Right ventricular involvement in transthyretin amyloidosis
(English)Manuscript (preprint) (Other academic)
Right heart involvement and dysfunction is frequently encountered in cardiomyopathies. In immunoglobulin light chain (AL) amyloidosis, right ventricular (RV) function has been described as a strong determinant of survival. However, RV involvement in transthyretin related amyloidosis (ATTR) has been somewhat overlooked. We therefore sought to establish the degree of RV involvement in ATTR amyloidosis and compare the findings with sarcomeric hypertrophic cardiomyopathy (HCM).
Patients and methods
Forty-two patients with diagnosed ATTR 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 sarcomeric HCM and 30 healthy controls were included in the study. Echocardiographic examinations were analysed and standard measurements of LV and RV dimensions and function were carried out. In addition, LV global and RV global and segmental strain were analysed. The degree of cardiac involvement and function was compared between non-cardiac ATTR patients and healthy controls and between cardiac ATTR patients and with those having HCM.
Non-cardiac ATTR amyloidosis patients did not differ from healthy controls, either in geometrical or functional measurements of the LV and RV. HCM patients had significantly thicker interventricular septal thickness (p=0.017) and thinner posterior wall thickness (p=0.004) as compared to cardiac ATTR. When comparing cardiac ATTR and HCM patients by means of RV structure and function only segmental strain differed between the two patient groups. In cardiac ATTR we found an RV apex-to-base strain gradient with highest deformation in the apex whereas the pattern was inverse in patients with HCM.
RV involvement is common only in ATTR patients with concomitant LV involvement. The present study also detected an apical sparing pattern for the RV in patients with ATTR cardiomyopathy, similar to what has previously been described for the LV. This pattern was not seen in HCM patients. Further studies are warranted to assess the clinical importance of these findings.
Cardiac amyloidosis, hypertrophic cardiomyopathy, HCM, strain, apical sparing
Cardiac and Cardiovascular Systems
IdentifiersURN: urn:nbn:se:umu:diva-113889OAI: oai:DiVA.org:umu-113889DiVA: diva2:890741