Brief Communications

1630

American

6. Koga Y, Itaya M, Takahashi H, Koga M, Ikeda H, et al. Apical hypertrophy and its genetic and acquired factors. J Cardiography 1985;15(suppl 6):65-74. 7. Webb KG, Sasson Z, Rakowski H, Liu P, Wigle D. Apical hypertrophic cardiomyopathy: clinical follow-up and diagnostic correlates. J Am Co11 Cardiol 1990;15:83-90. 8. Yamaguchi H, Ishimura T, Nishiyama S, Nagasaki F, Nakanishi S. Hypertrophic nonobstructive cardiomyopathy with giant negative T waves (apical hypertrophy): ventriculographic and echocardiographic features in 30 patients. Am J Cardiol 1979;44:401-12.- 9. Jarcho JA, McKenna W, Pare JAP, Solomon SD, Holcombe RF. et al. MaDDine a gene for familial hvpertrophic cardiomyopathy to ch&moiome 14ql. N Engl J-tied 1$89;321:1372-8. 10. Solomon SD, Jarcho JA, McKenna W, Geisterfer-Lowrance AAT, Germain R, et al. Familial hypertrophic cardiomyopathy is a genetically heterogeneous disease. J Clin Invest 1990;86:993-9. 11. Ko YL, Lien WP, Chen JJ, Wu CW, Tang KT, Liew CC. No evidence for linkage between a Chinese family with familial hypertrophic cardiomyopathy and chromosome 14ql locus D14S26: evidence for genetic heterogeneity. Hum Genet (In press) 12. Geisterfer-Lowrance AAT, Kass S, Tanigawa G, Vosverg HP, McKenna W, et al. A molecular basis for familial hypertrophic cardiomyopathy: a cardiac myosin heavy chain gene missense mutation. Cell 1990;62:999-1006.

Cardiac amyloidosis detected by indium-1 11 antimyosin imaging

and incomplete right bundle branch block. Twenty-four hour Holter monitoring showedfrequent ventricular premature beats and rare episodesof ventricular tachycardia. The cardiothoracic ratio on chest x-ray wasborderline, and a small pleural effusion on the right was evident. The echocardiogramshowedmild left ventricular hypertrophy with normal diameter, mild left atria1 enlargement, and normal systolic function; Doppler echocardiography showedI+ mitral and 2+ tricuspid insufficiency. Left- and right-sided cardiac catheterization was remarkable for mild elevation of pressurein the right atrium (10 mm Hg), right ventricle (42/Omm Hg), and pulmonary artery (41/17 mm Hg); pulmonary capillary wedge pressurewas 18 mm Hg, and cardiac output was 5 L/min. Coronary arteries were normal, and left ventricular ejection fraction was 50%. Results of thallium-201 perfusion scanning at rest werenormal (Fig. 1). Right ventricular endomyocardial biopsy showedhypertrophy of myocytes with degenerative changes.Congo-red staining was positive for amyloid in vesselwalls and interstitially. There were no signsof myocarditis.

Myosin-specific

Greece

One of the morelikely causesof restrictive cardiomyopathy is cardiac amyloidosis, the diagnosis of which may be missedfrequently during life.’ Radionuclide imaging with technetium-99m pyrophosphate yields valuable resuIts,2 whereasthe useof *231-labeledserumamyloid phosphorus component appears promising for the identification of amyloidosis.3We recently detected a caseof cardiac amyloidosis associatedwith multiple myeloma in which indiurn-111 antimyosin imageswere increasingly positive. A 57-year-old womanwas first seenwith exertional dyspnea, episodes of paroxysmal nocturnal dyspnea, and edema of the lower extremities. Physical examination findings included low arterial blood pressure(100/70 mm Hg), jugular venous distention, and hepatomegaly. ECG revealed low-voltage first-degree atrioventricular block From the Departments of Clinical Therapeutics and Nuclear Medicine, Alexandra University Hospital.

41414

requests: Greece. 1398

John

Lekakis,

MD,

80 Alkionis

St., P. Faliron

antibody

(Myoscint,

2 mCi of “lIn

was diluted

to a 10 ml volume

and

administered intravenously by slow injection. Planar imaging

was performed

175 62.

48 hours

later;

three

views

were

obtained (anterior and 45-degreeand 70-degreeleft anterior

oblique)

by means

of a 128 X 128 matrix

for

10

minutes/view. Diffuse and intense uptake of antimyosin was evident in both ventricles (Fig. 2), indicating myocyte damage.Determination of urinary and serumprotein concentrations

Reprint Athens,

monoclonal

Centocor Inc., Malvern, Pa.) was supplied as a sterile nonpyrogenic solution containing 0.5 mg of RI1 Dlo Fab DTPA, which is a mousemonoclonal antibody fragment that binds specifically to myosin. Antimyosin was radiolabeled by the addition of sterile I’In chloride. Approximately

John Lekakis, MD, John Nanas, MD, Chrysa Moustafellou, MD, John Darsinos, MD, John Germanidis, MD, Nicos Agapitos, MD, Panos Kostamis, MD, and Spyridon Moulopoulos, MD Athens,

December 1992 Heal Journal

by electrophoresis

and immunoelectrophoresis

yielded findings consistentwith plasmacell dyscrasia;bone marrow studiesestablisheda diagnosisshowinginfiltration by plasma cells. ‘llIn-labeled antimyosin monoclonal antibodies have been shownto bind specifically to areasof necrosisin vitro4; on postmortem imaging, tracer uptake matched the triphenyltetrazolium chloride staining, confirming that myosin-specific antibody binds specifically to damaged myocardial cells.5 Previous studies reported antimyosin uptake in patients with myocardial infarction, myocarditis, dilated cardiomyopathy, cardiac transplant rejection, and adriamycin cardiotoxicity.6 This is the first report showing antimyosin uptake in cardiac amyloidosis. The pattern of uptake is a typical diffuse one, analogousto that found in myocarditis, indicating a global effect of amyloid deposits on myocyte membranes.Rupture of the myocardial cell membrane and exposure of myosin are necessary for antimyosin to bind to the myocardium and showa positive scintigraphic result. In addition, although no data are available, the possibility that antimyosin binds to amyloid cannot be excluded. Identification of cardiac amyloidosis remains problem-

Volume124 Number 6

Brief Communications

1631

Fig. 1. Thallium-201 resting imagesshowingnormal per-

fusion

atic during life. Roberts and Waller’ reported the clinical and autopsy findings of 54 patients who died of cardiac amyloidosis; the diagnosiswas established during life in only 33:A of them. Radionuclide imagingmay be useful in detecting patients with this condition; Wizenberg et a1.2 reported 10 patients with tissue-proved amyloidosisand intense diffuse uptake of ggmT~pyrophosphate. Scintigraphy with 1231-labeled serumamyloid P componentappears promising.” The present report showsthat “lIn antimyosin imaging may alsobe useful in detecting cardiac amyloidosis,but there is no doubt that for the evaluation of this method a large number of patients with cardiac amyloidosis must be examined. REFERENCES

1. Roberts WC, Wailer BF. Cardiac amyloidosis causing cardiac dysfunction; analysis of 54 necropsy patients. Am J Cardiol 1983;52:137-46. 2. Wizenberg TA, Muz I, Sohn YH, Samlowski W, Weisslez AM. Value of positive myocardial technetium-99m pyrosphosphate scintigraphy in the noninvasive diagnosis of cardiac amyloidosis. AM HEART J 1982;103:468-79. 3. Hawkins PN, Lavender JP, Pepys MB. Evaluation of systemic amyloidosis by scintigraphy with 1231-labelled serum amyloid P component. N Engl J Med 1990;323:508-13. 4. Khaw BA, Fallon JT, Belier GA, Gaber E. Specificity of localization of myosin-specific antibody fragments in experimental myocardial infarction. Circulation 1979;60:1527-31. 5. Jain D, Crawley JC, Lahiri A, Rafley EB. Indium-111 antimyosin images compared with triphenyltetrazolium chIoride staining in a patient six days after myocardial infarction. J Nucl Med 1990;31:231-3. 6. Lekakis J, Vassilopoulos N, Psichoyiou H, Athanassiadis P, Gerali S, Kostamis P, Moulopoulos S. Doxorubicin cardiotoxicity detected by indium-Ill myosin specific imaging. Eur J Nucl Med 1991;18:225-6.

Fig. 2. “lIn antimyosin imagesshowingdiffuse uptake in both left and right ventricles.

QRST isointegral maps in patients with Romano-Ward syndrome OsamuKinoshita, MD, Yasuki Takabayashi, MD, Masao Tanaka, MD, Minoru Hongo, MD, and Morie Sekiguchi, MD Matsumoto, Japan

Patients

with

the

Romano-Ward

syndrome’s

2 show

a pro-

longed QT interval and an abnormal configuration of the T wave on the electrocardiogram; they are prone to ventricular fibrillation and sudden death. The disorder is predominantly a childhood illness; however, a marked variation in the degree of clinical expressionoccurs, and someaffected subjectsdo not showclinical signsuntil age 30. In general,the later the ageof onset,the milder the diseaseand the lessthe threat of suddendeath.3Clinical and experimental observationssupport the hypothesisthat the most likely pathogenetic mechanismof the long QT syndrome is an imbalance in the sympathetic innervation of the heart. The recovery processof myocardial fibers is markedly influenced by sympathetic activity, and regional From The First Department of Internal Medicine. Shinshu University School of Medicine. Reprints requests: Osamu Kinoshita, MD, The First Department of Internal Medicine, Shinshu University School of Medicine, 3-l-l Asahi, Matsumoto City, Nagano Prefecture, 390 Japan. 4/4141393