Abstract
Amyloidosis is a rare disorder characterized by the deposition of amorphous, extracellular, insoluble fibrillar protein in various tissues of the body. Pulmonary hypertension usually occurs in the last stages of the disease with co-existing left ventricular failure. Amyloidosis causing pulmonary hypertension in a patient with no evidence of left ventricular failure is rarely mentioned in literature. Here, we present a patient with IgA gammopathy presenting with pulmonary hypertension leading to progressive right heart failure and death.
Highlights
The caseOur patient is a 73-year-old AfricanAmerican lady who has had several hospital admissions over the past 2 years with the diagnosis of Congestive Heart Failure (CHF)
Amyloidosis is a disorder characterized by the deposition of amorphous, extracellular insoluble fibrillar protein in various tissues of our body causing widespread organ dysfunction and death [1]
Amyloidosis is a rare disorder characterized by the deposition of amorphous, extracellular, insoluble fibrillar protein in various tissues of the body
Summary
Our patient is a 73-year-old AfricanAmerican lady who has had several hospital admissions over the past 2 years with the diagnosis of Congestive Heart Failure (CHF). In September 2005 the patient suddenly worsened with severe and progressive shortness of breath (SOB) and increasing leg edema. Her weight increased from 72.5 kg to 113 kg and she became immobile. The patient had extreme dyspnea with swelling of the legs, thighs and abdomen. Chronic fibrosing mediastinitis could be the only cause of the calcifications that could explain the significant right-sided heart failure. With the possibility of amyloidosis as a cause for the patient’s symptoms, it was decided to biopsy the supraclavicular adenopathy but the patient refused this procedure. She was made a Do-Not-Resuscitate (DNR) patient and expired in a nursing home in a few days
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