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American lieart Journal

detecting the flail posterior leaflet of the pulmonic valve as well as the anterior leaflet as in the case described by Nakamura et al6 We suggest that echocardiographic examination of the pulmonic valve should be done if diastolic murmur is newly developed in a patient with suspected infective endocarditis.

REFERENCES

1. Sheikh MU, Ali N, Covarrubias E, Fox LM, Morjaria M, Dejo J: Right-sided infective endocarditis. An echocardiographic study. Am J Med 66:283, 1979.

2. Berser M. Delfin LA. Jelveh M. Goldbern E: Two-dimension-

Fig. 3. The excised pulmonic valve showing markedly destroyed left posterior pulmonic leaf-let (LPPL) with vegetation (arrow) and some shortening and thickening of the other leaflets. APL = anterior pulmonic leaflet; RPPL = right posterior pulmonic leaflet.

of the leaflets were markedly thickened. There was no pulmonic stenosis (Fig. 3). A VSD (10 X 10 mm) was present just below the crista supraventricularis. The leaf- lets and the chordae tendineae of the tricuspid valve were preserved except for a tiny vegetation (0.5 X 1 mm) attached to a chorda. There was a jet lesion on the right ventricular mural endocardium behind the anterior tricus- pid leaflet which was not infected. The puhnonic valve was excised and was replaced with a St. Jude Medical valve. The VSD was closed using a Teflon patch. The tiny vegetation attached to the chorda and the squashed tissue at the jet lesion were excised and curetted, respectively. Postoperatively the patient made uneventful recovery.

Two aspects of this case deserve mention: (1) severe pulmonic valve endocarditis in the presence of the mem- branous VSD and (2) echocardiographic demonstration of the flail pulmonic valve. In infective endocarditis in patients with VSD, vegetations usually lodge on the right ventricular mural endocardium at the jet lesion and/or on the tricuspid valvular endocardium.6 However, in this case, the pulmonic valve was most severely involved and destroyed, whereas only a tiny vegetation was there at a chorda of the tricuspid valve. A recent report4 described pulmonic valve endocarditis in patients with VSD. How- ever, VSD in those cases either was present at the subpulmonic region or was associated with pulmonic valvular stenosis as VSD was at the subcristal region. Thus, the clinical course of this patient was unique in that severe pulmonic valve endocarditis developed with sub- cristal VSD without pulmonic stenosis. Nakamura et al.5 reported 2DE demonstration of flail anterior leaflet of the pulmonic valve in a patient with infective endocarditis. The results in this case indicate that 2DE may be useful in

al echoca;diographic’ findings in right-sided infective endo- carditis. Circulation 61:855, 1980. Mehlman DJ, Furey W, Phair J, Effron M, Hartz R: Two- dimensional echocardiographic features diagnostic of isolated pulmonic valve endocarditis. AM HEART J 103:137, 1982. Nakamura K, Satomi G, Sakai T, Ando M, Hashimoto A, Koyanagi H, Hirosawa K, Takao A: Clinical and echocardio- graphic features of pulmonary valve endocarditis. Circulation 67398, 1983. Nakamura K, Suzuki S, Sakakibara S, Komatsu Y, Takaha- shi S, Hirosawa K: M-mode and cross-sectional echocardio- graphic features of flail pulmonary valve. A case report. Jpn Heart J 21:897, 1980. Edwards JE, Carey LS, Neufeld HN, Lester RG: Congenital heart disease. Philadelphia, 1965, W. B. Saunders Company, p. 107.

Two-dimensional echocardiographic documentation of accessory chordae tendineae accompanying isolated anterior mitral cleft

Yusuke Yamamoto, M.D., Rihei Shimada, M.D., Shunichi Kaseda, M.D., Hitonobu Tomoike, M.D., Akira Takeshita, M.D., and Motoomi Nakamura, M.D., Fukuoka, Japan

A cleft in the anterior leaflet of the mitral valve is often associated with endocardial cushion defect (ECD), but it may infrequently occur as an isolated anomaly and may result in congenital mitral regurgitation.1-3 The accessory chordae tendineae, not found in the normal heart, consti- tute one of the important features of this anterior mitral cleft.‘, 2. 4 We describe two-dimensional echocardiographic (2DE) findings of the accessory chordae tendineae which co-exist with the isolated anterior mitral cleft.

A 22-year-old man was admitted to our clinic for examination of heart murmur. He was asymptomatic. On

From Research Institute of Angiocardiology and Cardiovascular Clinic, Faculty of Medicine, Kyushu University.

Reprint requests: Motoomi Nakamura, M.D., Research Institute of Angio- cardiology and Cardiovascular Clinic, Faculty of Medicine, Kyushu Uni- versity, 3-l-l Maidashi, Higashi-ku, Fukuoka 812, Japan.

Volume 106

Number 6 Brief Communications 1555

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Fig. 1. Long-axis two-dimensional echocardiograms in diastole representing the accessory chordae tendineae (arrows) arising from the ventricular septum and attaching to the thickened anterior mitral leaflet. LV = left ventricle; RV = right ventricle; A0 = aorta; LA = left atrium.

c

Fig. 2. Short-axis 2DEs in diastole representing the anterior mitral cleft (panel A, arrow). Each free edge of the anterior mitral cleft is connected with the ventricular septum by the accessory chordae tendineae (panels B and C, arrows), Panel B, mid-diastole. Panel C, late-diastole. LV = left ventricle; RV = right ventricle.

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American Heart Journal

Fig. 3. The left ventriculogram representing mitral regurgitation. Mitral valve prolapse is not present.

physical examination, a grade 316 holosystolic murmur was heard at the apex, which radiated to the left sternal border. The first heart sound was reduced in intensity and a prominent third sound was present. An ECG showed left ventricular hypertrophy. Chest rentogenograms showed cardiomegaly with cardiothoracic ratio of 54% and small aortic knob with increased pulmonary vascularity. Long- axis 2DE (Fig. 1) showed an echo-reflective bundle (Fig. 1, arrows) which was attached to the ventricular septum at one end and to the thickened anterior mitral valve at the other. Mitral valve prolapse was not present. Short-axis 2DE (Fig. 2) showed that the anterior mitral valve was divided into two portions by the cleft (Fig. 2, A, arrow). The anomalous bundles (Fig. 2, B and C, arrows) were again noted between the ventricular septum and each free edge of the cleft anterior mitral leaflet. Thus, the bundles were identified as the accessory chordae tendineae. The cleft mitral valve, together with the accessory chordae tendineae, moved like a drawbridge. The cleft was located almost in the middle of the anterior mitral valve. Papillary muscles and the tricuspid valve were normal and the left ventricle was enlarged. The left ventriculogram (Fig. 3) showed moderate mitral regurgitation. The gooseneck deformity of the left ventricular outflow tract was not detected. Blood gas analysis revealed no intracardiac shunt. Accordingly, the clinical diagnosis was isolated anterior mitral cleft with accessory chordae tendineae, resulting in congenital mitral regurgitation.

Isolated anterior mitral cleft is considered to be an incomplete or a partial type of ECD.1*5 We could not confirm anatomic structures, since surgical intervention was not indicated. However, several studies3*4 relating complete or incomplete ECD described the presence of the

accessory chordae arising from the ventricular septum and attaching to the margins of the split leaflet. The 2DE findings in our case were consistent with the findings of these reports. The accessory chordae tendineae are clini- cally important since mitral regurgitation may persist unless the abnormal chordae are cut and the mitral valve is freed after the cleft is closed surgically.2.4 In the untreated state, the accessory chordae prevent the valvu- lar tissue bordering on the cleft from being flail.‘s4 Howev- er, they are not needed for valvular support after the cleft is closed. On the contrary, retention of the accessory chordae after the repair of the cleft may be an impedi- ment, because these chordae may exert a restraining action on the newly created single anterior mitral valve during ventricular systole.4 Therefore, it is important to document the accessory chordae preoperatively. In sum- mary, the present report provides the first 2DE documen- tation of the accessory chordae tendineae coexisting with isolated anterior mitral cleft.

REFERENCES

1. Perloff JK: Atria1 septal defect: The clinical recognition of congenital heart disease. Philadelphia, 1978, W.B. Saunders co., p 344.

2. Freedom RM: Congenital valvular regurgitation. Zn Keith JD, Rowe RD, Vlad P, editors: Heart disease in infancy and childhood. New York, 1978, Macmillan Publishing Co, Inc, p 828.

3. Davachi F, Moller JH, Edwards JE: Disease of the mitral valve in infancy. Circulation 43:670, 1971.

4. Edwards JE: The problem of mitral insufficiency caused by accessory chordae tendineae in persistent common atrioven- tricular canal. Mayo Clin Proc i&299, 1960.

5. Mints GS, Kotler MN, Segal BL, Parry WR: Two dimension- al echocardiographic evaluation of patients with mitral insuf- ficiency. Am J Cardiol 44:670, 1979.