Systolic and diastolic ventricular function in cardiac amyloidosis. Studies in six cases diagnosed with endomyocardial biopsy.

Five cases of cardiac amyloidosis diagnosed after endomyocardial biopsy are described. Systolic function was normal in three cases as assessed by cardiac index, ejection fraction and maximal velocity of contractile element shortening at zero developed pressure. The diastolic dip and plateau wave form was a characteristic but not an invariable finding. Increased ventricular end-diastolic pressure but normal end-diastolic volume indexes in all cases implied a disorder of diastolic compliance. The diastolic pressure-volume plots suggested a sudden decrease in distensibility after normal early filling of the ventricle. The functional defect did not necessarily differ from that of other diseases of heart muscle. Therefore, hemodynamic data should not be relied upon for diagnostic purposes. Electron microscopy was the most reliable aid in making the diagnosis from the endomyocardial biopsy specimens especially in the presence of fibrosis. It may not be valid to assume a diagnosis of cardiac amyloidosis from the finding of amyloid in other organs.