PULMONARY VASCULAR AMYLOID CAUSING PULMONARY-HYPERTENSION - REPORT OFA CASE AND REVIEW OF LITERATURE

Although amyloid may deposit in the lungs, it is rare for pulmonary va scular involvement to cause appreciable luminal obstruction. To our kn owledge, only three well-documented cases of pulmonary hypertension ca used by amyloidosis have been reported. We describe pulmonary hyperten sion in a 54-year-old woman with primary (amyloid light chain; AL) amy loidosis. Although myocardial deposition of amyloid had produced restr ictive hemodynamics, the diffuse and severely obstructive nature of pu lmonary vascular involvement was considered to be of greater importanc e in the production of pulmonary hypertension in this patient. Amyloid -associated pulmonary hypertension may be associated with an insidious clinical presentation and contribute to morbidity and mortality.