Մասնակից:Avandryan/Ավազարկղ

Վիքիպեդիայից՝ ազատ հանրագիտարանից

։

Բուժում[խմբագրել | խմբագրել կոդը]

Անախտանիշ հիվանդներ[խմբագրել | խմբագրել կոդը]

Հիպերտրոֆիկ կարդիոմիոպաթիայով մարդկանց զգալի մասը չունի որևէ ախտանիշ և ունեն կյանքի նորմալ տևողությունը, ուղղակի նրանք պետք է խուսափեն առանձնապես ծանր ֆիզիկական ծանրաբեռնվածությունից, սպորտային մրցումներից և պետք է հետազոտվեն սրտային հսանկարծամահության ռիսկի գործոնների համար։ In people with resting or inducible outflow obstructions, situations that will cause dehydration or vasodilation (such as the use of vasodilatory or diuretic blood pressure medications) should be avoided. Septal reduction therapy is not recommended in asymptomatic people.[1]

Դեղամիջոցներ[խմբագրել | խմբագրել կոդը]

Դեղամիջոցների օգտագործման հիմնական նպատակն է ախտանիշների՝ կրծքավանդակի ցավի, հևոցի և սրտխփոցի մեղամացումը։ Բետա բլոկերըները համարվում են առաջին շարքի պրեպարատներ, քանի որ կարող են նվազեցնել սրտի կծկումներին հաճախականությունը և էքստրասիստոլաների առաջացումը։ Այն մարդիկ, ում համար արդյունավետ չեն բետա բլոկերները, կարող են օգտագործել ոչ դիհիդրոպիրիդինային շարքի Ca բլոկատորներ(օրինակ վերապամիլ) սակայն դրանք վտանգավոր են, եթե հիվանդն ունի ցածր ճնշում կամ արտահայտված հևոց հանգստի ժամանակ։ Այս պրեպարատները նույնպես նվազեցնում են սրտի կծկումների հաճախականությունը, չնայած դրանց օգտագործումը արտատար տրակտի ծանր օբստրոկւցիայի, թոքային զարկերակի բարձր ճնշման և զարկերակային արյան ցածր ճնշման դեպքում պետք է կատարվի զգուշությամբ։ Պետք է խուսափել դիհիդրոպիրիդինային շարքի Ca բլոկատորների օգտագործումկից օբստրոկցիայի նշանների դեպքում։ Եթե վերոնշյալ դեղամիջոցները չեն թեթևացնում ախտանիշները,, հետագա բուժման համար կարելի է կիրառել դիզոպիրամիդ։ For people whose symptoms are not relieved by the above treatments, disopyramide can be considered for further symptom relief. Diuretics can be considered for people with evidence of fluid overload, though cautiously used in those with evidence of obstruction. People who continue to have symptoms despite drug therapy can consider more invasive therapies. Intravenous phenylephrine (or another pure vasoconstricting agent) can be used in the acute setting of low blood pressure in those with obstructive hypertrophic cardiomyopathy who do not respond to fluid administration.[1]

Վիրաբուժական սեպտալ միէկտոմիա[խմբագրել | խմբագրել կոդը]

Surgical septal myectomy is an open-heart operation done to relieve symptoms in people who remain severely symptomatic despite medical therapy. It has been performed successfully since the early 1960s.[2] Surgical septal myectomy uniformly decreases left ventricular outflow tract obstruction and improves symptoms, and in experienced centers has a surgical mortality of less than 1%, as well as 85% success rate.[3] It involves a median sternotomy (general anesthesia, opening the chest, and cardiopulmonary bypass) and removing a portion of the interventricular septum.[4] Surgical myectomy resection that focuses just on the subaortic septum, to increase the size of the outflow tract to reduce Venturi forces, may be inadequate to abolish systolic anterior motion (SAM) of the anterior leaflet of the mitral valve. With this limited resection, the residual mid-septal bulge still redirects flow posteriorly; SAM persists because flow still gets behind the mitral valve. It is only when the deeper portion of the septal bulge is resected that flow is redirected anteriorly away from the mitral valve, abolishing SAM. With this in mind, a modification of the Morrow myectomy termed extended myectomy, mobilization and partial excision of the papillary muscles has become the excision of choice.[5][6][7][8] In people with particularly large redundant mitral valves, anterior leaflet plication may be added to complete separation of the mitral valve and outflow.[8] Complications of septal myectomy surgery include possible death, arrhythmias, infection, incessant bleeding, septal perforation/defect, and stroke.[3]

Միջնապատի ալկոհոլային աբլացիա[խմբագրել | խմբագրել կոդը]

Alcohol septal ablation, introduced by Ulrich Sigwart in 1994, is a percutaneous technique that involves injection of alcohol into one or more septal branches of the left anterior descending artery. This is a catheter technique with results similar to the surgical septal myectomy procedure but is less invasive, since it does not involve general anaesthesia and opening of the chest wall and pericardium (which are done in a septal myectomy). In a select population with symptoms secondary to a high outflow tract gradient, alcohol septal ablation can reduce the symptoms of HCM. In addition, older individuals and those with other medical problems, for whom surgical myectomy would pose increased procedural risk, would likely benefit from the less-invasive septal ablation procedure.[4][9]

When performed properly, an alcohol septal ablation induces a controlled heart attack, in which the portion of the interventricular septum that involves the left ventricular outflow tract is infarcted and will contract into a scar. There is debate over which people are best served by surgical myectomy, alcohol septal ablation, or medical therapy.[10]

Միտրալ փականի կլիպավորում[խմբագրել | խմբագրել կոդը]

Since 2013, mitral clips have been implanted via catheter as a new strategy to correct the motion of the mitral valve in people with severe obstructive HCM. The device fastens together the mitral valve leaflets to improve the heart's blood outflow. The mitral clip has not yet established the long-term reliability of septal myectomy or alcohol septal ablation, but HCM specialists are increasingly offering the clip as a less-invasive treatment option.[11][12]

Ռիթմավարի կամ դեֆիբրիլյատորի իմպլանտացիա[խմբագրել | խմբագրել կոդը]

The use of a pacemaker has been advocated in a subset of individuals, in order to cause asynchronous contraction of the left ventricle. Since the pacemaker activates the interventricular septum before the left ventricular free wall, the gradient across the left ventricular outflow tract may decrease. This form of treatment has been shown to provide less relief of symptoms and less of a reduction in the left ventricular outflow tract gradient when compared to surgical myectomy.[13] Technological advancements have also led to the development of a dual-chamber pacemaker, which is only turned on when needed (in contrast to a regular pacemaker which provides a constant stimulus). Although the dual-chamber pacemaker has shown to decrease ventricular outflow tract obstruction, experimental trials have found only a few individuals with improved symptoms.[14] Unfortunately, researchers suspect that these reports of improved symptoms are due to a placebo effect.[3]

The procedure includes an incision on the anterolateral area below the clavicle. Two leads are then inserted; one into the right atrium and the other into the right ventricular apex via the subclavian veins. Once in place, they are secured and attached to the generator which will remain inside the fascia, anterior to the pectoral muscle.[3] Complications of this procedure include infection, electrical lead and generator malfunction which will require replacement.[3]

For people with HCM who exhibit one or more of the major risk factors for sudden cardiac death, an implantable cardioverter-defibrillator (ICD) or a combination pacemaker/ICD all-in-one unit may be recommended as an appropriate precaution.[1][2][15][16]

Սրտի փոխպատվաստում[խմբագրել | խմբագրել կոդը]

Հավելյալ տեղեկություն՝ Սրտի փոխպատվաստում

In cases that are unresponsive to all other forms of treatment, cardiac transplantation is one option. It is also the only treatment available for end-stage heart failure.[14] However, transplantation must occur before the onset of symptoms such as pulmonary vessel hypertension, kidney malfunction, and thromboembolism in order for it to be successful. Studies have indicated a seven-year survival rate of 94% in people with HCM after transplantation.[14]

A systematic review from 2002 concluded that: "Overall, HCM confers an annual mortality rate of about 1%... HCM may be associated with important symptoms and premature death but more frequently with no or relatively mild disability and normal life expectancy."[4]

Children[խմբագրել | խմբագրել կոդը]

Even though hypertrophic cardiomyopathy (HCM) may be present early in life and is most likely congenital, it is one of the most-uncommon cardiac malformations encountered in pediatric cardiology, largely because the presentation of symptoms is usually absent, incomplete, or delayed into adulthood. Most of the current information pertaining to HCM arises from studies in adult populations, and the implication of these observations for pediatric population is often uncertain.[17] Nonetheless, recent studies in pediatric cardiology have revealed that HCM accounts for 42% of childhood cardiomyopathies, with an annual incidence rate of 0.47/100,000 in children.[18] Further, in asymptomatic cases, sudden death is considered one of the most-feared complications associated with the disease in select pediatric populations. Consequently, the recommended practice is to screen children of affected individuals throughout childhood to detect cardiac abnormalities at an early stage, in the hope of preventing further complications of the disease.[17]

Generally, the diagnosis of HCM in a pediatric population is made during assessment for murmur, congestive heart failure, physical exhaustion, and genetic testing of children of affected individuals.[17] Specifically, echocardiogram (ECHO) has been used as a definitive noninvasive diagnostic tool in nearly all children. ECHO assesses cardiac ventricular size, wall thickness, systolic and diastolic function, and outflow obstruction. Thus, ECHO has been chosen as an ideal means to detect excessive wall thickening of cardiac muscle in HCM.[17]

For children with HCM, treatment strategies aim to reduce disease symptoms and lower the risk of sudden death.[19] Due to the heterogeneity of the disease, treatment is usually modified according to individual's needs.[19] β-blockers improve left ventricular filling and relaxation and thereby improve symptoms. In some children, β–blockers (e.g., propranolol) were shown effective to reduce the risk of sudden death.[19] Further, calcium channel blockers (verapamil) and antiarrhythmic drugs may be used as an adjunct therapy to β-blockers in symptomatic children. Nonetheless, further testing is needed to determine their definitive benefits.[19]

Other animals[խմբագրել | խմբագրել կոդը]

Echocardiography of hypertrophic-obstructive cardiomyopathy (HOCM) in a cat.

Feline hypertrophic cardiomyopathy (HCM) is the most common heart disease in domestic cats; the disease process and genetics are believed to be similar to the disease in humans.[20] In Maine Coon cats, HCM has been confirmed as an autosomal dominant inherited trait.[21] Numerous cat breeds have HCM as a problem in the breed.[22] The first genetic mutation (in cardiac myosin binding protein C) responsible for feline HCM was discovered in 2005 in Maine Coon cats.[23] A test for this mutation (A31P) is available.[24] About one-third of Maine Coon cats tested for the mutation are either heterozygous or homozygous for the mutation, although many of the cats that are heterozygous have no overt evidence of the disease on an echocardiogram (low penetrance). Some Maine Coon cats with clinical evidence of hypertrophic cardiomyopathy test negative for this mutation, strongly suggesting that another cause exists in the breed. The cardiac myosin binding protein C mutation identified in Maine Coon cats has not been found in any other breed of cat with HCM, but more recently another myosin binding protein C mutation has been identified in Ragdoll cats with HCM.[25][26] As in humans, feline HCM is not present at birth but develops over time. It has been identified for the first time in cats as young as 6 months of age and at least as old as 7 years of age.

Clinically, cats with hypertrophic cardiomyopathy commonly have a systolic anterior motion of the mitral valve (see graphic). Cats with severe HCM often develop left heart failure (pulmonary edema; pleural effusion) because of severe diastolic dysfunction of the left ventricle. They may also develop a left atrial thrombus that embolizes, most commonly, to the terminal aorta creating acute pain and rear limb paralysis (see below). Sudden death can also occur but appears to be uncommon.

There is no cure for feline HCM. Many but not all cats have a heart murmur. Many cats that have a heart murmur do not have HCM. Frequently the first signs that a cat has HCM are tachypnea/dyspnea due to heart failure or acute pain and paralysis due to systemic thromboembolism. While medication is commonly given to cats with HCM that have no clinical signs, no medication has been shown to be helpful at this stage and it has been shown that an ACE inhibitor is not beneficial until heart failure is present[27] (at which time a diuretic is most beneficial). Diltiazem generally produces no demonstrable benefit. Atenolol is commonly administered when a severe systolic anterior motion of the mitral valve is present.

Feline arterial thromboembolism (FATE) is a relatively common and devastating complication of feline HCM and other feline cardiomyopathies. The thrombus generally forms in the left atrium, most commonly the left auricle. The formation is thought to be primarily due to blood flow stasis. Classically, the thromboembolism lodges at the iliac trifurcation of the aorta, occluding either one or both of the common iliac arteries. Because this split is called the saddle, and is the most frequent location for the thrombus, FATE is commonly known as saddle thrombus.[28] Clinically this presents as a cat with complete loss of function in one or both hind limbs. The hind limbs are cold and the cat is in considerable pain. Emboli may, rarely, lodge in other locations, most commonly the right front limb and the renal arteries.

Clopidogrel is used to try to prevent left atrial thrombus formation in cats with HCM and a large left atrium. The FATCAT study at Purdue University demonstrated that it is superior to aspirin for the prevention of a second thrombus from forming in cats that have already experienced a clot. Thrombolytic agents (e.g., tissue plasminogen activator) have been used with some success to break down an existing aortic thromboembolism, but their cost is high and outcome appears to be no better than giving a cat time (48–72 hours) to break down its own clot. Pain management is extremely important. The prognosis for cats with FATE is often poor as they are likely to have significant HCM already and a recurrent bout of FATE is likely.[29] For this reason, euthanasia is often a valid consideration.

In July 2013, Rigo, a 42-year-old western lowland gorilla, resident in Melbourne Zoo and father of Mzuri, the first gorilla born by artificial insemination, died unexpectedly as a result of HCM. The condition is not uncommon in male gorillas over the age of 30, and in many cases, there is no sign of the disease until the individual's sudden death.[30]

Ծանոթագրություններ[խմբագրել | խմբագրել կոդը]

  1. ↑ 1,0 1,1 1,2 Gersh BJ, Maron BJ, Bonow RO, և այլք: (December 2011). «2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: executive summary». J. Thorac. Cardiovasc. Surg. 142 (6): 1303–38. doi:10.1016/j.jtcvs.2011.10.019. PMID 22093712.
  2. ↑ 2,0 2,1 Maron BJ, McKenna WJ, Danielson GK, և այլք: (Nov 2003). «American College of Cardiology/European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathy». J Am Coll Cardiol. 42 (9): 1687–1713. doi:10.1016/S0735-1097(03)00941-0. PMID 14607462.
  3. ↑ 3,0 3,1 3,2 3,3 3,4 Behr, Elijah; McKenna, William (2002). «Hypertrophic Cardiomyopathy». Current Treatment Options in Cardiovascular Medicine. 4 (6): 443–453. doi:10.1007/s11936-002-0039-8. PMID 12408787.
  4. ↑ 4,0 4,1 4,2 Maron BJ (March 2002). «Hypertrophic cardiomyopathy: a systematic review». JAMA. 287 (10): 1308–1320. doi:10.1001/jama.287.10.1308. ISSN 0098-7484. PMID 11886323.
  5. ↑ Sherrid MV, Chaudhry FA, Swistel DG (Feb 2003). «Obstructive hypertrophic cardiomyopathy: echocardiography, pathophysiology, and the continuing evolution of surgery for obstruction». Ann. Thorac. Surg. 75 (2): 620–32. doi:10.1016/S0003-4975(02)04546-0. PMID 12607696.
  6. ↑ Messmer BJ (Aug 1994). «Extended myectomy for hypertrophic obstructive cardiomyopathy». Ann. Thorac. Surg. 58 (2): 575–7. doi:10.1016/0003-4975(94)92268-3. PMID 8067875.
  7. ↑ Schoendube FA, Klues HG, Reith S, Flachskampf FA, Hanrath P, Messmer BJ (Nov 1995). «Long-term clinical and echocardiographic follow-up after surgical correction of hypertrophic obstructive cardiomyopathy with extended myectomy and reconstruction of the subvalvular mitral apparatus». Circulation. 92 (9 Suppl): II122–7. doi:10.1161/01.CIR.92.9.122. PMID 7586394.
  8. ↑ 8,0 8,1 Balaram SK, Sherrid MV, Derose JJ, Hillel Z, Winson G, Swistel DG (Jul 2005). «Beyond extended myectomy for hypertrophic cardiomyopathy: the resection-plication-release (RPR) repair». Ann. Thorac. Surg. 80 (1): 217–23. doi:10.1016/j.athoracsur.2005.01.064. PMID 15975370.
  9. ↑ Sigwart, Ulrich (22 July 1995). «Non-surgical myocardial reduction for hypertrophic obstructive cardiomyopathy». Lancet. 346 (8969): 211–214. doi:10.1016/S0140-6736(95)91267-3. PMID 7616800.
  10. ↑ Heldman AW, Wu KC, Abraham TP, Cameron DE (Jan 2007). «Myectomy or alcohol ablation surgery and percutaneous intervention go another round». J Am Coll Cardiol. 49 (3): 358–60. doi:10.1016/j.jacc.2006.10.029. PMID 17239718.
  11. ↑ Dimitrow PP, Rajtar-Salwa R (May 2016). «Obstructive Form of Hypertrophic Cardiomyopathy – Left Ventricular Outflow Tract Gradient: Novel Methods of Provocation, Monitoring of Biomarkers, and Recent Advances in the Treatment». BioMed Research International. 2016 (1575130): 1575130. doi:10.1155/2016/1575130. PMC 4877458. PMID 27247935.{{cite journal}}: CS1 սպաս․ չպիտակված ազատ DOI (link)
  12. ↑ Sorajja P, Pedersen WA, Bae R, Lesser JR, Jay D, Lin D, Harris K, Maron BJ (June 2016). «First Experience With Percutaneous Mitral Valve Plication as Primary Therapy for Symptomatic Obstructive Hypertrophic Cardiomyopathy» (PDF). J Am Coll Cardiol. 67 (24): 2811–2818. doi:10.1016/j.jacc.2016.03.587. PMID 27311518. Արխիվացված (PDF) օրիգինալից 2017-02-11-ին. Վերցված է 2017-02-08-ին.
  13. ↑ Ommen SR, Nishimura RA, Squires RW, Schaff HV, Danielson GK, Tajik AJ (Jul 1999). «Comparison of dual-chamber pacing versus septal myectomy for the treatment of patients with hypertrophic obstructive cardiomyopathy: a comparison of objective hemodynamic and exercise end points». J Am Coll Cardiol. 34 (1): 191–6. doi:10.1016/S0735-1097(99)00173-4. PMID 10400010.
  14. ↑ 14,0 14,1 14,2 Coats, Caroline; Elliott, Perry (2008). «Current management of hypertrophic cardiomyopathy». Current Treatment Options in Cardiovascular Medicine. 10 (6): 496–504. doi:10.1007/s11936-008-0042-9. PMID 19026180.
  15. ↑ Maron BJ, Spirito P, Shen W, և այլք: (2007). «Implantable Cardioverter-Defibrillators and Prevention of Sudden Cardiac Death in Hypertrophic Cardiomyopathy». JAMA. 298 (4): 405–412. doi:10.1001/jama.298.4.405. PMID 17652294.
  16. ↑ «ICDs and Pacemakers». Hypertrophic Cardiomyopathy Association. Արխիվացված օրիգինալից November 15, 2016-ին. Վերցված է November 14, 2016-ին.
  17. ↑ 17,0 17,1 17,2 17,3 Colan, Steven (October 2010). «Hypertrophic Cardiomyopathy in Childhood». Heart Fail Clin. 6 (4): 433–444. doi:10.1016/j.hfc.2010.05.004. PMC 2946944. PMID 20869644.
  18. ↑ Lipshultz, SE; Sleeper LA; Towbin JA (2003). «The incident of pediatric cardiomyopathy in two regions of the United States». N. Engl. J. Med. 348 (17): 1647–55. doi:10.1056/NEJMoa021715. PMID 12711739.
  19. ↑ 19,0 19,1 19,2 19,3 Maskatia, Shiraz (2012). «Hypertrophic cardiomyopathy: infants, children and adolescents». Congenit Heart Dis. 7 (1): 84–92. doi:10.1111/j.1747-0803.2011.00613.x. PMID 22222117.
  20. ↑ «Hypertrophic Cardiomyopathy (HCM) in Cats». Cornell University Hospital for Animals. Արխիվացված օրիգինալից 22 January 2018-ին. Վերցված է 24 February 2017-ին.
  21. ↑ Kittleson M, Meurs K, Munro M, Kittleson J, Liu S, Pion P, Towbin J (1999). «Familial hypertrophic cardiomyopathy in Maine coon cats: an animal model of human disease». Circulation. 99 (24): 3172–80. doi:10.1161/01.CIR.99.24.3172. PMID 10377082.
  22. ↑ Kittleson, Mark; Gompf, Rebecca; Little, Susan. «Feline Hypertrophic Cardiomyopathy: Advice for Breeders». Cat Fancier's Association. Արխիվացված է օրիգինալից May 13, 2008-ին.
  23. ↑ Meurs K, Sanchez X, David R, Bowles N, Towbin J, Reiser P, Kittleson J, Munro M, Dryburgh K, Macdonald K, Kittleson M (2005). «A cardiac myosin binding protein C mutation in the Maine Coon cat with familial hypertrophic cardiomyopathy». Hum Mol Genet. 14 (23): 3587–93. doi:10.1093/hmg/ddi386. PMID 16236761.
  24. ↑ «Genetics: Maine Coon Cat Hypertrophic Cardiomyopathy». North Carolina State University, College of Veterinary Medicine. Արխիվացված օրիգինալից September 3, 2019-ին. Վերցված է December 29, 2016-ին.
  25. ↑ Meurs KM, Norgard MM, Ederer MM, Hendrix KP, Kittleson MD (2007). «A substitution mutation in the myosin binding protein C gene in Ragdoll hypertrophic cardiomyopathy». Genomics. 90 (2): 261–264. doi:10.1016/j.ygeno.2007.04.007. PMID 17521870.
  26. ↑ «Genetics: Ragdoll Cat Hypertrophic Cardiomyopathy». North Carolina State University, College of Veterinary Medicine. Արխիվացված օրիգինալից December 14, 2016-ին. Վերցված է December 29, 2016-ին.
  27. ↑ MacDonald K, Kittleson M, Larson R, Kass P, Klose T, Wisner E (2006). «The effect of ramipril on left ventricular mass, myocardial fibrosis, diastolic function, an plasma neurohormones in Maine Coon cats with familial hypertrophic cardiomyopathy without heart failure». J Vet Intern Med. 20 (5): 1093–1105. doi:10.1111/j.1939-1676.2006.tb00707.x. PMID 17063701.
  28. ↑ «The Fragile Fate of FATEs: The Management and Prognosis of Feline Aortic Thromboembolism». Massachusetts Society for the Prevention of Cruelty to Animals-Angell. Արխիվացված օրիգինալից October 9, 2018-ին. Վերցված է October 8, 2016-ին.
  29. ↑ Borgeat K, Wright J, Garrod O, Payne JR, Fuentes VL (2014). «Arterial thromboembolism in 250 cats in general practice: 2004-2012». Journal of Veterinary Internal Medicine. 28 (1): 102–108. doi:10.1111/jvim.12249. PMC 4895537. PMID 24237457.
  30. ↑ Smith, Bridie (2013-07-26). «Silverback gorilla Rigo died of heart failure at Melbourne Zoo». The Age. Արխիվացված օրիգինալից 2017-01-03-ին. Վերցված է 2013-07-26-ին.

External links[խմբագրել | խմբագրել կոդը]


Կատեգորիա:Cardiomyopathy Կատեգորիա:Autosomal dominant disorders Կատեգորիա:Cardiogenetic disorders Կատեգորիա:Cat diseases Կատեգորիա:Cytoskeletal defects Կատեգորիա:Sports medicine Կատեգորիա:Articles containing video clips Կատեգորիա:RTT

Ստացված է «https://hy.wikipedia.org/w/index.php?title=Մասնակից:Avandryan/Ավազարկղ&oldid=7487805» էջից