Cardiac sarcoidosis

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The Thoracic and Cardiovascular Surgeon Official Organ of the German Society for Thoracic and Cardiovascular Surgery

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Cardiac Sarcoidosis

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Authors

F. A. Mitropoulos 1, C. S. Floudas 2, M. A. Kanakis 1, G. A. Vaiopoulos 2

Affiliations

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Key words " cardiomyopathy l " heart and lung transl plantation " heart disease l

Department of Pediatric Cardiac Surgery, Onassis Cardiac Center, Athens, Greece First Department of Internal Medicine, National and Kapodistrian University of Athens, Laiko General Hospital, Athens, Greece

Abstract !

Sarcoidosis is a systemic disease which affects many organs, including the heart. Cardiac sarcoidosis has a reported incidence of about 25 % and carries a poor prognosis. It can occur in the form of conduction abnormalities, pericardial and valvular heart disease, congestive heart failure, arrhythmias and sudden cardiac death. The diagnosis of cardiac sarcoidosis is difficult, requiring a high index of suspicion and the use of electrocardiography, echocardiography, nuclear med-

Introduction !

received

Sept. 13, 2008

Bibliography DOI 10.1055/s-0029-1185302 Thorac Cardiov Surg 2009; 57: 187–190 © Georg Thieme Verlag KG Stuttgart · New York · ISSN 0171‑6425 Correspondence Dr. Charalampos S. Floudas, M. D. First Department of Internal Medicine National and Kapodistrian University of Athens, Laiko General Hospital 17 Ag. Thoma 11527 Athens Greece Phone: + 30 69 74 91 28 80 Fax: + 30 21 07 78 88 30 [email protected]

Sarcoidosis is an idiopathic, systemic disease characterized pathologically by non-caseating granulomas. It is more common in younger adults and African-Americans, with an overall prevalence of 20 per 100 000 [1]. Sarcoidosis affects many organs including the heart. Cardiac sarcoidosis (CS) was first described by Bernstein et al. in 1929 [2]. Myocardial involvement occurs in at least 25% of patients with sarcoidosis [3]. In an autopsy study of 70 patients who died from CS it was found that the average age of death was 40 years [4]. The most common clinical manifestations were conduction disturbances, ventricular arrhythmias, and congestive heart failure [4]. Roberts et al. reported on 113 necropsy patients with CS. They showed that sarcoid granulomas and scars most often involved the left ventricular free wall, papillary muscles and the cephalad portion of the ventricular sep" Fig. 1). They claim that no portion of the tum (l heart is immune to infiltration by sarcoid granu" Fig. 2). CS can also involve the pericardilomas (l um, myocardium and endocardium. Furthermore, they observed that patients in whom sarcoidosis produced cardiac dysfunction showed little or no clinical evidence of involvement of any other or-

icine imaging, myocardial biopsy and magnetic resonance imaging. Corticosteroids have been the cornerstone of treatment of cardiac sarcoidosis, but other immunosuppressives have also been used, along with standard heart failure therapy, antiarrhythmic medications, pacemakers and implantable defibrillators. Cardiac transplantation is an option for patients who do not respond to medical treatment. We briefly review the current armamentarium for the diagnosis and treatment of cardiac sarcoidosis.

gan [5]. The types of cardiac dysfunction include 1) arrhythmias; 2) high degrees of atrioventricular block and complete bundle branch block; 3) sudden death; 4) congestive heart failure; 5) papillary muscle dysfunction; 6) acute myocardial infarction; 7) ventricular aneurysm; and 8) recurring pericardial effusion [4, 5]. Only 40 % to 50 % of patients with CS at autopsy had a correct diagnosis during their lifetime [6].

Diagnosis !

The diagnosis of this disorder requires a high index of clinical suspicion [7, 8]. In the medical literature there is a report of a patient who underwent orthotopic heart transplantation (OHT) for presumed idiopathic dilated cardiomyopathy, but on pathologic examination, the explanted heart revealed extensive CS [9]. The clinical diagnosis is difficult to make, since the manifestations depend on the location and extension of granulomas, and symptoms and signs vary and are nonspecific. The ECG may be normal or show any degree of heart block and any type of arrhythmia with nonspecific ST‑T changes [10]. Echocardiography may show an abnormal wall thickness, diastolic or systolic dysfunction, chamber dilation,

Mitropoulos FA et al. Cardiac Sarcoidosis Thorac Cardiov Surg 2009; 57: 187–190

This is a copy of the authorʼs personal reprint

This is a copy of the authorʼs personal reprint

2

Review

This is a copy of the authorʼs personal reprint

Fig. 1 Gross photograph of heart with sarcoidosis showing scars in unusual locations (image courtesy of Dr. Michael C. Fishbein, M. D.).

Fig. 2 Microscopic section showing typical discrete non-caseating granulomas (image courtesy of Dr. Michael C. Fishbein, M. D.).

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pericardial effusion. Papillary muscle infiltration may lead to valvular regurgitation, but primary valve leaflet involvement is uncommon [11]. Resting myocardial scintigraphy with 201Tl may show segmental areas of decreased uptake in up to 30 % of patients with sarcoidosis, even those without cardiac symptoms [12, 13]. These perfusion defects may disappear or decrease during exercise or after the administration of dipyridamole. This phenomenon (reverse distribution) helps to differentiate the granulomatous origin of the injury from coronary artery disease [14, 15]. In many cases, a coronary angiogram is required. In the presence of healthy coronary arteries, the perfusion defects on 201 Tl scan in a patient with known multisystem sarcoidosis strongly suggest myocardial involvement. However, normal findings on the 201Tl scan do not exclude the presence of cardiac involvement [16]. A positive finding on the 201Tl scan also has no prognostic value [17]. The combination of 201Tl and 67Ga provides more information [18]. These radionuclide studies remain nonspecific for the diagnosis of CS [19]. Furthermore, fasting 18F‑FDG PET (18F-labeled fluoro-2 deoxy-D glucose position emission tomography) can detect the early stage of CS, in which fewer perfusion abnormalities and high inflammatory activity are noted, prior to advanced myocardial impairment. One study showed that in patients with CS, fasting 18F‑FDG PET revealed a higher frequency of abnormal myocardial segments than 99mTc-MIBI SPECT (99mTc- methoxy-isobutyl isonitrile single photon emission computed tomography). The sensitivity of fasting 18F‑FDG PET in detecting CS was 100 %, significantly higher than that of 99mTc-MIBI SPECT (63.6)% or 67Ga scintigraphy (36.3%) [20]. Magnetic resonance imaging (MRI) has recently been used as a noninvasive method for the early diagnosis and follow-up of CS. It was demonstrated that cardiac MRI follow-up findings correlate well with clinical follow-up findings and can be used to evaluate and modify the treatment [21, 22]. Active sarcoidosis with myocardial inflammation appears as regions of bright signal intensity on both T2-weighted sequences and with delayed contrast enhancement technique (MRI technique which uses gadolinium paramagnetic contrast media). Furthermore, with this technique myocardial scars can be visualized as bright midwall or transmural areas with a “non-coronary” distribution [23].

Mitropoulos FA et al. Cardiac Sarcoidosis

Thorac Cardiov Surg 2009; 57: 187–190

Transvenous endomyocardial biopsy has a high specificity and therefore it is considered as the gold standard for definitive confirmation of the diagnosis of CS, since it reveals the presence of non-caseating granulomas. However, a negative finding cannot exclude the diagnosis, due to the low success rate of the method. This low sensitivity (20%) is likely due to the patchy nature of the granulomas and their preference for the cephalad portion of the ventricular septum, which is less accessible to the biopsy needle [24]. Differentiation of idiopathic giant cell myocarditis (IGCM) from CS may be difficult, particularly when there is limited extracardiac involvement by sarcoidosis. This distinction is important, since transplant-free survival is better than IGCM for patients with CS than IGCM. IGCM and CS have distinct histological features: although both diseases have multinucleated giant cells, myocyte necrosis and lymphocytic inflammatory infiltrate, CS is further characterized by the presence of at least one non-caseating granuloma, as well as more fibrosis and less necrosis, fewer foci of lymphocytic infiltrate and fewer eosinophils. On clinical grounds, the time elapsing from the onset of symptoms to presentation and diagnosis is longer in CS. Furthermore, syncope and atrioventricular block are more frequent in CS, whereas ventricular tachycardia is common in both diseases and left-sided congestive heart failure is more common with IGCM [25]. A recent article reported a case of a patient who underwent OHT for cardiomyopathy, and in whom pathologic examination of the native heart showed CS and concomitant deposition of immunoglobulin lambda II light-chain amyloidosis [26]. Recent reports indicate also the possibility of CS appearing as an arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) with ventricular tachycardia as a presenting symptom. This distinction is important, since corticosteroid therapy is not beneficial in ARVD/C, but may be of crucial importance in CS [27].

This is a copy of the authorʼs personal reprint

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Prognosis and Treatment

This is a copy of the authorʼs personal reprint

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The prognosis of patients with CS is poor, with a survival from onset of cardiac symptoms of 1 to 2 years. Sudden death is a frequent presentation, occurring in 65 % of patients. In 40 %, sudden death was the initial presentation [28]. Treatment of CS depends on cardiac manifestations (antiarrhythmic agents, pacemaker, implantable defibrillator, anticongestive therapy) along with corticosteroids [7, 29]. Mechanical support as a bridge to OHT has also been described [30]. Corticosteroid therapy has shown improvement in left ventricular systolic function, after 5 monthsʼ therapy, in a patient with CS [31, 32]. A retrospective study of 41 patients with CS concluded that CS should be treated with corticosteroids as soon as possible and another immunosuppressive treatment used where there is an insufficient therapeutic response or where there are contraindications to corticosteroids [33]. Until now, there are no randomized control trials reporting the efficacy of corticosteroids. OHT may improve the prognosis and quality of life for these patients who do not have active systemic disease and who have not responded to the above conventional therapy [7, 32]. A case of cardiac allograft recurrence, 6 months after OHT, has been reported and it was controlled with augmented corticosteroids. Interestingly, in spite of sarcoid recurrence, cardiac function remained normal [34]. Transmission via cardiac transplantation has also been published [35]. These reports supported the observation that the disease was transmissible through human sarcoid tissue, thus possibly caused by an infectious agent [36]. Cardiac allograft has to be followed by serial graft biopsy. Endomyocardial biopsy not only identifies and establishes the diagnosis of rejection, but also gives some clue as to the possible outcome. Mild and moderate rejection episodes during conventional treatment can usually be overcome by antirejection therapy [37]. Contrastenhanced MRI can be used for follow-up as well after OHT [22]. The armamentarium of immunosuppressive agents has recently been enforced. Even acute heart allograft rejection resistant to common immunosuppressive therapy can be controlled by new agents [38]. CS is a severe disease associated with a poor prognosis. When the usual medical treatment fails, heart transplantation is the final option for these patients.

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Review

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34 Oni AA, Hershberger RE, Norman DJ, Ray J, Hovaguimian H, Cobanoglu AM et al. Recurrence of sarcoidosis in a cardiac allograft: control with augmented corticosteroids. J Heart Lung Transplant 1992; 11: 367–369 35 Burke WM, Keogh A, Maloney PJ, Delprado W, Bryant DH, Spratt P. Transmission of sarcoidosis via cardiac transplantation. Lancet 1990; 336: 1579 36 Mitchell DN, Rees RJ. A transmissible agent from sarcoid tissue. Lancet 1969; 2: 81–84

37 Gokel JM, Reichart B, Struck E. Human cardiac transplantation–evaluation of morphological changes in serial endomyocardial biopsies. Pathol Res Pract 1985; 179: 354–364 38 Ankersmit HJ, Roth G, Zuckermann A, Moser B, Obermaier R, Taghavi S et al. Rapamycin as rescue therapy in a patient supported by biventricular assist device to heart transplantation with consecutive ongoing rejection. Am J Transplant 2003; 3: 231–234

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Thorac Cardiov Surg 2009; 57: 187–190

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