In ApHCM, there is typically no LV outflow tract obstruction from systolic anterior motion of the anterior mitral valve leaflet and therefore no associated mitral regurgitation. We sought to characterize cardiac magnetic resonance imaging (MRI) findings among apical HC patients. Epub 2018 Dec 31. Apical Hypertrophic Cardiomyopathy ABSTRACT Apical hypertrophic cardiomyopathy (AHCM) is one form of hyper-trophic cardiomy¬opathy that is the most common hereditary car-diac disease and the most frequently found cardiomyopathy. Sixteen patients had chest pain. Apical hypertrophic cardiomyopathy: diagnosis, medical and surgical treatment. 2020;9, Long‐term outcome in patients with apical hypertrophic cardiomyopathy, Giant T wave inversion as a manifestation of asymmetrical apical hypertrophy (AAH) of the left ventricle. Apical hypertrophic cardiomyopathy is an uncommon phenotypic variant of hypertrophic cardiomyopathy, characterised by large negative precordial T-waves on electrocardioram and spade-like configuration of the left ventricular cavity. National Center for Biotechnology Information, Unable to load your collection due to an error, Unable to load your delegates due to an error. *Correspondence to: Gabriella Captur, MD, PhD, MRCP, MSc, Institute of Cardiovascular Science, University College London, Gower Street, London WC1E 6BT, United Kingdom. Customer Service In one ApHCM study of 105 patients, 94% had abnormal ECGs with voltage criteria for LVH (65%) and T‐wave inversion (93%), but only 47% had giant negative T‐waves.1 Maximal T‐wave negativity weakly correlated with apical wall thickness, and electrocardiography does not well differentiate mixed and pure ApHCM variants.1 Giant negative T‐waves have also been identified in other types of HCM and cardiac disease, including coronary artery disease, so are not a pathognomonic feature. Cureus. At rest, continuous wave Doppler across the point of distal ventricular obstruction demonstrates a midsystolic peaking jet, followed by a drop in velocity prior to second peak representing paradoxical early diastolic jet flow, with gradients of 54 and 39 mm Hg, respectively (Ci). Apical hypertrophic cardiomyopathy: prevalence and correlates of apical outpouching. 15, No. AHCM can be an incidental finding, or patients may present with chest pain, palpitations, dyspnea, syncope, atrial fibrillation, myocardial infarction, embolic events, ventricular fibrillation and congestive heart failure. ApHCM can exist with or without midventricular obstruction and cavity obliteration (MVOCO) and with or without apical aneurysm formation.15 It can be subclassified into 3 forms: (1) “pure,” with isolated apical hypertrophy; (2) “mixed,” with both apical and septal hypertrophy16 but with the apex thickest1; and (3) “relative” ApHCM, believed to be an early ApHCM phenotype. CMR pixelwise inline perfusion maps at rest (A), stress (B) in (i) basal, (ii) mid, (iii) apical short axis and (iv) 2‐chamber views in a patient with ApHCM and MVOCO. Unauthorized Athletes with pure apical LVH had normal ECGs (no T‐wave inversion28), and the phenotype was postulated to reflect athletic training, rather than true HCM. Apical hypertrophic cardiomyopathy is a rare form of hypertrophic cardiomyopathy that involves thickening of the distal portion of the left ventricular wall. Circulation. Although sustained monomorphic VT is uncommon in classic HCM, a case series reported monomorphic VT in ApHCM from reentry in a region of apical scar. Professor Moon is directly and indirectly supported by the University College London Hospitals NIHR Biomedical Research Center and Biomedical Research Unit at Barts Hospital, respectively. 30 Citations. Apical hypertrophic cardiomyopathy; Electrocardiogram. Relative ApHCM may simply represent early disease that with time progresses to overt ApHCM, eventually meeting conventional criteria, as with other HCM variants where penetrance is age dependent. This editorial refers to ‘Apical hypertrophic cardiomyopathy with left ventricular apical aneurysm: prevalence, cardiac magnetic resonance characteristics, and prognosis’, by K. Yang et al., pp. We describe a patient with asymptomatic apical hypertrophic cardiomyopathy (AHCM) who later developed cardiac arrhythmias, and briefly discuss the diagnostic modalities, differential diagnosis and treatment option for this condition. The electrocardiographic changes and symptoms The phenotypic spectrum and natural history of ApHCM (“pure,” “mixed,” and “relative”) is being clarified, as is the impact of sarcomere gene mutations, sex, and other clinical and environmental factors on phenotype expression. Relative ApHCM is diagnosed when electrocardiography shows characteristic precordial T‐wave inversion and CMR shows loss of the usual apical wall thickness tapering due to apical wall thickness exceeding basal wall thickness, although failing to reach the ApHCM diagnostic cutoff of wall thickness ≥15 mm.17 As the normal heart exhibits tapering of wall thickness towards the apex, loss of this is abnormal. Small aneurysms are often overlooked on echocardiography and may be difficult to delineate without advanced imaging.15 In ApHCM, it is hypothesized that apical aneurysms and obstructive physiology arise from regional myocardial scarring caused by repeatedly exposing the apical myocardium to increased LV wall stress and high systolic pressures, leading to pressure overload, increased oxygen demand, impaired coronary perfusion, and ischemia.25 The dyskinetic/akinetic aneurysm confers risk of apical thrombus formation and thromboembolic stroke.25 Apical aneurysms have been associated with LVH severity, SCD, monomorphic VT,24 LV systolic dysfunction, and heart failure.25. Figure 1. NLM Dr Captur is supported by the National Institute for Health Research Rare Diseases Translational Research Collaboration (NIHR RD‐TRC, #171603) and by NIHR University College London Hospitals Biomedical Research Center. In systole, the apical aneurysm becomes apparent (Ei; Eii) and contains LGE (Fi; Fii). When two-dimensional echocardiography is limited by a poor acoustic window, patients are often referred for MRI. Apical Hypertrophic Cardiomyopathy Nabil S. Zeineh, M.D., and Gustav Eles, D.O. apical hypertrophic cardiomyopathy; Issue Section: Articles. CMR may detect early ApHCM phenotypes better than echocardiography. Distinguishing between morphological HCM subtypes has conferred little in terms of personalized management strategies, with one distinctive exception: ApHCM. Clipboard, Search History, and several other advanced features are temporarily unavailable. Apical hypertrophic cardiomyopathy (AHCM) is a rare form of hypertrophic cardiomyopathy (HCM), first introduced by Sakamoto et al in 1976, who described a cardiac disorder manifested by negative T-waves on electrocardiography. Figure 4. G… Enhanced American College of Cardiology/American Heart Association strategy for prevention of sudden cardiac death in high‐risk patients with hypertrophic cardiomyopathy, Genotype‐phenotype correlations in apical variant hypertrophic cardiomyopathy, Sarcomere protein gene mutations in patients with apical hypertrophic cardiomyopathy, Cardiac muscle cell disorganization in apical hypertrophic cardiomyopathy a cardiac biopsy study, Hypertrophic nonobstructive cardiomyopathy with giant negative T waves (apical hypertrophy): ventriculographic and echocardiographic features in 30 patients, Apical hypertrophic cardiomyopathy: present status, Apical hypertrophic cardiomyopathy: correlations between echocardiographic parameters, angiographic left ventricular morphology, and clinical outcomes, Diagnosis of apical hypertrophic cardiomyopathy: T‐wave inversion and relative but not absolute apical left ventricular hypertrophy, CMR assessment of the left ventricle apical morphology in subjects with unexplainable giant T‐wave inversion and without apical wall thickness >/=15 mm, Ineffective and prolonged apical contraction is associated with chest pain and ischaemia in apical hypertrophic cardiomyopathy, Long‐term outcome of catheter ablation for atrial fibrillation in patients with apical hypertrophic cardiomyopathy, Prevalence and determinants of elevated high‐sensitivity cardiac troponin T in hypertrophic cardiomyopathy, Serum cardiac troponin I is related to increased left ventricular wall thickness, left ventricular dysfunction, and male gender in hypertrophic cardiomyopathy, Significance of apical cavity obliteration in apical hypertrophic cardiomyopathy, Apical hypertrophic cardiomyopathy presenting with sustained monomorphic ventricular tachycardia and electrocardiographic changes simulating coronary artery disease and left ventricular aneurysm, Prevalence, clinical significance, and natural history of left ventricular apical aneurysms in hypertrophic cardiomyopathy, Hypertrophic cardiomyopathy with left ventricular apical aneurysm: implications for risk stratification and management, Cardiovascular magnetic resonance demonstration of the spectrum of morphological phenotypes and patterns of myocardial scarring in Anderson‐Fabry disease, The upper limit of physiologic cardiac hypertrophy in highly trained elite athletes, Distinguishing hypertrophic cardiomyopathy from athlete's heart: a clinical problem of increasing magnitude and significance, Assessing myocardial extracellular volume by T1 mapping to distinguish hypertrophic cardiomyopathy from athlete's heart, Clinical profile of athletes with hypertrophic cardiomyopathy, Phenotypic spectrum and clinical characteristics of apical hypertrophic cardiomyopathy: multicenter echo‐Doppler study, Clinical and echocardiographic predictors of outcomes in patients with apical hypertrophic cardiomyopathy, Detection of apical hypertrophic cardiomyopathy by cardiovascular magnetic resonance in patients with non‐diagnostic echocardiography, Comparison of morphologic assessment of hypertrophic cardiomyopathy by magnetic resonance versus echocardiographic imaging, Significance of magnetic resonance imaging in apical hypertrophic cardiomyopathy, Frequency and distribution of late gadolinium enhancement in magnetic resonance imaging of patients with apical hypertrophic cardiomyopathy and patients with asymmetrical hypertrophic cardiomyopathy: a comparative study, Myocardial scarring in asymptomatic or mildly symptomatic patients with hypertrophic cardiomyopathy, Evaluation of apical subtype of hypertrophic cardiomyopathy using cardiac magnetic resonance imaging with gadolinium enhancement, Distinct subgroups in hypertrophic cardiomyopathy in the NHLBI HCM Registry, Myocardial native T1 time in patients with hypertrophic cardiomyopathy, Rest perfusion abnormalities in hypertrophic cardiomyopathy: correlation with myocardial fibrosis and risk factors for sudden cardiac death, Hypertrophic cardiomyopathy in cardiac CT: a validation study on the detection of intramyocardial fibrosis in consecutive patients, Resting “Solar Polar” map pattern and reduced apical flow reserve: characteristics of apical hypertrophic cardiomyopathy on SPECT myocardial perfusion imaging, Myocardial perfusion SPECT in the diagnosis of apical hypertrophic cardiomyopathy, Pharmacological treatment options for hypertrophic cardiomyopathy: high time for evidence, Substrate characterization and catheter ablation for monomorphic ventricular tachycardia in patients with apical hypertrophic cardiomyopathy, 2014 ESC guidelines on diagnosis and management of hypertrophic cardiomyopathy, Dual chamber pacemaker therapy for mid‐cavity obstructive hypertrophic cardiomyopathy, Journal of the American Heart Association, Apical Hypertrophic Cardiomyopathy: The Variant Less Known, Creative Commons Attribution‐NonCommercial‐NoDerivs, Copyright © 2020 The Authors. Bull's‐eye plots are shown (rest C, stress D). Two‐dimensional strain or speckle tracking demonstrate regional apical dyskinesis and reduced LV “twist,” which can be attributable to cavity obliteration negating the effect of apical twist in systolic contraction. 1 – 4 HCM is caused primarily by mutations in sarcomere proteins and is inherited in an autosomal dominant manner. This “MI pattern” of LGE adds credence to the hypothesis that apical myocardial ischemia is key in ApHCM. An implantable cardioverter defibrillator is recommended for high risk patients. Apical hypertrophic cardiomyopathy. One study investigating outcomes in patients with apical aneurysms irrespective of the HCM morphological subtype, identified aneurysms in 4.8%.26 Authors identified 2 distinct patterns of LVH in those with aneurysms: segmental thickening confined to the distal LV in 51%, and in the remaining 49% diffuse thickening of the septum and free wall, resulting in an “hourglass” configuration with midventricular muscular narrowing, creating discrete proximal and distal chambers.26 Thromboembolic events were 2‐fold more common (P=0.06) in those with apical aneurysms compared with those without, and this subgroup also experienced a 3‐fold greater adverse event rate, at 6.4%/year. Advance article alerts. Healthy volunteer stress MBF is 2 to 4 mL/g per minute. Author information: (1)Department of Medicine, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA 02215, USA. Other modalities, including left ventriculography, multislice spiral computed tomography, and cardiac magnetic resonance imagings are also valuable tools and are frequently used to differentiate AHCH from other conditions. Apical hypertrophic cardiomyopathy is a rare form of hypertrophic cardiomyopathy (HCM) recognized by a unique spadelike configuration on the left ventriculogram. Symptoms of AHCM might vary from none to others mimic coronary artery disease including acute coronary syndrome, thus resulting in inappropriate hospitalization. Fabry disease causes progressive LVH that potentially mimics ApHCM. Crossref Medline Google Scholar; 159. Quantitative perfusion mapping in ApHCM. Indian Heart J. NIH J Am Coll Cardiol 2020;Nov 20:[Epub ahead of print]. They should be essential in everyday clinical decision making. Methods Between 1976 and 2006, 193 patients with ApHCM (120 men; overall mean age, 61 ± 17 years) were evaluated. Over half of patients with apical hypertrophic cardiomyopathy are thought to be asymptomatic but the most common presenting symptom is chest pain, followed by palpitations, dyspnea and syncope. In a study looking at genotype‐phenotype correlations in ApHCM, those that carried a pathogenic sarcomere gene mutation had a stronger family history of HCM (39% versus 26%; P=0.4) but no phenotypic features were not significantly different.11 European Society of Cardiology (ESC) and American College of Cardiology Foundation/American Heart Association HCM guidelines provide no ApHCM‐specific genotyping or family screening recommendations. 2018 Dec;15(4):246-253. doi: 10.5114/kitp.2018.80922. 1 January 1990:83-
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