A 32-year-old woman with a history of an undifferentiated connective tissue disease (CTD) was admitted with arthralgias and severe hypotension.
History of the present illness
The patient had been in her usual state of health until 6 days earlier, when she developed acute bilateral wrist and hand arthralgias, left-sided pleuritic chest pain, and dyspnea on exertion. She noted stiffness of approximately 30 minutes' duration. Her dyspnea had worsened progressively and she presented to an outside hospital. She denied fever, rash, night sweats, Raynaud's phenomenon, hair loss, sores in the mouth and nose, nasal congestion, epistaxis, cough, hemoptysis, palpitations, myalgia, muscle weakness, and paresthesia.
Evaluation in the emergency department showed a blood pressure of 90/60 mm Hg and tachycardia, with a regular heart rate of approximately 130 beats/minute. The patient was tachypneic, with a respiratory rate of 28 breaths/minute, and had 93% O 2 saturation on room air. She was afebrile. Diagnostic study results included a normal chest radiograph and a computed tomography angiogram that was negative for pulmonary emboli. An electrocardiogram (EKG) revealed a sinus tachycardia with mild ST-segment depression in leads V4, V5, and V6. The patient's troponin 1 and creatine kinase (CK) concentrations were elevated, as was the myocardial fraction of CK: troponin 1 level 10.6 ng/ml (normal value Ο½0.10), CK level 263 units/liter (normal value Ο½165), and MB fraction 44.3 ng/ml (normal value Ο½5). A transthoracic echocardiogram (TTE) revealed normal left ventricular cavity size, a left ventricular ejection fraction (LVEF) of 20% with severe global hypokinesis, increased left ventricular wall thickness (septal thickness 16 mm), and severely reduced right ventricular function.
Over the course of the first hospital day, the patient developed worsening hypotension that required the placement of an intraaortic balloon pump and the use of inotropic pressor support. An emergent cardiac catheterization revealed normal coronary arteries. On the day following admission, she was transferred to our institution for further evaluation and treatment of cardiogenic shock.