Cardiac sarcoidosis

Case contributed by Dr Joachim Feger

Presentation

Known history of sarcoidosis. Few extrasystoles on ECG. Previous endomyocardial biopsy.

Patient Data

Age: 50 years
Gender: Female
MRI

Cardiac MRI

Findings:

Heart rate:  58 bpm, BSA: 1,94 m²

Image quality: no limitations

Morphology and functional analysis:

  • LV-EDVI:  73 ml/m²
  • LV-ESVI:  28 ml/m²
  • LV-SVI:  45 ml/m²
  • LV-EF:  61 %
  • Cardiac output:  5.0 l/min
  • Cardiac index: 2,6 l/min/m²
  • LV-ED mass + papillary muscle:  43 g/m²
  • Septum thickness: 8 mm

No regional left ventricular wall motion abnormalities.

Visually normal atrial size. Persistent foramen ovale (PFO).

Mild calcification of the anterior mitral leaflet. Mild mitral insufficiency.

No intercavitary thrombi found.

Myocardial tissue properties 1:

No regional or global myocardial edema.

Patchy intramyocardial and subepicardial late gadolinium enhancement (LGE) in the midventricular/apical inferior segments, best visible on the 2-chamber view (2ch).

T2 mapping: 51 ms

T1 mapping native: 990-1040 ms [950-1060ms*], extracellular volume (ECV):  < 30%

*native T1 reference range based on local data

Extensive, circular pericardial effusion.

Impression:

Patchy intramural and subepicardial late gadolinium enhancement visible in the apical/midventricular inferior segments and extensive pericardial effusion consistent with cardiac sarcoidosis.

No signs of acute myocardial inflammation.

Annotated image

Short tau inversion recovery (STIR) imaging / T2 mapping:

No focal myocardial edema can be seen.

T2 signal intensity ratio between myocardium and skeletal muscle within the same image was calculated and was normal: <1.9 - a T2 SI ratio of ≥2 is considered abnormal, provided images were acquired with the MRI integrated body coil (Q-body) 2.

T2 mapping:  51 ms (within normal range).

T1 mapping native and postcontrast:

native T1  ≈ 1025 ms, z-score of ≈ 0.8 (within normal limits)

extracellular volume (ECV) was < 30% in all segments  ≈ 27.6 % (within normal limits)

Late gadolinium enhancement (LGE):

Patchy intramural/subepicardial late gadolinium enhancement can be seen in the inferior midventricular/apical segments on IR-GE images (red arrows) and reflects a granuloma or postinflammatory scar tissue.

CT

Thoracic CT

Findings:

Thyroid nodules (up 35 mm in size).

Bilateral, symmetric hilar, right paratracheal and subcarinal lymph node enlargement, some lymph node calcifications.

Pericardial effusion.

Multiple small pulmonary (micro-)nodules with a perilymphatic distribution. Most are located along the fissures and the peribronchovascular interstitium and the subpleural surface. There is an upper and middle zone predominance.

Spleen is not enlarged.

Impression:

CT-findings are consistent with pulmonary sarcoidosis.

Multiple thyroid nodules, indicative of thyroid involvement.

Pericardial effusion as an indicator of cardiac involvement.

 

MRI

Cardiac MRI - 4 years earlier

Findings:

Heart rate:  67 bpm, BSA: 1,88 m²

Image quality: no limitations

Morphology and functional analysis:

  • LV EDVI:  93 ml/m²
  • LV- ESVI:  43 ml/m²
  • LV SVI:  50 ml/m²
  • LV EF:  53 %
  • Cardiac output:  6.7 l/min
  • Cardiac index: 3,6 l/min/m²
  • LV-ED mass + papillary muscle:  52 g/m²
  • Septum thickness: 8 mm

Mild global hypokinesia. No regional left ventricular wall motion abnormalities.

Visually normal atrial size.

Mild calcification of the anterior mitral leaflet. Mild mitral insufficiency.

No intercavitary thrombi found.

Myocardial tissue properties:

No regional myocardial edema. Mildly increased relative water content on STIR images.

Early gadolinium enhancement (EGE) ratio was significantly increased.

Patchy intramyocardial and subepicardial enhancement visible in the apical/midventricular inferior segments, best visible on the 2-chamber view (2ch).

Extensive, circular pericardial effusion.

Impression:

Cardiac MRI findings are consistent with non-ischemic myocardial inflammation.

Massive circular pericardial effusion. Mild systolic dysfunction.

In view of the clinical information and the previous thoracic CT, the findings are consistent with cardiac sarcoidosis.

Immediately after the cardiac MRI four years ago, the patient received systemic immunotherapy and was then referred to a major cardiac centre, where she received another cardiac MRI two months after the first. They found that late gadolinium enhancement (LGE), mild systolic dysfunction and pericardial effusion was unchanged, but they did not find any early gadolinium enhancement (EGE) or cardiac oedema.

A subsequent endomyocardial biopsy showed focal interstitial fibrosis, with inflammatory cells and granulomatous inflammation consistent with the cardiac manifestation of sarcoidosis. Eosinophil and giant cell myocarditis as a potential differential diagnosis for the late gadolinium enhancement were histologically ruled out and thus the diagnosis of cardiac sarcoidosis was established.

Case Discussion

This case illustrates the findings of cardiac and pulmonary sarcoidosis.

The pulmonary and mediastinal findings are typical and comprise:

  • symmetric, bilateral hilar and right paratracheal lymphadenopathy, also known as Garland's triad or 1-2-3 sign, together with subcarinal lymphadenopathy
  • a micronodular lung pattern with a perilymphatic distribution predominant in the upper and middle zones

The cardiac findings are:

  • an extensive, circular pericardial effusion
  • a patchy intramyocardial/subepicardial late gadolinium enhancement
  • acute inflammatory changes of the myocardium in the first MRI, which apparently had resolved after a course of systemic immunosuppressive therapy

There are reports in the literature, indicating that increased myocardial T2 signal representing cardiac edema in conjunction with late gadolinium enhancement (LGE) can predict cardiac arrhythmia risk in cardiac sarcoidosis 4. T1 and T2 mapping seem to have the potential to characterize disease activity in addition to LGE and to monitor the response to immunosuppressive therapy 3-7.  

The cardiac MRI, which is shown first in this case (last in the timeline), was conducted to find out if there were acute inflammatory changes responsible for the patient's ventricular extrasystoles and whether her anti-inflammatory medications had to be adjusted.

The late gadolinium enhancement (LGE) is unchanged in comparison to the previous MRI, but this time there were no acute inflammatory signs and cardiac function was normal.

On grounds of both clinical and MRI findings, the patients anti-inflammatory medications were left unchanged.

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Case information

rID: 74548
Published: 4th Mar 2020
Last edited: 22nd May 2020
System: Cardiac, Chest
Inclusion in quiz mode: Included