Left atrial volumetric and functional properties in hemophilia – insights from a three-dimensional speckle-tracking echocardiographic MAGYAR-Path study

Abstract

Hemophilia is an inherited disorder due to deficiencies in factor VIII (type A) and factor IX (type B). Abnormalities in myocardial mechanics could be theorized due to hemophilia-associated hypocoagubility and related quantitative and qualitative changes of the blood. The present study aimed a detailed assessment of left atrial (LA) volumetric and functional properties in patients with hemophilia using three-dimensional speckle-tracking echocardiography (3DSTE). The study consisted of 12 subjects with hemophilia type A and 2 cases with hemophilia type B (mean age: 40.8 ± 19.1 years, all males). Results of hemophilia patients were compared to that of 23 age-, gender- and risk factor-matched controls (42.4 ± 9.0 years, all males). Routine two-dimensional Doppler echocardiography and 3DSTE were performed in all subjects. LA volumes respecting cardiac cycle did not differ between controls and hemophilia patients. From LA volume-based functional properties, LA stroke volumes were similar between the groups examined in all phases of LA function. While total atrial emptying fraction featuring LA reservoir function was reduced in patients with hemophilia compared to that of controls, passive and active atrial emptying fraction characterizing LA conduit and booster pump functions were similar between the groups. From LA strains, peak mean segmental circumferential and longitudinal LA strains were impaired in patients with hemophilia, other peak LA strains were similar between the groups. LA strains at atrial contraction did not differ between groups of hemophilia patients and controls. Hemophilia is not associated with LA volumetric changes, but mild LA functional abnormalities are present.