Left ventricular systolic and diastolic time intervals in familial amyloid polyneuropathy

To determine left ventricular (LV) systolic and diastolic properties in patients with familial amyloid polyneuropathy (FAP), we analyzed simultaneous recordings of the electrocardiogram, phonocardiogram, carotid arterial pulse, and apex cardiogram in 14 patients and 21 normal subjects. LV systolic and diastolic time intervals were measured. Correlations between the intervals and neurologic disabilities, duration of illness, and age were examined. We found that in patients with FAP, 1) LV relaxation was impaired, especially in early diastole, whereas systolic function was generally preserved, 2) abnormal LV relaxation could be seen before the development of both clinically identifiable heart disease and the abnormalities in systolic function, 3) indexes of LV systolic function and relaxation decreased in parallel with the progress of the disease, duration of illness, and age, and 4) electromechanical delay was markedly increased.