Besides the "Fluid," Something Else
A 39-year-old man presented to emergency department with sudden onset of sharp pleuritic chest pain and increasing dyspnea for three days. He denied any orthopnea, paroxysmal nocturnal dyspnea or leg swelling. He had a past medical history of hypertension, chronic kidney disease, systemic lupus erythematosus and pulmonary fibrosis. His medications included amlodipine, hydroxychloroquine, mycophenolate mofetil and prednisolone.
Upon admission, vital signs were notable for sinus rhythm with heart rate of 90 beats per minute, a blood pressure of 139/88 mm Hg, tachypnea with respiratory rate of 40 breaths per minute and oxygen saturation of 100% on 50% oxygen via face mask. Pericardial rub and distended jugular vein were noted on physical examination. His electrocardiogram showed ST depressions in the inferolateral leads and PR depressions in lead II and PR elevation in lead aVR (Figure 1). Cardiomegaly was shown on chest X-ray.
Urgent echocardiogram showed a large circumferential pericardial effusion and echocardiographic evidence of cardiac tamponade. There was significant respiratory variation in the mitral and tricuspid inflows, i.e. ~26% and ~-62% respectively. Inferior vena cava (IVC) was plethoric with a diameter of 2.9cm and no change in size during respiration (Figure 2A). There appeared diastolic collapse of right ventricular outflow tract (Figure 2B). Pericardiocentesis was then performed and yielded 600 ml slightly turbid yellowish fluid which was confirmed to be exudative.
However, patient still had pleuritic chest pain after pericardiocentesis. Echocardiogram was then repeated and showed a small circumferential pericardial effusion. There was significant respiratory variation in the mitral inflow (~26%), and IVC was still plethoric, but there was no diastolic chamber collapse. There was a ventricular septal shift (i.e., cyclic movement of the ventricular septum toward the left ventricle with inspiration and towards the right ventricle with expiration) (Figure 3A). There was hepatic vein expiratory diastolic reversal (Figure 3B). Tissue Doppler imaging of mitral annulus velocities showed "annulus reversus", which the septal e' velocity (12 cm/s) was greater than the lateral one (10 cm/s) (Figure 3C, 3D).
Which of the following is the likely diagnosis?