Yamaguchi syndrome or apical hypertrophic cardiomyopathy is a rare subtype of non-obstructive hypertrophic cardiomyopathy that is defined as the focused hypertrophy of the left ventricular apex. It is typically seen in Asian populations. Herein, we present a rare case of Yamaguchi syndrome seen in a Hispanic female.

Apical hypertrophic cardiomyopathy, also called Yamaguchi syndrome, is a rare variant of hypertrophic cardiomyopathy. Yamaguchi syndrome is characterized by hypertrophy almost confined to the apical region of the left ventricle rather than the left ventricular septum. A case of 65-year-old Saudi man presented to the ER with angina, and the ECG, echocardiogram, and nuclear study confirmed the diagnosis with Yamaguchi. Reporting this case serves to help physicians broaden their vision in approaching patients with symptoms mimicking acute coronary syndrome.

Apical hypertrophic cardiomyopathy is a rare variant of hypertrophic cardiomyopathy characterized by abnormal heart muscle thickening, specifically affecting the left ventricle\'s apex. Classically revealing both giant T-wave inversions in the precordial leads of an electrocardiogram and a spade-like configuration of the left ventricular cavity on ventriculograms, the diagnosis of the apical variant has evolved with cardiac magnetic resonance imaging. Despite being well known among East Asian populations, the diagnosis of apical hypertrophic cardiomyopathy is often underestimated and overlooked among American patients due to the non-specific nature of echocardiography. In this case report, we present the diagnosis of apical hypertrophic cardiomyopathy in a middle-aged African American male with chronic palpitations. The diagnosis was confirmed using cardiac magnetic resonance imaging, which revealed extensive myocardial fibrosis. Ultimately, the patient was treated with an implantable cardioverter-defibrillator. Our case aims to enhance the understanding and facilitate the recognition and management of apical hypertrophic cardiomyopathy, particularly among non-Asian individuals. Current challenges revolve around robust risk stratification strategies for patients at high risk for sudden cardiac death that require device therapy.

Apical hypertrophic cardiomyopathy (ApHCM) is thought to be an uncommon variant of hypertrophic cardiomyopathy (HCM). This article is a literature review focusing on the characteristic electrocardiogram (EKG) and 2D echocardiogram findings as currently there are no specific ACC/AHA/ESC guidelines set as diagnostic criteria for ApHCM.

Apical hypertrophic cardiomyopathy (ApHCM), also known as Yamaguchi syndrome represents an uncommon morphologic variant of hypertrophic cardiomyopathy (HCM) in which the myocardial hypertrophy predominantly involves the apex of the left ventricle (LV). It is exemplified by \"giant\" negative precordial T-waves on electrocardiography and a peculiar \"spade-like\" configuration of LV cavity on ventriculography historically, and more recently, on echocardiography with use of image enhancing agents. The disease entity was first described in 1976. Available literature reveals that it is prevalent largely among the East-Asian population but is rare among non-Asians. Here, we report a case of a 66-year-old Hispanic male with multiple cardiac histories including persistent atrial fibrillation, non-ST-elevation myocardial infarction (NSTEMI), and ventricular fibrillation cardiac arrest with multiple inconclusive evaluations, who later in life was found to have ApHCM. This case highlights the rare incidence of the disease among the Hispanic population and underlines the challenging diagnosis that requires a high index of suspicion in patients with cardiac symptoms, as ApHCM can masquerade as ischemic coronary heart disease. Our case also describes an unusual clinical course for ApHCM presenting with extreme clinical features, including ventricular arrhythmias and cardiac arrest, unlike the usual benign natural history of this disease.

Electrocardiogram (ECG) still remains a very useful diagnostic method in modern cardiology. Its broad availability, noninvasiveness and good sensitivity explain why it plays a capital role in the very beginning of the process of diagnosis for every patient, with or without cardiac-related complaints. For the practitioner, good training in ECG interpretation is mandatory. Sometimes, the ECG trace reveals particular aspects that may cause confusion and complicate decision-making. In this article, we present several less common situations underlying the general context and ECG features. The syndromes studied have a high pathological significance and may range from acute emergencies that call for a rapid therapeutical response to chronic syndromes that require prolonged observation, monitoring and risk stratification.

Apical hypertrophic cardiomyopathy is a relatively rare subtype of hypertrophic cardiomyopathy with a wide range of clinical manifestation. The most frequent symptom is chest pain and thus it can mimic the acute coronary syndrome and due to unfamiliarity of this condition by some physicians, the diagnosis is frequently missed or delayed. In this case, report our purpose is to emphasize the importance of keeping apical hypertrophic cardiomyopathy as one of the differential diagnoses in a young patient presented with chest pain.

The HeartMate 3 is a ventricular assist device that supports the heart with a centrifugal continuous flow. It contains a fully levitated rotor to minimize hemolysis and was initially designed as an apical intrapericardial implant. It can be used as a bridge to a transplant, to recovery, or to destination therapy. After we excise the ventricles, we implant 2 HeartMate 3 devices as a total artificial heart (HeartMate 6). The patient was 35 years old when the devices were implanted and had been diagnosed with Yamaguchi syndrome (apical hypertrophic cardiomyopathy) at 13 years of age. Being listed for a transplant was not an option due to secondary pulmonary hypertension. Furthermore, the conventional method of apically implanting a left ventricular assist device was not possible due to the underlying pathology. A HeartMate 6 implant as a bridge to transplant therapy was planned. Additionally, a CardioMEMS HF System was implanted to monitor the pulmonary artery pressure. The video tutorial provides step-by-step instructions for implanting 2 HeartMate 3 devices as a total artificial heart.

Apical hypertrophic cardiomyopathy (ApHCM) is a rare variant of hypertrophic cardiomyopathy, characterized by a spade-like left ventricular cavity. A 58-year-old African-American female with past medical history of hypertension presented for evaluation of recurrent exertional chest tightness, palpitations and headache. Prior workup including multiple stress tests and angiogram was non-conclusive. Electrocardiogram (EKG) showed characteristic marked T-waves inversions in inferior leads and left ventriculography revealed left ventricle apical hypertrophy with spade like left ventricular cavity that was typical of Yamaguchi syndrome. This case highlights the rare incidence of the disease among African American as well as the challenging diagnostic and presentation features of the disease.