Year: 2024 | Month: October-December | Volume: 9 | Issue: 4 | Pages: 435-453
DOI: https://doi.org/10.52403/ijshr.20240449
Yamaguchi Syndrome (Ace of Spades) in an Elderly Male. Case Report and Literature Review
Akhil Mehrotra1, Faiz Illahi Siddiqui2
1Chief, Pediatric and Adult Cardiology, Prakash Heart Station, Nirala Nagar, Lucknow, UP, India.
2Cardiac Technician, Prakash Heart Station, Nirala Nagar, Lucknow, UP, India.
Corresponding Author: Dr. Akhil Mehrotra
ABSTRACT
Apical hypertrophic cardiomyopathy (AHCM) is a rare phenotypic variant of hypertrophic cardiomyopathy (HCM), most commonly seen in Asian men (Yamaguchi syndrome). It is characterized by hypertrophy, predominantly affecting the cardiac apex, with an "ace of spades"-shaped left ventricular (LV) cavity best seen on the 4-chamber view of a transthoracic echocardiogram (TTE). However, TTE can be falsely negative in 30% of AHCM cases, largely due to difficulties in delineating endocardial border. The diagnostic criteria for apical cardiac hypertrophy are: 1) asymmetric LV hypertrophy - predominantly at the apex of the ventricle; 2) LV wall thickness of 15 mm or more during diastole; and 3) apical to posterior wall thickness ratio of 1.5 or more determined by 2-dimensional echocardiography or cardiac magnetic resonance imaging. Here, we are presenting a case of elderly male who visited our cardiac OPD for a check-up due to atypical chest pain, cough and mild fever for 5 days. His resting ECG showed left ventricular hypertrophy with deep T wave inversion in precordial leads V2-V6, consistent with AHCM. An exhaustive transthoracic echocardiography (TTE) with contrast study (CS) and speckle tracking imaging by 4Dimensional XStrain speckle tracking echocardiography (STE) was performed to provide a clinching diagnosis.
Keywords: Hypertrophic cardiomyopathy, Apical hypertrophic cardiomyopathy, Ace of spades, Strain echocardiography, Contrast imaging in apical hypertrophic cardiomyopathy, Yamaguchi syndrome, 4Dimensional XStrain echocardiography.