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CA5 Yamaguchi's syndrome: A case of apical hypertrophic cardiomyopathy. (Cardiology).


CA5 YAMAGUCHI'S SYNDROME: A CASE OF APICAL HYPERTROPHIC CARDIOMYOPATHY. Eron Crouch, MD, Timothy Mixon, MD, and Tahir Tak, MD, PhD. Scott & White Memorial Hospital, Texas A&M University System Health Science Center, College of Medicine, Temple.

A 47-year-old white man, with no significant medical history, presented with complaints of dyspnea and fatigue. Physical examination revealed a grade 3/6 midsystolic ejection murmur at the left sternal sternal /ster·nal/ (ster´n'l) of or relating to the sternum.

ster·nal
adj.
Of, relating to, or occurring near the sternum.



sternal

pertaining to the sternum.
 border and a prominent S4. The electrocardiogram electrocardiogram /elec·tro·car·dio·gram/ (-kahr´de-o-gram?) a graphic tracing of the variations in electrical potential caused by the excitation of the heart muscle and detected at the body surface.  (ECG) showed sinus rhythm with deep, symmetrical negative T-waves in leads V3-V6. Cardiac enzymes were negative. Echocardiography Echocardiography Definition

Echocardiography is a diagnostic test that uses ultrasound waves to create an image of the heart muscle. Ultrasound waves that rebound or echo off the heart can show the size, shape, and movement of the heart's valves and
 (ECHO) revealed apical hypertrophy, with apical cavity obliteration during systole systole /sys·to·le/ (sis´to-le) the contraction, or period of contraction, of the heart, especially of the ventricles.systol´ic

aborted systole
. These findings were confirmed by left ventricular angiography and by contrast ECHO showing a typical spade-like configuration during diastole diastole /di·as·to·le/ (di-as´tah-le) the dilatation, or the period of dilatation, of the heart, especially of the ventricles.diastol´ic

di·as·to·le
n.
. The patient was diagnosed with apical hypertrophic cardiomyopathy (Yamaguchi's syndrome) and started on [beta]-blocker therapy, which resulted in amelioration of symptoms. Outpatient follow-up with 24-hour continuous ECG recording showed no evidence of atrial or ventricular arrhythmias. Yamaguchi's syndrome is a rare form of hypertrophic cardiomyopathy frequently characterized by chest pain, dyspnea, fatigue, and in rare instances, sudden death. ECG often reveals deep negative T-waves in the precordial leads. ECHO shows a characteristic picture of an ace of spades in diastole and cavity obliteration at end-systole. Magnetic resonance imaging magnetic resonance imaging (MRI), noninvasive diagnostic technique that uses nuclear magnetic resonance to produce cross-sectional images of organs and other internal body structures.  is useful in patients who are difficult to image by ECHO. Apical hypertrophy was first described in a Japanese population where it accounted for up to 25% of all cases of hypertrophic cardiomyopathy. However, the prevalence of hypertrophic cardiomyopathy is approximately 0.2% in the general population throughout the world. The apical form of hypertrophic cardiomyopathy is generally thought to have a more favorable prognosis in terms of morbidity and mortality Morbidity and Mortality can refer to:
  • Morbidity & Mortality, a term used in medicine
  • Morbidity and Mortality Weekly Report, a medical publication
See also
  • Morbidity, a medical term
  • Mortality, a medical term
.
COPYRIGHT 2001 Southern Medical Association
No portion of this article can be reproduced without the express written permission from the copyright holder.
Copyright 2001, Gale Group. All rights reserved. Gale Group is a Thomson Corporation Company.

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Publication:Southern Medical Journal
Article Type:Brief Article
Geographic Code:1USA
Date:Dec 1, 2001
Words:279
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