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Number 11-05: Cardiac Sarcoidosis - A Severe Presentation.
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Number 11-05: Cardiac Sarcoidosis - A Severe Presentation.

Case from: Daniel M Sado,1 Gianni Quarta,1 Viviana Maestrini,1 Alison Muir,1 Michael Ashworth,2 Antonis Pantazis,1 Perry Elliott,1 James Moon1

Institute: 1Department of Inherited Cardiac Disease, The Heart Hospital, 16-18 Westmoreland Street, London, England, W1G 8PH. 2Department of Histopathology, Great Ormond Street Hospital for Children, London, England, WC1N 3JH.

 

Clinical history: A 52 year old gentleman presented with a 3-month history of tiredness, severe reduction in exercise tolerance (NYHA 3), orthopnoea, weight loss and progressive ascites.

 

 

 

Figure 1 - click mouse to enlarge

 

Resting 12 lead ECG (Figure 1) showed an unusual presentation of 2nd degree atrioventricular node block (mobitz 1) with Holter monitoring documenting intermittent 2nd degree atrioventricular node block (mobitz type 2) and 3rd degree atrio-ventricular block

 

 

 

Movie 1 

 

Transthoracic echocardiography (Movie 1) was performed showing mildly dilated left ventricular (LV) dimensions, but severe systolic impairment (EF: 12% using Simpsons Rule). The right ventricle (RV) had moderate dilatation and severe systolic impairment.

A coronary angiogram showed no flow limiting coronary disease.

Cardiovascular magnetic resonance (CMR) was performed to investigate these findings in more detail.

 

   

 Figure 2                                                                                                       Figure 3

 

The Black blood transverse stack images depict thoracic lymphadenopathy (Figure 2) and left basal lung parenchymal changes (Figure 3).

 

  

Movie 2


Cine images show severe bi-ventricular dilatation and systolic dysfunction (Movie 2). The majority of the LV segments were akinetic. Of note, the wall thickness of the dysfunctional segments was not reduced.

On T2 weighted imaging, there was no evidence of oedema (not shown)

 

 

Movie 3  


Late-gadolinium enhancement (LGE) images (Movie 3), revealed extensive fibrosis (some of which was transmural) involving the left and right ventricle.

 

 

Figure 4 

 

Following CMR, an endomyocardial RV biopsy was performed showing non-caseating granuloma consistent with the diagnosis of Sarcoidosis (Figure 4).

The patient was treated with optimisation of his heart failure medication and insertion of a biventricular pacemaker with implantable cardioverter defibrillator. Steroid treatment and Methotrexate were also initiated.

Perspective: CMR is a very useful investigation for patients with impaired left ventricular systolic function, often suggesting the underlying cause. There are a variety of conditions associated with dilated cardiomyopathy and conduction disease: inflammatory/infective (sarcoidosis, giant cell myocarditis, Chagas disease), genetic (laminopathies, end-stage HCM, ARVC, LVNC) and infiltrative/metabolic (Danon disease, AMP-kinase disease, end-stage forms of Anderson-Fabry disease, cardiac amyloidosis, cardiac iron overload).1 CMR is very useful in the differential diagnosis and may guide clinical management, identifying patients that may benefit from invasive procedures like endomyocardial biopsy. In the case described, both cardiac and extracardiac features of sarcoidosis could be identified by CMR, which along with the abnormal ECG findings was highly suggestive of the diagnosis.2-5 This was then confirmed by endomyocardial biopsy, allowing specific therapy to be administered.

References:

1) Lakdawala NK, Givertz MM. Dilated cardiomyopathy with conduction disease and arrhythmia. Circulation. 2010;122:527-34.

2) Doughan AR, Williams BR. Cardiac sarcoidosis. Heart. 2006 Feb;92:282-8.

3) Smedema JP, Snoep G, van Kroonenburgh MP, van Geuns RJ, Dassen WR, Gorgels AP, Crijns HJ. Evaluation of the accuracy of gadolinium-enhanced cardiovascular magnetic resonance in the diagnosis of cardiac sarcoidosis. J Am Coll Cardiol.2005;45:1683-90.

4) Mehta D, Lubitz SA, Frankel Z, Wisnivesky JP, Einstein AJ, Goldman M, Machac J, Teirstein A. Cardiac involvement in patients with sarcoidosis: diagnostic and prognostic value of outpatient testing. Chest. 2008;133:1426-35.

5) Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med. 2007;357:2153-65.

 

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COTW handling editor: Giovanni Quarta 

 

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Re: Number 11-05: Cardiac Sarcoidosis: a Severe Presentation
Thanks,I know the borderline indication, but this patient,'s heart with advanced AV block (and therefore likely to be paced once the device was in) and very poor LV EF, may have suffered from RV pacing only... ...
On: 03/11/2011 By: gquarta Read more?

Re: Number 11-05: Cardiac Sarcoidosis: a Severe Presentation
GQ:Very nice case indeed.Why was Bi-Ventricular pacemaker placed. If the QRS was between 120-130 it is a soft indication for Bi-V unless you had more evidence of Dyssynchrony from 2D Echo. ...
On: 03/10/2011 By: vikasrathi Read more?

Re: Number 11-05: Cardiac Sarcoidosis: a Severe Presentation
I agree that CMR is a key step in the clinical diagnosis of cardiac involvement from sarcoidosis, but it needs to be seen in the context of the clinical scenario, in this case, conduction abnormalities and lynphoadenopathies; but I think...
On: 03/10/2011 By: gquarta Read more?

Re: Number 11-05: Cardiac Sarcoidosis: a Severe Presentation
I think the problem is that sarcoidosis is essentially a form of myocarditis so for the majority of studies the two will be indistinguishable.I have seen the odd case where i have favoured sarcoid on the Cardiac findings...
On: 03/10/2011 By: drflett Read more?

Re: Number 11-05: Cardiac Sarcoidosis: a Severe Presentation
Ok, I will kick off with a question: Sometimes the LGE pattern of sarcoidosis and myocarditis could be quite similar. Any tips in differentiatating the two?Immagine if you also have a vague clinical hx and no mediastianal...
On: 03/09/2011 By: chiarabd Read more?

Number 11-05: Cardiac Sarcoidosis: a Severe Presentation
This is case just went online and now open for discussion. ...
On: 03/09/2011 By: chiarabd Read more?

 

 

 

 

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