Importance of the echocardiographic evaluation of right ventricular function in patients with AL amyloidosis

Abstract

Background: Patients with AL amyloidosis often present with signs of congestive heart failure. Aim: This study was prospectively designed to assess the significance of RV dysfunction in AL amyloidosis. Methods and results: Seventy-four patients with biopsy proven AL amyloidosis underwent a thorough echocardiographic evaluation. A tricuspid annular plane systolic excursion (TAPSE) b 17 mm was taken as marker of RV dysfunction. Plasma NT-proBNP determinations were performed in all cases. RV function was normal in 60 patients and reduced in 14 patients. Patients with RV dysfunction had thicker left ventricular (LV) walls (p b 0.01), lower LV end-diastolic volumes (p b 0.01), lower LV ejection fraction (p b 0.01) and more frequently a restrictive LV filling pattern (p b 0.01). RV dimensions and RV free wall thickness were not significantly different in the two groups. A thick interventricular septum and a reduced TAPSE were associated with high NT-proBNP levels (both p b 0.01). Seven patients died during a median follow-up period of 19 months; TAPSE b 17 mm was the only echocardiographic parameter associated with poor survival. Conclusion: In patients with AL amyloidosis, RV dysfunction is associated with more severe involvement of the left ventricle, higher plasma levels of NT-proBNP and with poor prognosis.

Prognostic value of depressed midwall systolic function in cardiac light-chain amyloidosis

“…This piece of information adds on previous demonstration of reduced longitudinal contractile dysfunction [35,36,[55][56][57][58]. Moreover, midwall systolic dysfunction was able to further stratify patients' prognosis in both Mayo Stage II and Stage III patients.…”

“…As expected, cardiac amyloidosis was associated with left ventricular concentric hypertrophy with preserved ejection fraction and evident diastolic dysfunction (all P <0.01 vs. patients without cardiac involvement). Also longitudinal systolic function was depressed, as estimated by tissue Doppler systolic velocity (P <0.01) and by mitral and tricuspid annulus excursion (P <0.01) [36]. This was associated with increased NT-proBNP and TnI serum levels [277 (126-558) vs. 5170 (1948-13 079) pg/ml, 0.011 (0.005-0.026) vs. 0.130 (0.044-0.269) ng/ml, respectively; P <0.01 for both when compared with patients with noncardiac light-chain amyloidosis].…”

Liver AL amyloidosis as a possible cause of high liver stiffness values

“…In the strong suspicion of amyloidosis, with kidney, heart and liver involvement, a transthoracic echocardiography was performed, which showed a thick interventricular septum with mild diastolic dysfunction, typical echocardiographic findings of initial heart amyloidosis [3]. Neither direct nor indirect signs of congestive heart failure were collected.…”

Primary (AL) amyloidosis with gastrointestinal involvement

“…Ten patients underwent aprotinin scintigraphy [14] for information on amyloidosis involvement in other organs. Echocardiography [15], 24-h urine protein excretion [16], and clinical evaluation of the peripheral nervous system [1] were also used to assess organ involvement. The median (range) delay from initial symptoms to diagnosis was 7 (0Á24) months.…”