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syndrome. This multisystem condition is more extensive than previously recognized> When evaluating the patient with syndrome myxoma, we recommend that first-degree relatives (parents, siblings, and children) of affected patients have a complete physical examination, with special emphasis on the cardiovascular system, study of the skin in natural and ultraviolet light, and palpation of the testes. Echocardiography should be done. Adrenal function should be tested by determining the 24-hour excretion of free cortisol in the urine. Until more is known of syndrome myxoma, we recommend that these investigations be repeated yearly in affected persons6r7 In the era of echocardiography’* and cardiothoracic surgery, cardiac myxomas are more than a medical curiosity. Although such myxomas are infrequently encountered, there seem to be two types (sporadic and syndrome). Recognition of the existence of syndrome myxoma is important because of its clinical, surgical, and genetic implications.‘-6
REFERENCES
1 Braunwald E. Heart disease: a textbook of cardiovascular medicine, vol 2. 2nd ed. Philadelphia: WB Saunders, 1984: 1460. 2. Vidaillet HJ Jr, Seward JB, Fyke E III, Tajik AJ. NAME syndrome (nevi, atrial myxoma, myxoid neurofibroma, enhelides): a new and unrecognized subset of patients with cardiac myxoma. Minn Med 1984;67:695. 3. Carney JA, Gordon H, Carpenter PC, Shenoy BV, Go VLW. The complex of myxomas, spotty pigmentation, .and endocrine overactivity. Medicine (Baltimore) 1985;64:270. 4. Carney JA. Differences between nonfamilial and familial cardiac myxoma. Am J Surg Path01 1985;9:53. 5. McCarthy PM, Piehler JM, Scheff HV, Pluth JR, Orszulak TA, Vidaillet HJ Jr, Carney JA. The significance of multiple, recurrent, and “complex” cardiac myxomas. J Thorac Cardiovaac Surg 1986;91:389. 6. Vidaillet HJ Jr, Seward JB, Fyke FE III, SU WPD, Tajik AJ. “Syndrome myxoma”: a subset of patients with cardiac myxoma associated with pigmented skin lesions and peripheral and endocrine neoplasms. Br Heart J 1987;57:247. 7. Carney JA, Hruska LS, Beauchamp GD, Gordon H. Dominant inheritanceof the complexof myxomas,spotty pigmentation, and endocrine overactivity. Mayo Clin Proc 1986; 61:165. 8. Gorlin RJ, Anderson RC, Blaw M. Multiple lentigenes syndrome: complex comprising multiple lentigenes, electrocardiographic conduction abnormalities, ocular hypertelorism, pulmonary stenosis, abnormalities of genitalia, retardation of growth, sensorineural deafness, and autosomal dominant hereditary pattern. Am J Dis Child 1969;117:652. 9. Kleid JJ, Klugman J, Haaa J, Battock D. Familial atrial myxoma. Am J Cardiol 1973;32:361. 10. Atherton DJ, Pitcher DW, Wells RS, MacDonaldDM. A syndrome of various cutaneous pigmented lesions, myxoid neurofibromate and atrial myxoma: the NAME syndrome. Br J Dermatol 1980;103:421. 11. Rhodes AR, Silverman RA, Harrist TJ, Perez-Atayde AR. Mucocutaneous lentigines, cardiomucocutaneous myxomas, and multiple blue nevi: the “LAMB” svndrome. J Am Acad Dermatol i984;10:72. 12. Fyke FE III, Seward JB, Edwards WD, Miller FA Jr, Reeder GS, Schattenberg ‘IT, Shub C, Callahan JA, Tajik AJ. Primary cardiac tumors: experience with 30 consecutive patients since the introduction of two-dimensional echocardiography. J Am Co11 Cardiol 1985;5:1465.
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Doppler color flow mapping demonstration of diastolic mitral regurgitation in severe acute aortic regurgitation Jean-Luc Vandenbossche,M.D., and Marc Englert, M.D. Brussels, Belgium
Although diastolic mitral regurgitation was documented by angiography for the first time in 1966+* controversy still remains regarding whether premature closure of the mitral valve, occurring in the setting of acute aortic regurgitation, is the result only of a reversal of the atrioventricular pressuregradient or is also related to the back flow of blood from the aortic regurgitation.3 In the present observation we show that Doppler can easily document the presenceof diastolic regurgitation, indicating that premature closure of the mitral valve is associated with a reversal of the atrioventricular gradient. A 50-year-old man was referred to our echocardiography laboratory for evaluation of aortic regurgitation secondary to bacterial endocarditis. The cross-sectionalechocardiogram showed thickening of the aortic valve and a dilated and hyperkinetic left ventricle. On M-mode examination there was typical premature mitral valve closure, with fluttering of the anterior mitral leaflet. The Doppler color flow mapping (Fig. 1) demonstrated on both Mmodeand cross-sectionalmode the presenceof retrograde (blue coded) flow in the left atrium occurring in late diastoleand disappearingin systole.The extension of flow was visible up to 3 cm away from the mitral valve closure point. Maximal velocities of the regurgitating flow were measured at 1.5 m/set, indicating a small (8 mm Hg) retrograde gradient between ventricle and atrium. The regurgitating aortic flow showed a pressurehalf-time of 100 msec, with cessation of back flow before the QRS complex. Locking of the mitral valve requires not only a reversal of pressuregradient between left ventricle and atrium but also creation of tension in the mitral valve apparatus produced by a properly timed ventricular systole.’ For instance, atrioventricular conduction abnormalities are commonly associatedwith diastolic mitral regurgitation5 In acute aortic regurgitation, premature mitral closure is actually followed by a smallamount of separationbetween the leaflets until after ventricular systole. In addition, it has been repeatedly shown that premature closureof the mitral valve wasassociatedwith a reversedmitral diastolic gradient.6*7Hence, these two conditions contribute to the occurrence of diastolic mitral regurgitation. Observation by Doppler of diastolic mitral regurgitation may have clinical implications: first, it should help to differentiate From the Cardiology Department, Saint-Pierre University Hospital. Reprint requests: J. L. Vsndenbossche, M.D., Cardiology Depsrtment, Saint-Pierre gium.
University
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Rue Haute,
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Octobrr 1907 Heart Journal
Fig. 1. On the left, M-mode color flow mapping of the mitral valve, left ventricle, and left atrium, obtained from apical position. Premature closure of the mitral valve is indicated by the white arrow. A blue area, indicating backward flow, appears below the mitral valve echocardiogram at the time of premature closure and disappearsin early systole. Note the relatively uniform blue color, without aliasing, indicating rather low velocities. On the right, long-axis apical cross-sectionalcolor flow mapping, frozen in late diastole. Mitral regurgitation is illustrated by a blue area extending 3 cm in the left atrium. (AR= aortic regurgitation; LA = left atrium; LV = left ventricle; MR = mitral regurgitation.)
angiographic diastolic mitral regurgitation from organic systolic insufficiency, which can occur in the setting of aortic valve endocarditis caused by jet lesion on the anterior mitral leaflet. Moreover, its presence in the course of acute aortic regurgitation indicates that the left atrium and the pulmonary vasculature are not protected from elevation of pressureby the premature closure, as commonly thought. REFERENCES
1. Aldridge HE, Lansdown EL, Wigle ED. Diastolic mitral insufficiencv. Circulation 1966;34:42. 2. Lochaya ST Igarashi M, Shaffer AB. Late diastolic mitral regurgitation secondary to aortic regurgitation: Its relationship to the Austin Flint murmur. AM HEART J 1967;74:161. 3. Feigenbaum H. Echocardiography, ed 4. Philadelphia: Lea & Febiger, 1986z212. 4. Little RC. The mechanism of closure of the mitral valve: a continuing controversy. Circulation 1979;59:615. 5. Panidis II?, Ross J, Munley B, Nestico P, Mintz GS. Diastolic mitral regurgitation in patients with atrioventricular conduction abnormalities: a common fmding by Doppler echocardiography.J Am Coll Cardiol1986;7:768. 6. Oliver GC Jr, Gaeetopoulos N, Deuchar DC. Reversed mitral diastolic gradient in aortic incompetence. Br Heart J 1967;293239. 7. Botvinick EH. Schiller NB, Wickramasekaran R, Klausner SC, Gertz E.’ Echocardiographic demonstration of early mitral valve closure in severe aortic insufficiency. Circulation 1975;51:836.
Right and left coronary artery to left ventricle fiitula detected by color D@@W flow mapping Toshio Nishikimi, M.D., Hisao Oku, M.D., Kazuyoshi Hirota, M.D., Kayoko Murai, M.D., Takahiko Kawarabayashi, M.D., Minoru Yoshiyama, M.D., Kaname Akioka, M.D., Masakazu Teragaki, M.D., Mitsutaka Yasuda, M.D., Kazuhide Takenuchi, M.D., Tadanao Takeda, M.D. Osaka City, Japan
Coronary artery fistulas are rare congenital anomalies consisting of a communication between the coronary arteries and intracardiac chambers. The majority of the casesof coronary artery fistulas drain into the right sideof the heart, that is the pulmonary artery, coronary sinus, right atrium, or right ventricle.’ Fistulas between the coronary artery and the left ventricle are extremely rare.2 From the Fit Department of Internal Medicine, Osaka City Univereity Medical School. Reprint,requests: Tcshio Niahikimi, M.D., First Department of Internal Medicine, Osaka City University Medical School, l-5-7, Abeno-ku, Aeahimachi, Osaka City, 545, Japan.