Yamaguchi Syndrome: A Difficult Diagnosis in the Differential Diagnosis of Acute Coronary Syndrome

Year 2023, Volume: 25 Issue: 2, 209 - 211, 30.08.2023

Ali Batur , Hasan Can Sağlam , Ahmet Karakaya , Bülent Erbil

https://doi.org/10.18678/dtfd.1293614

Abstract

Apical hypertrophic cardiomyopathy (ApHCM) (Yamaguchi Syndrome) with hypertrophy of the ventricular apex constitutes 8% of the hypertrophic cardiomyopathies (HCMs). ApHCM can cause ventricular malignant dysrhythmias, atrial fibrillation, and ischemic chest pain. Definitive diagnosis is made by electrocardiography (ECG) and transthoracic echocardiography. A 73-year-old male patient was admitted to the emergency department with chest pain. The patient's vital signs were within the normal range. In the ECG, there were giant negative T wave in leads V4-5-6, 0.5 mm ST segment depression, and left ventricular hypertrophy in the inferior derivations. The left ventricular apex thickness was measured as 14 mm (reference range: 6-11). Although the HEART score was 4, the preliminary diagnosis of the patient was determined as ApHCM. Beta-blocker and antiplatelet therapy were started. The mortality and morbidity rates of ApHCM are higher among HCMs. Clinicians should be aware of such ECG and echocardiography findings to prevent possible morbidity and mortality.

Keywords

Apical, hypertrophic cardiomyopathy, Yamaguchi syndrome, acute coronary syndrome, echocardiography

Yamaguchi Sendromu: Akut Koroner Sendrom Ayırıcı Tanısında Zor Bir Tanı

Abstract

Ventriküler apeksin hipertrofisi ile seyreden apikal hipertrofik kardiyomiyopati (ApHKM) (Yamaguchi Sendromu) hipertrofik kardiyomiyopati (HKM)’lerin %8'ini oluşturmaktadır. ApHKM ventriküler malign disritmilere, atriyal fibrilasyona ve iskemik göğüs ağrısına neden olabilir. Kesin tanı elektrokardiyografi (EKG) ve transtorasik ekokardiyografi ile konur. 73 yaşında bir erkek hasta göğüs ağrısı şikayetiyle acil servise başvurdu. Hastanın vital bulguları normal sınırlardaydı. EKG'de V4-5-6 derivasyonlarında dev negatif T dalgası, 0,5 mm ST segment depresyonu ve inferior derivasyonlarda sol ventrikül hipertrofisi vardı. Sol ventrikül apeks kalınlığı 14 mm (referans aralığı: 6-11) olarak ölçüldü. HEART skoru 4 olmasına rağmen hastanın ön tanısı ApHKM olarak belirlendi. Beta-bloker ve antiplatelet tedavi başlandı. HKM'ler arasında ApHKM'nin mortalite oranları daha yüksektir. Klinisyenler olası morbidite ve mortaliteyi önlemek için bu tür EKG ve ekokardiyografi bulgularının farkında olmalıdır.

Keywords

Apikal, hipertrofik kardiyomiyopati, Yamaguchi sendromu, akut coroner sendrom, ekokardiyografi

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