The obstructive variant of hcm, hypertrophic obstructive cardiomyopathy hocm, has also historically been known as idiopathic hypertrophic subaortic stenosis ihss and asymmetric septal hypertrophy ash. Esc clinical practice guidelines aim to present all the relevant evidence to help physicians weigh the benefits and risks of a particular diagnostic or therapeutic procedure on hypertrophic cardiomyopathy. Histopathology from surgical specimens of 3 patients with obstructive hcm who underwent surgical septal myectomy for progressive heart failure symptoms. Pathophysiology and treatment of hypertrophic cardiomyopathy. Hcm is caused primarily by mutations in sarcomere proteins and is inherited in an autosomal dominant manner. Pediatric hypertrophic cardiomyopathy hcm, hypertrophic. The ventricle size often remains normal, but the thickening may block blood flow out of the ventricle.
Patients with severe obstructive hypertrophic cardiomyopathy and associated symptoms may require catheterbased on surgical interventions to relieve the obstruction. Hypertrophic obstructive cardiomyopathy sciencedirect. Hypertrophic obstructive cardiomyopathy the lancet. Hypertrophic cardiomyopathy hcm is a genetic disorder that is characterized by left ventricular hypertrophy unexplained by secondary causes, and a. Hypertrophic obstructive cardiomyopathy annals of cardiothoracic. The thickening makes it harder for the heart to contract and pump blood out to the body. Hypertrophic obstructive cardiomyopathy is a common genetic disorder associated with heart failure and scd. Overview hypertrophic cardiomyopathy hcm is one of the most commonly encountered heart disease in cats. Hypertrophic obstructive cardiomyopathy is reported as a main cause of symptoms of angina and syncope in humans with this disease 11, 12. A comparison of the outcomes in patients with hypertrophic obstructive cardiomyopathy in patients 60 years old. Hypertrophic cardiomyopathy treatment and services.
Hypertrophic cardiomyopathy hcm is a genetic disorder of the myocardium. Going beyond the septum for complete repair of obstructive hypertrophic cardiomyopathy david blitzer 1, stephanie nguyen, hiroo takayama 1 1. Hcm manifests as otherwise unexplained thickening of the left. Often, leakage of the mitral valve causes the blood in the lower chamber left ventricle to leak back into the upper chamber left atrium. Hypertrophic cardiomyopathy distribution of lvh 600 patients anterior and inferior septum 31% anterior septum only 25% klues hg, jacc 1995. Hypertrophic cardiomyopathy hcm is a genetically determined heart muscle disease most often 60 to 70 percent caused by mutations in one of several sarcomere genes which encode components of the contractile apparatus of the heart. Men and women have the condition at the same frequency.
As cardiomyopathy progresses, the heart becomes weaker. Full text full text is available as a scanned copy of the original print version. Mavacamten, an orally administered, smallmolecule modulator of cardiac myosin, targets underlying biomechanical abnormalities in obstructive hypertrophic cardiomyopathy ohcm. Hypertrophic obstructive cardiomyopathy springerlink. An introduction to hypertrophic cardiomyopathy hcm. Hypertrophic cardiomyopathy hcm is the most common heritable cardiomyopathy, manifesting as left ventricular hypertrophy in the absence of a secondary cause.
Hypertrophic cardiomyopathy hcm is a condition in which a portion of the heart becomes. Hypertrophic cardiomyopathy hcm is a disorder which causes hypertrophy of the interventricular septum of the heart, leading to obstruction of left ventricular outflow during systole. Hypertrophic cardiomyopathy is the most common primary cardiomyopathy and can cause exertional dyspnea, presyncope, atypical chest pain, heart failure, and sudden cardiac death. Hypertrophic cardiomyopathy is a genetic disease associated with the development of abnormal myocytes resulting in ventricular hypertrophy even in the absence of ventricular overload. Dec 28, 2016 familial hypertrophic cardiomyopathy hcm is an inherited heart condition characterized by thickening of the heart muscle. Hc is associated with a small, hyperdynamic ventricle with a large ejection fraction. Hypertrophic obstructive cardiomyopathy treatment at emory. A 28yearold man presents with a twoyear history of increasing dyspnea on strenuous exertion and is found to have hypertrophic cardiomyopathy, with a septal thickness of 23 mm and a left ventricu. Hypertrophic cardiomyopathy morbidity mortality mortality 1%3% per year some remain stable or improve. Hypertrophic obstructive cardiomyopathy definition of.
Hypertrophic obstructive cardiomyopathy is an inherited myocardial disease defined by cardiac hypertrophy wall thickness. Some people have a type of hypertrophic cardiomyopathy called obstructive hcm that interferes with the smooth flow of blood leaving the heart. Hypertrophic obstructive cardiomyopathy an overview. Hypertrophic obstructive cardiomyopathy hocm ecg example 1. The care team at emory hypertrophic cardiomyopathy clinic creates a treatment plan based on your specific condition. Surgical treatment for hypertrophic obstructive cardiomyopathy. Your team may refer you to a specially trained stanford cardiovascular interventionist to discuss alcohol septal ablation, a type of cardiac ablation. Hypertrophic cardiomyopathy hcm is a genetic disorder of the heart muscle, characterized by a small left ventricular cavity and marked hypertrophy of the myocardium with myocyte disarray. Septal reduction in hypertrophic obstructive cardiomyopathy. In some patients, a catheter can be used to inject alcohol into the hypertrophied enlarged ventricular septum which separates the left and right ventricles.
Nonsurgical septal reduction therapy for hypertrophic obstructive cardiomyopathy. Columbia university, department of surgery, new york, ny, united states. Hypertrophic cardiomyopathy hcm is a genetic cardiac disorder caused by mutations in one of at least 12 sarcomeric or nonsarcomeric genes and is recognized as the most common cause of sudden cardiac death scd in the young and an important substrate for disability at any age. It is a heterogeneous disorder, produced by mutations in multiple genes coding for sarcomeric proteins e. In the present study, the anatomic and physiologic characteristics of childhood hypertrophic obstructive cardiomyopathy were highly heterogeneous, with simultaneous right ventricular outflow tract obstruction in 22 patients 18. Hypertrophic cardiomyopathy hcm is a genetic disorder that is characterized by left ventricular hypertrophy unexplained by secondary.
Please use one of the following formats to cite this article in your essay, paper or report. Cardiomyopathy kardeomiopahthee refers to diseases of the heart muscle. The role of myectomy and percutaneous septal ablation in drugrefractory disease. Lv outflow tract obstruction systematically exclude obstruction unrelated to sam, including subaortic membranes, mitral valve leaflet abnormalities and midcavity obstruction. In rare cases, the muscle tissue in the heart is replaced with scar tissue. Life with hypertrophic cardiomyopathy hypertrophic cardiomyopathy 14 15 asymmetrical septal hypertrophy with obstruction in some cases, the thickening of the septum obstructs the flow of blood through the left ventricular outflow tract when the heart contracts. Hypertrophic cardiomyopathy hcm is an important genetic heart muscle disease for which prevalence in the general population has not been completely resolved. Spark erosion myectomy in hypertrophic obstructive cardiomyopathy. The heart muscle in abnormally thickened or hypertrophied. Patients with hypertrophic obstructive cardiomyopathy have a high risk for sudden cardiac death. Cardiomyopathy is a disease of the heart muscle that causes the heart muscle to become enlarged, thick, or rigid. Hypertrophic cardiomyopathy circulation research aha journals. Hypertrophic cardiomyopathy hcm american heart association.
The thickening most often occurs in the muscle wall that separates the left and right ventricles from each other interventricular septum. This results in the heart being less able to pump blood effectively. Hypertrophic cardiomyopathy hcm is a common inherited heart disease with diverse phenotypic and genetic expression, clinical presentation, and natural history. New treatment strategies for hypertrophic obstructive cardiomyopathy. Hypertrophic obstructive cardiomyopathy and concomitant systemic hypertension can present a challenging diagnostic and therapeutic dilemma. How can hypertrophic obstructive cardiomyopathy be prevented.
Patient information hypertrophic obstructive cardiomyopathy. Cardiac magnetic resonance with late gadolinium enhancement in a patient with hypertrophic obstructive cardiomyopathy steady state free precession a sequences are used to assess morphology and. Get a printable copy pdf file of the complete article 4. Twenty to 30% of hc patients develop septal hypertrophy that can obstruct the aortic outflow tract. Diagnosis and treatment of hypertrophic cardiomyopathy november 2011 developed in collaboration with the american association for thoracic surgery, american society of echocardiography, american society of nuclear cardiology, heart failure society of america, heart rhythm society, society for cardiovascular angiography and interventions, and. Approximately 20 30% of patients with hc can develop obstruction of aortic outflow if inadequate filling of the ventricle occurs or a hyperdynamic state develops. It is commonly asymmetric with the most severe hypertrophy involving the basal interventricular septum. Hcm is a condition where areas of heart muscle become thickened and stiff. Hypertrophic non obstructive cardiomyopathy is a genetic condition in a majority of the affected individuals, and therefore, there are no guidelines or specific methods for preventing the condition from developing.
Therapy for hypertrophic cardiomyopathy is directed at the dynamic left ventricular outflow tract obstruction which is present in 30 to 50 percent of patients fig. Hypertrophic cardiomyopathy hcm, also called hypertrophic obstructive cardiomyopathy hocm occurs in one out of 500 people. Ccfaha guideline for the diagnosis and treatment of hypertrophic cardiomyopathy. A balloon catheter is passed, the wire is removed and the balloon inflated to occlude the artery. Hypertrophic cardiomyopathy, septal myectomy, alcohol septal. A 28yearold man presents with a twoyear history of increasing dyspnea on strenuous exertion and. This is known as asymmetrical septal hypertrophy with obstruction. Background information hypertrophic obstructive cardiomyopathy hocm is a cardiac abnormality which leads to the muscle in the wall of the heart growing and thickening to the point that it blocks blood flow exiting the heart. The initial descriptions of hypertrophic cardiomyopathy hcm focused on the presence of left ventricular lv outflow tract obstruction as a defining feature of the. During periods of strenuous exertion andor dehydration, the degree of obstruction to blood flow. The diagnosis and treatment of hypertrophic cardiomyopathy. Topic collections contact me when new articles are published in these topic areas. Widely accepted as a monogenic disease caused by a mutation in 1 of or more sarcomeric. Familial hypertrophic cardiomyopathy genetic and rare.
New perspectives on the prevalence of hypertrophic cardiomyopathy. Types of cardiomyopathy heart muscle diseases in children. Hypertrophic obstructive cardiomyopathy hocm topic. Arrhythmogenic right ventricular cardiomyopathydysplasia dilated cardiomyopathy familial dilated cardiomyopathy congestive cardiomyopathy idiopathic dilated cardiomyopathy. Surgical myectomy is a wellestablished and effective treatment for over 5 decades. Hypertrophic cardiomyopathy can cause obstruction of blood as it exits the heart left ventricle. This disease is characterized by an abnormal thickening hypertrophy of one or several areas of the walls of the heart, usually of the left ventricle. The generally accepted definition of hypertrophic cardiomyopathy hcm, is a disease state characterized by unexplained left ventricular lv hypertrophy. Another, nonobstructive variant of hcm is apical hypertrophic cardiomyopathy. Obstructive sleep apnea is associated with nonsustained. Pediatric hypertrophic cardiomyopathy hcm, hypertrophic obstructive cardiomyopathy diagnosis and treatment see online here hypertrophic cardiomyopathy hcm is the preferred term for both, what was earlier classi. Compiled consensus on the most important diagnostic modalities and genetic testing tools for the treatment of hypertrophic cardiomyopathy hcm. These treatment options can prevent sudden cardiac death, fix irregular heartbeats and keep your heart beating well. The genetic underpinnings of hcm arise largely from mutations of sarcomeric proteins.
Links to pubmed are also available for selected references. Hypertrophic obstructive cardiomyopathy is a genetic condition in a majority of the affected individuals, and therefore, there are no guidelines or specific methods for preventing the condition from developing. It is characterized by marked myocardial hypertrophy 15 mm that cannot be explained by pressure load or the presence. Denise antle, arnp, msn, ccrn, ccns critical care arnpcns. Obstruction in hypertrophic cardiomyopathy introduction hypertrophic cardiomyopathy hcm is a genetic disorder of the heart muscle, characterized by a small left ventricular cavity and marked hypertrophy of the myocardium with myocyte disarray. First experience with percutaneous mitral valve plication as primary therapy for symptomatic obstructive hypertrophic cardiomyopathy pdf. Hypertrophic cardiomyopathy hc is a genetic disease associated with the development of abnormal myocytes.
All 3 patients had intimal and medial hypertrophy of the intramural septal branches with luminal narrowing. Hypertrophic obstructive cardiomyopathy hocm usually responds very well to drug therapy. Hypertrophic cardiomyopathy occurs if heart muscle cells enlarge and cause the walls of the ventricles usually the left ventricle to thicken. Septal ablation in hypertrophic obstructive cardiomyopathy. The presence of a central or anteriorly directed jet of mitral regurgitation should raise suspicion of an intrinsic. Obstructive sleep apnea osa is highly prevalent and independently associated with atrial fibrillation in patients with hcm. Hypertrophic cardiomyopathy core concepts anesthesia. Symptoms can occur from increased afterload from both dynamic outflow obstruction as well as the elevated systemic vascular resistance. Oct 08, 2018 how can hypertrophic non obstructive cardiomyopathy be prevented. Septal reduction in hypertrophic obstructive cardiomyopathy 1 the ohio state university wexner medical center disclosures research grants from. Longterm effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy. Adapted from the 2011 accfaha guideline for the diagnosis.
Cardiac magnetic resonance with late gadolinium enhancement in a patient with hypertrophic obstructive cardiomyopathy steady state free precession. Hypertrophic cardiomyopathy hcm is a genetically determined cardiac disease. Clinical practice from the new england journal of medicine hypertrophic obstructive cardiomyopathy. It is frequently accompanied by dynamic left ventricular outflow tract obstruction and sympt. Jan 26, 2015 hypertrophic cardiomyopathy hcm is a global disease with cases reported in all continents, affecting people of both genders and of various racial and ethnic origins. The hypertrophy initially develops in the septum and extends to the free walls, often giving a picture of concentric hypertrophy. Hypertrophic cardiomyopathy hcm is associated with arrhythmias and cardiovascular death. Some patients have labile obstruction that is absent at rest but provoked with changes in preload, afterload, and contractility. In cardiomyopathy, the heart muscle becomes enlarged, thick, or rigid. To characterize the effect of mavacamten on left ventricular outflow tract lvot gradient. Hcm has been recognized for 55 years, but recently substantial advances. If this happens, the condition is called obstructive hypertrophic cardiomyopathy.
New treatment strategies for hypertrophic obstructive cardiomyopathy alcohol ablation of the septum. These diseases have many causes, signs and symptoms, and treatments. Hypertrophic obstructive cardiomyopathy circulation research. The first page of the pdf of this article appears above. Childhood hypertrophic obstructive cardiomyopathy and its.
Hess, md, ulrich sigwart, md, frcp bern and geneva, switzerland hypertrophic cardiomyopathy is a primary myocardial disorder with an autosomal pattern of inheritance characterized by inappropriate myocardial. Evaluation of left ventric ular filling pressures by doppler echocardiography in patients with hy pertrophic cardiomyopathy. Hypertrophic cardiomyopathy hcm is a condition in which a portion of the heart becomes thickened without an obvious cause. Hypertrophic obstructive cardiomyopathy and uncontrolled.
Highlights of prescribing information these highlights do. Cardiomyopathy can have many causes, signs and symptoms, and some people never have symptoms. Typical symptoms include dyspnoea, chest pain, palpitations, and syncope. Obstructive hypertrophic cardiomyopathy hcm is a relatively common genetic malformation of the heart with a prevalence of approximately 1 case in 500 births. Management of symptoms in hypertrophic cardiomyopathy. Hypertrophic cardiomyopathy hcm is a primary disease of cardiac muscle characterized by a thickening of the left ventricular wall and often predominantly affecting the interventricular septum. However, the authors have interrogated a number of relevant advances in cardiovascular medicine, including widespread feeforservice genetic. The diagnosis is usually suspected on clinical examination and. There is concern that a positive inotrope and afterload. The murmur that is pathognomonic for hcm is a crescendodecrescendo holosystolic murmur best heard at the left sternal boarder. Apr 01, 2011 hypertrophic cardiomyopathy hcm is the most common hereditary disease of the heart. They should be essential in everyday clinical decision making. Hypertrophic obstructive cardiomyopathy hocm, also known as hypertrophic cardiomyopathy hcm, is an inherited condition that affects the muscle of the. Comparison of outcomes in patients with nonobstructive.
We present a case of an obstructive hypertrophic cardiomyopathy in an 84yearold italian woman with a left ventricular outflow tract lvot peak gradient with the valsalva maneuver of 188 mm hg. For the past 20 years, most data have supported the occurrence of hcm at about 1 in 500. Pdf spark erosion myectomy in hypertrophic obstructive. New perspectives on the prevalence of hypertrophic. Cardiomyopathy information guide c a r d i o m y o p a t h y m a r d i g i a n w e l l n e s s r e s o u r c e c e n t e r page 1 other names. Pacemaker therapy in hypertrophic obstructive cardiomyopathy. Hypertrophic cardiomyopathy hcm is a genetic disorder that is characterized by left ventricular hypertrophy unexplained by secondary causes, and a nondilated left ventricle with preserved or increased ejection fraction. Guideline for the diagnosis and treatment of hypertrophic.
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