Teaching Case

A 34-year-old man showing left arm paresthesia, reports use of cocaine and ecstasy, during recent cardiological examination echocardiography reported left ventricular dilatation with systolic biventricular dysfunction (EF 25-30%), moderate mitral and tricuspid regurgitation, left atrial enlargement, moderate pulmonary hypertension. (Fig. 1)

Chest X-ray: multiple bilateral parenchymal thickening in the middle and upper lung right field and upper left. (Fig. 2)
Bronchoscopy with BAL: normal bronchial cells detected, transbronchial biopsy not performed for persistent abnormal INR, insights reveal deficits of factorVII.
Head CT without administration of contrast: no significant alterations.
ECG: sinus rhythm, right bundle branch block and complete right axis deviation.
Chest HRCT: diffuse parenchymal micronodules in both lung fields.
Peri-broncho-vascular and sub-pleural distribution, aspects of bilateral apical fibrotic retraction. (Fig. 3)
Thoraco-abdominal CT with contrast shows multiple enlarged lymph nodes in the hilum-mediastinal, partly overlapping without colliquative aspects, enlarged heart, multiple hypodensity in splenic parenchyma, as confirmed by echography.
CMR: dilated and hypokinetic left ventricle with reduction of EF (25%), slightly dilated and hypokinetic right ventricle (EF 25%), after administration of contrast medium areas of delayed enhancement subepicardial, transmural in some locations, without coronary distribution (Fig.4)
Right ventricular endomyocardial biopsy showed non-caseificant granulomas compatible with sarcoidosis. Steroid therapy started.

Sarcoidosis is a systemic disease of unknown aetiology characterised by noncaseating epithelioid granulomas in many organs and tissues, it occurs most commonly with lung involvement and bilateral hilar lynphadenopathy, the spleen is involved microscopically in about three-quarters of the cases.
Myocardial involvement, which is observed in 5 to 10% of patients, can cause heart failure and fatal conduction defects. Sarcoidosis has an unpredictable course characterised by either progressive chronicity or periods of activity interspersed with remissions, sometimes permanent, that may be spontaneous or induced by corticosteroid therapy. Most patients seek medical attention for the insidious onset of respiratory abnormalities or nonspecific signs and symptoms; in this case the patient develops symptoms of neurological disorders.
Ultrasound examination in our clinical case revealed abnormal cardiac chamber size, ejection fraction decreased bilaterally, which led to the decision to continue the study. HRCT and volumetric CT chest abdomen showed a picture of diffuse interstitial fibrosis with aspects of interstitial pulmonary fibrosis, commitment of mediastinal lymphnodes and nodules in the spleen. MRI has allowed us to accurately assess the size, kinetics and function of cardiac chambers, also the distribution of non-coronary heart delayed enhancement was properly directed to a cardiac involvement not secondary to ischemia but not in a systemic framework.
CMR is very useful in the differential diagnosis and may guide clinical management.