Cardiac Sarcoidosis Millee Singh DO - PowerPoint Presentation

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Cardiac Sarcoidosis

Millee Singh DO Non Invasive CardiologyFirst Coast Heart and Vascular

Slide2

Introduction

Multisystem granulomatous disease of unknown etiology characterized by

non-

caseating

granulomas in involved organs The prevalence is 10-40/100,000 persons in the United States and Europe Increased prevalence in African- Americans compared to Caucasians with a ratio ranging from 17 : 1

Sekhri

et al. Arch

Med

Sci

2011; 7, 4: 546-554

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Introduction

Sarcoidosis is more prevalent in women than in men

Organs involved Include :

Lymph

nodes, skin, lung, central nervous system, and eye Cardiac involvement in sarcoidosis occurs in 20- 30% of patients in reported pathology seriesA review of 84 autopsy cases of pulmonary sarcoidosis and found myocardial granulomas in 27% of patients In Sarcoidosis, deaths from

Cardiac sarcoid appear to have a regional connection :

United States, 13 to

25% Japan, 47 to 85%

Sekhri

et al. Arch

Med

Sci

2011; 7, 4: 546-554

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Introduction

Genetic link:A Case Controlled Etiologic Sarcoidosis Study (ACCESS) concluded that first-degree relatives of patients with sarcoidosis had a relative risk of sarcoidosis that was

five times

that of control subjects

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Pathophysiology

Three aspects :Exposure

to

antigen

Acquired cellular immunity directed against the antigen mediated through antigen presenting cells and antigen specific T lymphocytesAppearance of immune effector cells that promote a more nonspecific inflammatory responseALL LEADING TO GRANULOMA FORMATION AND PROGRESSION TO FIBROSIS OF TISSUE

Sekhri

et al. Arch

Med

Sci 2011; 7, 4: 546-554

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Arch Med

Sci

4, August / 2011

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Pathology

Characteristic lesion of sarcoidosis is a discrete, compact,

noncaseating

epitheloid cell granulomaCardiac sarcoidosis is associated with noncaseating granulomas which may involve the left ventricular free wall, basal ventricular septum, right ventricle, papillary muscles, right atrium, and left atrium 3 successive histological stages: Edema Granulomatous infiltration

F

ibrosis leading to post- inflammatory scarring

Roberts et al. ;Am J Med 1977; 63: 86-108

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Pathology

Pathological slides: Myocardium show the presence

of numerous lymphocytes located at the border zones around the

granulomas

A dense band of fibroblasts, collagen fibers, and pro- teoglycans usually encase this aggregate of inflammatory cells

Slide9

Clinical

Presensation/Diagnosis Symptoms which include fever, fatigue, malaise, and weight loss

can be very vague presentation overall

When multi

-systemic sarcoidosis has already been diagnosed – Cardiac diagnosis of symptoms are easier to diagnosedWhen cardiac dysfunction is the only manifestation of sarcoidosis, the diagnosis is frequently not entertained

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Clinical Presentation/Diagnosis

Sekhri

et al. Arch

Med

Sci

2011; 7, 4: 546-554

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Clinical Presentation/Diagnosis

Early diagnosis and treatment is essential since treatment improves

prognosis

Serious cardiac dysfunction is detected in 5% to 10% of

cases In a significant proportion of patients with cardiac sarcoidosis, the initial presentation is sudden deathSarcoid granulomas may serve as foci for abnormal automaticity or reentrant tachycardiasAtrial arrhythmias are less common 15-17% of casesResult

of atrial dilatation or pulmonary involvement rather than the result of atrial granulomas

Fleming HA. Sarcoid heart disease. Br Heart J 1974; 36: 54-68.

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Complete Heart Block

Occurs in younger age in patients with sarcoidosis than in patients with complete heart block of other causes Rate of occurrence :

Complete

heart

block - 23-30%Bundle branch block 12-32%Caused by the involvement of basal septum by scar tissue, granulomas, or involvement of the nodal artery causing ischemia in the conduction system Sarcoidosis pts who present with syncope/pre- syncope should be evaluated for CHB

Yoshida

et al.

Am Heart J 1997; 134: 382-6.

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Ventricular arrhythmias

Sudden death caused by ventricular tachyarrhythmia's may account for 25-65% of deaths caused by cardiac sarcoidosis

M

ay

be the initial presentation in 40% of patients with cardiac sarcoidosis

Sekiguchi

et al.

I Sarcoidosis. Jpn Circ J 1980; 44: 249-63.

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Congestive Heart

Failure

Accounts for 25

% to 75% of cardiac deaths in patients with cardiac

sarcoidosis Heart failure may be secondary to left- sided cardiac involvement with either systolic or diastolic dysfunction DDx: Idiopathic dilated cardiomyopathy (IDC) can be difficult to differentiateSarcoidosis HF tend to have more progression to CHB then IDC

Yazaki

et al. Am

J Cardiol 2001; 88: 1006-10.

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Other Cardiac Manifestations

Pericardial Effusions< 10 % of Cases- Small in sizeValvular

Involvement

< 3 % , mostly Mitral Regurgitation noted

Ventricular Aneurysms 10% of casesAnterior and septal segmentsUsually associated with steroid treatment for sarcoid rather than a direct cause

Garrett et al; Am

Heart J 1984; 107:

394

Pesola et a; Sarcoidosis 1987; 4: 42-4

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Diagnosis

Thorough clinical evaluation

documenting

a syndrome consistent with

sarcoidosisANDBiopsy documentation of the presence of non- caseating granulomas

Uemura

et al. ;Am

Heart J 1999; 138:299

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Endomyocardial

Biopsy “ Gold standard”Definite diagnosis of cardiac sarcoidosis can be made

The sensitivity IS LOW-usually

less than 20%

Despite its low sensitivity, early myocardial biopsy can be considered when the diagnosis of cardiac sarcoidosis is still used

Uemura

et al. ;Am

Heart J 1999; 138:299

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DDX- Differential Diagnosis

Connective tissue diseases Lyme

disease

R

heumatoid arthritisDermatomyositisCardiac amyloidosis alcohol related cardiomyopathy

Sekhri

et al. Arch

Med

Sci 2011; 7, 4: 546-554

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Imaging

Doppler echocardiography A

bnormal

septal

thickening or thinningDilatation of the left ventricle Systolic dysfunction of the left ventricle Radionuclide studies fibrogranulomatous lesions in the myocardium display segmental areas of decreased uptake in nuclear imagingPET

18F

-Fluorodeoxyglucose (FDG) PET appears to detect active cardiac sarcoidosis with high sensitivity

Non-specific for sarcoidosis, and uptake of 18F-FDG is seen in other inflammatory myocardial diseases

Skold

et al. J

Intern Med 2002; 252: 465-

71

.Haywood

et al; J

Natl

Med

Assoc

1983; 63: 478-

82

Fields

et al. ;

South Med J 1990; 83: 339-

42

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Imaging- CMR

Technique of choice in the evaluation of sarcoidosis

zone

of increased

intramyocardial signal intensityMore pronounced on T2 weighted images because of edema These images can be enhanced on gadolinium Focal myocardial thickening is often seen as a result of the edema Delayed enhanced MRI is considered a useful method for the early identification of cardiac sarcoidosis

Tadamura

et al. AJR

Am J Roentgenol 2005; 185: 110-5.

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Treatment and

Prognosis Threshold for treatment is lower because of increased risk of sudden

death

Goal is to reduce inflammation

Corticosteroids, the most common initial therapyShould be started in patients with a definite probability of cardiac sarcoidosis on different imaging studies, even with a negative myocardial biopsy

Chiu

et al.

Am

J Cardiol 2005; 95: 143-6.

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Treatment

Clinical Trials have suggested that Steroid treatments:

(

LVEF) > 55% may prevent LV remodeling and altered cardiac

function Most benefited with LVEF < 54% who showed significant reduction in LV volumes and LVEF improvement LVEF < 30%, steroid therapy did not improve the LV volume or functionEarly/Middle stage disease, steroid therapy may be protective or therapeutic but may not be as effective in the late

stages

Chiu

et al.

Am J

Cardiol

2005;

95

: 143-6.

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Other Treatment Options

Methotrexate

, azathioprine, or cyclophosphamide can be used as steroid sparing agents and in those whose disease is refractory to high-dose

steroids

Improvement in cardiac conduction abnormalities have been seen with the use of infliximab, a TNF alpha inhibitor Avoid using :Anti Arrhythmic drugs- Increased SCDBeta Blockers – Increased Heart BlockICD implantation in Cardiac sarcoid does have a lower threshold then other Heart Failure guidelines

Baughman

et al. Sarcoidosis

Vasc

Diffuse Lung Dis 2001; 18:70-4Lower et al . Am J Med

Sci

1990; 299: 153-

7

Baughman et al. Sarcoidosis

Vasc

Diffuse Lung Dis 2000; 17: 60-

6

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Cardiac

Transplantation Cardiac

transplantation is reserved for end-stage disease unresponsive to medical therapy

Major

indications for cardiac transplantation : Resistant ventricular tachyarrhythmia Severe intractable heart failure ( Stage D)

Valantine

et al. J

Heart Transplant 1987; 6: 244-50.

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KEY POINTS

Cardiac sarcoidosis may occur alone or alongside systemic sarcoidosis but is frequently clinically

silent

Diagnosis is still a major clinical challenges CMR and PET may be suggestive but are not diagnosticEndo-myocardial biopsy lacks sensitivity but still is “ Gold Standard”There is no clear-cut consensus on which diagnostic methodologies are best to evaluate disease presence and

progression

S

uspect cardiac sarcoidosis in young adults with unexplained cardiac clinical manifestations , with a histologic (or clinical) diagnosis of extra-cardiac sarcoidosis, particularly in those who develop arrhythmias, conduction disease, or heart

failure