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Source publication
Patient: Female, 53
Final Diagnosis: Apical hypertrophic cardiomyopathy
Symptoms: Chest pain • dizziness • palpitations
Medication: —
Clinical Procedure: —
Specialty: Cardiology
Objective
Rare disease
Background
Apical hypertrophic cardiomyopathy (ApHCM) is a relatively rare form of hypertrophic cardiomyopathy that predominantly affects the apex...
Context in source publication
Context 1
... heart catheter- ization demonstrated no significant epicardial coronary artery disease. Left ventriculogram was suggestive of a spade-like ap- pearance ( Figure 2); however, the interpreting cardiologist did not comment on this until later. During admission, our patient was noted to have several pre-syncopal episodes with non-sustained ventricular tachycardia (NSVT), the longest run lasting 25 beats. ...
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The natural history and etiology of SCAD remain unclear. This was a young female with chest pain and inferior ST elevations. Cardiac catheterization revealed type 1 angiographic SCAD of the LMCA. This case was unusual in that the injury pattern of inferior posterior MI was from a LMCA SCAD.
Citations
... Specifically, in this case, chest pain may have been misattributed to apical HCM. It is known that approximately 14% of patients with HCM are known to complain of chest pain symptoms [11] , which are thought to be caused by microvascular dysfunction, reduced capillary density, and myocardial bridging [ 12 ,13 ]. ...
Epipericardial fat necrosis (EFN) is a rare benign cause of chest pain, that is frequently overlooked. EFN involves the necrosis of fat tissue in the mediastinum, and presents on computed tomography (CT) as an ovoid lesion of fat attenuation surrounded by a rim of soft tissue attenuation. This case report describes a case of a 50-year-old man diagnosed with EFN on cardiac CT, which was incidentally associated with apical hypertrophic myocardiopathy. Notably, the detection of EFN proved difficult on arterial phase images during coronary CT angiography, whereas it was much easier to detect on delayed phase images. EFN should be considered in the differential diagnosis of chest pain, and careful examination of mediastinal fat is crucial for accurate diagnosis.
... Management should be aimed at symptom control, genetic counseling, control of comorbidities, and prevention of major cardiovascular events 8 . Beta-blockers or non-hybrid pyridine calcium antagonists could be a therapeutic option for reducing oxygen consumption, preventing ventricular arrhythmias, and decreasing heart rate to improve diastolic filling 9 . Some case reports and series have proposed catheter ablation and surgical management in patients who develop ventricular arrhythmias and left ventricular outflow tract obstruction, respectively 10 . ...
... [9] The aim of treatment for ApHCM is mainly to control symptoms and prevent complications, which are done by means of medications such as beta-blockers or calcium channel blockers to control the heart rate and also ACE inhibitors to reduce left ventricular after-load. [10] For a patient who is persistently symptomatic or has a high risk of sudden cardiac death an ICD will be inserted. The first-degree family member of the patient will be screened for having a similar condition with genetic testing and echocardiography also. ...
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.
... Los resultados a largo plazo de la terapia con beta-bloqueadores en los pacientes con ApCMPH son desconocidos y se necesitan más estudios. Los bloqueadores de los canales de calcio no dihidropiridínicos son una terapia de segunda línea si los pacientes no pueden tolerar los beta-bloqueadores 14,16 . ...
Apical hypertrophic cardiomyopathy is an uncommon form of hypertrophic cardiomyopathy characterized by a compromise of the left ventricular apex and is distinguished by the ace morphology in the ventriculography. The heterogeneity and variability in the presentation of this pathology and the clinical course, make a diagnosis easily omitted. We describe a clinical case of a patient with apical hypertrophic cardiomyopathy that debuted with precordial pain and syncope of cardiogenic origin. The purpose of this report is to review the main characteristics of a rare pathology, epidemiology, diagnosis, prognosis and treatment and thus, to provide medical personnel with the approach of this pathology in clinical practice.
... It presents signs of left ventricular overload (LVO) with deeply inverted T-waves in an electrocardiogram (ECG) (Fig. 1) [1]. Some variants of the disease present ventricular aneurysm and left ventricular outflow tract obstruction [1,2] ( In Japan, AHCM corresponds to 25% of all cases of hypertrophic cardiomyopathy (HCM), although in other countries, particularly in the west, the incidence corresponds to 1 to 3% of the cases of HCM [3]. Despite being generally considered as benign, AHCM may achieve a cardiovascular morbidity rate of between 25 and 30%, and a mortality rate of between 4 and 29% of cases [2]. ...
... Some variants of the disease present ventricular aneurysm and left ventricular outflow tract obstruction [1,2] ( In Japan, AHCM corresponds to 25% of all cases of hypertrophic cardiomyopathy (HCM), although in other countries, particularly in the west, the incidence corresponds to 1 to 3% of the cases of HCM [3]. Despite being generally considered as benign, AHCM may achieve a cardiovascular morbidity rate of between 25 and 30%, and a mortality rate of between 4 and 29% of cases [2]. ...
... Clinically, around 54% of patients are symptomatic, most frequently presenting with: dyspnea, syncope, palpitation and chest pain [2]. The most frequent complication is atrial fibrillation (AF) [4]. ...
Background
Apical hypertrophic cardiomyopathy (AHCM) is a rare cardiomyopathy, in which hypertrophy occurs predominantly in the ventricular apex, and in some cases with a high risk of sudden cardiac death.
Objective
The aim of this paper is to present a case series of patients with AHCM and describe their main clinical, echocardiographic and electrocardiographic characteristics, the recommendation for an implantable cardioverter-defibrillator (ICD) and the frequency of sudden cardiac death (SCD).
Methods
A retrospective case series was conducted at the referral center of a federal teaching hospital, between the years 2005 to 2020, involving patients with an echocardiographic diagnosis of AHCM. The parameters of the American College of Cardiology and the European Society of Cardiology were used to assess the risk of SCD.
Results
A total of 11 individuals were assessed with a mean age of 55.3 years, mean follow-up of 41.2 months, most of whom were symptomatic at diagnosis (72.7%). The most frequent symptom was dyspnea (27.3%). A family history of SCD was described in 45.5% of cases. Due to a high risk of SCD, four patients received ICDs. One patient presented sudden cardiac death after having refused the ICD.
Conclusions
Symptoms and alterations in the imaging exams are significant factors in the clinical and prognostic assessment of patients with AHCM.
... Apical Hypertrophic Cardiomyopathy (ApHCM), a rare form of HCM, manifests as hypertrophy of the left ventricular apex and has nonobstructive physiology. Research has found sarcomere mutations can cause apical hypertrophy, [3]. Obstruction in BiApHCM is due to midsystolic contact of the prominent RV muscle bundles in the RV outflow tract. ...
Apical Hypertrophic Cardiomyopathy (ApHCM) is a rare variant of hypertrophic cardiomyopathy with a low prevalence in the general population. ApHCM with right ventricular involvement (BiApHCM) is largely unreported and may not be detected with conventional transthoracic echocardiogram (TTE) alone. Cardiac Magnetic Resonance (CMR) has been demonstrated to be a proficient imaging modality to diagnose BiApHCM. We present a case of BiApHCM that was diagnosed with TTE and further characterized by CMR. This imaging modality may be utilized more in the future to help diagnose and detect the prevalence of BiApHCM.
... Clinically, the manifestations can vary from apical aneurysms, atrial tachycardia or atrial fibrillation, ventricular arrhythmias and sudden cardiac death. About 54% of patients are asymptomatic [4]. Nevertheless, one third of patients experience serious cardiovascular complications, such as myocardial infarction and arrhythmias [5]. ...
... The finding of apical hypertrophic cardiomyopathy in this patient likely does not suggest an increase in mortality or morbidity due to its historically benign course. However, recent studies have shown increased morbidity and mortality in this subset of patients [4]. Factors like advanced age, hypertension, diabetes, baseline atrial fibrillation, left atrial volume index and apical aneurysm on cardiac magnetic resonance are predictors of poor prognosis. ...
Abstract Background Left ventricular apical hypertrophic cardiomyopathy is a rare presentation of hypertrophic cardiomyopathy associated with thickening of the apical segment of the left ventricle. It was initially described in Japan in 1976 and is characterized by electrocardiogram findings showing giant T wave inversions in the precordial leads as well as a spade shaped appearance of the apical cavity on imaging (Abugroun et al., Cardiol Res 8:265-268, 2017). In this case, we present a patient with a heart transplant with a stable post-transplant course who was found to have apical hypertrophic cardiomyopathy. There have been a few cases of apical hypertrophy in a transplanted heart documented in the literature. Making this case even more unique is that this presentation is evident 17 years after heart transplantation. Case presentation Fifty-four year-old male with a history of orthotropic heart transplant in 2001 on immunosuppressive therapy presented with palpitations and associated lightheadedness. He had a blood pressure of 184/89 mmHg on arrival but otherwise had stable vital signs and physical examination. Cardiac biomarkers revealed a CK of 59 U/L and a troponin of 0.11NG/ML(normal
... Recent discovery also showed that ACEI will reduce the adverse cardiac remodeling and fibrosis in ApHCM. 3 For a patient who is persistently symptomatic or has high risk of sudden cardiac death, an ICD will be inserted. The first-degree family member of the patient will be screened for having a similar condition with genetic testing and also ECHO. ...
Yamaguchi syndrome, or apical hypertrophic cardiomyopathy (ApHCM), is a nonobstructive subtype of hypertrophic cardiomyopathy which predominantly affects the left ventricular apex. Due to the nature of its presentation that mimics acute coronary syndrome and also to the unfamiliarity of the condition by some physicians, the diagnosis of ApHCM is frequently missed or delayed. Here, we report a case of a young male who presented with chest pain and breathlessness. His cardiac enzyme was normal and electrocardiogram showed giant negative T-waves. He was treated as unstable angina and was then subjected to a line of diagnostic procedures including coronary angiogram before he subsequently underwent ventriculogram which reveal a characteristic “spade-like” configuration over the left ventricle, in keeping with the diagnosis of ApHCM. The purpose of this case report is to emphasize the importance of keeping ApHCM as one of the differential diagnoses in a young patient presented with chest pain.
... The evolution of imaging diagnostic resources has made AHCM evaluation more elaborate, often providing information that helps therapeutical conduction, allowing an increase in survival with a significant improvement in quality of life. right ventricular hypertrophy with right bundle branch block, encephalic changes and transient changes during coronary angiography due to apical hypertrophy [12]. ...
... The descriptions of AHCM date back to the 19th century. In 1979, Yamaguchi and his colleagues published a series of 30 patients with cardiomyopathy with apical presentation, a study that gained great repercussion and denominated Yamaguchi's Disease [3,12]. ...
... The syndrome is most common in Japan, where it was first described. Outside Japan, it is a very rare cause of hypertrophic cardiomyopathy [12,13]. ...
... обычно предпочтение отдаётся β-Аб без вазодилатирующего эффекта во избежание нарастания градиента при сжо. в случае непереносимости или противопоказаний β-Аб, препаратами второй линии являются недигидропиридиновые блокаторы кальциевых каналов [48]. действие верапамила и дилтиазема опосредовано их отрицательными инотропным и хронотропным эффектами, что приводит к уменьшению боли в груди, увеличению времени диастолического наполнения лж и улучшению миокардиальной перфузии. ...
The review provides information about apical hypertrophic cardiomyopathy (HCM), which is a relatively rare phonotype of HCM. Previously, it was believed that apical HCMP has a favorable prognosis, but recent studies have demonstrated an increased risk of fatal arrhythmias and sudden cardiac death, especially among elderly patients, women and those with comorbidities such as arterial hypertension and diabetes mellitus. Patients with apical HCMP complain of chest pain, shortness of breath and palpitations. The diagnosis is based on data from various investigation methods, among which imaging techniques such as echocardiography and cardiac magnetic resonance imaging are crucial. For apical HCMP, a characteristic sign is the identification of the configuration of the left ventricular cavity at the end of the systole resembling to the type "Ace-of-spades". Treatment includes pharmacotherapy using beta-blockers, calcium channel blockers, cardiac myosin inhibitors and surgical interventions. The lack of information on unfavorable prognostic factors, the lack of specific clinical data and the complexity of instrumental diagnosis emphasize the relevance of studying the problem of apical HCMP.
