Circulation Cardiovascular Case Series

A 34-year-old man with a 2-year history of unexplained steroid-refractory polyneuropathy, lower-extremity edema, and monoclonal gammopathy presented to our tertiary care center with 3 days of rapidly progressive dyspnea. The patient’s vital signs were notable for a temperature of 37.0oC, heart rate of 122 bpm, blood pressure of 80/60 mm Hg, respiratory rate of 22 breaths per minute, and oxygen saturation of 94% on 2 L supplemental oxygen. Physical examination revealed a cachectic young man in mild respiratory distress with a jugular venous pressure of 12 cm H 2O and a positive Kussmaul sign, pulsus paradoxus of 6 mm Hg, regular tachycardic rhythm without murmur or gallop, but a prominent P2 sound with heave at the left sternal border, bilateral fine crackles, and decreased bibasilar breath sounds. Abdominal examination revealed mild hepatomegaly. Peripheral examination was notable for warm and well-perfused lower extremities with 2+ pitting edema extending to the sacrum but without evidence of venous stasis dermopathy, leg tenderness, erythema, warmth, or palpable cord. There was also mild, diffuse skin hyperpigmentation.