title: Right ventricular dilation in primary amyloidosis: an independent predictor of survival.
abstract: This study was designed to characterize the geometry and function of the right ventricle and its prognostic significance in patients with primary (AL) cardiac amyloidosis. AL amyloidosis is an infiltrative systemic disease that can result in thickening of heart structures and rapidly progressive congestive heart failure due to restrictive ventricular physiology and eventual systolic dysfunction. Thirty-seven patients with AL amyloid heart involvement and 20 normal control subjects were evaluated using 2-dimensional and Doppler echocardiography. Based on the ratio of left-to-right end-diastolic ventricular chamber areas, patients were classified into 2 groups: 25 patients with disproportionate right ventricular (RV) dilation (left ventricular to RV ratio < or = 2) and 12 with a ventricular area ratio > 2. Patients with a relatively dilated right ventricle (ratio < or = 2) had a shorter median survival (4 months) compared with patients with an area ratio > 2 (10 months, p <0.003). Of multiple clinical, echocardiographic, and Doppler features entered into a multifactorial model, a ventricular area ratio < or = 2 remained the only independent predictor of survival. Patients with AL amyloid heart disease represent a heterogeneous population with regard to both prognosis and the relative degree of right to left ventricular dilation. RV dilation in patients with amyloid heart disease appears to be associated with more severe involvement and is associated with a very poor prognosis with a median survival of only 4 months.
authors.affiliation: Evans Memorial Department of Clinical Research, Arthritis Research Center, Boston University Medical Center, Massachusetts 02118, USA.
authors.forename: A R
authors.initials: AR
authors.lastname: Patel
authors.forename: S W
authors.initials: SW
authors.lastname: Dubrey
authors.forename: L A
authors.initials: LA
authors.lastname: Mendes
authors.forename: M
authors.initials: M
authors.lastname: Skinner
authors.forename: A
authors.initials: A
authors.lastname: Cupples
authors.forename: R H
authors.initials: RH
authors.lastname: Falk
authors.forename: R
authors.initials: R
authors.lastname: Davidoff
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D015150:Echocardiography, Doppler; D005260:Female; D006801:Humans; D017380:Hypertrophy, Right Ventricular / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D015588:Observer Variation; D011237:Predictive Value of Tests; D016019:Survival Analysis; D016278:Ventricular Function, Right