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Published on: June 16, 2020
Cardiac involvement in sarcoidosis
Jane C Deng1, Robert P Baughman, Joseph P Lynch
1Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Michigan Medical Center, Ann Arbor 48109-0360, USA.
Insights
Cardiac sarcoidosis diagnosis is difficult, often requiring treatment without definitive proof. Early recognition and aggressive therapy, including corticosteroids and device implantation, are crucial for managing this life-threatening condition.
Area of Science:
- Cardiology
- Immunology
- Pathology
Background:
- Cardiac sarcoidosis presents a diagnostic challenge, with varied clinical manifestations from benign findings to fatal outcomes.
- Histologic confirmation of myocardial sarcoidosis is often difficult, complicating management decisions.
Purpose of the Study:
- To review the clinical features, diagnostic approaches, treatment strategies, and prognosis of cardiac sarcoidosis.
- To highlight the challenges in diagnosing and managing cardiac sarcoidosis.
Main Methods:
- Review of existing literature on cardiac sarcoidosis.
- Discussion of diagnostic tools including thallium scintigraphy, echocardiography, and other radionuclide techniques.
- Evaluation of treatment options such as corticosteroids, immunosuppressants, and device implantation.
Main Results:
- Definitive diagnosis can be challenging, necessitating treatment based on clinical suspicion.
- Thallium-201 scintigraphy is a key diagnostic tool, with echocardiography offering complementary information.
- Aggressive, long-term treatment with corticosteroids or immunosuppressants is often required.
- Automatic cardioverter-defibrillators may be necessary for recurrent tachyarrhythmias.
Conclusions:
- Prompt diagnosis and aggressive treatment are vital for managing potentially fatal cardiac sarcoidosis.
- Optimal diagnostic strategies and management protocols require further clarification.
- Clinicians face significant obstacles in recognizing and effectively managing cardiac sarcoidosis.
Abstract:
Cardiac sarcoidosis is a challenging diagnostic entity. Manifestations range from an incidentally discovered, benign condition to fatal cardiomyopathy and arrhythmias. In this review, we discuss the salient clinical features, diagnostic evaluation, treatment, and prognosis of cardiac sarcoidosis. A definitive histologic diagnosis of myocardial sarcoidosis is difficult, and treatment may be necessary in some patients with suspected cardiac involvement even in the absence of histologic confirmation. Optimal strategies to diagnose cardiac involvement have not been clarified. Thallium (201) radionuclide scintigraphy has been most extensively studied; 2-D echocardiography is less sensitive, but may have a complementary role. Additional radionuclide techniques (e.g., gallium (67) or technetium (99) ) may have a role in complex cases. Given the life-threatening nature of cardiac involvement, prompt and aggressive treatment with corticosteroids or immunosuppressive agents, or both, is warranted. Long-term, possibly lifelong, therapy may be required. Serious, recurrent tachyarrhythmias may require implantation of an automatic cardioverter-defibrillator. Recognizing cardiac sarcoidosis and selecting a sensible management strategy remain the major obstacles facing clinicians.
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