DOI: 10.25881/BPNMSC.2020.89.65.034


Vakhromeeva M.N., Solomannikov V.M., Vakhrameeva A.Yu., Sivokhina N.Yu.

Pirogov National Medical and Surgical Center, Moscow


Apical non-obstructive hypertrophic cardiomyopathy also known as Yamaguchi syndrome is a rare inheritable disorder that affects the inner structure of myocardium of the left ventricle giving it a certain ace-of-spades appearance on ventriculograms, echocardiography and cardiac MRI. First described my H. Yamaguchi in 1979 this rare disorder is still a challenge for physicians around the world. Given that this condition might manifest in a number of clinical syndromes or simply coexist with other cardiac pathologies, single-photon emmition tomography seems like a viable option for differential diagnosis of coronary artery disease in this group of patients. Therefore we present a clinical observation of such a patient and a detailed illustrated description of all SPECT findings. Our case report is accompanied by a small review of literature on the matter, which, we hope, shall facilitate the work of future physicians that will have to work with patients with this rare and complex disorder.

Keywords: SPECT, hypertrophic cardiomyopathy, Yamaguchi syndrome, echocardiography.


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For citation

Vakhromeeva M.N., Solomannikov V.M., Vakhrameeva A.Yu., Sivokhina N.Yu. Cardiac SPECT in diagnosis of Yamaguchi syndrome. Bulletin of Pirogov National Medical & Surgical Center. 2020;15(3-2):188-192. (In Russ.)