Sarcoidosis: Cardiac Involvement

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J Am Soc Echocardiogr. 2001 May;14(5):399-402.

Transesophageal echocardiographic abnormalities in a case of cardiac sarcoidosis.

Hourigan LA, Burstow DJ, Pohlner P, Clarke BE, Donnelly JE.

Department of Cardiology, The Prince Charles Hospital, Rode Road, Chermside, Brisbane, Australia.

Sarcoidosis is a granulomatous disease that may involve multiple organ systems, including the heart. Manifestations include atrial and ventricular arrhythmias, conduction abnormalities, congestive cardiac failure, pericarditis, and sudden death. Whereas cardiac involvement is a relatively common finding at autopsy, antemortem diagnosis is often missed because the clinical manifestations are nonspecific, and the sensitivity and specificity of investigations are low. We report a case of a 62-year-old woman who had clinically significant cardiac sarcoidosis associated with echocardiographic abnormalities that had not been reported previously in association with this condition.

Circ J. 2003 Feb;67(2):169-71.

Cardiac sarcoidosis mimicking right ventricular dysplasia.

Shiraishi J, Tatsumi T, Shimoo K, Katsume A, Mani H, Kobara M, Shirayama T, Azuma A, Nakagawa M.

Second Department of Medicine, Kyoto Prefectural University of Medicine, Japan.

A 59-year-old woman with skin sarcoidosis was admitted to hospital for assessment of complete atrioventricular block. Cross-sectional echocardiography showed that the apical free wall of the right ventricle was thin and dyskinetic with dilation of the right ventricle. Thallium-201 myocardial imaging revealed a normal distribution. Both gallium-67 and technetium-99m pyrophosphate scintigraphy revealed no abnormal uptake in the myocardium. Right ventriculography showed chamber dilation and dyskinesis of the apical free wall, whereas left ventriculography showed normokinesis, mimicking right ventricular dysplasia. Cardiac sarcoidosis was diagnosed on examination of an endomyocardial biopsy specimen from the right ventricle. A permanent pacemaker was implanted to manage the complete atrioventricular block. After steroid treatment, electrocardiography showed first-degree atrioventricular block and echocardiography revealed an improvement in the right ventricular chamber dilation. Reports of cardiac sarcoidosis mimicking right ventricular dysplasia are extremely rare and as this case shows, right ventricular involvement may be one of its manifestations.

Masui. 2001 Jan;50(1):62-4.

Anesthesia for a patient with cardiac sarcoidosis.

Imai M, Hayashi Y, Ono R, Shibata C, Mashimo T.

Department of Anesthesiology, Osaka University Faculty of Medicine, Suita 565-0871.

Anesthesia for a 49-year-old man with cardiac sarcoidosis is reported. Preoperative cardiac examination showed left ventricular dysfunction (ejection fraction = 27%) and myocardial conduction defects. In addition, his previous history included atrial fibrillation and cerebral infarction. Transesophageal echocardiography showed thrombus in the left appendage. Anesthesia was induced with fentanyl and diazepam and maintained with fentanyl and isoflurane. Perioperative hemodynamic monitoring included direct arterial pressure, central venous and pulmonary artery pressure and continuous cardiac output. In addition, transesophageal echocardiography was useful for watching thrombus in the left appendage. His hemodynamic condition was stable and no neurological complication was noted after anesthesia.

Cardiol Rev. 2003 Jul-Aug;11(4):163-8.

Rapidly progressing congestive heart failure due to cardiac sarcoidosis involving papillary muscles: a case report and brief review of the literature.

Desai MY, Fallert MA.

Division of Cardiology, Allegheny General Hospital, Pittsburgh, Pennsylvania, USA.

This is the case of a patient who presented with congestive heart failure, which appeared to be on the basis of severe mitral regurgitation secondary to granulomatous infiltration of the papillary muscles. In addition, conduction system disease and generalized myocardial involvement were documented. The patient responded well to an aggressive course of corticosteroids.

Curr Opin Pulm Med. 2004 Sep;10(5):397-400.

Cardiac sarcoidosis with presentation of large left atrial mass.

Abrishami B, O'Connel C, Sharma O.

Department of Pulmonary and Critical Care Medicine, University of Southern California/Los Angeles County Medical Center, Los Angeles, California 90012, USA.

PURPOSE OF REVIEW: The purpose of discussing this rare clinical presentation is to provide an opportunity to review difficulties encountered in diagnosing myocardial sarcoidosis. RECENT FINDINGS: Recent advances in diagnosis and management of cardiac sarcoidosis are discussed in this article. SUMMARY: A 45-year old man was brought to the emergency department after he fainted in a marketplace. An electrocardiogram showed a complete heart block; an echocardiogram revealed a large atrial mass consistent with the appearance of a myxoma. A biopsy of the cardiac mass showed noncaseating granuloma.

Heart. 2004 Nov;90(11):1275-80.

Early detection of cardiac involvement in patients with sarcoidosis by a non-invasive method with ultrasonic tissue characterisation.

Hyodo E, Hozumi T, Takemoto Y, Watanabe H, Muro T, Yamagishi H, Yoshiyama M, Takeuchi K, Yoshikawa J.

Department of Internal Medicine and Cardiology, Osaka City University School of Medicine, Osaka, Japan.

OBJECTIVES: To clarify the value of cycle dependent variation of myocardial integrated backscatter (CV-IB) analysis, which non-invasively measures acoustic properties of the myocardium, for early detection of cardiac involvement in patients with sarcoidosis. METHODS: The study population consisted of 22 consecutive patients with biopsy proven sarcoidosis who did not have any abnormal findings on conventional two dimensional echocardiogram. Cardiac sarcoidosis was diagnosed by radionuclide testing including thallium-201 scintigraphy, gallium-67 scintigraphy, and cardiac fluorine-18-deoxyglucose positron emission tomography. The magnitude and delay of the CV-IB were analysed in the basal mid septum and the basal mid posterior wall of the left ventricle of all patients. RESULTS: The patients were divided into two groups: 8 patients with cardiac involvement and 14 patients without cardiac involvement. In the basal septum, a major reduction in the magnitude (mean (SD) 1.8 (4.4) v 6.6 (1.3), p = 0.012) and an increase in the time delay (1.3 (0.5) v 1.0 (0.1), p = 0.038) of CV-IB were observed in patients with cardiac sarcoidosis even in the absence of two dimensional echocardiographic abnormalities. The sensitivity for detecting cardiac involvement was such that the magnitude of CV-IB in the basal septum discriminated 75% of patients with cardiac sarcoidosis from those with non-cardiac sarcoidosis, whereas two dimensional echocardiographic parameters did not discriminate between these two groups. CONCLUSIONS: The CV-IB is decreased in the basal septum in patients with cardiac sarcoidosis even in the absence of two dimensional echocardiographic abnormalities. Analysis of CV-IB may be a useful method to detect early myocardial involvement in patients with sarcoidosis.

Rev Mal Respir. 2003 Nov;20(5 Pt 1):777-81.

Comment in: Rev Mal Respir. 2003 Nov;20(5 Pt 1):665-7.

The role of exercise testing in the management of systemic sarcoidosis.

Leroy S, Just N, Zanetti C, Palem A, Maurage CA, Neviere R, Wallaert B.

Clinique des Maladies Respiratoires, Hopital Albert Calmette, CHRU, Lille, France.

INTRODUCTION: Patients suffering from sarcoidosis sometimes report dyspnoea of effort. The restrictive or obstructive ventilatory problems contribute to this disability, but the systemic manifestations of sarcoidosis should be considered as other possible causes of the dyspnoea. CASE REPORT: We report a case of systemic sarcoidosis in a 29 year old man from the Ivory Coast. The disorder presented in December 1999 with generalised symptoms and grade II dyspnoea (Sadoul). Investigations confirmed pulmonary, mediastinal, hepatic, lachrymal and salivary involvement. Lung function tests at rest showed a combined restrictive and obstructive ventilatory defect. The initial exercise test showed evidence of severe limitation of aerobic metabolism with impaired oxygen uptake without limitation of ventilation. The absence of cardiac involvement, confirmed by echocardiography and myocardial isotope scanning, suggested that the dyspnoea was, at least in part, secondary to a "peripheral" muscular abnormality. After 3 months treatment with oral corticosteroids, the clinical and physiological parameters improved. Exercise testing confirmed an improvement in maximum work rate and oxygen uptake. The relative stability of the functional measurements and the subsequent development of myalgia prompted a biopsy of the quadriceps that showed granulomata within the muscle tissue, confirming a sarcoid myopathy. A combination of steroids and methotrexate allowed the patient to increase his exercise capacity without any major change in the resting lung function tests. CONCLUSIONS: This case report underlines the role of exercise testing in determining the cause of dyspnoea. In this patient it contributed to the diagnosis of sarcoid myopathy and lead to modification of his treatment.

Cardiovasc Drugs Ther. 1996 Nov;10(5):495-510.

Cardiac sarcoidosis: diagnostic, prognostic, and therapeutic considerations.

Sekiguchi M, Yazaki Y, Isobe M, Hiroe M.

1st Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto City, Japan.

Cardiac involvement in patients with sarcoidosis is an important consideration for those who are concerned with this strange disease. Sarcoidosis is not an acute malignant disease but may be noticed at the time of sudden, expected death as fatal myocardial sarcoidosis at autopsy. Even with modern advances in our ability to diagnose heart disease, cardiac sarcoidosis is still often overlooked because of its subclinical disease progression. In view of this, an extensive review of previously published literature and of our own case analyses has been carried out because of the authors' long-term experience with performing Konno's endomyocardial biopsy, which was originally developed in 1962 at the author's institution. However, the sensitivity of endomyocardial biopsy in detecting sarcoid granuloma is low (20-30%), and, instead, various kinds of nongranulomatous pathologies are often seen. During the course of our research it was found that there might exist a racial difference in cardiac sarcoidosis. Cardiac death was much more frequent in Japanese patients. The possibility that heart disease in sarcoidosis is caused by cor pulmonale due to advanced pulmonary fibrosis should be reevaluated because only a limited amount of background data is available. The author's review clarified the fact that cardiac sarcoidosis is caused by myocardial or pericardial involvement, resulting in various kinds of bradyarrhythmias or tachyarrhythmias and/or congestive heart failure. Electrocardiographic (ECG) and Holter monitor readings provide a simple and effective method for early detection of this disease. The incidence of ECG abnormalities in a total of 963 sarcoidosis patients was 22.1%, which was more frequent than that of the sex- and age-matched healthy control subjects (17.9%; p < 0.025). Echocardiography and radionuclide studies also provide useful clinical information. Careful follow-up and early corticosteroid administration followed by small maintenance doses may prevent the progression of the disease and improve prognosis. Owing to the progress in antiarrhythmic drugs and pacemaker implantation, the primary cause of death in cardiac sarcoidosis has changed from sudden death (1976 report) to congestive heart failure (1985 report).

J Am Coll Cardiol. 2005 May 17;45(10):1683-90. Epub 2005 Apr 25.

Evaluation of the accuracy of gadolinium-enhanced cardiovascular magnetic resonance in the diagnosis of cardiac sarcoidosis.

Smedema JP, Snoep G, van Kroonenburgh MP, van Geuns RJ, Dassen WR, Gorgels AP, Crijns HJ.

Department of Cardiology, University Hospital Maastricht, Maastricht, The Netherlands.

OBJECTIVES: This study analyzed the accuracy of gadolinium-enhanced cardiovascular magnetic resonance (CMR) for the diagnosis of cardiac sarcoidosis (CS). BACKGROUND: The diagnosis of CS was made according to the guidelines of the Japanese Ministry of Health and Welfare (1993); CMR has not been incorporated into the guidelines, and the diagnostic accuracy of CMR for the diagnosis of CS has not yet been evaluated. METHODS: We performed an analysis of 12-lead electrocardiograms (ECGs), 24-h ambulatory ECGs, echocardiograms, thallium scintigrams, and gadolinium-enhanced CMR studies in 58 biopsy-proven pulmonary sarcoidosis patients assessed for CS. The diagnostic accuracy of CMR for CS was determined using modified Japanese guidelines as the gold standard. RESULTS: The diagnosis of CS was made in 12 of 58 patients (21%); CMR revealed late gadolinium enhancement (LGE), mostly involving basal and lateral segments (73%), in 19 patients. In 8 of the 19 patients, scintigraphy was normal, while patchy LGE was present. The sensitivity and specificity of CMR were 100% (95% confidence interval, 78% to 100%) and 78% (95% confidence interval, 64% to 89%), and the positive and negative predictive values were 55% and 100%, respectively, with an overall accuracy of 83%. CONCLUSIONS: In patients with sarcoidosis, CMR is a useful diagnostic tool to determine cardiac involvement. New diagnostic guidelines should include CMR.

Sarcoidosis Vasc Diffuse Lung Dis. 2005 Mar;22(1):63-5.

Relationship between basal thinning of the interventricular septum and atrioventricular block in patients with cardiac sarcoidosis.

Uemura A, Morimoto S, Kato Y, Hiramitsu S, Ohtsuki M, Kato S, Sugiura A, Miyagishima K, Iwase M, Hishida H.

Department of Internal Medicine, Fujita Health University School of Medicine, Toyoake, Japan.

BACKGROUND: Basal thinning of the interventricular septum (IVS) and atrioventricular block (AVB) are characteristic features of cardiac sarcoidosis. Since the conduction system passes along IVS, it has been considered that a close connection exists between basal thinning of IVS and AVB. However, neither the incidence of cases showing basal thinning of IVS nor the relation between it and AVB has been clarified. We thus investigated to elucidate these two issues. METHODS: Thirty-five patients with cardiac sarcoidosis were selected for this study and underwent echocardiographic examination. The wall thickness of IVS was measured at a site 1 cm below the aortic valve inserted point of IVS. Thickness of this site < or = 5 mm was defined as thinning. Twelve-lead and Holter electrocardiograms were obtained to determine the presence/absence and degree of AVB. RESULTS: Basal thinning of IVS was noted in 7 of the 35 patients (20%). AVB was present in 4 of these 7 (57%), and was first degree in 3 (43%) and third degree in one (14%). AVB was not present in 3 patients. Basal thinning of IVS was not apparent in 28 of the 35 patients (80%). AVB was observed in 14 of the 28 patients, 3 had first degree block, 2 had second degree block, and 9 had third degree block. AVB was not observed in 14 of the 28 patients. CONCLUSIONS: These results clarified that basal thinning of IVS is not as common as previously thought in cardiac sarcoidosis, basal thinning of IVS and the presence/absence and degree of AVB are not necessarily correlated.

Sarcoidosis Vasc Diffuse Lung Dis. 2005 Mar;22(1):43-50.

Association of small fiber neuropathy with cardiac sympathetic dysfunction in sarcoidosis.

Hoitsma E, Faber CG, van Kroonenburgh MJ, Gorgels AP, Halders SG, Heidendal GA, Kessels AG, Reulen JP, Drent M.

Dept of Neurology, Management Center, University Hospital Maastricht, the Netherlands.

BACKGROUND AND AIM: Recently we found that small fiber neuropathy (SFN) occurs frequently in sarcoidosis. Autonomic dysfunction may be a feature of SFN. Since cardiac autonomic dysfunction has been identified as a strong predictor of morbidity and mortality, recognition of cardiac autonomic involvement is of clinical relevance. It was hypothesised that SFN might be related to cardiac sympathetic denervation in sarcoidosis. METHODS: In the present study 45 consecutive sarcoidosis patients (13 without SFN assessed by thermal threshold testing (TTT), 32 with SFN (abnormal TTT) were enrolled. To rule out bias due to myocardial ischemia, cases with abnormal Thallium (201Tl) perfusion scintigraphy were excluded (n = 2). Cardiovascular autonomic function testing (Ewing tests) and 123I-MIBG (metaiodobenzylguanidine) scintigraphy were used to assess cardiac autonomic function. Further cardiac diagnostic work-up included ECG, Holter recording and echo Doppler cardiography. RESULTS: Mild to moderate heterogeneity of 123I-MIBG uptake regional in the myocardium was demonstrated in a substantial number of the studied sarcoidosis population, especially in those with SFN (abnormal TTT). Mean inferior-anterior ratios were 0.85+/-0.17 (SFN) and 1.0+/-0.17 (no SFN; p = 0.003), respectively. Four out of the 14 cases with abnormal MIBG scintigraphy and SFN showed an abnormal Ewing test. CONCLUSION: Cardiac sympathetic dysfunction assessed by use of 123I-MIBG myocardial scanning appeared to be heterogeneous in sarcoidosis patients and dependent on the presence or absence of SFN. MIBG scintigraphy may be of additional value in the management and follow-up of sarcoidosis patients. Future study is warranted to explore possible prognostic and therapeutic implications of these findings in sarcoidosis.

Eur Heart J. 2005 Apr 4;

Focal uptake on 18F-fluoro-2-deoxyglucose positron emission tomography images indicates cardiac involvement of sarcoidosis.

Ishimaru S, Tsujino I, Takei T, Tsukamoto E, Sakaue S, Kamigaki M, Ito N, Ohira H, Ikeda D, Tamaki N, Nishimura M.

First Department of Medicine, Hokkaido University School of Medicine, N15, W7, Kita-ku, Sapporo, Hokkaido 060-8638, Japan.

AIMS: To evaluate the value of (18)F-fluoro-2-deoxyglucose positron emission tomography ((18)F-FDG PET) in detecting cardiac sarcoidosis. METHODS AND RESULTS: Thirty-two patients with sarcoidosis and thirty controls were recruited. All subjects underwent cardiac (18)F-FDG PET after a 6 h fasting period, and subjects with sarcoidosis underwent blood testing, ECG, echocardiography, and (67)Ga and (99m)Tc-sestamibi (MIBI) scintigraphy. We classified (18)F-FDG PET images into four patterns ('none', 'diffuse', 'focal', and 'focal on diffuse') and found that all the control subjects exhibited either none (n = 16) or diffuse (n = 14) pattern. In contrast, fifteen subjects with sarcoidosis exhibited none, seven exhibited diffuse, eight exhibited focal, and two ex hibited focal on diffuse patterns, with the prevalence of the focal and focal on diffuse patterns being significantly higher in the sarcoidosis group when compared with the control group (P < 0.001). None of the 32 subjects with sarcoidosis exhibited abno rmal findings on (67)Ga scintigraphy, and 4 exhibited abnormal findings on (99m)Tc-MIBI scintigraphy. CONCLUSION: Focal uptake of the heart on (18)F-FDG PET images is a characteristic feature of patients with sarcoidosis. Furthermore, (18)F-FDG PET has th e potential to detect cardiac sarcoidosis that cannot be diagnosed by (67)Ga or (99m)Tc-MIBI scintigraphy.

Mod Pathol. 2005 Mar;18(3):451-5.

Sarcoidosis and immunoglobulin lambda II light-chain amyloidosis diagnosed after orthotopic heart transplantation: a case report and review of the literature.

Treaba DO, Benson MD, Assad LW, Dainauskas JR.

Rush Presbyterian St Luke's Medical Center, Chicago, IL, USA.

Cardiac involvement by sarcoidosis and concomitant deposition of AL amyloid is an uncommon association. We describe the case of a 53-year-old African-American man with a 7-year history of dilated nonischemic cardiomyopathy and severe cardiac failure who u nderwent orthotopic heart transplantation. His prior cardiac biopsies had only mild myocyte hypertrophy and minimal interstitial fibrosis. After surgery, numerous sarcoid granulomas and amyloid deposition were identified in the native heart. Six days afte r the transplant the patient died due to aspiration bronchopneumonia and acute renal failure. At autopsy, both sarcoidosis and immunoglobulin (Ig) lambda light-chain amyloidosis were present in the native atria, lungs, thyroid, liver, spleen, and kidneys. Sarcoid granulomas alone were present in the parathyroids, lymph nodes, and bone marrow. Amyloid deposition alone was present in the aorta, stomach, large bowel, and urinary bladder. There was no evidence of plasma cell dyscrasia, or underlying gammopath y. This unusual association was described in only two other cases in the medical literature. However, this is the first case of sarcoidosis and AL amyloidosis with successful sequencing and identification of Ig lambda light-chain amyloid, and in which the re was no evidence of plasma cell dyscrasia.

Medicine (Baltimore). 2004 Nov;83(6):315-34.

Cardiac sarcoidosis: a retrospective study of 41 cases.

Chapelon-Abric C, de Zuttere D, Duhaut P, Veyssier P, Wechsler B, Huong du LT, de Gennes C, Papo T, Bletry O, Godeau P, Piette JC.

Service de Medecine Interne, Hopital de la Pitie, Paris, France.

This retrospective study concerned 18 female and 23 male patients with cardiac sarcoidosis (CS). The average age at CS diagnosis was 38 years. CS was observed in white (73% of cases) and in black or Caribbean patients (27% of cases). All patients had extr acardiac histologic proof of sarcoid tissue. In 63% of cases, the CS arose during the follow-up of systemic sarcoidosis. Systemic sarcoidosis was not specific except for a high frequency of neurosarcoidosis. Revealing cardiac signs were clinical in 63% of cases and electrical in 22%. In most patients these signs were associated with an abnormal echocardiography (77%) and/or a defect on thallium-201 or sestamibi imaging (75%). Thirty-nine patients received steroid therapy (initial dose mostly equal to 1 mg /kg per day), associated in 13 cases with another immunosuppressive treatment. In 26% of cases the immunosuppressive treatment was associated with a specific cardiac treatment. In the long-term follow-up (average follow-up, 58 mo), 87% of the cases showed an improvement, and 54% were cured from a clinical and laboratory point of view (electrocardiogram, 24-hour monitoring, echocardiography, radionuclide imaging). There was no sudden death. Two patients worsened, which can be explained in 1 case by very la te treatment and in the other case by lack of treatment, except for a pacemaker. Our experience leads us to treat CS with corticosteroids as soon as possible and to use another immunosuppressive treatment where there is an insufficient therapeutic respons e or where there are contraindications to corticosteroids.

Clin Cardiol. 2004 Apr;27(4):217-22.

Ventricular tachyarrhythmia associated with cardiac sarcoidosis: its mechanisms and outcome.

Furushima H, Chinushi M, Sugiura H, Kasai H, Washizuka T, Aizawa Y.

The First Department of Internal Medicine, Niigata University School of Medicine, Niigata, Japan.

BACKGROUND: Cardiac sarcoidosis is increasingly recognized and is associated with poor prognosis. Ventricular tachycardia (VT) associated with cardiac sarcoidosis is the most likely cause of sudden death in most patients, but the mechanism has not been we ll established. HYPOTHESIS: This study investigated the mechanisms and outcome of VT associated with cardiac sarcoidosis. METHODS: The study included eight consecutive patients (five men, three women, aged 54 +/- 19 years) who had sustained monomorphic VT associated with cardiac sarcoidosis in our hospital. RESULTS: The average ejection fraction was 43 +/- 11%. Twenty-two VTs were observed in these patients, and mean heart rate during VT was 192 +/- 29 beats/min (range 144-259). The phenomenon of transien t entrainment was documented in 10 of 22 (45%) VTs by ventricular pacing (eight in the active phase). Another five (23%) VTs could not be entrained, but could be initiated by programmed stimulation and terminated by rapid pacing, reproducibly. In 3 of the 22 (14%) VTs, cardioversion was required urgently because of the fast rate, while the remaining 4 (18%) could be induced during electrophysiologic study. CONCLUSIONS: In this study, there was a high possibility that the mechanism of 15 (68%) VTs was reen try. Reentrant substrate is formed not only in association with the healing of cardiac granulomas in the inactive phase of cardiac sarcoidosis but also in the active phase. Ventricular tachycardia with cardiac sarcoidosis, even if this mechanism is reentr y, has different inducibility between the active and inactive phases in an electrophysiologic study. This makes the therapy for cardiac sarcoidosis (e.g., corticosteroids, antiarrhythmic agents, and catheter ablation) difficult. The implantable cardiovert er-defibrillator is an effective treatment for ventricular tachyarrythmia with cardiac sarcoidosis.

Rev Med Interne. 2004 May;25(5):357-62.

Diagnosis of cardiac sarcoidosis and follow-up of 24 consecutive patients.

Paule P, Braem L, Heno P, Miltgen J, Verrot D, Fourcade L, Bonnet D.

Service de cardiologie, hopital d'instruction des armees Alphonse-Laveran, boulevard Laveran, 13998 Marseille Armees, France.

PURPOSE: Cardiac sarcoidosis is responsible for 50% of deaths which mainly occur by ventricular arrhythmia or conduction disorders. The aim of this study is to determine the value of cardiac explorations for an early diagnosis of these localizations, whic h are often underestimated and can cause sudden death. PATIENTS AND METHODS: We prospectively studied 24 consecutive patients, aged 33 +/-10 years, presenting with a sarcoidosis. Nine (38%) were asymptomatic and had no treatment. Fifteen (62%) were sympto matic: two (8%) had only pulmonary lesions and 13 (54%) had a polyvisceral disease. Seven (30%) were treated. Thirteen (54%) had an elevation of the disease activity markers. The patients had a 12-lead ECG, an echocardiography (TTE), a Holter ECG and a Ma gnetic Resonance Imaging (MRI) at inclusion. RESULTS: Realization rate was: 100% ECG (24), 83% TTE (20), 75% Holter ECG (18) and 62% MRI (15). Only two patients (8%) had a cardiac involvement. The first one had a polyvisceral sarcoidosis presenting with a hypokinetic cardiomyopathy and a complete AV block and the second one presented with a complete AV block which revealed sarcoidosis. Both patients had a MRI septal hypersignal and disease activity markers. They were treated with cardiac stimulation and c orticotherapy: the first patient died suddenly, the second one remains asymptomatic after a 14 months follow-up. The 22 patients (92%) with normal explorations did not present any cardiac involvement during the follow-up (3.7 +/-1.6 years). CONCLUSIONS: T his study confirms the rarity of cardiac involvement in sarcoidosis. An exhaustive cardiac check-up does not seem very productive even for patients presenting with polyvisceral disease or an elevation of disease markers. A systematic 12-lead ECG seems to be the most useful and simple tool for the early diagnosis of cardiac sarcoidosis. The other explorations will be realized according to clinical data. The absence of abnormal findings seems to have a good negative predictive value allowing to rule out a c ardiac problem.

J Assoc Physicians India. 2004 Mar;52:210-4.

Uncommon manifestations of sarcoidosis.

Sharma SK, Mohan A.

Department of Medicine, All India Institute of Medical Sciences, New Delhi 110 029, India.

OBJECTIVE: To study the uncommon manifestations in patients of sarcoidosis. METHODS: This is a prospective study of uncommon manifestations observed in 93 of the 210 biopsy proved patients (mean age 43.8 +/- 6.4 years; 58 males) of sarcoidosis seen by us over the last 22 years at the All India Institute of Medical Sciences, New Delhi. RESULTS: Clinical presentation of acute sarcoidosis in the form of Lofgren's syndrome (n = 5) was uncommonly observed. Rare pulmonary manifestations included seasonal dyspno ea with audible wheezing mimicking bronchial asthma (n = 30); narrowing of main bronchi, fixed upper airways obstruction (n = 1); pleural involvement (n = 7); bullous lung disease (n = 2); chronic respiratory failure (n = 2); and vocal cord involvement (n = 4). Other notable features were glaucoma (n = 4); digital clubbing (n = 3); sarcoid nephritis (n = 1); and sicca syndrome (n = 1). Uncommon neurological manifestations included bilateral sequential facial nerve palsy (n = 2); optic atrophy (n = 2); opt ic neuritis, proximal myopathy, multiple brainstem lesions, pituitary stalk lesion (one patient each). Cardiac involvement occurred in 15 patients. This included complete heart block (n = 2); congestive heart failure (n = 4); supraventricular ectopics (n = 6); ventricular ectopics (n = 2); and recurrent ventricular tachycardia requiring radiofrequency ablation (n = 1).

Angiology. 2005 Jan-Feb;56(1):115-7.

A rare type of alternating bundle branch block in a patient with cardiac sarcoidosis--a case report.

Konno T, Shimizu M, Ino H, Araki T, Yamaguchi M, Inoue M, Tofuku Y, Mabuchi H.

Molecular Genetics of Cardiovascular Disorders, Division of Cardiovascular Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan. kontetsu@im2.m.kanazawa-u.acjp

The authors report a rare type of alternating bundle branch block observed in a patient with cardiac sarcoidosis. Not only alternation of complete right and left bundle branch block but also narrow QRS complexes were observed on electrocardiogram. The mec hanism of these unusual findings is briefly discussed.

Neth J Med. 2005 Feb;63(2):70-3.

Sarcoidosis mimicking ischaemic ventricular arrhythmia and pulmonary embolism.

de Jager CP, Jessurun ER, Jansen EK, Verheij J, Girbes AR, Strack van Schijndel RJ.

Department of Intensive Care, VU University Medical Centre, Amsterdam, The Netherlands.

Sarcoidosis is a multisystem granulomatous disorder characterised pathologically by the presence of noncaseating granulomas in the organs involved. Cardiac involvement, although well known, is rare. We describe a 72-year-old patient who was admitted to th e intensive care unit after coronary artery bypass grafting. She developed refractory right and left ventricular failure complicated by multiple organ failure and died three days later. Postmortem examination revealed extensive sarcoidosis. On hindsight, preoperative ventricular tachycardia and an abnormal perfusion-ventilation scintigraphy of the lungs were manifestations of an underlying sarcoidosis.

AJR Am J Roentgenol. 2005 May;184(5):1420-6.

Myocardial late enhancement in contrast-enhanced cardiac MRI: distinction between infarction scar and non-infarction-related disease.

Hunold P, Schlosser T, Vogt FM, Eggebrecht H, Schmermund A, Bruder O, Schuler WO, Barkhausen J.

Department of Diagnostic and Interventional Radiology and Neuroradiology, University Hospital Essen, Hufelandstrasse 55, 45122 Essen, Germany.

OBJECTIVE: Our objective was to assess and compare the patterns of late enhancement (LE) in contrast-enhanced cardiac MRI caused by myocardial infarction and different myocardial diseases that are not related to ischemic infarction. MATERIALS AND METHODS: A total of 811 consecutive contrast-enhanced cardiac MRI studies performed for different indications were reviewed for left ventricular myocardial LE after gadopentetate dimeglumine administration. MRI studies were performed on a 1.5-T scanner using an i nversion recovery turbo FLASH sequence (TR/TE, 8/4 msec; flip angle, 25 degrees). The LE pattern of ischemic infarction scar was compared with that in nonischemic myocardial disease. RESULTS: LE was found in 421 (52%) patients. In all patients with myocar dial infarction, LE included the subendocardial layer. Nineteen patients without history of myocardial infarction and angiographically excluded coronary artery disease showed different patterns of LE caused by myocarditis, sarcoidosis, arrhythmogenic righ t ventricular dysplasia, cardiomyopathy, endomyocardial fibrosis, and iatrogenic scars after biopsy, ablation of septal hypertrophy, and myocardial laser revascularization. CONCLUSION: LE in contrast-enhanced cardiac MRI is not specific for ischemic infar ction. LE in ischemic infarction always involves the subendocardial layer, whereas it does not necessarily do so in other myocardial diseases. Therefore, if LE omit the subendocardial layer, different nonischemic myocardial diseases have to be considered. The pattern of LE might be helpful for the differential diagnosis of myocardial disease and in distinguishing it from ischemic disease.

Schweiz Rundsch Med Prax. 2004 Oct 13;93(42):1727-32.

The red face.

Schuster Ch, Burg G.

Dermatologische Klinik, Universitatsspital Zurich.

Facial erythema may not only present clinically as a distinct entity, but can also be a symptom of other diseases. It is seen in common dermatoses such as eczema, psoriasis, acne and urticaria, as well as in rarer conditions such as disorders of keratiniz ation, infectious diseases, porphyrias and neoplasia. Facial erythema may also present as a symptom of carcinoid syndrome, drug allergies, cardiac disease or in rare cases as a feature of Bloom's syndrome, sarcoidosis, lymphoma, amyloidosis and other dise ase processes. We would like to concentrate on the practical aspects of facial erythema as a presenting symptom, rather than discussing every disease in detail.

Can J Cardiol. 2004 Jan;20(1):89-93.

Sarcoid heart disease.

Syed J, Myers R.

Internal Medicine and Cardiology, University of Toronto, Toronto, Ontario, Canada.

BACKGROUND: Sarcoidosis is a multisystem inflammatory disease of unknown cause. Due to its protean and nonspecific clinical manifestations, the optimal diagnostic and therapeutic strategies, as well as prognosis, are not well defined. OBJECTIVE: To review the literature on sarcoid heart disease and present an approach to its diagnosis, prognosis and therapy. METHODS: MEDLINE and PreMEDLINE searches of all available English-language articles and case reports from 1966 to 2002 were performed. Search terms i ncluded 'sarcoidosis' and 'heart diseases', with a variety of medical subject subheadings. RESULTS: Clinical disease often includes heart block, dilated cardiomyopathy and ventricular arrhythmias. Patients with sarcoidosis are at increased risk of sudden death. Because the yield of endomyocardial biopsy for definitive diagnosis is low, the diagnosis is often made with a combination of electrocardiography, Holter monitoring, echocardiography, myocardial perfusion imaging and, most recently, magnetic resona nce imaging. For symptomatic patients, medical therapy may include a trial of steroids and immunosuppressive therapy. Monoclonal antibodies against tumour necrosis factor may be employed in refractory cases. Heart block warrants a permanent pacemaker, whi le ventricular tachyarrhythmias are typically amiodarone-unresponsive, requiring implantation of an implantable cardioverter defibrillator. CONCLUSIONS: Although there are no large, randomized trial data on cardiac sarcoidosis, the literature suggests tha t making the diagnosis is clinically important. Unlike the situation in isolated pulmonary disease, cardiac involvement implies a poor prognosis. The diagnosis often requires high clinical suspicion with a combination of radiological and nuclear imaging. Therapy with steroids may halt progression of left ventricular dysfunction, while arrhythmias warrant device therapy.

Int J Cardiol. 2004 Nov;97(2):173-82.

Sarcoid heart disease: clinical course and treatment.

Bargout R, Kelly RF.

Division of Adult Cardiology, Cook County Hospital, Chicago, IL 60612, USA.

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 tr eatment 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.

Circ J. 2002 May;66(5):519-21.

Spontaneous development of left ventricular aneurysm in a patient with untreated cardiac sarcoidosis.

Haraki T, Ueda K, Shintani H, Hayashi T, Taki J, Mabuchi H.

Department of Internal Medicine, Komatsu Municipal Hospital, Japan.

A young adult patient with untreated sarcoidosis spontaneously developed a left ventricular (LV) aneurysm in the anterolateral free wall. Single-photon emission computed tomography (SPECT) using Gallium-67 clearly demonstrated widespread abnormal uptake, including the LV aneurysm. Thallium-201 SPECT revealed a perfusion defect in the anterolateral wall, and abnormal uptake of technetium-99m pyrophosphate was seen, especially in the borders of the defect lesion.

Arch Mal Coeur Vaiss. 2001 Jul;94(7):739-42.

Localization of isolated atrial involvement of cardiac sarcoidosis.

Phung H, Leroy G, Bouksibi F, Meddane M, Fuchs L, Lewy P, Mouseaux E, Halphen C, Haiat R.

Service de cardiologie et urgences cardiovasculaires, CHI Poissy-Saint-Germain-en-Laye, 20, rue Armagis, 78105 Saint-Germain-en-Laye.

The authors report the first case of cardiac sarcoidosis involving only the interatrial septum and the atrioventricular node in a 30 year old man with known lymph node sarcoidosis. The diagnosis was suspected after the finding of an apparently innocent 1s t degree atrioventricular block and confirmed by transoesophageal echocardiography and by IMATRON computerised tomography. Isolated atrial involvement of sarcoidosis is rare in the absence of any left ventricular disease. This explains the negative findin gs at transthoracic echocardiography and radio-isotopic investigations usually recommended for diagnosing cardiac sarcoidosis.

Am Heart J. 1997 Sep;134(3):382-6.

Incidence of cardiac sarcoidosis in Japanese patients with high-degree atrioventricular block.

Yoshida Y, Morimoto S, Hiramitsu S, Tsuboi N, Hirayama H, Itoh T.

Cardiovascular Center, Nagoya Dai-ni Red Cross Hospital, Japan.

In Japan the majority of sarcoidosis-related deaths are due to cardiac sarcoidosis. One of the most common electrocardiographic abnormalities in patients with this disease is atrioventricular block. This study surveyed the incidence of cardiac sarcoidosis in Japanese patients (40 men and 49 women; mean age, 69.1 years) with high-degree atrioventricular block who were admitted to the hospital to receive a permanent pacemaker. We excluded cases in which sarcoidosis had been diagnosed from the involvement of other organs. Patients with the characteristic signs of sarcoidosis underwent echocardiography, radionuclide imaging, and biopsy. Ten cases (11.2%) of cardiac sarcoidosis were diagnosed, most frequently in women aged 40 to 69 years (8 of 25, 32%). Thus t he possibility of cardiac sarcoidosis should be carefully considered in middle-aged or elderly Japanese women who show high-degree atrioventricular block.

Clin Cardiol. 1995 Apr;18(4):234-5.

A case of cardiac sarcoidosis with remarkable atrophy of the left ventricular septum on two-dimensional echocardiography.

Matsumura A, Yanase O, Motomiya T, Tokuyasu Y, Sakurada H, Nomura S, Teshima T, Hiyoshi Y, Sugiura M.

Department of Cardiology, Tokyo Metropolitan Hiroo General Hospital, Japan.

Sarcoidosis is a multisystem granulomatous disorder of unknown etiology which mainly affects the lungs, skin, the lymphoreticular system, and the heart. We report a case of cardiac sarcoidosis in which a remarkably thin ventricular septum was demonstrated on two-dimensional echocardiography.

Chest. 1996 Jan;109(1):62-6.

Doppler echocardiographic detection of left ventricular diastolic dysfunction in patients with pulmonary sarcoidosis.

Fahy GJ, Marwick T, McCreery CJ, Quigley PJ, Maurer BJ.

Department of Cardiology, University College and St. Vincent's Hospital, Dublin, Ireland.

STUDY OBJECTIVE: To determine the prevalence of left ventricular diastolic dysfunction in patients with biopsy specimen-proved pulmonary sarcoidosis without clinical evidence of cardiac disease. DESIGN: A cross-sectional study. SETTING: A large tertiary c are university teaching hospital. PATIENTS AND CONTROL SUBJECTS: Fifty consecutive subjects had biopsy specimen-proved pulmonary sarcoidosis without suspected cardiac involvement. Those with other conditions known to affect diastolic function were exclude d. The control group comprised 30 healthy hospital workers. INTERVENTIONS: Clinical examination, 12-lead ECG, and combined echocardiographic/phonocardiographic examination. MEASUREMENTS: Indexes of left ventricular diastolic function, including isovolumic relaxation time, peak velocity of early (E) and late (A) ventricular filling, deceleration rate of early diastolic flow, and the sum of the time velocity integrals of E and A were obtained in each patient and control subject. Systolic function was determ ined using a modification of Simpson's rule. RESULTS: Diastolic dysfunction was present in 7 (14%) patients, 6 of whom had normal systolic function and normal two-dimensional echocardiographic examination. Those with diastolic dysfunction had a longer dur ation of illness (15 +/- 7 vs 6 +/- 5 years; p = 0.0004), were significantly older (52 +/- 11 vs 38 +/- 9 years; p = 0.0009), and had higher systolic BP (130 +/- 13 vs 117 +/- 12 mm Hg; p = 0.01) than the sarcoid patients with normal diastolic function. C ONCLUSIONS: These results demonstrate a significant prevalence of left ventricular diastolic dysfunction in patients with pulmonary sarcoidosis. The cause of this abnormality may be a subclinical sarcoid cardiomyopathy.

Lijec Vjesn. 1992 May-Aug;114(5-8):137-42.

Echocardiography in the diagnosis of cardiac sarcoidosis.

Sarac R, Agbaba-Primorac R, Rozman A, Gomercic D.

Magistra medicinskih znanosti, Bolnica za bolesti pluca, Zagreb.

This paper deals with echocardiographic changes in the heart in patients with active intrathoracic sarcoidosis. Heart changes were registered with 10 (38%) out of 26 patients, in all radiological stages of sarcoidosis. Out of 16 patients first radiologica l stage (BHL) was found with 4 (25%), out of 8 patients second radiological stage was found with 4 (25%), and third radiological stage was found with 2 patients. Out of 10 patients with echocardiographic changes in the heart 4 patients (40%) had increased echoes and hypokinesis of the proximal part of interventricle septum, 3 (30%) had significantly reduced diffusive capacity and pulmonary hypertension. Increased echoes of papillary muscles were registered in two cases, one of them with relatively mitral insufficiency. In one case dilatational incipient cardiomyopathy with second stage of relatively mitral insufficiency was found and in one case increased echoes and hypokinesis of the back wall of the left ventricle were registered. Changes in electrocard iogram were found with 5 (19%) patients suffering from pulmonary sarcoidosis.

Postgrad Med J. 1992;68 Suppl 1:S52-6.

Doppler echocardiographic evaluation of left ventricular diastolic function in patients with systemic sarcoidosis.

Angomachalelis N, Hourzamanis A, Vamvalis C, Gavrielides A.

Aristotle University of Thessaloniki, Department of Internal Medicine, G. Papanicolaou General Hospital, Macedonia, Greece.

In order to estimate left ventricular diastolic function in systemic sarcoidosis, Doppler echocardiography was utilized in 10 normal female subjects (aged 45 +/- 13 years) and in 10 female patients (aged 48 +/- 14 years), with biopsy proven sarcoidosis an d without any clinical evidence of other cardiac disease or hypertension. Left ventricular systolic function assessed by two-dimensional echocardiography was normal in both groups (ejection fraction greater than 60%). The following Doppler echocardiograph ic parameters were measured: peak early diastolic flow velocity (E), peak late atrial diastolic flow velocity (A), the ratio (E/A) and deceleration of the flow velocity in early diastole (E-F slope). The results showed that 5 patients had two or more abno rmal Doppler diastolic indexes, indicating impaired left ventricular relaxation, while the remaining 5 patients had values within or just outside normal limits. The mean values for the patients as a whole showed increased A velocity (0.77 +/- 0.11 m/s ver sus 0.61 +/- 0.10 m/s; P + 0.05), decreased E/A ratio (1.05 +/- 0.32 versus 1.4 +/- 0.30; P + 0.01), normal E velocity and normal E-F slope, compared with the mean values of the normal group. We concluded that a large percentage (50%) of patients with sys temic sarcoidosis, had reduced diastolic performance of the left ventricle, at a stage of the disease when systolic dysfunction is not yet evident.

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