Pulmonary hypertension in patients with amyloidosis

Pulmonary hypertension (PH) with right-sided cardiac failure is a rare comp lication of amyloidosis, and its natural history is not well-defined. The a im of our study was to evaluate patients who were seen at our institution w ho had PH and amyloidosis and to describe the natural history of this compl ication. The study was a retrospective chart review of patients seen at the Mayo Clinic with both PH and amyloidosis listed as major diagnoses between January 1, 1980, and December 31, 1999. Patients with known causes of PH w ere excluded. Five patients met our criteria (four women and one man). Four patients had light-chain amyloidosis and one had amyloid A deposition seco ndary to familial Mediterranean fever. All patients had symptoms related to PH without echocardiographic evidence of left ventricular dysfunction. The median survival time after the diagnosis of amyloidosis was 2.8 years, and PH was found a median of 73 days before death. Five patients died of cardi ac complications, including one with sudden cardiac death. PH is an unusual complication of amyloidosis. Patients develop PH late in the disease proce ss and do not have a worse prognosis compared to other patients with cardia c amyloidosis. PH is a marker of advanced amyloidosis.