Prognostic value of right ventricular systolic function in cardiac amyloidosis

Abstract

Background: Right ventricular (RV) dysfunction is a strong predictor of poor outcomes in heart failure. Its prognostic meaning in cardiac amyloidosis (CA) is under-investigated.

Methods: Hundred and twenty nine patients with suspected CA and an interventricular septum thickness (IVST) ≥ 12 mm underwent echocardiography with measurement of left ventricular (LV) and RV longitudinal strain (LS), late gadolinium-enhancement (LGE) cardiac MRI, and standard evaluation.

Results: Among 82 confirmed CA, types were immunoglobulin light chain (AL, n = 26), hereditary transthyretin (m-TTR, n = 37) and senile (WT-TTR, n = 19). Compared to those without, CA patients had significantly lower RV fractional shortening (RV-FS), tricuspid annular plane systolic excursion (TAPSE), tissue Doppler systolic velocity, and global RV-LS, without any difference among the CA types. RV-LGE, observed in 62% of CA patients, was associated with lower global and basal RV-FS. Median follow-up was 8(2; 16) months. Using multivariate analysis, NYHA-class and low TAPSE independently predicted major adverse cardiac event (MACE) defined as death, heart transplantation and acute heart failure. Independent determinants of TAPSE < 14 mm, the best cut-off value, were LV ejection fraction (LVEF), estimated filling pressure (E/E′), NT-proBNP and pulmonary artery pressure, but not RV-LGE.

Conclusions: RV dysfunction is common in CA. Its routine evaluation by a simple TAPSE may be an aid in assessing the prognosis of CA patients.

• Cardiac amyloidosis
• echocardiography
• heart failure
• prognosis
• right ventricle

Acknowledgements

We thank all the physicians involved in the Amyloidosis Network of the Henri Mondor Hospital who participated in the assessment and care of the patients included in this study. We also thank A Wolfe MD for editing the English-language manuscript.

Declaration of interest

The authors report no conflicts of interest.

This work was supported by the non-profit organisation AREMCAR (Association pour la Recherche et l’Etude des Maladies Cardiovasculaires).

Supplementary material available online.