Right ventricular involvement in transthyretin amyloidosis

Abstract

Background: The extent of right ventricular (RV) involvement in transthyretin amyloidosis (ATTR) is unknown.

Objectives: This study sought to establish the degree of RV involvement in ATTR amyloidosis, and compare findings with RV involvement in hypertrophic cardiomyopathy (HCM).

Methods: Forty-two patients with ATTR amyloidosis and echocardiographic evidence of cardiac amyloidosis (cardiac ATTR), 19 ATTR patients with normal left ventricular (LV) wall thickness (non-cardiac ATTR), 25 patients with diagnosed HCM and 30 healthy controls were included in this study. Echocardiographic measurements for conventional parameters, as well as RV global and segmental strain, were recorded.

Results: When comparing RV structure and function between cardiac ATTR amyloidosis and HCM patients, only segmental strain differed between the two groups. In cardiac ATTR amyloidosis, we found an RV apex-to-base strain gradient with highest deformation in the apex. This pattern was reversed in patients with HCM.

Conclusions: RV involvement is common in cardiac ATTR patients. The present study also detected an RV apical sparing pattern in patients with ATTR cardiomyopathy, similar to what has previously been described for the left ventricle in these patients. This pattern was not seen in HCM patients. Further studies are needed to assess the clinical importance of these findings.

Keywords:

• Hypertrophic cardiomyopathy
• apical sparing
• strain
• amyloid cardiomyopathy
• right ventricle

Disclosure statement

No potential conflict of interest was reported by the authors.

Additional information

Funding

This study was supported by grants from the Swedish Heart-Lung Foundation (http://www.hjartlungfonden.se/) [PL, OBS], from a regional agreement between Umeå University and Västerbotten County Council/ALF [VLL 365301 for funding of SA], and the Heart Foundation of Northern Sweden (http://www.hjartfonden.com/) [SA].