Apical hypertrophic cardiomyopathy (AHC)

ECG features of AHC
  • The classic ECG finding in apical HCM is giant T-wave inversion in the precordial leads. Inverted T waves are also commonly observed in the inferior and lateral leads
  • Localised hypertrophy of LV apex, causing an “ace of spades” configuration of the LV cavity on ventriculography

Non-obstructive variant of Hypertrophic Cardiomyopathy (HCM). This relatively uncommon form of HCM is seen most frequently in Japanese patients (13-25% of all HCM cases in Japan).

Also known as Yamaguchi syndrome after the Hiroshi Yamaguchi MD and his original papers on hypertrophic non-obstructive cardiomyopathy with giant negative T waves (apical hypertrophy) in 1976 and 1979.

ECG HCM Apical

1979 Yamaguchi et al reviewed 1,002 consecutive left heart catheterization patients. Of the total, 30 patients exhibited the pathognomonic “ace of spades” shape of the left ventricle (LV).

2003 Kitaoka et al. showed a prevalence of 15% in an unselected Japanese cohort compared with 3% in an unselected Minneapolis cohort. They found an increased incidence among men, confirmed by Husser et al (2018) who found the incidence increased with age and further favoured men as age increased  


Ace-of-spades sign 
ace of spades sign ecg

Pathognomonic left ventricule configuration seen in apical hypertrophic cardiomyopathy. Marked ventricular wall thickening at the apex resulting in cavity narrowing at the apex with a relatively normal appearance of the mid-ventricular to basal wall and cavity.

The sign can be demonstrated on echocardiography, ventriculography, and cardiac MRI

Ventriculogram

ace of spades Yamaguchi syndrome Apical hypertrophic cardiomyopathy
4: Left ventriculograms in hypertrophic non-obstructive cardiomyopathy obtained at (A) end diastole and (B) end-systole in the right anterior oblique (RAO) projection. Note the spade-like configuration at end diastole with a marked increase in the free wall thickness toward the apex (arrows).
5: Left ventriculograms in hypertrophic obstructive cardiomyopathy at (A) end-diastole the free wall is equally thickened in all segments (arrows) and the left ventricular silhouette is shaped like a kidney or banana. (B), at end systole.
Yamaguchi 1979
Ace of spades Yamaguchi 1979
Ventriculographic patterns of hypertrophic cardiomyopathy: end-diastolic configurations. Yamaguchi 1979

Cardiac MRI

A cardiac MRI with findings suggestive of apical hypertrophic cardiomyopathy. The left ventricle is observed in the right view at the end of diastole with “ace of spades” shape, characteristic of Yamaguchi syndrome

Cardiac MRI Yamaguchi syndrome
Cardiac MRI ace of spades sign in Yamaguchi syndrome. Morales 2018

Examples
Example 1
ECG Apical HCM HOCM

This ECG shows the typical pattern of apical HCM:

  • Large precordial voltages.
  • Giant T wave inversions in the precordial leads
  • Inverted T waves are also seen in the inferior and lateral leads.

This great ECG is reproduced from Hansen & Merchant (2007).


Example 2
Apical hypertrophic cardiomyopathy (AHC) Yamaguchi syndrome
Shah FA et al. Cureus. 2021

This ECG shows the typical pattern of apical HCM:

  • T wave inversions in leads I, II, and V2-V6

Videos
HCM video education



Advanced Reading

References

Online

Textbooks


LITFL Further Reading

ECG LIBRARY

Electrocardiogram

MBBS (UWA) CCPU (RCE, Biliary, DVT, E-FAST, AAA) Adult/Paediatric Emergency Medicine Advanced Trainee in Melbourne, Australia. Special interests in diagnostic and procedural ultrasound, medical education, and ECG interpretation. Editor-in-chief of the LITFL ECG Library. Twitter: @rob_buttner

Emergency physician MA (Oxon) MBChB (Edin) FACEM FFSEM with a passion for rugby; medical history; medical education; and asynchronous learning #FOAMed evangelist. Co-founder and CTO of Life in the Fast lane | Eponyms | Books | Twitter |

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