2017
DOI: 10.1136/bcr-2016-218051
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Yamaguchi syndrome presenting as atrioventricular nodal re-entrant tachycardia in an African-American patient

Abstract: Apical hypertrophic cardiomyopathy (Yamaguchi syndrome) is a rare subtype of hypertrophic cardiomyopathy. The syndrome is more common in Japan where it was first described. Outside Asia, it is a very rare cause of hypertrophic cardiomyopathy. Apical hypertrophic cardiomyopathy is usually detected incidentally and has a good long-term outcome. We present a case of apical hypertrophic cardiomyopathy in an African-American patient manifesting as atrioventricular nodal re-entrant tachycardia.

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Cited by 3 publications
(5 citation statements)
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“…The diagnosis of apical HCM can be achieved by multiple different modalities. The key EKG finding is the “giant negative T waves” (greater than or equal to 10 mm) in the precordial leads and high QRS voltage in the absence of notable coronary artery disease [ 8 , 10 , 11 ]. These deep, inverted T waves are often mistaken for ACS [ 12 ].…”
Section: Discussionmentioning
confidence: 99%
See 1 more Smart Citation
“…The diagnosis of apical HCM can be achieved by multiple different modalities. The key EKG finding is the “giant negative T waves” (greater than or equal to 10 mm) in the precordial leads and high QRS voltage in the absence of notable coronary artery disease [ 8 , 10 , 11 ]. These deep, inverted T waves are often mistaken for ACS [ 12 ].…”
Section: Discussionmentioning
confidence: 99%
“…Characteristics associated with a poor prognosis include young age at diagnosis, family history of sudden cardiac death, and NYHA class II or greater [ 12 ]. Patients should be followed up annually for repeat echocardiograms [ 11 ].…”
Section: Discussionmentioning
confidence: 99%
“…The syndrome is most common in Japan, where it was first described. Outside Japan, it is a very rare cause of hypertrophic cardiomyopathy [12,13].…”
Section: Discussionmentioning
confidence: 99%
“…Complementary imaging has become indispensable for the therapeutic planning of patients with AHCM [11]. In unusual presentations such as apical morphology, the determination of aspects such as myocardial fibrosis, valvar regurgitation, systolicdiastolic dysfunction, complex arrhythmias, myocardial ischemia, among others, shows the importance of diagnostic resources in order to allow longevity with quality of life as In the case described above [11][12][13][14][15][16].…”
Section: Discussionmentioning
confidence: 99%
“…Paraclinical diagnosis. Electrocardiography will reveal characteristically giant negative T waves, greater than 10 mm, and left ventricular hypertrophy, which can be confirmed using the Sokolov-Lyon criteria and calculating whether the sum of the S wave in V1 and the R wave in leads V5, V6 is greater than 35 mm [50]. These ECG changes should be presented as stand-alone and without the presence of an important underlying coronary artery disease or hypertension.…”
Section: Yamaguchi Syndromementioning
confidence: 99%