PMCCPMCCPMCC

Search tips
Search criteria 

Advanced

 
Logo of thijTexas Heart Institute JournalSee also Cardiovascular Diseases Journal in PMCSubscribeSubmissionsTHI Journal Website
 
Tex Heart Inst J. 2009; 36(5): 501–503.
PMCID: PMC2763448

Cardiac Sarcoidosis

A Clinical Entity Uncommonly Recognized
Raymond F. Stainback, MD, Section Editor
Department of Adult Cardiology, Texas Heart Institute at St. Luke's Episcopal Hospital, 6624 Fannin Street, Suite 2480, Houston, TX 77030

A 47-year-old black woman with no relevant medical history was brought to the emergency room after a syncopal episode. The electrocardiogram obtained at admission showed an ectopic atrial rhythm with trifascicular block (Fig. 1). Transthoracic echocardiography revealed a normal left ventricular ejection fraction with mild left ventricular hypertrophy and regional wall motion abnormalities in the basal septum and anterolateral wall. Myocardial perfusion scanning with technetium 99m sestamibi showed multiple fixed perfusion defects in the noncoronary distribution (Fig. 2), but subsequent selective coronary angiography showed no evidence of significant coronary artery disease. Cardiac magnetic resonance imaging revealed delayed hyperenhancement (myocardial fibrosis) in the lateral wall of the left ventricle (Fig. 3) and in the basal interventricular septum (Fig. 4), which was similar to the findings on the myocardial perfusion scan. At this point, we strongly suspected the presence of cardiac sarcoidosis, so a computed tomographic scan of the chest was performed. It showed multiple mediastinal lymph nodes. Therefore, a guided biopsy of these abnormal mediastinal lymph nodes was performed by means of mini-video-assisted thoracic surgery. Histopathologic examination of the specimen revealed noncaseating granulomas (Fig. 5). The diagnosis of cardiac sarcoidosis was confirmed.

figure 35FF1
Fig. 1 A 12-lead surface electrocardiogram shows ectopic atrial rhythm with 1st-degree atrioventricular block, right bundle branch block, and left anterior fascicular block (trifascicular block).
figure 35FF2
Fig. 2 Technetium 99m sestamibi scan reveals multiple fixed perfusion defects.
figure 35FF3
Fig. 3 Gadolinium-enhanced inversion-recovery magnetic resonance imaging shows hyperenhancement in the lateral wall of the left ventricle.
figure 35FF4
Fig. 4 Gadolinium-enhanced inversion-recovery magnetic resonance imaging shows hyperenhancement in the basal interventricular septum.
figure 35FF5
Fig. 5 Photomicrograph of the mediastinal lymph node biopsy shows noncaseating granulomas with multinucleated giant cells characteristic of sarcoidosis (H & E, orig. ×40).

Comment

Sarcoidosis, an inflammatory disorder of unknown origin and characterized by noncaseating granulomas, can affect the lungs, lymph nodes, heart, liver, spleen, skin, eyes, phalangeal bones, parotid glands, and other organs and tissues.1 The annual incidence of sarcoidosis in the United States is higher in the black population (35.5 per 100,000) than in the white population (10.9 per 100,000).2 Another U.S. study has found on autopsy that cardiac sarcoidosis occurs in up to 25% of patients with sarcoidosis,3 and it accounts for 13% to 25% of deaths from the disease.4 Cardiac sarcoidosis usually occurs in young adults. Sarcoid granulomas can be located anywhere in the heart, including the pericardium, endocardium, and, most frequently, the myocardium.1

Conduction abnormalities have been reported in 12% to 62% of patients with cardiac sarcoidosis.1,5–7 Infiltration of the conduction system by sarcoidosis can cause various abnormalities, such as left or right bundle branch block, atrioventricular conduction block,1,5 and, rarely, sinus arrest.8 Complete heart block is the most common presenting conduction abnormality (in 23%–30% of patients), and most frequently presents as syncope,1 but it can also occur without any notable evidence of cardiomyopathy. Syncope in this population can also be due to ventricular arrhythmias, which are usually caused by direct granulomatous involvement of the myocardium.1 The most feared complication—and often the initial presentation of sarcoidosis—is sudden death due to ventricular arrhythmia or complete heart block. In the U.S., sudden death occurs in 24% to 65% of all patients whose deaths are related to cardiac sarcoidosis,1,5 and in 17% of patients, sudden death is the initial presentation of cardiac sarcoidosis.5

Footnotes

Address for reprints: Mahboob Alam, MD, Section of Cardiology, Department of Medicine, BCM 620, 1709 Dryden St., Suite 9.90, Houston, TX 77030 E-mail: ude.cmt.mcb@malam

References

1. Roberts WC, McAllister HA Jr, Ferrans VJ. Sarcoidosis of the heart. A clinicopathologic study of 35 necropsy patients (group 1) and review of 78 previously described necropsy patients (group 11). Am J Med 1977;63(1):86–108. [PubMed]
2. Rybicki BA, Major M, Popovich J Jr, Maliarik MJ, Iannuzzi MC. Racial differences in sarcoidosis incidence: a 5-year study in a health maintenance organization. Am J Epidemiol 1997; 145(3):234–41. [PubMed]
3. Perry A, Vuitch F. Causes of death in patients with sarcoidosis. A morphologic study of 38 autopsies with clinicopathologic correlations. Arch Pathol Lab Med 1995;119(2):167–72. [PubMed]
4. Silverman KJ, Hutchins GM, Bulkley BH. Cardiac sarcoid: a clinicopathologic study of 84 unselected patients with systemic sarcoidosis. Circulation 1978;58(6):1204–11. [PubMed]
5. Fleming HA, Bailey SM. Sarcoid heart disease. J R Coll Physicians Lond 1981;15(4):245–6, 249–53. [PubMed]
6. Matsui Y, Iwai K, Tachibana T, Fruie T, Shigematsu N, Izumi T, et al. Clinicopathological study of fatal myocardial sarcoidosis. Ann N Y Acad Sci 1976;278:455–69. [PubMed]
7. Smedema JP, Snoep G, van Kroonenburgh MP, van Geuns RJ, Dassen WR, Gorgels AP, Crijns HJ. Cardiac involvement in patients with pulmonary sarcoidosis assessed at two university medical centers in the Netherlands. Chest 2005;128(1): 30–5. [PubMed]
8. Ahmad K, Kim YH, Spitzer AR, Gupta A, Han IH, Herskovic A, Sakr WA. Total nodal radiation in progressive sarcoidosis. Case report. Am J Clin Oncol 1992;15(4):311–3. [PubMed]

Articles from Texas Heart Institute Journal are provided here courtesy of Texas Heart Institute