CARDIAC SARCOID

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CARDIAC SARCOID

• Dr. Prateek Suri

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Key Points

• Cardiac involvement in sarcoidosis
• Diagnosing cardiac sarcoid
• Role of cardiac biopsy
• Role of MRI
• Treatment options
• prognosis

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INITIAL PRESENTATION

• NOV 2003 45 Yr old patient referred with a 2
  month history of progressive SOBE,PND,orthopnea
  to Maitland hospital

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B/G

• No history of previous cardiac disease
• Non smoker
• No h/o dm,htn,dyslipidemia
• No family history of IHD,Cardiomyopathy
• No preceding viral infection

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B/G

• Biopsy proven pulmonay sarcoidosis 1996 after
  asymptomatic lymph nodes were detected in
  screening chest Xray-on no treatment.
• Coal mine worker by profession 20 yrs

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O/E

• BP -120/75
• PR- 100/MIN REGULAR
• B/L Pedal oedema
• Jvp- elevated
• Chest b/l inspiratory crackles

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lab

• Fbc/euc normal
• Serum ACE 36 nmol/L(N32)
• Cxray cardiomegaly with interstitial oedema

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ECHO

• LV dilatation with severe lv dysfunction
• Restrictive ventricular filling
• Moderate left atrial enlargement
• Moderate mitral regurgitation
• Moderate pulmonary hypertension.

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TREATMENT

• STARTED on lasix ,irbesartan,and carvedilol
• Symptoms improved and patient discharged and
  given an appointment to see Dr. Bastian.

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Cardiac cathetorisation 2004

• RA (a,v,y,m)20,4.12,4.11,11.7
• RV (s,d,a) 71,-3,11
• PA (s,d,m) 70,31,50
• PAW(a,v,y,m)32.3,37,28.3
• LV(s,d,a)98,-17,34
• Normal coronary arteries

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2004 Cardiac Mri

• Several enlarged aortopulmonary and subcarinal
  lymp nodes
• Cardiomegaly with biventricular dilatation
• Marked thinning of left ventricular posterior
  wall.

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Role of MRI

• Cardiac sarcoidosis is associated with patchy
  LGE(late gadolinium enhancement) that does not
  correspond to a coronary artery territory In
  addition, zones with increased intensity may be
  seen on T2-weighted images suggestive of
  myocardial edema, and septal and left ventricular
  wall thickness may be increased. The size and
  signal intensity of LGE may be reduced with
  steroid treatment

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Pattern of LGE

• Ischaemic its usually subendocardial or
  transmural in the distribution of a vascular
  territory
• Non ischaemic- patchy and predominantly
  epicardial or mid anterior wall.

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How good is MRI

• Senstivity 100 and specificity 78 in a series
  of 19 patients (american j.cardiology 2005)

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LUNG FUNCTION TESTS

• PRED ACTUAL
• FEV1 4.16 3.87 93
• FVC 5.15 5 97
• FEV1/FVC 81 77 96
• VC 5.38 5.14 96
• FRC 3.64 3.17 87
• TLC 7.70 7.08 92
• DLC0 4.84 4.11 85

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Medications now

• Digoxin
• Irbesartan
• Lasix
• Carvedilol
• spironolactone

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TRIAL OF STEROIDS 2006

• After being started on 50 mg of
  prednisolone,patient was tapered to receive 15 mg
  daily
• Some improvement in Sob and patient referred for
  cardiac transplant.

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• Patient turned down by transplant team in Sydney
  and they suggested prophylactic defibrillator and
  felt mitral valve repair would probobaly not
  benefit.

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June 2006

• Single chamber AICD inserted in 2006 for
  prophylaxis

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May 2007

• Patient represented with heart failure
• Initially given inotropes
• Cardiac synchrony studies carried out and
  dyssynchrony index 8 and patient was upgraded to
  biventricular pacing

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MULTIPLE PRESENTATIONS WITH SYMPTOMATIC HEART
FAILURE

• Patient kept presenting to Maitland and jhh with
  heart failure
• By now developed steroid complications diabetes
  ,osteoporosis,
• Renal dysfunction secondary to diuretics

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Jan 09

• Patient represented with heart failure
• By now patient had developed diabetes and was on
  gliclazide and januvia(sitagliptin)

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Discharge medications

• Hydrochlorthiazide was added .
• Spironolactone dose was increased
• Irbesartan was halved
• Januvia(sitagliptin) was ceased.

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• Patient improved and discharged
• Follow up arranged with Dr. Bastian

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SARCOIDOSIS

• A disease of unknown aetiolgy characterised by
  the presence of non caseating granulomas in the
  body leading to multisystem organ dysfunction.

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HISTORY

• In 1899 Caesar Boeck a Norvegian dermatologist
  coined the term to describe skin nodules
  characterised by compact sharply defined foci of
  epitheloid cells and giant cells that resembled
  sarcoma

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EPIDEMIOLOGY

• Peak incidence is between 20 -40 yrs
• Northern Europe 5- 40/100,000
• Japan 1-2/100,000
• U.S. blacks 35.5/100,000 and 10.9/100,000 in
  whites
• Slight female preponderance
• Only 5 people with sarcoid develop clinical
  cardiac involvement however nearly 25 people
  have involvement on autopsy

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Guidelines for the diagnosis of cardiac
sarcoidosis
Adapted from Hiraga, H, Yuwai, K, Hiroe, M, et
al. Guidelines for the diagnosis of cardiac
sarcoidosis Study Report of Diffuse Pulmonary
Diseases. The Japanese Ministry of Health and
Welfare 1993 pp. 23-24.
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Cardiac involvement

• Conduction abnormalities
• Ventricular arrhythmias
• Heart failure
• Valvular dysfunction
• Supraventricular arrhythmias

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Heart failure

• Extensive granulomatous inflamation of the
  myocardium gives rise to both systolic and
  diastolic dysfunction.
• Steroid treatment can convert granulomas to scar
  tissue and contribute to aneurysmal dilatation.
• Progressive heart failure accounts for between
  25- 75 of deaths in different series .

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Role of cardiac biopsy

• An established diagnostic tool since its
  introduction in1962.
• Problems with biopsy include
• -non homogeneous cardiac
• involvement
• -sarcoid granulomas tend to be
• basal whereas biopsy is usually
• obtained from apical septum
  

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Net effect

• There is a low sensitivity approaching about 20
  in a series of 26 patients.(american heart jounal
  1999)

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ROLE OF thallium scanning

• Segmental areas of decreased uptake correspond to
  fibrogranuolomatous involvement
• In contrast to CAD ,the perfusion defects
  decrease in exercise during exertion.

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Treatment options

• Steroids
• Other immune suppressive agents
• Use of AICD
• Cardiac transplant.

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ROLE OF AICD

• For primary prevention in symptomatic patients
  with EF lt35
• Secondary prevention in patients who have
  survived sudden death or refractory VT

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ALTERNATE IMMUNE SUPPRESIVES

• Methotrexate can be used as an alternative to
  steroids when there are side effects however no
  differences in survival or disease outcomes were
  noted.(cochrane data base 2006)

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Cardiac transplant for sarcoid accounts for 1 of
all cardiac transplants

• 65 patients database of patients undergoing
  transplant with a mean age of 46 from the united
  network for organ sharing between 1986 and 2005 1
  yr post transplant survival was 87.7 compared to
  84.5 in other groups
• 5 yr survival rate 80.

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Prognosis

• 5 yr survival was 75 compared to 10 for
  patients not treated with steroids(American .j.
  cardiology 2006) in a report involving 75
  patients
• Best out come was noted in patients in whom
  steroids were started when EF gt50

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Thank you.