PULMONARY-EDEMA IN MIXED CONNECTIVE-TISSUE DISEASE FOLLOWING PERICARDIOCENTESIS

A 22-year-old female patient with an 8-year history of mixed connectiv e tissue disease (systemic sclerosis overlapping with systemic lupus e rythematosus) presented with marked respiratory distress, sinus tachyc ardia (135 bpm), and pulsus paradoxus. The chest x-ray showed an enlar gement of the cardiac silhouette, which was due to a 3-cm-wide, circul ar pericardial effusion, as demonstrated by two-dimensional echocardio graphy. Pericardiocentesis performed to decompress cardiac tamponade d id not lead to clinical improvement. The increase in dyspnea was cause d by a rise in pulmonary wedge pressure from 21 to 40 mm Hg following an acute increase of mitral valve regurgitation. In the presence of gl obal hypokinesia of the left ventricle, cardiac output decreased from 3.25 to 2.63 l/min. Intensive care including hemodialysis and plasmaph eresis as well as high-dose application of cyclophosphamide and steroi ds led to a stabilization of the hemodynamic situation over a period o f days. The case report presented here supports the general recommenda tion to perform pericardiocentesis in a stepwise manner under hemodyna mic monitoring. This holds true primarily for patients with mitral val ve regurgitation and/or cardiac involvement in connection with an unde rlying disease.