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

Eur J Heart Fail. 2007 Aug;9(8):808-13. doi: 10.1016/j.ejheart.2007.05.006. Epub 2007 Jun 22.

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)<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<0.01), lower LV end-diastolic volumes (p<0.01), lower LV ejection fraction (p<0.01) and more frequently a restrictive LV filling pattern (p<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<0.01). Seven patients died during a median follow-up period of 19 months; TAPSE<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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Amyloidosis / physiopathology*
  • Echocardiography, Doppler
  • Female
  • Heart Ventricles / diagnostic imaging*
  • Humans
  • Male
  • Middle Aged
  • Natriuretic Peptide, Brain / blood
  • Peptide Fragments / blood
  • Prognosis
  • Prospective Studies
  • Stroke Volume
  • Ventricular Dysfunction, Left
  • Ventricular Dysfunction, Right / diagnostic imaging*
  • Ventricular Function, Right*

Substances

  • Peptide Fragments
  • pro-brain natriuretic peptide (1-76)
  • Natriuretic Peptide, Brain