Abstract

A 35-year-old male patient suffering from dyspnoea on effort for 8 months, with abdominal and jugular venous distension, was previously studied in another hospital and discharged with a diagnosis of restrictive cardiomyopathy. Physical examination revealed a blood pressure of 110/60 mm Hg and absence of pericardial knock and also of paradoxical pulse. Chest X-ray showed no cardio-pulmonary alterations. Transthoracic echocardiography showed mild LV dysfunction (LVEF 46%) and lack of pericardial effusion and thickening. Doppler interrogation of transmitral flow showed a restrictive pattern. Computed tomography showed diffusely thickened pericardium, with the absence of calcification and of pericardial effusion. Cardiac magnetic resonance confirmed pericardial thickening and showed lack of myocardial alterations. Mild LV dysfunction was noted with dyskinesia of interventricular septum. The patient underwent cardiac catheterization, demonstrating an equalisation of RV and LV diastolic pressures with “square root” sign. The patient underwent pericardiectomy with consequent resolution of his symptoms and improvement of LV function.

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