Physical Examination and Work-up
The physical examination reveals an alert, well-appearing female, with a regular pulse rate of 95 beats/min, blood pressure of 110/70 mm Hg, respiratory rate of 24 breaths/min, and an oxygen saturation of 91% while breathing room air. Her neck is supple and without jugular venous distention.
The cardiac examination reveals a normal S1, a loud P2, and no audible murmurs, rubs or gallops. Examination of the chest shows decreased air entry at the lung bases, but no wheezes, rhonchi, or rales are noted. The abdomen is soft, nontender, and without organomegaly. No pedal edema, cyanosis, or clubbing is observed. The peripheral arterial pulses are palpable. The neurologic examination findings are normal.
Blood tests, including a complete blood cell count, comprehensive metabolic panel, B-type natriuretic peptide, and serial troponins, are all within normal limits. A blood gas test on room air shows a pH of 7.46, pCO2 of 35 mm Hg, and pO2 of 70 mm Hg.
Chest radiography shows bilateral hilar adenopathy, with increased markings bilaterally. Pulmonary function tests (PFTs) performed 1 month before presentation showed an FVC of 1.9 L (63% of predicted), FEV1 of 1.65 L (70% of predicted), FEV1/FVC ratio of 89%, a residual volume of 0.83 L (38% of predicted), total lung capacity of 2.74 L (53% of predicted), and a diffusing capacity (DLCO) of 8.73 mL/min/mm Hg (38% of predicted). These results are unchanged from an examination performed 6 months ago.
A 12-lead ECG is obtained (Figure 1). A 2-dimensional (2D) echocardiography examination is subsequently obtained, and right-heart catheterization performed.
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