BACKGROUND: Transthyretin cardiac amyloidosis (ATTR-CA) is an under-recognized cause of heart failure with preserved ejection fraction. In the United States, the valine-to-isoleucine substitution (Val122Ile) is the most common inherited variant. Data on sex differences in presentation and outcomes of Val122Ile ATTR-CA are lacking.
METHODS AND RESULTS: In a retrospective, single-center study of 73 patients diagnosed with Val122Ile ATTR-CA between 2001-2018, sex differences in clinical and echocardiographic data at the time of diagnosis were evaluated. Pressure-volume analysis using non-invasive single beat techniques was used to compare chamber performance. Compared to men (n=46), women (n=27) were significantly older at diagnosis (76 years vs 69 years, p<0.001). End-systolic pressure-volume relationship (5.1mmHgm(2)/mL vs 4.3 mmHgm(2)/mL, p=0.27), arterial elastance (5.5mmHgm(2)/mL vs 5.7mmHgm(2)/mL, p=0.62), and left ventricular capacitance were similar between sexes as was pressure-volume areas indexed to a left ventricular end-diastolic pressure of 30 mmHg, a measure of overall pump function. Three-year mortality rates were also similar (34% vs 43%, p=0.64).
CONCLUSIONS: Despite being significantly older at time of diagnosis with Val122Ile ATTR-CA, women have similar overall cardiac chamber function and rates of mortality to men, suggesting a less aggressive disease trajectory. These findings should be confirmed with longitudinal studies.
Tags: Amyloid, Noninvasive PV Loops