title: Pulmonary arterial hypertension in primary amyloidosis.
abstract: Amyloidosis involves extravascular deposition of fibrillar proteins within tissues and organs. Primary light chain amyloidosis represents the most common form of systemic amyloidosis involving deposition of monoclonal immunoglobulin light chains. Although pulmonary amyloid deposition is common in primary amyloidosis, clinically significant pulmonary amyloidosis is uncommon, and elevated pulmonary artery pressures are rarely observed in the absence of other underlying etiologies for pulmonary hypertension, such as elevated filling pressures secondary to cardiac amyloid. In this case report, we present a patient with primary light chain amyloidosis and pulmonary arterial hypertension in the setting of pulmonary vascular and right ventricular myocardial amyloid deposition.
authors.affiliation: Division of Cardiovascular Medicine, Department of Medicine, University of Utah, Salt Lake City, Utah, USA; Internal Medicine-Pediatrics Residency Program, Department of Medicine, University of Utah School of Medicine, Salt Lake City, Utah, USA.
authors.forename: Meghan M
authors.initials: MM
authors.lastname: Cirulis
authors.affiliation: Division of Anatomic Pathology, Department of Pathology, University of Utah, Salt Lake City, Utah, USA.
authors.forename: Lyska L
authors.initials: LL
authors.lastname: Emerson
authors.affiliation: Division of Cardiothoracic Surgery, Department of Surgery, University of Utah, Salt Lake City, Utah, USA.
authors.forename: David A
authors.initials: DA
authors.lastname: Bull
authors.affiliation: Division of Pulmonary Medicine, Department of Medicine, University of Utah, Salt Lake City, Utah, USA.
authors.forename: Nathan
authors.initials: N
authors.lastname: Hatton
authors.affiliation: Division of Cardiovascular Medicine, Department of Medicine, University of Utah, Salt Lake City, Utah, USA.
authors.forename: Jose
authors.initials: J
authors.lastname: Nativi-Nicolai
authors.affiliation: Division of Hematology and Hematologic Malignancies, Department of Medicine, University of Utah, Huntsman Cancer Institute, Salt Lake City, Utah, USA.
authors.forename: Gerhard C
authors.initials: GC
authors.lastname: Hildebrandt
authors.affiliation: Division of Cardiovascular Medicine, Department of Medicine, University of Utah, Salt Lake City, Utah, USA.
authors.forename: John J
authors.initials: JJ
authors.lastname: Ryan
keywords: Congo red stain; medial hypertrophy; multiple myeloma; pulmonary vascular disease