Sandra Arvidsson1, Michael Y Henein2, Gerhard Wikström3, Ole B Suhr2, Per Lindqvist1,4. 1. a Department of Clinical Physiology , Heart Centre, Umeå University , Umeå , Sweden. 2. b Department of Public Health and Clinical Medicine , Umeå University , Umeå , Sweden. 3. c Department of Medical Sciences , Cardiology, Uppsala University , Uppsala , Sweden. 4. d Department of Surgical and Perioperative Sciences , Umeå University , Umeå , Sweden.
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.
BACKGROUND: The extent of right ventricular (RV) involvement in transthyretinamyloidosis (ATTR) is unknown. OBJECTIVES: This study sought to establish the degree of RV involvement in ATTRamyloidosis, and compare findings with RV involvement in hypertrophic cardiomyopathy (HCM). METHODS: Forty-two patients with ATTRamyloidosis and echocardiographic evidence of cardiac amyloidosis (cardiac ATTR), 19 ATTRpatients 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 ATTRamyloidosis and HCM patients, only segmental strain differed between the two groups. In cardiac ATTRamyloidosis, 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 ATTRpatients. The present study also detected an RV apical sparing pattern in patients with ATTRcardiomyopathy, 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.
Entities:
Keywords:
Hypertrophic cardiomyopathy; amyloid cardiomyopathy; apical sparing; right ventricle; strain
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