Accelerating restrictive cardiomyopathy after liver transplantation in a patient with familial amyloidotic polyneuropathy: a case report

We describe the case of a man of Irish ancestry with Familial Amyloidotic Polyneuropathy and no clinical history of cardiac involvement. Shortly after orthotropic liver transplantation, he developed congestive heart failure. He was subsequently diagnosed with an accelerating post-transplant restrictive cardiomyopathy due to amyloid infiltration.A liver transplant induced cardiomyopathy in Familial Amyloidotic Polyneuropathy can be observed in patients without any history of cardiac symptoms. All patients with Familial Amyloidotic Polyneuropathy should be followed after transplantation to assess for a deterioration in cardiac function.Familial Amyloid Polyneuropathy (FAP) is a disease process that has been described to affect the cardiovascular system. Clinical manifestations include hypotension, conduction disturbances, and most problematic, restrictive cardiomyopathies [1]. The majority of those with FAP have bothersome neuropathies, and are spared significant cardiovascular complications. Orthotropic liver transplantation (OLT) has been shown to stabilize and at times improve the neuropathic symptoms. Based on the pathogenesis of FAP, if OLT is performed prior to any of the clinical manifestations of cardiac amyloidosis, the likelihood of a patient succumbing to an amyloid cardiomyopathy should be significantly decreased.A 61 year old man presented with increasing dyspnea on exertion, ascites, and lower extremity edema. His medical history was remarkable for FAP which initially manifested ten years earlier as nausea and vomiting due to gastroparesis. In addition, he developed a painful peripheral neuropathy approximately two years later. Due to his progressive symptoms, he underwent OLT at another institution in August, 2006. Prior to his transplant, he had no cardiovascular complaints. His preoperative evaluation included a 2-dimensional echocardiogram. He did not have a cardiac catheterization. Approximately 2 months after his transplant, he began feeling dyspne