Combined cardiac and liver transplantation for the treatment of familial amyloidosis
Brian G. Schwartz, MD, Johannes J. Kuiper, MD, Göran B. Klintmalm, MD, PhD, Marvin J. Stone, MD, MACP, and Jeffrey M. Schussler, MD
A 55-year-old white man presented with weight loss
and diarrhea in 2002. An extensive workup, including
colonoscopy and gastrointestinal biopsy, led to a
diagnosis of familial transthyretin amyloidosis (Appalachian
variant). The patient had a steady decline over the next
2 years, including progressive weakness, peripheral neuropathy,
and wasting. Because of the clinical progression, he underwent
evaluation for liver transplantation. During this workup, an
echocardiogram demonstrated right ventricular enlargement,
pulmonary pressures in excess of 50 mm Hg (based on tricuspid
regurgitation), a small pericardial effusion, and an increased
echogenicity of the myocardium (Figure 1). These findings were
consistent with cardiac amyloid.
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