scholarly journals Left Ventricular Apical Hypertrophy (Yamaguchi Disease)

2021 ◽  
Author(s):  
Hatim Al Lawati ◽  
Marwa Al Habsi ◽  
Adil B Al Riyami
Keyword(s):  
Chest Pain ◽  
Hypertrophic Cardiomyopathy ◽  
Coronary Angiography ◽  
Left Ventricular ◽  
Apical Hypertrophic Cardiomyopathy ◽  
Cardiac Evaluation ◽  
Apical Hypertrophy

Abstract Apical Hypertrophic Cardiomyopathy (Ap-HCM) also known as Yamaguchi Disease is characterized by limited involvement of the left ventricular (LV) apex. It can present with typical ischaemic chest pain, dyspnoea and arrhythmias. We report the case of patient with ischaemic chest pain who was diagnosed with Ap-HCM at the time of coronary angiography. We share representative images from his cardiac evaluation.

Apical hypertrophic cardiomyopathy: what are the risks in our diverse military population?

2018 ◽  
Vol 165 (3) ◽  
pp. 206-209
Author(s):  
Leanne Jane Eveson ◽  
A Williams
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Normal Sinus Rhythm ◽  
Cardiopulmonary Exercise Testing ◽  
Left Ventricular ◽  
Inferior Wall ◽  
Military Population ◽  
Apical Hypertrophic Cardiomyopathy ◽  
Normal Sinus ◽  
Wave Inversion ◽  
Apical Hypertrophy

We present the case of a 50-year-old, fit, asymptomatic gurkha officer. At a routine medical, an ECG showed T-wave inversion in the chest leads V3–6. Transthoracic echo showed left ventricular apical hypertrophy and cavity obliteration consistent with apical hypertrophic cardiomyopathy (ApHCM). Cardiac magnetic resonance imaging showed apical and inferior wall hypertrophy in the left ventricle with no aneurysm or scarring. A 24-hour monitor showed normal sinus rhythm with no evidence of non-sustained ventricular tachycardia. Eighteen-panel genetic testing revealed no specific mutations. Cardiopulmonary exercise testing demonstrated a V̇O2 max, anaerobic threshold and peak V̇O2 consistent with above average cardiopulmonary capacity. There was no family history of either ApHCM or sudden cardiac death (SCD). Risk of SCD by the European Society of Cardiology’s HCM calculator was low. This case generates discussion on the prognosis of ApHCM, factors that worsen prognosis, occupational limitation considerations and appropriate monitoring in this patient group.

scholarly journals Extended Myectomy for Apical Hypertrophic Cardiomyopathy: A Case Report

2021 ◽  
Author(s):  
Daiki Saitoh ◽  
Mike Saji ◽  
Schuichiro Takanashi
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Left Ventricle ◽  
Papillary Muscle ◽  
Left Ventricular ◽  
Left Ventricular Aneurysm ◽  
Apical Hypertrophic Cardiomyopathy ◽  
End Diastolic Volume ◽  
First Case ◽  
Surgical Myectomy ◽  
Apical Hypertrophy

Abstract BackgroundApical hypertrophic cardiomyopathy is a variant of hypertrophic cardiomyopathy that predominantly affects the left ventricle apex and rarely involves the right ventricular apex or both apexes. Traditional treatment for apical hypertrophic cardiomyopathy is heart transplantation. Although surgical myectomy approaching the apex has been available for decades, its safety and accuracy greatly depend on the surgeon’s skills and experience.Case presentationThe first case is of a 63-year-old man with apical hypertrophic cardiomyopathy in whom a preoperative contrast computed tomography revealed apical hypertrophy and complete apical cavity obliteration. The patient underwent extended myectomy. The inside of the apex cavity was filled with abnormal muscles. Using the transaortic approach, the location of the bilateral papillary muscle was confirmed, which provided the required orientation. The abnormal muscle mass was successfully resected, and postoperative end-diastolic volume was extremely increased. The second case is of a 43-year-old man with an apical left ventricular aneurysm and mid- hypertrophic cardiomyopathy obstruction. The thin-walled apical aneurysm contained a large apical-basal band. After detecting the bilateral papillary muscle, mid-ventricular myectomy was performed from the apex. During postoperative catheterization, there was no pressure gradient between the left ventricle and aorta.ConclusionsWe reviewed two cases of apical hypertrophic cardiomyopathy, efficiently treated using extended apical myectomy. Although apical myectomy is an uncommon procedure, the cases presented herein show that it can successfully manage apical hypertrophic cardiomyopathy. However, it is important to secure the postoperative left ventricular end-diastolic volume.

scholarly journals Extended myectomy for apical hypertrophic cardiomyopathy: a case report

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Daiki Saitoh ◽  
Mike Saji ◽  
Schuichiro Takanashi
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Left Ventricle ◽  
Papillary Muscle ◽  
Left Ventricular ◽  
Left Ventricular Aneurysm ◽  
Apical Hypertrophic Cardiomyopathy ◽  
End Diastolic Volume ◽  
First Case ◽  
Surgical Myectomy ◽  
Apical Hypertrophy

Abstract Background Apical hypertrophic cardiomyopathy is a variant of hypertrophic cardiomyopathy that predominantly affects the apex of the left ventricle and rarely involves the right ventricular apex or both apexes. Heart transplantation is the traditional treatment for apical hypertrophic cardiomyopathy. Although surgical myectomy approaching the apex has been available for decades, its safety and accuracy greatly depend on the surgeon’s skills and experience. Case presentation The first case involved a 63-year-old man with apical hypertrophic cardiomyopathy, wherein preoperative contrast computed tomography findings revealed apical hypertrophy and complete apical cavity obliteration. The patient underwent extended myectomy, which revealed the apex cavity filled with abnormal muscles. Using the transaortic approach, the location of the bilateral papillary muscle was confirmed, thereby providing the required orientation. The abnormal muscle mass was successfully resected, and the postoperative end-diastolic volume was extremely increased. The second case involved a 43-year-old man with an apical left ventricular aneurysm and mid-hypertrophic cardiomyopathy obstruction. The thin-walled apical aneurysm contained a large apical-basal band. Upon detecting the bilateral papillary muscle, mid-ventricular myectomy was performed from the apex. During postoperative catheterization, there was no pressure gradient between the left ventricle and aorta. Conclusions We reviewed two cases of apical hypertrophic cardiomyopathy, efficiently treated using extended apical myectomy. Although it is an uncommon procedure, the cases presented show how it can be used to successfully manage cases of apical hypertrophic cardiomyopathy. However, it is important to secure the postoperative left ventricular end-diastolic volume.

Abstract 3265: A Comparison of Echocardiographic and Electrocardiographic Characteristics of Patients with Apical Hypertrophic Cardiomyopathy

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Josepha Binder ◽  
Brandon R Grossardt ◽  
Christine Attenhofer Jost ◽  
Kyle W Klarich ◽  
Michael J Ackerman ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Ejection Fraction ◽  
Left Ventricular ◽  
Right Atrial ◽  
Apical Hypertrophic Cardiomyopathy ◽  
T Wave ◽  
Atrial Enlargement ◽  
Lv Ejection Fraction ◽  
The Relationship ◽  
T Waves

Background: Apical hypertrophic cardiomyopathy (apical HCM) is a less common subtype of HCM characterized by a focal thickening in the left ventricular apex. “Classic” ECG features have been described, however, apical HCM can persist for many years without detection. We investigated the relationship between ECG findings and echocardiographic morphometry in a large referral series of patients with apical HCM. Methods: We enumerated all patients diagnosed with apical HCM prior to Sept. 30, 2006 using the Mayo Clinic HCM database. We compared echocardiographic measures separately for patients with positive status for two ECG indices of left ventricular hypertrophy (LVH); the Sokolow-Lyon index and the Romhilt-Estes (RE) point-score. We also compared echocardiographic measurements in patients with and without negative T-waves in the precordial leads. Results: Apical HCM was detected in 177 patients (111 men and 68 women). Only 51% had positive Sokolow criteria and 51% had positive RE criteria. The agreement between Sokolow and RE status was high (agreement = 75.0%; kappa = 0.50; 95% CI = 0.38 – 0.62). In particular, Sokolow positive patients had increased LV ejection fraction (P = 0.02), and decreased LV end-systolic diameter (P = 0.03) compared with Sokolow negative patients. The prevalence of right atrial enlargement (47 vs. 28%; P = 0.02) and intracavity obstruction (22 vs. 8%; P = 0.01) were more common in Sokolow positive patients. Positive RE criteria was associated with a greater thickness of the basal septal and basal posterior walls (P = 0.001 and 0.02, respectively), and with a higher frequency of intracavity obstruction (21 vs. 9%; P = 0.04). Most patients (89%) exhibited at least one negative T-wave in the precordial leads; however, only 10% of patients had a negative T-wave of greater than 1.0 mV. We found that patients with an inverted T-wave larger than 0.4 mV (median) had a significantly increased LV ejection fraction (P = 0.03) compared with patients who had smaller or no negative T-waves. Conclusions: Among patients with apical HCM, nearly half do not have ECG evidence of LVH based on classic criteria and most do not have marked T-wave inversions. However, the majority did have at least a mild expression of negative T-waves.

Coronary artery–left ventricular shunt: an important cause of chest pain in patients with hypertrophic cardiomyopathy

2018 ◽  
Vol 33 (11) ◽  
pp. 1267-1274
Author(s):  
Mareomi Hamada ◽  
Akiyoshi Ogimoto ◽  
Kiyotaka Ohshima ◽  
Shigehiro Miyazaki ◽  
Norio Kubota ◽  
...  
Keyword(s):  
Chest Pain ◽  
Coronary Artery ◽  
Hypertrophic Cardiomyopathy ◽  
Left Ventricular

scholarly journals Semiotics of apical hypertrophic cardiomyopathy in magnetic resonance Imaging

2020 ◽  
Vol 19 (2) ◽  
pp. 18-23
Author(s):  
I. E. Obramenko
Keyword(s):  
Magnetic Resonance Imaging ◽  
Hypertrophic Cardiomyopathy ◽  
Magnetic Resonance ◽  
Left Ventricle ◽  
Heart Diseases ◽  
Fibrous Tissue ◽  
Left Ventricular ◽  
Left Ventricular Cavity ◽  
Resonance Imaging ◽  
Apical Hypertrophic Cardiomyopathy

Introduction. About 0.2 % of the adult population all over the world suffers from hypertrophic cardiomyopathy. Early and timely diagnosis of the apical form of hypertrophic cardiomyopathy remains an urgent medical problem, since the disease has a wide variability of clinical manifestations and often occurs asymptomatic or with symptoms of other heart diseases. Magnetic resonance imaging is an informative method of radiation diagnosis of hypertrophic cardiomyopathy. The aim is improving of radiology diagnostics in applying to the apical form of hypertrophic cardiomyopathy. Materials and methods. 98 patients with apical hypertrophic cardiomyopathy aged 19 to 76 years were еxamined. There were 48 men and 50 women. All subjects were examined by a cardiologist, all patients underwent electrocardiography and echocardiography, 45 patients underwent magnetic resonance imaging (MRI) of the heart. Results. In our study 13 patients had MRI determined the isolated form of apical form of hypertrophic cardiomyopathy, 32 had combined one. 42.2% of the patients with symmetrical hypertrophy of all apical segments had sawtoothed configuration of the LV revealed by MRI. The symptom of left ventricular cavity obliteration was determined in 19 patients. The symptom of LV cavity sequestration was determined in 5 subjects. 5 patients had an aneurysm on the top of the left ventricle, 1 – on the top of the right ventricle. Signs of left ventricular outflow tract obstruction were visualized in 2 patients, intraventricular obstruction at the level of the middle segments of the left ventricle was determined in 5 cases. Akinesis and hypokinesis were detected in areas of fibrous changes (n=21) or in areas of cardiosclerosis (n=2). In 17.8 % of subjects identified non-compacted myocardium, in 3 cases it was combined with apical form of hypertrophic cardiomyopathy. With contrast enhancement in 29 patients, foci (n=22) or zones (n=7) of pathological accumulation of contrast agent were determined, which indicated the replacement of myocardium with fibrous tissue.

Hypertrophic cardiomyopathy: diagnosis and assessment of symptoms

ESC CardioMed ◽  
2018 ◽  
pp. 1450-1455
Author(s):  
Albert Alain Hagège
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Exercise Testing ◽  
Imaging Modality ◽  
Functional Limitation ◽  
Cardiopulmonary Exercise Testing ◽  
Signs And Symptoms ◽  
Left Ventricular ◽  
Myocardial Segment ◽  
Apical Hypertrophy ◽  
Electrocardiogram Monitoring

The diagnosis of hypertrophic cardiomyopathy (HCM) in adults is based on the presence of left ventricular wall thickness greater than 15 mm using any imaging modality on at least one myocardial segment and not explained solely by abnormal cardiac loading conditions; a 13 mm threshold should be applied for familial screening in first-degree relatives. Diagnosis in children, in the elderly, in hypertensive individuals, and in elite athletes may be challenging. Initial evaluation should include a family pedigree, evaluation of signs and symptoms, electrocardiogram, and 48 h Holter electrocardiogram monitoring, exercise testing, imaging, and biochemistry. The presence of an intraventricular left ventricular obstruction, present in two-thirds of the patients at rest, during Valsalva or exercise, should be systematically evaluated using echocardiography. Cardiovascular magnetic resonance imaging with late gadolinium enhancement should always be considered, particularly to assess apical hypertrophy, left ventricular aneurysms, and fibrosis. This systematic approach is recommended to assist in the detection of HCM not caused by mutations in cardiac sarcomere protein genes (up to 10% of patients). Genetic tests should be performed and interpreted after a careful and complete clinical evaluation and genetic counselling. HCM is characterized by symptoms of dyspnoea, fatigue, chest pain, palpitations, and syncope, which are highly variable. Functional limitation may be difficult to evaluate and often necessitates cardiopulmonary exercise testing.

Hypertrophic cardiomyopathy: diagnosis and assessment of symptoms

ESC CardioMed ◽  
2018 ◽  
pp. 1450-1455
Author(s):  
Albert Alain Hagège
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Exercise Testing ◽  
Imaging Modality ◽  
Functional Limitation ◽  
Cardiopulmonary Exercise Testing ◽  
Signs And Symptoms ◽  
Left Ventricular ◽  
Myocardial Segment ◽  
Apical Hypertrophy ◽  
Electrocardiogram Monitoring

The diagnosis of hypertrophic cardiomyopathy (HCM) in adults is based on the presence of left ventricular wall thickness greater than 15 mm using any imaging modality on at least one myocardial segment and not explained solely by abnormal cardiac loading conditions; a 13 mm threshold should be applied for familial screening in first-degree relatives. Diagnosis in children, in the elderly, in hypertensive individuals, and in elite athletes may be challenging. Initial evaluation should include a family pedigree, evaluation of signs and symptoms, electrocardiogram, and 48 h Holter electrocardiogram monitoring, exercise testing, imaging, and biochemistry. The presence of an intraventricular left ventricular obstruction, present in two-thirds of the patients at rest, during Valsalva or exercise, should be systematically evaluated using echocardiography. Cardiovascular magnetic resonance imaging with late gadolinium enhancement should always be considered, particularly to assess apical hypertrophy, left ventricular aneurysms, and fibrosis. This systematic approach is recommended to assist in the detection of HCM not caused by mutations in cardiac sarcomere protein genes (up to 10% of patients). Genetic tests should be performed and interpreted after a careful and complete clinical evaluation and genetic counselling. HCM is characterized by symptoms of dyspnoea, fatigue, chest pain, palpitations, and syncope, which are highly variable. Functional limitation may be difficult to evaluate and often necessitates cardiopulmonary exercise testing.

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