Right Atrial Deformation Analysis in Cardiac Amyloidosis - Results from the Three-Dimensional Speckle-Tracking Echocardiographic MAGYAR-Path Study

Abstract

BackgroundLight-chain (AL) cardiac amyloidosis (CA) is characterized by fibril deposits, which are composed of monoclonal immunoglobulin light chains. The right ventricle is mostly involved in AL-CA and impairment of its function is a predictor of worse prognosis.ObjectivesTo characterize the volumetric and functional properties of the right atrium (RA) in AL-CA by three-dimensional speckle-tracking echocardiography (3DSTE).MethodsA total of 16 patients (mean age: 64.5 ± 10.1 years, 11 males) with AL-CA were examined. Their results were compared to that of 15 age- and gender-matched healthy controls (mean age: 58.9 ± 6.9 years, 8 males). All cases have undergone complete two-dimensional Doppler and 3DSTE. A two-tailed p value of less than 0.05 was considered statistically significant.ResultsSignificant differences could be demonstrated in RA volumes respecting cardiac cycle. Total (19.2 ± 9.3% vs. 27.9 ± 10.7%, p = 0.02) and active atrial emptying fractions (12.1 ± 8.1 vs. 18.6 ± 9.8%, p = 0.05) were significantly decreased in AL-CA patients. Peak global (16.7 ± 10.3% vs. 31.2 ± 19.4%, p = 0.01) and mean segmental (24.3 ± 11.1% vs. 38.6 ± 17.6%, p =0.01) RA area strains, together with some circumferential, longitudinal and segmental area strain parameters, proved to be reduced in patients with AL-CA. Global longitudinal (4.0 ± 5.2% vs. 8.2 ± 5.5%, p = 0.02) and area (7.8 ± 8.1% vs. 15.9 ± 10.3%, p = 0.03) strains at atrial contraction and some circumferential and area strain parameters at atrial contraction were reduced in AL-CA patients.ConclusionSignificantly increased RA volumes and deteriorated RA functions could be demonstrated in AL-CA.

Normal values of left ventricular rotational parameters in healthy adults—Insights from the three‐dimensional speckle tracking echocardiographic MAGYAR‐Healthy Study

“…This phenomenon was first reported in noncompaction cardiomyopathy (NCCM) . Our group has reported this phenomenon in a number of pathological states such as cardiac amyloidosis, acromegaly and congenital heart diseases . It is not known whether this is a sign of a yet subclinical pathology or undiagnosed disease; however, to the best of our knowledge, this phenomenon has not been previously reported in the healthy population.…”

Change of left ventricular “rigid body rotation” during dipyridamole‐induced vasodilation: A case from the three‐dimensional speckle tracking echocardiographic MAGYAR‐Stress Study

“…Moreover, in some pathologic circumstances, the LV basal and apical regions move in the same clockwise or counterclockwise direction, resulting in the near absence of LV twist called LV‐RBR . It could also happen that, although the apical and basal LV segments move in the same direction, there is an overrotation of certain regions leading to an apicobasal rotational gradient . Although LV‐RBR is known to be associated with different cardiomyopathies, congenital and infiltrative disorders, and other rare diseases, its prevalence in CAD is not known.…”

“…It could also happen that, although the apical and basal LV segments move in the same direction, there is an overrotation of certain regions leading to an apicobasal rotational gradient . Although LV‐RBR is known to be associated with different cardiomyopathies, congenital and infiltrative disorders, and other rare diseases, its prevalence in CAD is not known.…”

Left ventricular rigid body rotation in a diffuse large B-cell lymphoma patient with cardiac involvement: A case from the three-dimensional speckle-tracking echocardiographic MAGYAR-Path Study

“…RBR has previously only been demonstrated in a limited number of disorders, including cardiomyopathies (peripartum, 17 hypertensive, 18 dilated with reduced EF 19 and noncompaction 20 ), infiltrative disorders (amyloidosis) 21 and congenital heart disease (univentricular heart 22 and hypoplastic right heart syndrome 23 ). The underlying mechanism is unknown, but the findings presented could be explained by infiltration of the myocardium by lymphoma tissue, as confirmed in some areas by PET/CT.…”