Congenital Complete Absence of Pericardium Masquerading as Pulmonary Embolism
We present the case of a 23-year-old white male, who presented to our hospital with complaints of pleuritic chest pain and exertional dyspnea of two weeks’ duration. There was a history of trauma to the left leg, with swelling, two weeks prior. The patient′s past history was significant for hepatitis C, substance abuse, and smoking. The patient was otherwise physically active and used to exercise for two hours daily. His vital signs were within normal limits and physical examination, including cardiovascular and respiratory system, failed to reveal any abnormality. An electrocardiogram (EKG) showed that the patient had poor r-wave progression and severe left axis deviation of minus 125 degrees. This would be typical of a heart that had rotated into the left chest, and a chest X-ray showed deviation of the cardiac silhouette to the left and clear lung fields. The Snoopy's sign was present []. The anterior clear space was prominent [].\nA chest CT with contrast and a pulmonary embolism (PE) protocol showed evidence of multiple emboli, predominantly in the left lung. The patient was started on heparin and warfarin therapy. Further history and workup for deep vein thrombosis (DVT) and a hereditary hypercoagulable state failed to identify a cause for PE. The patient was discharged, but later on readmitted to the hospital with chest pain and palpitations.\nTelemetry monitoring of the patient showed that the heart rate changes were from 30/minute to episodes where it went up to 112/minute. There was evidence of premature atrial contractions (PACs) and sinus arrythmias.\nA repeat chest CT with contrast showed no significant change from the previous CT scan, showing the heart to be abnormally rotated to the left side of the chest [Figures and ]. The pericardium was absent.\nAn echocardiogram confirmed the abnormal orientation of the heart, with the apex of the left ventricle sitting deeply in the left chest and a moderate amount of pulmonary hypertension []. A diagnosis of congenital absence of pericardium was made. The misdiagnosis of pulmonary embolism was attributed to the motion artifact of the swinging heart ( showing abnormal CT scan compared with a normal chest CT; ). As the patient's symptoms did not prevent him from carrying out his daily activities, he was discharged with regular Outpatient follow-up. He was told to cease his daily regimen of an intense two-to-three hour workout. His symptoms usually came during this work out.