A Novel Two-Dimensional Echocardiographic Finding in Cardiac Amyloidosis

Background: A number of echocardiographic findings characteristic of cardiac amyloidosis (CA) have been described, each with limitations. Methods: A distinctive wall motion pattern of preserved myocardial thickening at left ventricular apex with hypokinesis in basal and midsegments was observed in two patients with biopsy proven CA. Following this observation, endomyocaradial biopsy files beginning in 2007 were reviewed. Seven consecutive patients with documented CA were identified. Two-dimensional (2D) echocardiograms for each were reviewed in consensus by two experienced echocardiographers. Clinical and electrocardiographic data were obtained from chart review. Results: All patients were men with class II-IV heart failure. Six had light chain CA, 1 senile CA. Six patients had coronary angiography. One had a 60% left anterior descending coronary artery stenosis. Five had nonobstructive disease. Echocardiograms for all seven patients demonstrated the distinctive pattern of preserved myocardial thickening at apex with hypokinesis in basal and midsegments. Reduced ejection fraction was present in six and increased wall thickness and myocardial echogenicity in seven. Other echo signs of amyloid were variably present. Three had low voltage on electrocardiogram. Conclusion: A distinctive 2D echocardiographic pattern of preserved segmental wall motion at left ventricular apex with hypokinesis in basal to midsegments was consistently identified in seven consecutive patients with endomyocardial biopsy-proven CA. (Echocardiography 2010;27:1171-1176).