ST segment/T wave abnormalities, and fragmented QRS complexes were associated with cardiac events.
Electrocardiographic abnormalities occurred prior to cardiac events in extracardiac sarcoidosis. Patients with electrocardiographic abnormalities may require further evaluation for cardiac involvement and careful follow-up.
Sarcoid heart disease: Clinical course and treatment
2004, International Journal of Cardiology
Sarcoidosis is a rare granulomatous disease of unknown etiology that can affect any organ. Cardiac involvement, although uncommon, has a wide spectrum of clinical manifestations and is potentially fatal. Although there is no agreement upon a strategy for the diagnosis (which is difficult to make based on clinical information alone), the introduction of newer technology is promising and may be useful both for the early diagnosis of cardiac involvement and for the evaluation of response to therapy.
Early treatment is crucial in improving symptoms and prognosis. ICD implantation and cardiac transplantation may offer improvements in management, as steroid therapy and pacemaker implantation has led to improved outcomes over the past three decades.
Diagnosis of cardiac sarcoidosis and evaluation of the effects of steroid therapy by gadolinium-DTPA-enhanced magnetic resonance imaging
2001, The American journal of medicine
Citation Excerpt :
Electrocardiography and echocardiography are widely used to detect cardiac involvement in patients with sarcoidosis. Electrocardiographic changes are found in as many as 50% of patients with systemic sarcoidosis without clinical evidence of cardiac involvement (12–14); the most common findings are repolarization changes, arrhythmias, and conduction disturbances (11). Evidence of transmural infarction is less common (15,16).
BACKGROUND: Cardiac involvement is an important prognostic factor in patients with sarcoidosis. In this study, we evaluated the usefulness of gadolinium-DTPA (diethylene triamine pentaacetic acid)–enhanced magnetic resonance imaging (Gd-MRI) for diagnosing cardiac sarcoidosis and evaluating the effects of steroid therapy.
METHODS: Sixteen patients with sarcoidosis diagnosed by histology or by Japanese Ministry of Health and Welfare criteria for cardiac sarcoidosis underwent Gd-MRI with a 1.5-Tesla superconducting magnet system using a T1-weighted spin-echo sequence.
RESULTS: Gd-MRI showed localized enhancement of signal intensity, indicating interstitial edema, in the left ventricle in 8 of the 16 patients. Two patients with enhancement also had thinning of the left ventricular septal wall. After 1 month of prednisolone therapy (60 mg every other day or 30 to 40 mg every day), the localized high-intensity signals were markedly diminished in all 8 patients.
CONCLUSIONS: Images of the heart obtained by Gd-MRI may reflect active inflammation with interstitial edema in patients with sarcoidosis. Gd-MRI may be a useful noninvasive method for early detection of cardiac sarcoidosis and for evaluating the effects of steroid therapy.
Sarcoidosis: An updated review
1984, Journal of the American Academy of Dermatology
Sarcoidosis, a multisystem disease of unknown cause characterized by the formation of noncaseating granulomas, may involve any organ of the body, but the commonest sites of predilection are the lungs, lymph nodes, skin, and eyes. Sarcoidosis can be associated with polyclonal hyperglobulinemia, hypercalcemia, hypercalciuria, circulating immune complexes, cutaneous anergy, and in vitro depressed cell-mediated immunity. Recent studies of the cellular composition and cellular interactions of sarcoid-involved tissue have resulted in some insight into the pathophysiology of the disease. In early stages the predominance of activated T-helper cells in affected sites may account for many of the immunologic aberrations. The production of macrophage chemotactic substances by these cells may be the initial stimulus for the formation of the granuloma. The natural history of the disease is highly variable; the acute forms tend to resolve spontaneously, whereas the chronic forms rarely involute. Although multiple therapeutic modalities have been used and some have reportedly been effective, systemic corticosteroids remain the most effective available therapeutic agents.
Cardiac sarcoidosis: A comprehensive review
2011, Archives of Medical Science
Cardiac sarcoidosis - A cardinal diagnosis
2007, Hart Bulletin

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From the Johns Hopkins University School of Medicine, and Veterans Administration Medical Center, Baltimore

Manuscript received September 28; revision accepted December 5.

Supported in part by grants HL-18799 and HL-17655 from the National Heart, Lung, and Blood Institute, National Institutes of Health and by the Veterans Administration

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Chest

Clinical InvestigationsSystemic Sarcoidosis and Electrocardiographic Conduction Abnormalities: Electrophysiologic Evaluation of Two Patients

Section snippets

Case 1

Discussion

Chest

J Electrocardiol

Am Heart J

Cardiac sarcoid: A clinicopathologic study of 84 unselected patients with systemic sarcoidosis

Circulation

Clinical Investigations
Systemic Sarcoidosis and Electrocardiographic Conduction Abnormalities: Electrophysiologic Evaluation of Two Patients