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.

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.

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.

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 Apical hypertrophic cardiomyopathy associated with circumflex to left ventricular fistulae: a case report of two rare subtypes of rare conditions occurring together

2021 ◽  
Vol 5 (2) ◽  
Author(s):  
Samuel Conway ◽  
Anna S Herrey ◽  
Roby D Rakhit
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Systolic Function ◽  
Beta Blockers ◽  
Left Ventricular ◽  
Wave Inversion ◽  
Cardiac Symptoms ◽  
Coronary Steal ◽  
Artery Disease ◽  
Apical Hypertrophy ◽  
Cavity Obliteration

Abstract Background  Coronary arterial fistulae are rare yet have been associated with hypertrophic cardiomyopathy (HCM). We present a patient who was found to have a left circumflex (LCx) to left ventricular (LV) fistula in combination with apical HCM. Case summary  A 72-year-old female presented with syncope after exercise. She sustained facial injuries including fracture of her nasal bones. There were no previous episodes, no cardiac history, and she denied chest pain or anginal symptoms. Electrocardiogram showed sinus rhythm with T-wave inversion throughout the chest leads. Echocardiography suggested apical HCM with hypertrophy of the LV apex but good systolic function. This was confirmed on cardiac magnetic resonance imaging with a characteristically spade-shaped LV cavity. Coronary angiography demonstrated a distal LCx to LV fistula from the apical hypertrophy but no coronary artery disease. She was started on beta-blockers and has had no further episodes, remaining well. Discussion  Coronary fistulae are present in 0.002% of the population but clinical outcomes are poorly understood. The majority are asymptomatic but anginal chest pains can occur through the ‘coronary steal’ phenomenon. Apical HCM is a subtype of HCM characterized by spade-shaped LV cavity obliteration. It is unclear whether the association between fistulae and HCM occur because of the increased vascularization and fibrosis associated with HCM or whether congenital malformation leads to hypertrophy. Both can produce a constellation of cardiac symptoms. Our patient has the previously unreported combination of apical HCM and an LCx fistula; two rarer subtypes of rare conditions appearing together.

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.

Apical hypertrophic cardiomyopathy with subendocardial late gadolinium enhancement in an adolescent

2020 ◽  
pp. 1-3
Author(s):  
Simona Boroni Grazioli ◽  
Marc-Philip Hitz ◽  
Inga Voges
Keyword(s):  
Late Gadolinium Enhancement ◽  
Hypertrophic Cardiomyopathy ◽  
Resonance Imaging ◽  
Gadolinium Enhancement ◽  
Apical Hypertrophic Cardiomyopathy ◽  
T Wave ◽  
Wave Inversion ◽  
Cardiology Department ◽  
History Of ◽  
Rare Diagnosis

Abstract A 17-year-old boy with a history of dyspnea attacks and chest pain was referred to our paediatric cardiology department. Electrocardiogram at presentation showed T-wave inversion in the inferior leads. Cardiovascular magnetic resonance imaging revealed the rare diagnosis of apical hypertrophic cardiomyopathy with subendocardial late gadolinium enhancement, missed by echocardiography.

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