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Calcific constrictive pericarditis

A 40-year-old man suffered from gradually worsening dyspnea and swollen ankles for six months prior to his evaluation. He underwent a chest X-ray, lung function tests, a 24-hour Holter heart rhythm monitoring, and a polysomnography study. A working diagnosis of sleep apnea syndrome was made in another institution and the patient was advised to use a continuous positive airway pressure (CPAP) mask. With the use of the mask, his symptomatology got worse: he had severe dyspnea at rest and leg edema, which was extending up to the knees. He also noticed an increased abdominal girth. The patient was assessed by a cardiologist in his hometown who perfomed a Doppler echocardiography and diagnosed a thrombus of the right ventricle and an abnormally moving intraventricular septum. These findings prompted an emergency referral to the hospital.

On physical examination the patient was hemodynamically unstable with a blood pressure of 90/65 mmHg, pulse rate 105 per minute, respiratory rate 25 per minute, and temperature 38,2 0 C. He was severely dyspneic at rest and was mildly obese (Body Mass Index of 27,75). The first and second heart sounds were normal but there was a pericardial knock. Pulsus paradoxus was present as well. There were few crackles of the lung fields up to the midzone. Abdominal examination revealed splenomegaly and signs of free peritoneal fluid. He had edema of the legs up to the level of the knees.

An initial workup revealed the presence of mild anemia (Ht: 37.6%), an increased erythrocyte sedimentation rate (ESR = 50 mm at the first hour), an elevated C-reactive protein (CRP = 16.36 mg/dl with upper normal limits: 0.5 mg/dl) and decreased serum albumin levels (2.7 gr/dl). Measurement of other routine biochemical parameters was normal. Tuberculin skin testing was negative. An electrocardiogram showed decreased voltage. Imaging included a chest X-ray which showed calcification of the pericardium and a small left pleural effusion, and an echocardiogram which showed a localized pericardial effusion causing tamponade of the right ventricle and calcification of a thickened pericardium. A CT scan of the chest is shown in Figure 1.


Figure 1