DOCSLIB.ORG

Coexistence of Asymmetric Septal Hypertrophy and Aortic Valve Disease in Adults

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Coexistence of Asymmetric Septal Hypertrophy and Aortic Valve Disease in Adults Thorax: first published as 10.1136/thx.34.1.91 on 1 February 1979. Downloaded from Thorax, 1979, 34, 91-95 Coexistence of asymmetric septal hypertrophy and aortic valve disease in adults M V J RAJ, V SRINIVAS, I M GRAHAM, AND D W EVANS From the Regional Cardiac Unit, Papworth Hospital, Papworth Everard, Cambridge CB3 8RE, UK ABSTRACT Echocardiography detected asymmetric septal hypertrophy (ASH) in five of 200 adults being assessed for aortic valve surgery. Four of these were among 119 patients with dominant aortic stenosis, which was severe in three. ASH was confirmed at the time of aortic valve replacement in two of these patients; the third declined operation. The finding of ASH in only one of 81 patients with free aortic reflux is consistent with chance association. While the same explanation could apply to the higher prevalence in those with aortic stenosis, it may be that a long-standing pressure overload can trigger inappropriate septal hypertrophy in predisposed individuals. Brock (1957) recorded subaortic hypertrophy in pressure gradients, estimated echocardiographically association with hypertension and postulated its (Bennett et al, 1975) in all, and measured at development in response to chronic left ventri- cardiac catheterisation in 55, ranged from 20 to copyright. cular pressure load from aortic valvar stenosis. 100 mmHg. Of the 118 with gradients higher than Hurst and Logue (1966) suggested, on the basis of 40 mmHg 117 had pressures of a similar order operative and necropsy data, that it might be recorded at operation. One, with a valvar gradient present in about 10% of those with aortic valve of 100 mmHg at rest, refused the offer of surgical disease. Another necropsy study (Parker et al, help. http://thorax.bmj.com/ 1969) yielded ten instances of subaortic hyper- trophy in association with aortic valve stenosis. Table 1 119 patients with dominant aortic stenosis In echocardiography we now have a sensitive method for detecting excessive (asymmetric) septal Age 18-67 yr (mean 47) hypertrophy (ASH) during life (Henry etal, 1973). Sex Men 72 We used it to screen a series of patients under- Women 47 going investigation with a view to aortic valve Aetiology replacement and report our findings with respect Congenital 3 to Calcific 112 on September 25, 2021 by guest. Protected dominance of pressure or volume load on the Unknown 4 left ventricle. Symptoms Dyspnoea 110 Patients and methods Angina 82 Syncope or faintness 40 Congestive heart failure 4 Echocardiography was carried out in 220 adults undergoing assessment for aortic valve replace- ment. Technically satisfactory records could not Of the 81 patients who had free aortic reflux, of be obtained from 20 of them. The remaining 200 varied aetiology, which was thought to be the patients (74 women, 126 men) form the basis of basis of their symptoms (table 2), 80 underwent this study. Their ages ranged from 18 to 67 years aortic valve replacement. In one the investigations (mean 47). None gave a family history suggestive showed only moderately severe reflux, with co- of cardiomyopathy. existing ASH, and operation was not advised. One hundred and nineteen patients had domi- The echocardiograms were recorded with the nant aortic stenosis. Their symptoms and some patients supine, using a Cl 1 transducer with an aetiological details are shown in table 1. Their Ekoline 20 ultrasonoscope and a Cambridge photo- peak systolic-left ventricular-systemic arterial graphic recorder. Echocardiograms showed both 91 Thorax: first published as 10.1136/thx.34.1.91 on 1 February 1979. Downloaded from 92 M V J Raj, V Srinivas, I M Graham, and D W Evans Table 2 81 patients with aortic reflux surfaces of the interventricular septum (IVS), the posterior basal left ventricular wall (PLVW), and Age 28-66 yr (mean 46) the peak excursion of the mitral leaflets (fig 1). Sex Men 54 Diastolic IVS and PLVW thickness was measured Women 27 at a point in the cardiac cycle immediately before Aetiology The left ventricular internal diamen- Rheumatic 67 atrial systole. Bacterial 6 sions in systole (Ds) and diastole (Dd) were also Syphilitic 2 measured. Mean circumferential fibre shortening Aortic dissection 1 Rickettsial carditis 1 velocity (Vcf) was calculated by the method of Unknown 4 Cooper et al (1972). Symptoms The chief echocardiographic criterion used for Dyspnoea 8 1 Angina 20 diagnosing ASH in this study was an IVS: PLVW Syncope or faintness 12 thickness ratio in excess of 1-3: 1 (Henry et al. Congestive heart failure 10 1973). Systolic anterior movement of the mitral copyright. N . 23 mm http://thorax.bmj.com/ '" D D ~'' / on September 25, 2021 by guest. Protected PLVW 16 mmr IL 67 yrs Fig 1 Echocardiogram showing IVS and PLVW thickness and left ventricular internal dimensions at peak .systole and end diastole. ASH is present in this case. Thorax: first published as 10.1136/thx.34.1.91 on 1 February 1979. Downloaded from Coexistence of asymmetric septal hypertrophy and aortic valve disease in adults 93 valve (SAM), high Vcf, and small left ventricular One woman (aged 63) with aortic reflux was cavity (Shah et al, 1969) were also sought. found to have an IVS: PLVW ratio in excess of 1-3: 1. Her echocardiogram also showed typical Results SAM and cardioangiography confirmed asym- metric hypertrophy while suggesting that her aortic Table 3 shows the numbers, sex distribution, and reflux was not severe enough to require aortic echocardiographic findings in those with dominant valve replacement. aortic stenosis and table 4 in those with aortic All 195 patients without echocardiographic reflux. Four patients with dominant stenosis (three evidence of ASH were operated on, and no evi- women aged 63, 64, and 67 and one man aged 53) dence of unusual septal hypertrophy was noted. were found to have IVS: PLVW ratios higher than 1-3: 1. All four exhibited typical SAM of the Discussion anterior mitral leaflet (fig 2), though in one only after inhalation of amyl nitrite. Retrograde left Nanda et al (1974) pioneered the use of echo- ventricular catheterisation showed systolic aortic cardiography for detecting idiopathic subaortic valvar gradients of 20, 65, 75, and 100 mmHg, stenosis coexisting with aortic valve disease. We without subvalvar gradients, and showed ventri- used the technique in an attempt to establish the culographic features of hypertrophic cardiomyo- prevalence of excessive asymmetric septal hyper- pathy in all four. The patient with the gradient of trophy in a series of patients undergoing assess- 100 mmHg declined operation. Excessive septal ment with a view to aortic valve replacement. hypertrophy was confirmed at the time of aortic Since Henry et al (1973) had suggested that in valve replacement in the other two with critical adults without right ventricular hypertension an stenosis, and a myotomy was carried out in one. IVS: PLVW ratio in excess of 1 3 offers a suffi- cient basis for the diagnosis, we used this criterion suggested Table 3 Age and sex distribution and in our study. Popp (1976) subsequently echocardiographic findings in subjects with aortic that a ratio in excess of 1-5: 1 is necessary. Had copyright. stenosis we used that criterion, we would have failed to identify two examples of ASH (subsequently con- IVS/PLVW Wall ratio firmed by ventriculography and at operation) in <1 3 >1 3 association with disease of the aortic valve. In a 115 4 which No still more recent study (Chahine et al, 1977), http://thorax.bmj.com/ Men 71 1 pointed the lack of any pathognomonic feature, Women 44 3 three finally considered to have hyper- Range Mean Range Mean patients Age (yr) 18-66 47 52-67 62 trophic cardiomyopathy had echocardiographic IVs (mm) 10-22 15 17-31 22 IVS: PLVW ratios between 1 0 and 1 2. PLVW (mm) 10-20 15 12-16 14 IVS/PLVW 0-7-1-3 1.0 1-42-0 1-7 Only three of the patients in this study had Ds(mm) 16-47 28 15-25 20 coexistent ASH and disease of the aortic valve Dd(mm) 30-60 46 30-41 35 Vcf(sec -) 1-02-0 1.5 1-2-2-0 1-7 severe enough to require operation. On this evi- SAM Absent Present dence, the prevalence of ASH in adults with critical aortic stenosis may be of the order of on September 25, 2021 by guest. Protected 2 5%. The figure rises to 3-4% if the patient with Table 4 Age and sex distribution and a peak systolic gradient of 20 mmHg (and resting eclhocardiographic findings in subjects with aortic left ventricular pressure circa 160 mmHg) is in- reflux cluded. Others have noted coexistence of ASH IIVS/PL VW Wall ratio with aortic stenosis, and with systemic hyperten- <1*3 >1*3 sion and coarctation of the aorta (Moreyra et al, et Feizi and Emanuel, 1975). No 80 1 1970; Block al, 1973; Men 54 0 The possibility exists that frequent or sustained Women 26 1 excessive pressure loads may trigger inappropriate Range Mean (asymmetric) ventricular hypertrophy in predis- Age 28-66 46 62 IVs (mm) 10-18 13 23 posed individuals. Alternatively, the association PLVW (mm) 10-16 13 15 may be coincidental; however, in our series none IVS/PLVW 0-8-1-3 1.0 1-5 Ds (mm) 26-70 42 24 of the four patients with ASH gave a family Dd (mm) 55-90 63 41 history suggestive of cardiomyopathy. Vcf(sec 1) 06-1*8 1*4 1*6 Among the 81 patients with aortic reflux, only SAM Absent Present one was found to have coexisting ASH. This Thorax: first published as 10.1136/thx.34.1.91 on 1 February 1979.
Load more

Recommended publications
  • Idiopathic Isolated Right Ventricular Apical Hypertrophy
    Acta Cardiol Sin 2018;34:288-290 Letter to the Editor doi: 10.6515/ACS.201805_34(3).20180122A Idiopathic Isolated Right Ventricular Apical Hypertrophy Debika Chatterjee1 and Pradeep Narayan2 INTRODUCTION creased RV compliance or RV dysfunction. Isolated right ventricular hypertrophy is extremely rare and reports in the literature are very sparse. We re- DISCUSSION port a case of focal apical right ventricular hypertrophy without involvement of the left ventricular cavity or the RV hypertrophy is usually reported in association inter-ventricular septum. with left ventricular hypertrophy which in turn could be secondary to hypertrophic cardiomyopathy (HCM), hy- pertensive left ventricular hypertrophy (LVH) or other CASE infiltrative conditions.1 However, isolated involvement of right ventricle is extremely rare in these conditions. The A 50 year old male, asymptomatic, normotensive only situation where isolated RV hypertrophy is seen is in patient on routine medical evaluation was found to have presence of idiopathic pulmonary artery hypertension. inverted T-waves on the electrocardiogram (Figure 1). However, the hypertrophy in these cases is rarely focal. He had no history of angina or any other symptoms. Isolated apical hypertrophic cardiomyopathy is an However, despite complete lack of symptoms, because extremely unusual nonobstructive hypertrophy that is of the abnormality on the electrocardiography (ECG) an localized to the cardiac apex.2 Even in these cases spar- echocardiography was carried out for further evaluation. ing of the left ventricular apex with involvement of only Echocardiography revealed focal hypertrophy of the the right ventricular apex is even more uncommon. The right ventricular (RV) apex almost obliterating the apex most common presenting symptom in patients with api- (Figure 2A) but sparing the inter-ventricular septum.
    [Show full text]
  • Antithrombotic Therapy in Atrial Fibrillation Associated with Valvular Heart Disease
    Europace (2017) 0, 1–21 EHRA CONSENSUS DOCUMENT doi:10.1093/europace/eux240 Antithrombotic therapy in atrial fibrillation associated with valvular heart disease: a joint consensus document from the European Heart Rhythm Association (EHRA) and European Society of Cardiology Working Group on Thrombosis, endorsed by the ESC Working Group on Valvular Heart Disease, Cardiac Arrhythmia Society of Southern Africa (CASSA), Heart Rhythm Society (HRS), Asia Pacific Heart Rhythm Society (APHRS), South African Heart (SA Heart) Association and Sociedad Latinoamericana de Estimulacion Cardıaca y Electrofisiologıa (SOLEACE) Gregory Y. H. Lip1*, Jean Philippe Collet2, Raffaele de Caterina3, Laurent Fauchier4, Deirdre A. Lane5, Torben B. Larsen6, Francisco Marin7, Joao Morais8, Calambur Narasimhan9, Brian Olshansky10, Luc Pierard11, Tatjana Potpara12, Nizal Sarrafzadegan13, Karen Sliwa14, Gonzalo Varela15, Gemma Vilahur16, Thomas Weiss17, Giuseppe Boriani18 and Bianca Rocca19 Document Reviewers: Bulent Gorenek20 (Reviewer Coordinator), Irina Savelieva21, Christian Sticherling22, Gulmira Kudaiberdieva23, Tze-Fan Chao24, Francesco Violi25, Mohan Nair26, Leandro Zimerman27, Jonathan Piccini28, Robert Storey29, Sigrun Halvorsen30, Diana Gorog31, Andrea Rubboli32, Ashley Chin33 and Robert Scott-Millar34 * Corresponding author. Tel/fax: þ44 121 5075503. E-mail address: [email protected] Published on behalf of the European Society of Cardiology. All rights reserved. VC The Author 2017. For permissions, please email: [email protected]. 2 G.Y.H. Lip 1Institute of Cardiovascular Sciences, University of Birmingham and Aalborg Thrombosis Research Unit, Department of Clinical Medicine, Aalborg University, Denmark (Chair, representing EHRA); 2Sorbonne Universite´ Paris 6, ACTION Study Group, Institut De Cardiologie, Groupe Hoˆpital Pitie´-Salpetrie`re (APHP), INSERM UMRS 1166, Paris, France; 3Institute of Cardiology, ‘G.
    [Show full text]
  • Cardiac Hypertrophy, Hypertrophic Cardiomyopathy, and Hyperparathyroidism-An Association
    Br Heart J: first published as 10.1136/hrt.54.5.539 on 1 November 1985. Downloaded from Br HeartJ 1985; 54: 539-42 Cardiac hypertrophy, hypertrophic cardiomyopathy, and hyperparathyroidism-an association C SYMONS, F FORTUNE, R A GREENBAUM, P DANDONA From the Departments of Cardiology and Human Metabolism, the Royal Free Hospital, London SUMMARY Left ventricular hypertrophy (symmetric, asymmetric, or hypertrophic cardio- myopathy) is an almost invariable accompaniment of primary hyperparathyroidism. Five of 18 patients with hypertrophic cardiomyopathy had raised serum concentrations of parathyroid hor- mone with normal serum calcium concentrations. Left ventricular hypertrophy did not occur in any of the six patients with hypercalcaemia alone. These relations suggest that parathyroid hormone rather than a rise in the extracellular calcium concentration is associated with a spectrum of left ventricular hypertrophy. All patients with increased circulating parathyroid hormone concentrations should have echo- cardiographic examination of the left ventricle. Conversely, parathyroid hormone concentrations should be measured in all patients with left ventricular hypertrophy from an unknown cause, especially those with hypertrophic cardiomyopathy. copyright. Calcium has powerful positive inotropic and chro- included.) Sixteen of these patients were found to notropic effects on cardiac muscle.' Any factor that have primary hyperparathyroidism and six of these promotes transmembrane calcium flux could be had had operations to remove either a parathyroid
    [Show full text]
  • Hypertension and Arrhythmia
    2005; 6: No. 24 HYPERTENSION AND ARRHYTHMIA Jean-Philippe Bagueta, Serap Erdineb, Jean-Michel Malliona From aCardiology and Hypertension Department, Grenoble University Hospital, BP 217, 38043 Grenoble cedex 09, France and b Istanbul University Cerrahpasa School of Medicine, Göztepe I. Orta Sok, 34 A/9 Istanbul, Turkey Correspondence: Jean-Philippe Baguet, Cardiologie et Hypertension artérielle, CHU de Grenoble - BP 217, 38043 Grenoble Cedex 09, France, tel +334767654.40, fax 334767655.59, [email protected] Introduction rapid or if there is some underlying problem with left ventricular Arrhythmia-both atrial and ventricular-is a common comorbidity function (either systolic or diastolic) (10). AF can also cause with hypertension (HT). Underlying mechanisms are many and episodes of dizziness or even syncope. Finally, in the various, including left ventricular hypertrophy (LVH), myocardial Framingham study, a correlation was observed between AF and ischemia, impaired left ventricular function and left atrial enlarge- mortality in both sexes, and this independently of other variables ment. Any form of arrhythmia may be associated with LVH but (11). ventricular arrhythmia is more common as well as being more dangerous. Treatment of atrial arrhythmia Preventing AF in hypertensive subjects depends on controlling Atrial arrhythmia blood pressure in order to reduce the risk of hypertensive car- Prevalence diomyopathy (or at least mitigating the consequences thereof). After supraventricular extrasystole, atrial fibrillation (AF) is the Antihypertensive therapy has been shown to reverse some of the next most common form of arrhythmia associated with HT. The structural cardiac changes caused by HT, including LVH and atri- relative risk of developing AF in HT is modest compared with other al enlargement (12, 13).
    [Show full text]
  • Hypertrophic Cardiomyopathy: a Systematic Review
    CLINICAL CARDIOLOGY CLINICIAN’S CORNER Hypertrophic Cardiomyopathy A Systematic Review Barry J. Maron, MD Context Throughout the past 40 years, a vast and sometimes contradictory litera- ture has accumulated regarding hypertrophic cardiomyopathy (HCM), a genetic car- YPERTROPHIC CARDIOMYOP- diac disease caused by a variety of mutations in genes encoding sarcomeric proteins athy (HCM) is a complex and and characterized by a broad and expanding clinical spectrum. relatively common genetic Objectives To clarify and summarize the relevant clinical issues and to profile rap- cardiac disease that has been idly evolving concepts regarding HCM. Hthe subject of intense scrutiny and in- Data Sources Systematic analysis of the relevant HCM literature, accessed through vestigation for more than 40 years.1-10 Hy- MEDLINE (1966-2000), bibliographies, and interactions with investigators. pertrophic cardiomyopathy is an impor- Study Selection and Data Extraction Diverse information was assimilated into tant cause of disability and death in a rigorous and objective contemporary description of HCM, affording greatest weight patients of all ages, although sudden and to prospective, controlled, and evidence-based studies. unexpected death in young people is per- Data Synthesis Hypertrophic cardiomyopathy is a relatively common genetic car- haps the most devastating component of diac disease (1:500 in the general population) that is heterogeneous with respect to disease- its natural history. Because of marked causing mutations, presentation, prognosis, and treatment strategies. Visibility at- heterogeneity in clinical expression, tached to HCM relates largely to its recognition as the most common cause of sudden natural history, and prognosis,11-20 HCM death in the young (including competitive athletes).
    [Show full text]
  • Atrial Fibrillation in Hypertrophic Cardiomyopathy: Prevalence, Clinical Impact, and Management
    Heart Failure Reviews (2019) 24:189–197 https://doi.org/10.1007/s10741-018-9752-6 Atrial fibrillation in hypertrophic cardiomyopathy: prevalence, clinical impact, and management Lohit Garg 1 & Manasvi Gupta2 & Syed Rafay Ali Sabzwari1 & Sahil Agrawal3 & Manyoo Agarwal4 & Talha Nazir1 & Jeffrey Gordon1 & Babak Bozorgnia1 & Matthew W. Martinez1 Published online: 19 November 2018 # Springer Science+Business Media, LLC, part of Springer Nature 2018 Abstract Hypertrophic cardiomyopathy (HCM) is the most common hereditary cardiomyopathy characterized by left ventricular hyper- trophy and spectrum of clinical manifestation. Atrial fibrillation (AF) is a common sustained arrhythmia in HCM patients and is primarily related to left atrial dilatation and remodeling. There are several clinical, electrocardiographic (ECG), and echocardio- graphic (ECHO) features that have been associated with development of AF in HCM patients; strongest predictors are left atrial size, age, and heart failure class. AF can lead to progressive functional decline, worsening heart failure and increased risk for systemic thromboembolism. The management of AF in HCM patient focuses on symptom alleviation (managed with rate and/or rhythm control methods) and prevention of complications such as thromboembolism (prevented with anticoagulation). Finally, recent evidence suggests that early rhythm control strategy may result in more favorable short- and long-term outcomes. Keywords Atrial fibrillation . Hypertrophic cardiomyopathy . Treatment . Antiarrhythmic agents Introduction amyloidosis) [3–5]. The clinical presentation of HCM is het- erogeneous and includes an asymptomatic state, heart failure Hypertrophic cardiomyopathy (HCM) is the most common syndrome due to diastolic dysfunction or left ventricular out- inherited cardiomyopathy due to mutation in one of the sev- flow (LVOT) obstruction, arrhythmias (atrial fibrillation and eral sarcomere genes and transmitted in autosomal dominant embolism), and sudden cardiac death [1, 6].
    [Show full text]
  • Avalus™ Pericardial Aortic Surgical Valve System
    FACT SHEET Avalus™ Pericardial Aortic Surgical Valve System Aortic stenosis is a common heart problem caused by a narrowing of the heart’s aortic valve due to excessive calcium deposited on the valve leaflets. When the valve narrows, it does not open or close properly, making the heart work harder to pump blood throughout the body. Eventually, this causes the heart to weaken and function poorly, which may lead to heart failure and increased risk for sudden cardiac death. Disease The standard treatment for patients with aortic valve disease is surgical aortic valve Overview: replacement (SAVR). During this procedure, a surgeon will make an incision in the sternum to open the chest and expose the heart. The diseased native valve is then Aortic removed and a new artificial valve is inserted. Once in place, the device is sewn into Stenosis the aorta and takes over the original valve’s function to enable oxygen-rich blood to flow efficiently out of the heart. For patients that are unable to undergo surgical aortic valve replacement, or prefer a minimally-invasive therapy option, an alternative procedure to treat severe aortic stenosis is called transcatheter aortic valve replacement (TAVR). The Avalus Pericardial Aortic Surgical Valve System is a next- generation aortic surgical valve from Medtronic, offering advanced design concepts and unique features for the millions of patients with severe aortic stenosis who are candidates for open- heart surgery. The Avalus Surgical Valve The Avalus valve, made of bovine tissue, is also the only stented surgical aortic valve on the market that is MRI-safe (without restrictions) enabling patients with severe aortic stenosis who have the Avalus valve to undergo screening procedures for potential co-morbidities.
    [Show full text]
  • Left Ventricular Remodeling in Hypertrophic Cardiomyopathy: an Overview of Current Knowledge
    Journal of Clinical Medicine Review Left Ventricular Remodeling in Hypertrophic Cardiomyopathy: An Overview of Current Knowledge Beatrice Musumeci, Giacomo Tini , Domitilla Russo, Matteo Sclafani, Francesco Cava, Alessandro Tropea, Carmen Adduci, Francesca Palano, Pietro Francia and Camillo Autore * Cardiology, Department of Clinical and Molecular Medicine, Faculty of Medicine and Psychology, Sapienza University of Rome, 00189 Rome, Italy; [email protected] (B.M.); [email protected] (G.T.); [email protected] (D.R.); [email protected] (M.S.); [email protected] (F.C.); [email protected] (A.T.); [email protected] (C.A.); [email protected] (F.P.); [email protected] (P.F.) * Correspondence: [email protected]; Tel.: +39-06-3377-5577 Abstract: While most patients with hypertrophic cardiomyopathy (HCM) show a relatively stable morphologic and clinical phenotype, in some others, progressive changes in the left ventricular (LV) wall thickness, cavity size, and function, defined, overall, as “LV remodeling”, may occur. The interplay of multiple pathophysiologic mechanisms, from genetic background to myocardial ischemia and fibrosis, is implicated in this process. Different patterns of LV remodeling have been recognized and are associated with a specific impact on the clinical course and management of the disease. These findings underline the need for and the importance of serial multimodal clinical and instrumental evaluations to identify and further characterize the LV remodeling phenomenon. A more complete definition of the stages of the disease may present a chance to improve the management of Citation: Musumeci, B.; Tini, G.; Russo, D.; Sclafani, M.; Cava, F.; HCM patients. Tropea, A.; Adduci, C.; Palano, F.; Francia, P.; Autore, C.
    [Show full text]
  • Hypertrophic Cardiomyopathy
    Clinical Update Adapted from: 2020 ACC/AHA Guideline for the Diagnosis and Treatment of Patients with Hypertrophic Cardiomyopathy ACC/AHA Applying Class of Recommendation and Level of Evidence to Clinical Strategies, Interventions, Treatments, or Diagnostic Testing in Patient Care (Updated May 2019)* HCM Hypertrophic Cardiomyopathy (HCM) is a Globally Prevalent & Common Genetic Heart Disease Inheritance Pattern Sex Distribution Disease Prevalence Triggers for Evaluation +/- +/+ Symptoms 50% 50% Cardiac Event Heart Murmur +/- Abnormal EKG Women diagnosed Estimated Cardiac Imaging Autosomal Dominant less commonly 1:200 – 1:500 Family Studies Other non-HCM Causes of LV Hypertrophy ⅔ have LVOTO Metabolic & Multi-organ Syndromes RASopathies Mitochondrial myopathies LV Outflow Tract Glycogen / Lysosomal storage diseases Amyloidosis Obstruction Sarcoidosis (LVOTO) Hemochromatosis Danon disease Secondary Causes Athlete’s heart HCM ⅓ do not have LVOTO Hypertension Valvular & subvalvular stenosis Abbreviations: EKG, indicates electrocardiogram; RAS, reticular activating system. 3 Ommen, SR et al. 2020 ACC/AHA Guideline for the Diagnosis and Treatment of Patients with Hypertrophic Cardiomyopathy. Circulation. XXX:XX-XX. HCM Defining Hypertrophic Cardiomyopathy in 2020 • Morphologic expression confined solely to the heart • Characterized by left ventricular (LV) hypertrophy Basal anterior septum in continuity with the anterior free wall = most common • No other cardiac, systemic or metabolic disease capable of producing the magnitude of hypertrophy
    [Show full text]
  • Changes in Cardiac Geometry Due to Hypertrophy
    Changes in Cardiac Geometry Due to Hypertrophy By Marta Ellen Pedersen A Master’s Paper Submitted in Partial Fulfillment of The Requirements for the Degree of Master of Science in Clinical Exercise Physiology Dr. Joseph O’ Kroy Date University of Wisconsin-River Falls 2014 Introduction For any given body size, men have larger hearts than women, athletes have larger hearts than nonathletes, and often times, an enlarged heart is a symptom of an underlying disorder that is causing the heart to work harder than normal. This review will emphasize the differences between a pathologically enlarged heart and an athletically enlarged heart. Pathologically induced hypertrophy (myopathy) When heart cells get bigger, (often is the case when heart disease is present) the total heart works less efficiently. Some people suffer from conditions like hypertrophic cardiomyopathy, which includes significant heart muscle enlargement, and can be genetic or caused by high blood pressure. Cardiomyopathy decreases the size of the heart's chambers, reducing blood flow. Hypertrophy, or thickening, of the heart muscle can occur in response to increased stress on the heart. The most common causes of Cardiomyopathy are related to increased blood pressure. The extra work of pumping blood against the increased pressure causes the ventricle to thicken over time, the same way a body muscle increases in mass in response to weightlifting. Cardiomyopathy can occur in both the right and left atrium and the right and left ventricles. Blood travels through the right ventricle to the lungs. If conditions occur that decrease pulmonary circulation, extra stress can be placed on the right ventricle, and can lead to right ventricular myopathy.
    [Show full text]
  • Heart Failure in Aortic Stenosis — Improving Diagnosis and Treatment Michael R
    The new england journal of medicine perspective Heart Failure in Aortic Stenosis — Improving Diagnosis and Treatment Michael R. Zile, M.D., and William H. Gaasch, M.D. The development of heart failure in patients with tional to the stroke volume divided by the square aortic stenosis is associated with a high mortality root of the pressure gradient. Therefore, if the stroke rate — unless aortic-valve replacement is per- volume declines, as it does in some patients with formed. There is an especially high risk of death aortic stenosis in whom heart failure has developed, among patients with aortic stenosis and a decreased there is a proportional decline in the pressure gra- ejection fraction. Before surgery is performed in dient. Under these low-flow conditions, the cal- such patients, initial management must include an culated effective aortic-valve area may indicate the evaluation of the severity of the stenotic lesion and presence of severe aortic stenosis, despite a low the functional state of the left ventricle; in addition, transvalvular pressure gradient. A mean pressure the heart failure must be treated and the patient’s gradient that is less than 30 mm Hg in a patient with condition stabilized. It is possible to pursue both what appears to be severe aortic stenosis (an aortic- of these goals simultaneously with echocardio- valve area of <1 cm2) indicates what is referred to as graphic techniques or cardiac catheterization tech- “low-gradient aortic stenosis.” niques, together with selected pharmacologic in- An example of a low pressure gradient in a pa- terventions. Proper evaluation and treatment require an un- derstanding of the pathophysiology of heart failure ABC in patients with aortic stenosis.
    [Show full text]
  • How to Estimate Left Ventricular Hypertrophy in Hypertensive Patients
    Review 389 How to estimate left ventricular hypertrophy in hypertensive patients Dragan Lovic, Serap Erdine1, Alp Burak Çatakoğlu2 Clinic for internal disease, InterMedica; Nis-Serbia 1Department of Cardiology, Cerrahpaşa Faculty of Medicine, İstanbul University; İstanbul-Turkey 2Department of Cardiology, Liv Hospital; İstanbul-Turkey ABSTRACT Left ventricular hypertrophy (LVH) is a structural remodeling of the heart developing as a response to volume and/or pressure overload. Previous studies have shown that hypertension is not an independent factor in the development of LVH and occurrence does not depend on the length and severity of hypertension, but the role played by other comorbidities such as triglycerides, age, gender, genetics, insulin resis- tance, obesity, physical inactivity, increased salt intake and chronic stress. LVH develops through three phases: adaptive, compensatory, and pathological phase. Contractile dysfunction is reversible in the first two phases and irreversible in the third. According to the Framingham study, LVH develops in 15-20% of patients with mild arterial hypertension, and in 50% of patients with severe hypertension. The pathophysiology of LVH includes hypertrophy of cardiomyocytes, interstitial and perivascular fibrosis, coronary microangiopathy and macroangiopathy. Individuals with LVH have 2-4 times higher risk of having adverse CV events compared to patients without LVH. (Anadolu Kardiyol Derg 2014; 14: 389-95) Key words: arterial hypertension, left ventricular hypertrophy, pathophysiology, cardiovascular events Introduction increases concurrently with the increase of body size in both genders. From puberty on, the growth rate of the heart is higher Arterial hypertension is a major cause of organ damage in men than in women, implying that men and women have spe- including the heart and left ventricular hypertrophy (LVH) is a cific cardiac growth curves.
    [Show full text]