Cardiac Amyloidosis Presenting as Isolated Severe Pulmonary Artery Hypertension

Abstract

A 58-year-old woman presented with dyspnea on exertion and bilateral pedal edema for 3 months. She was found to have severe pulmonary hypertension (PH) with the right ventricular failure. The detailed evaluation did not reveal any secondary cause for PH (cardiac disease, pulmonary embolism, interstitial or other lung disease, collagen vascular disease, portal hypertension, or chronic liver disease). Meanwhile, her workup revealed multiple myeloma. Abdominal fat pad biopsy was performed because PH as an isolated manifestation of cardiac amyloidosis was previously reported. Thus, she was diagnosed to have systemic amyloidosis secondary to myeloma. PH was attributed to cardiac amyloidosis. We present a patient with PH and amyloidosis secondary to multiple myeloma. PH and primary systemic amyloidosis without cardiac or parenchymal lung involvement are extremely rare with only a few cases reported in the past.