MITRAL VALVE PROLAPSE IN KLINEFELTER SYNDROME

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    cancer growth rapidly progresses to reach the clinical threshold fordiagnosis within a year. If the patient responds to adjuvantchemotherapy, subclinical tumour growth can be temporarilyarrested; hope of cure is unrealistic.

    (7) The hazards of polychemotherapy are substantial. Mortalitydue to chemotherapy may be as high as 4 .4%.11 Side effects such asanorexia, nausea, vomiting, gastroenteritis, hepatic toxicity, bonemarrow depression, hair loss, and cystitis are common, and thepatients feel ill. Palmer and co-workers 12 discontinued their trial ofadjuvant chemotherapy because of severe, partly "unbearable"side-effects.In view of the many uncertainties and controversies about

    adjuvant chemotherapy, which itself has serious health hazards, nopatient should be routinely subjected to this kind of "treatment".

    Departments of Obstetrics-Gynecologyand of Pharmacology,

    School of Medicine,University of New Mexico,Albuquerque, New Mexico 87131, U.S.A. HELMUTH VORHERR

    MITRAL VALVE PROLAPSE IN KLINEFELTERSYNDROME

    SIR,The frequency of mitral valve prolapse ("floppy valvesyndrome" 1,2) in the normal population is 5-10%.3,4 Thepossibility of female sex preponderance in this syndrome iscontroversial. 4,5 The incidence of the syndrome is considerablyincreased in certain hereditary disorders, particularly in diseaseswith abnormal collagen structure or metabolism-e.g., Marfan, 1,6Ehlers-Danlos, and von Willebrand syndromes. Mitral valveprolapse syndrome has also been found in a higher frequency ingonadal dysgenesis (Ullrich-Turner syndrome).9 As far as we know,the prevalence of mitral valve prolapse in Klinefelters syndrome hasnot been reported.We investigated sixteen consecutive patients with Klinefelter

    syndrome, the karyotypes being 47,XXY in eleven, 48,XXYY intwo, and 48,XXXY, 49,XXXXY, and 46,XY/47,XXY mosaicism(one each). The mean age was 29 years, the youngest patient(49,XXXXY) being 10 and the oldest (mosaic) 50. Six patients hadvaricose veins, and in three this was severe. Osteoporosis waspresent in three patients. On auscultation, a mid-systolic click washeard in ten cases a "click-murmur syndrome"2 being detected insix of them. Echocardiograms revealed prolapse of the posteriormitral leaflet into the left atrium in nine patients (see figure).M-mode and sector scan techniques were applied in all cases and infour patients mitral valve prolapse could be elicited by provocation(handgrip or orciprenaline infusion).The severity of mitral valve prolapse was graded by a modification

    of the Markiewicz method. (see figure). Severe pansystolic

    11. Milstead RAV, Fox RM, Tattersall MHN, Woods RL. Cancer chemotherapy: Whathave we achieved? Lancet 1980; i: 1343-46.

    12. Palmer BV, Walsh GA, McKinna JA, Greening WP. Adjuvant chemotherapy forbreast cancer: side effects and quality of life. Br Med J 1980; 281: 1594-97.

    1. Read RC, Thal AP, Wendt VE. Symptomatic valvular myxomatous transformation(the floppy valve syndrome): a possible forme fruste of the Marfan syndrome.Circulation 1965; 32: 897-910.

    2. Barlow JB, Bosman CK, Pocock WA, Marchand P. Late systolic murmurs and non-ejection (mid-late) systolic click: an analysis of 90 patients. Br Heart J 1968; 30:203-18

    3. Braunwald E. Valvular heart disease: The mitral valve prolapse syndrome. In:Braunwald E, ed. Diseases of heart. Philadelphia: WB Saunders, 1980, 1121-127.

    4. Darsee JR, Mikolich JR, NicoloffNB, Lesser LE. Prevalence of mitral valve prolapsein presumably healthy young men. Circulation 1979; 59: 619-22.

    5. Markiewicz W, Stoner J, London E, Hunt S, Popp RL. Mitral valve prolapse in onehundred presumably healthy young females. Circulation 1976; 53: 464-73.

    6. Khl I, Fricke G, Kardiovaskulre Manifestationsformen des Marfan-Syndroms. KlinWschr 1973; 51: 1129-142.

    7. Jaffe AS, Geltman EM, Rodey GE, Uitto J. Mitral valve prolapse: a consistentmanifestation of type IV Ehlers-Danlos syndrome. Circulation 1981; 64: 121-25.

    8. Pickering NJ, Brody JI, Barrett MJ. von Willebrands syndrome and mitral valveprolapse. N Engl J Med 1981; 305: 131-34

    9. Barlow JB, Pocock WA. Mitral valve prolapse, the specific billowing mitral leafletsyndrome, or an insignificant non-election systolic click. Am Heart J 1979; 97:277-85.

    KARYOTYPE

    Mitral valve prolapse in Klinefelter syndrome.

    prolapse of both posterior and anterior mitral leaflets was notencountered. Haemodynamically significant mitral regurgitationwas suggested by pulsed Doppler echocardiography in two patientswith mitral valve prolapse (karyotypes 48,XXYY and 49,XXXXY).Moderate to marked mitral valve prolapse has been a regular findingin our patients with 47 chromosomes or more. Seven patientsshowed essential normal mitral valve kinetics at rest as well as

    during provocation.We conclude that mitral valve prolapse is a frequent finding in

    Klinefelter syndrome (56% in our series). The mechanism isunknown. Perhaps the weakness of connective tissues in thesepatients, which predisposes to osteoporosis and varicosis 10, 11 alsoplays a role in the alterated structure of the mitral valve. Androgendeficiency may also influence the metabolism of collagene fibrils.Age does not seem important in the development of mitral prolapse,compared with the composition of the karyotype. In Klinefeltersyndrome a thorough examination of the heart, clinically andechocardiographically, is advisable since mitral valve prolapse, withits attendant risks of cardiac arrhythmia, endocarditis, chest pain,and even sudden death, seems common in such patients.

    Medical Policlinic,University of Bonn,D-5300 Bonn 1, West Germany

    G. R. FRICKEH. J. MATTERNH. U. SCHWEIKERT

    PERINATAL MORTALITY AND SOCIAL CLASS IN THEWEST MIDLANDS

    SiR,Since 1975, when a computer aided system was introducedby the Office of Population Censuses and Surveys to link individualregistered births with the corresponding perinatal deaths, accuratenational data have been available on social class trends by region andarea. The data for 1975-78 are substantial and homogenous whengrouped together; but in 1979 the assignments of occupations tosocial classes were changed so that subsequent years are not directlycomparable. 2An unexpected feature of the data for 1975-78 is that, while for

    England and Wales as a whole, there is a distinct gradient forperinatal mortality (PNM) rates by social class, rates for social classv being twice these of social class I (24 and 12 per 1000 live andstillbirths, respectively), in the West Midlands region the gradient isless steep, with PNM rates of 25 and 16 for social classes v and I,

    10. Zuppinger K, Engle E, Forbes AP, Mantooth L, Claffey J. Klinefelters syndrome: aclinical and cytogenetic study in twenty-four cases. Acta Endocrinol 1967; 54: Suppl113, 5-48.

    11. Hornstein O. Zur Klinik und Histopathologie des mnnlichen primrenHypogonadismus: II. Mitteilung. Arch klin Exp Derm 1963; 217: 149-95.

    1. Adelstein AM, Davies IMM, Weatherall JAC. Stud Med Popul Subj 1980; no 41.2. OPCS Monitor DH3 81/1.