DOI: 10.1111/j.1540-8175.2010.01238.x
CME
C 2010, Wiley Periodicals, Inc.
A Novel Two-Dimensional Echocardiographic Finding in Cardiac Amyloidosis Robert N. Belkin, M.D., F.A.C.C., F.A.S.E.,∗ Andrew C. Kupersmith, M.D.,∗ Omar Khalique, M.D.,∗ Wilbert S. Aronow, M.D., F.A.C.C., F.A.H.A.,∗ Kiran Chilappa, M.D.,∗ Chandrasekar Palaniswamy, M.D.,∗ Warren D. Rosenblum, M.D.,∗ Alan Gass, M.D.,∗ Tauseef Ahmed, M.D.,† and Umadevi S. Katta, M.D.‡ ∗
Department of Medicine, Division of Cardiology; †Department of Hematology and Oncology; and ‡Department of Pathology, New York Medical College and Westchester Medical Center, Valhalla, New York
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) Key words: cardiac amyloidosis, echocardiography Cardiac amyloidosis (CA) is an uncommon but fatal disorder that may be underdiagnosed.1 Early recognition and prompt initiation of therapy may be important because the median survival following onset of heart failure symptoms is severely reduced. This will particularly apply to AL amyloid, the most common form of CA, for which a number of therapeutic options are available.1 Nevertheless, recognition of CA with noninvasive techniques may be difficult. Although a number of echocardiographic findings characteristic of this disorder have been described, including the finding of increased wall thickness with low voltage on electrocardiogram (ECG), each of these findings has limitations.2–4 We describe here a distinctive, previously unrecognized twodimensional (2D) echocardiographic finding in seven consecutive patients with CA confirmed by endomyocardial biopsy. We found in each of these patients an unusual but characteristic wall motion pattern of preserved myocardial thicken-
Address for correspondence and reprint requests: Wilbert S. Aronow, F.A.C.C., F.A.H.A., Cardiology Division, New York Medical College, Macy Pavilion, Room 138, Valhalla, NY 10595. Fax: +(914) 235-6274; E-mail:
[email protected]
ing at left ventricular apex with hypokinesis in basal and midsegments. Methods: Following initial anecdotal observation of this wall motion pattern in two patients with CA, endomyocardial biopsy files beginning in 2007 were searched for patients with proven disease. The pathologic diagnosis of amyloidosis was made on the basis of Congo red staining, immunohistochemical stains, and direct immunofluorescence. Seven patients with endomyocardial biopsy proven cardiac amyoidosis were identified. Two-dimensional echo Doppler studies were read in consensus by two experienced academic, board certified echocardiographers. Harmonic imaging was employed for all studies. Among patients with more than one echocardiographic study, the one closest in time to the date of endomyocardial biopsy was reviewed. Results: Demographic findings, amyloid type, and echocardiographic findings of the seven patients are shown in Table I. All seven patients were men; their ages ranged from 55 to 82 years. All seven 1171
Belkin, et al.
TABLE I Demographic Characteristics and Two-Dimensional Echocardiographic Findings Age
Amyloid Type
Wall Motion Pattern
Estimated LVEF
Increased Wall Thickness∗
Increased Echogenicity∗∗
Pericardial Effusion
Restrictive Inflow Pattern∗∗∗
1 2 3 4 5 6 7
69 57 55 74 82 74 75
AL AL AL AL AL AL SCA
Yes Yes Yes Yes Yes Yes Yes
30–39% >50% 40–49% 30–39% 30–39%
