Recent onset of cardiac failure in a young athlete (ECR 2014 Case of the Day)

Clinical History

36-year-old male triathlete (recreational sport)
• No comorbidities, no risk factor, no medical history, no hypertension
• Recent and progressive onset of dyspnoea during exercise
• Cardiac ultrasound (decrease in LV ejection fraction, LV dilatation, subtle mitral insufficiency, the diagnosis after US was dilated cardiomyopathy.)
• Coronary arteries were normal
• CMR was required

Imaging Findings

Parameters of the LV systolic function (images 1, 2)
• LVEF: 33%
• EDV: 142ml/m2
• ESV: 94ml/m2
• Mass: 80g/m2
--> eccentric remodelling

• Functional mitral insufficiency
• Dilation of left and right atrium as well as both ventricles
• Segmental akinesia of the infero-basal wall with wall thinning
• No coronary lesion (coronarography)
• No valvular disease

LGE reveal large myocardial enhancement (Fig. 3)
Scout images showed lung involvement that was confirmed by CT (Fig. 4).
CT reveal lymph node enlargement and lymphatic micronodulation (Fig. 5, 6)
Figure 7 and 8 showed MR acquired on 2 different patients with cardiac sarcoidosis to show that this disease might show a different phenotype.

Discussion

Background:
• Sarcoidosis is a multisystem granulomatous disease
• Recent evidence suggests that the granulomas are an immunological response to an unidentified antigenic trigger(s)
• Cardiac involvement in at least 25% of patients with sarcoidosis (in United States)
• Cardiac involvement in sarcoidosis accounts for 13% to 25% of deaths of patient with sarcoidosis
Cardiac manifestation might be the first sign of the disease, and the radiologist might be the first to advocate the diagnosis.
Clinical perspective:
• Cardiac involvement: Wide variety of signs and symptoms related to the location and extent of granulomatous involvement
• Signs and symptoms (minimally symptomatic or asymptomatic, pericardial effusion, congestive heart failure, conduction abnormalities, arrhythmia and sudden death)
• Reference for the diagnosis is endomyocardial biopsies but MRI appears to be the best examination to look for cardiac involvement [2-4]
Imaging perspective:
• There is no specific sign on MR that characterises sarcoidosis
• Cardiac involvement during sarcoidosis could mimic a lot of diseases (HCM, DCM, ARVD, myocarditis, infarction, cystic fibrosis cases); there is no specific pattern of enhancement [5-7]
Endomyocardial biopsy:
• Highly specific/low sensitivity
• Should not be routinely performed as a diagnostic test: risks associated with an invasive technique [2]
• Most useful in patients with an unexplained cardiomyopathy where other diagnostic entities are also being considered [1]
• Diagnosis based on histology is also possible in the other organs
• Preferably peripheric biopsy (salivary gland, lymph nodes, cutaneous lesion…)
Treatment and prognosis:
• Therapy for cardiac sarcoidosis is not yet standardised (implantable cardioverter-defibrillator for primary prevention reasons, corticosteroids are regarded as the first-line drug)
CS: 5-year survival ranging from 60% to 90% (correlation between the extent of Gd-enhancement and the clinical severity of cardiac involvement [5], excellent correlation during follow-up of changes in MRI and clinical course of the patients)
Outcomes:
The patient died due to sudden cardiac death the day before his appointment in cardiology for DAI implantation.
Take home messages:
• Rare but potentially fatal condition that can mimic more common conditions
• It is therefore important to search for its presence in all patients diagnosed with sarcoidosis with cardiac MRI [5]
• MRI +++, very sensitive but no specific imaging pattern

Differential Diagnosis List

Final Diagnosis

Sarcoidosis with cardiac involvement

URL:	https://www.eurorad.org/case/11777
DOI:	10.1594/EURORAD/CASE.11777
ISSN:	1563-4086