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Cardiac sarcoidosis
- 1. CARDIAC SARCOIDOSIS BY-DR PIYUSH KUMAR MODERATOR-DR M SUDHAKAR RAO
- 13. Genetic Predisposition Familial clustering indicates a strong genetic element in sarcoidosis 1st degree relative : 5-fold increase in risk of developing sarcoidosis HLA DQB*0601 & tumour necrosis factor allele TNFA2 in Japanese patient with cardiac sarcoidosis.
- 14. Presentation Variable and vague Syncope and presyncope, palpitation, heart failure symptoms Manifeastation of CS-AV Block, Atrial and ventricular tachyarrythmia, heart failure Silent CS- non specific chest pain , dyspnea , fatigue
- 18. Screening
- 20. Very few data comparing the sensitivity and specificity of various screening test
- 44. Conduction abnormality Atrial arrythmia Ventricular arrythmia Heart failure Risk stratification for SCD-ICD therapy
- 48. IMMUNOSUPRESSION
- 49. Steroid
- 50. ATRIAL TACHYARRYTHMIA True prevalance is unknown but observational studies shows that it can be up to 32% of sarcoidosis patients Most common-AF All non-AF atrial arrythmia seem to be related to scar identified on electroanatomic maping and mechanism is diverse(focal abn,automaticity,microrentry) HRS recommendations -AC based on CHA2DS2VASc Score -EPS MAY BE CONSIDERED(Ib) -Beta blocker, CCB, SOTALOL, DOFETILIDE AND AMIODARONE CAN BE USED
- 51. VENTRICULAR ARRYTHMIA MECHANISM- Most common macrorentry around the granulomatous scar Immunosuppression + Antiarrythmic medication followed by catheter ablation if VT persisted Immunosuppression- contrasting data but the consensus statement advocate steroid use specially early in the disease in the presence of preserved LV function Antiarrythmic- amiodarone and sotalol are the most widely used HRS 2014
- 58. PROGNOSIS Poorer prognosis with cardiac involvement Extent of lv dysfunction is the most important predictor of survival in the pt with clinical manifestation Pt with normal EF-survival rate at 10 yrs was 100% Pt with EF < 30% -1 year-91% -5 year- 57% -10 year- 19%
- 59. THANK YOU
Editor's Notes
- The disease of sarcoidosis was 1st described by Jonathan Hutchinson in 1877 as skin changes associated with sarcoidosis. In 1899 sezer peter beck published 1st comprehensive description of such skin changes and included its pathologic analysis showing the non caseating non necrotising granulomas and he named the disease beck sarcoid particularly bcoz the cell he saw resemble sarcoma cells.
- And 30 yrs later in 1929 dr Mitchell Bernstein described the 1st case of sarcoidosis that involved the heart and here we r after 100 yr later we don’t know the exact pathophysiology of sarcoidosis
- If we look for incidence we can see in different countries………….it is more common in female and In African American …..age more common in between 25-70 yrs and rare in less than 15 and over 70 yr.
- Burden of this disease is very profound ,
- Mechanism is formation of non necrotising granuloma and it is inflammatory cascade usually in genetically predisposed individuals who may have exposure of some sort ,and that results in activation of immune system particularly IL 17 AND TNF as well as macrophages and all of them can form granulomas.
- As far as the cardiac involvement is concerned they have a very high rate of hf……
- As it is becoming more and more prevalent as we r improving in imaging modalities and more and more pt r getting diagnosed with this ds, in finland alone
- Association have been described with hladqb ….
- Depending on which part of heart is involved ,that really depicts what kind of cardiac manifestation pt is going to have. If it affects conduction system– av block can occur, if there is inflammation and scar formation they can act as reentrant area frm where vt can occur. U can have heart failure…. Interestingly u can have valvular inflammation . Pt can have AR, MR
- This data shown here shows some imp data from finland, there is 351 pt with cardiac sarcoidosis, and the most common presentation is av block around 42 %. However sudden death is also very common presentation…. So we should keep in mind that if pt presents with VT or VF or HEART BLOCKS THAT CAN ALSO be life threatening arrythmia. When look at the mode of death SCD is around 80%. So it is imp to assess the pt, so that arrythmia related death can be prevented with ICD.
- In biopsy proven extra CS with …. 2. even if u have no extra cs that is biopsy proven , if we have a young pt with significant av block that we can not explain, with no tick bite or not on medication that can cause av block, then we should think of cs 3…
- But this is 1 such article and I want u to focus on this 1.this is small study as this is a rare ds , 62 pt, ant they found that the presence of a +ve screening variable in any of the following . If u hv positive history of cardiac symptoms or……
- Qrs fragmentation-presence oo 2 or more notch in r or s in 2 contiguous leads
- Scar,structure and function. Rules out mimickers like hcm,arvc T2 cmr may enable detection of active inflammation
- High fat diet to promote fatty acid metabolism in the heart to suppress background utilisation in the myocardium. So during fdg pet only area with active inflammation will take up fdg. No approved quantisation of fdg uptake for follow up
- Findings u expect ………
- If u belong to this grp,its higly likely that u have cardiac sarcoidosis .when u hv both an abnormal perfusion and abnormal fdg uptake is + its higly suugestive of cs. …. In the same area, it usually tells us active inflammation in the same location 3. ……..in focal or multifocal uptake in a diff area 2.
- So the same consensus divided the fdg finding in accordance with likelihood of cs Probably no sarcoid if no defect 1 indicative of scar 2.Early onset of ds
- If u have focal fdg uptake with abnormal perfusion defect in diff area -----cs If u have multifocal fdg uptake higly probable that u hv cs
- Published in bleinkstein paper…3 fold increase in adverse event- death or appropriate icd therapy
- Incidentally the same study find an abnormal fdg uptake in rv side
- Technologies has been developed to perform fused pet/cmr which enables concurrent imaging of the 2 stages of the ds(inflammation and fibrosis/scar
- Only 20-30 % have positive EMB.
- Low sensitivity
- Looking at he diagnostic yield…… ,This is from a large series from Italy showing
- 2012 HRS guidelines
- Cs specific recommendation
- Case series studied for high grade av block.
- Out of 84 pt 30 pt had more than 10 appropriate icd therapy. It was also found that most of the pt with appropriate icd therapy had lvef > 35 %. So pt with ef > 35 are also at risk
- CHIU ET AL 2005