I-Common Genetic Heart Disorder
I-cardiomyopathy ye-hypertrophic (HCM) isifo esiyinto ejwayelekile kakhulu ye-genetic (esithinta abantu abangaba ngu-1 kubantu abangu-500), okungabangela izinkinga eziningana, kuhlanganise nokuhluleka kwenhliziyo nokufa okungazelelwe. Kodwa-ke, ubukhulu beHCM buhlukahluka kusukela kumuntu kuya komuntu, futhi abantu abaningi abane-HCM bangadla impilo ejwayelekile.
Izimbangela
I-HCM ibangelwa yiyodwa noma enye yezinguquko eziningana zezakhi zofuzo ezikhiqiza ukuphazamiseka ekukhuleni kwezinhliziyo ze-muscle fibers.
I-HCM idluliselwa njengendlela "ye-autosomal", okusho ukuthi uma igciwane elingavamile lizuze umzali oyedwa, ingane izoba nesifo.
Kodwa-ke, cishe isigamu seziguli ezine-HCM, isifo sofuzo asizukuzuzwa njengefa, kodwa senzeke njengendlela yokuguqula izakhi zofuzo - uma kunjalo, abazali kanye nabantwana bakithi besiguli ngeke babe engozini enkulu ye-HCM. Noma kunjalo, lokhu "okusha" kokuguqulwa kungadluliselwa esizukulwaneni esilandelayo.
Imiphumela ye-Cardiac
E-HCM, izindonga ze-muscular of the ventricles (amakamelo aphansi enhliziyo) ajwayelekile ngokweqile - isimo esibizwa ngokuthi "hypertrophy." Lokhu kuqina kubangela ukuba imisipha yenhliziyo isebenze ngokungavamile, okungenani ubukhulu obuningi. Uma kunzima, i-hypertrophy ingabangela ukuhluleka kwenhliziyo kanye nama-arrhythmias enhliziyo .
Ngaphezu kwalokho, uma kuba ngokweqile i-hypertrophy ingabangela ukuphazamiseka ema-ventricles, okungaphazamisa umsebenzi we-valve ye-aortic ne-valve mitral, ephazamisa ukugeleza kwegazi ngenhliziyo.
I-HCM ingabangela okungenani izinhlobo ezinhlanu zezinkinga ezinkulu zenhliziyo:
1) I-HCM ingabangela ukungasebenzi kwe-diastolic. "Ukukhubazeka kwe-Diastolic" kubhekisela "ekutheni" okungavamile kosizi lwe-ventricular, okwenza kube nzima kakhulu ukuthi ama-ventricles agcwalise igazi phakathi kwe-heart beat.
Ku-HCM, i-hypertrophy ngokwayo ikhiqiza okungenani ukungasebenzi kahle kwe-diastolic. Uma kunzima ngokwanele, lokhu ukungasebenzi kahle kwe-diastolic kungaholela ekuhlulekeni kwenhliziyo ye-diastolic, nezimpawu ezinzima ze- dyspnea (ukuphefumula) nokukhathala. Ngisho nokungasebenzi kahle kwe-diastolic kwenza kube nzima kakhulu iziguli ezine-HCM ukuba zibekezelele ama-arrhythmiya enhliziyo, ikakhulukazi i-fibrillation ye-atrial .
2) I-HCM ingabangela "ukuvinjelwa kwe-ventricular outflow obstruction. (I-LVOT). " Ku-LVOT, kunesithiyo esinqunyiwe esenza kube nzima ku-ventricle kwesokunxele ukulahla igazi layo ngokushaya kwenhliziyo ngayinye. Le nkinga nayo ikhona nge- aortic valve stenosis , lapho i-valve ye-aortic iqina khona futhi ihluleka ukuvula ngokujwayelekile. Kodwa-ke, kuyilapho i-aortic stenosis ibangelwa yizifo enhliziyweni ye-valve ngokwayo, i-LVOT ne-HCM ibangelwa ukuqina kwesisipha senhliziyo ngaphansi kwe-valve ye-aortic. Lesi sifo sibhekwa ngokuthi "i-subvalvular stenosis." Njengoba nje nge-aortic stenosis, i-LVOT ebangelwa i-HCM ingabangela ukuhluleka kwenhliziyo.
3) I-HCM ingabangela ukubuyiswa kwe-mitral. Ekuvuseleleni kwe-mitral , i-valve ye-mitral ehluleka ukuvala ngokujwayelekile uma i-ventricle yesokunxele ibetha, ivumela igazi ukuba ligijime emuva ("regurgitate") engxenyeni yesokunxele.
Ukubuyiswa kwe-mitral okubonwe nge-HCM akubangelwa inkinga ye-valve yenhliziyo yangaphakathi, kodwa kunalokho, kubangelwa ukuphazamiseka ngendlela okwenziwa ngayo izinkontileka ze-ventricle, ezibangelwa ukuqina kwesisu se-ventricular. Ukubuyiswa kwe-Mitral kungenye indlela abantu abanayo i-HCM abangase bahlakulele ukuhluleka kwenhliziyo.
4) I-HCM ingabangela i-ischemia ye-muscle yenhliziyo. I-Ischemia - ukushiywa kwe- oksijeni - kutholakala kakhulu kwiziguli ezine- coronary artery disease (CAD) , lapho ukuvinjelwa emthonjeni we-coronary kuvimbela ukugeleza kwegazi kuyingxenye yesisu senhliziyo. Nge-HCM, imisipha yenhliziyo ingaba yinkimbinkimbi kangangokuthi ezinye izingxenye zomzimba zivele nje zingatholi ukugeleza kwegazi okwanele, ngisho nalapho imishanguzo ye-coronary ijwayelekile.
Uma lokhu kwenzeka, i- angina ingenzeka (ikakhulukazi ngokuzikhandla), futhi i- infarction ye-myocardial (ukufa kwesisu senhliziyo) kungenzeka.
5) I-HCM ingabangela ukufa okungazelelwe. Ukufa okungazelelwe ku-HCM ngokuvamile kubangelwa yi- ventricular tachycardia noma i- fibrication ye-ventricular , futhi ngokuvamile ihlobene nokuzikhandla ngokweqile. Kungenzeka ukuthi i-ischemia ye-muscle yenhliziyo iveza eziningi uma ingekho ama-arrhythmias amaningi aholela ekufeni okungazelelwe ezigulini ezine-HCM. Ngenxa yalesi sizathu, iziguli eziningi ezine-HCM zidinga ukuvimbela ukuzivocavoca kwazo.
Izimpawu
Izimpawu ezitholwa abantu abane-HCM ziyahlukahluka kakhulu. Kuvamile iziguli ezinezifo ezincane ukuthi zingabi nazo izimpawu nhlobo. Kodwa-ke, uma izinkinga zenhliziyo ezishiwo nje zikhona, okungenani ezinye izimpawu kungenzeka. Izimpawu ezivame ukubhekana neziguli ezine-HCM ziyi-dyspnea enokuzivocavoca , i- orthopnea , i- dyspnea ye-paroxysmal nocturnal , i- palpitations , iziqephu zesikhumba esiphezulu, ubuhlungu besifuba, ukukhathala noma ukuvuvukala kwamazinyo. I-Syncope (ukulahlekelwa kwengqondo) kunoma ubani one-HCM, ikakhulu uma ihlotshaniswa nokuzivocavoca, yindaba ebaluleke kakhulu, futhi ingabonisa ingozi enkulu kakhulu yokufa okungazelelwe. Noma iyiphi isiqephu se-syncope sidinga ukuhlolwa ngokushesha ngudokotela.
Ukuxilongwa
Ngokuvamile, i- echocardiogram iyindlela engcono yokuhlola i-HCM. I-echocardiogram ivumela ukulinganisa okunembile kobuningi bezindonga ze-ventricular, futhi ingabona i-LVOT kanye nokuphindaphinda kwe-mitral.
Umshini we- electrocardiogram (ECG) ungadalula i-ventricular hypertrophy, futhi usetshenziswe njengethuluzi lokuhlola ukuhlola i-HCM kubadlali abasha.
Kokubili i-ECG kanye ne-echocardiogram kufanele kwenziwe ngezihlobo eziseduze zomuntu otholakala nge-HCM, futhi i-echocardiogram kufanele yenziwe kunoma yimuphi umuntu okukhulunywa ngaye nge-ECG noma ukuhlolwa kwangokwenyama kubonisa ukuxhuma kwe-ventricular hypertrophy.
Ukwelapha
I-HCM ayikwazi ukuphulukiswa, kodwa ezimweni eziningi ukuphathwa kwezokwelapha kungalawula izimpawu futhi kuthuthukise imiphumela yomtholampilo. Kodwa-ke, ukuphathwa kwe-HCM kungaba yinkimbinkimbi kakhulu, futhi noma ubani onezibonakaliso ngenxa ye-HCM kufanele alandelwe isazi se-cardiologist.
Abavimbela i-Beta ne- calcium blockers bangasiza ekunciphiseni "ukuqina" emzimbeni wezinhliziyo ezinzima. Ukugwema ukungcola amanzi kubalulekile kuziguli ezine-HCM ekunciphiseni izimpawu ezihlobene ne-LVOT. Kwezinye iziguli ukuhlinzeka ukususa izingxenye zomzimba wenhliziyo eqinisiwe kuyadingeka ukuze kukhululwe i-LVOT.
I-fibrillation ye-atrial, uma kwenzeka, ivame ukudala izimpawu ezinzima nezidingo okumele ziphathwe ngokweqile kwiziguli ezine-HCM kunabantu abaningi.
Ukuvimbela Ukufa Okungazelelwe
I-HCM yiyona imbangela evame kakhulu yokufa ngokuzumayo kubadlali abasha Nakuba ukufa ngokungazelelwe kuyinkinga ebhubhisa kakhulu, ikakhulukazi uma kwenzeka kubantu abasha. Ngenxa yalesi sizathu, ukuzikhandla ngokweqile nokuvivinya umzimba kufanele kuvinjelwe ezigulini ezine-HCM.
Izindlela eziningi ziye zazama ukunciphisa ingozi yokufa okungazelelwe ezigulini ezine-HCM - kuhlanganise nokusebenzisa i-beta blockers kanye ne-calcium blockers, kanye nezidakamizwa eziphikisayo . Noma kunjalo, lezi zindlela azizange zibonwe ziphumelele ngokwanele. Manje kubonakala kucacile ukuthi, ezigulini ezine-HCM ezengozini yokufa okungazelelwe zivela phezulu, i- defibrillator engasetshenziselwa kufanele ihlolwe ngokuqinile.
Imithombo:
UMassie, BM "Ukuhluleka Inhliziyo" ku: Goldman L no-Ausiello D (Eds). I-Cecil Textbook of Medicine, i-WB Saunders, ka-2003.
Nishimura RA, Holmes DR Jr. Ukuzikhandla kwemitholampilo. I-hypertrophic obstructive cardiomyopathy. N Engl J Med 2004; 350: 1320.
Gersh BJ, Maron BJ, Bonow RO, et al. 2011 ACCF / AHA isiqondiso sokuthola ukwelashwa nokuphathwa kwe-cardiomyopathy ye-hypertrophic: summary executive: umbiko we-American College of Cardiology Foundation / i-American Heart Association Task Force on Guidelines Practice. Ukujikeleza ngo-2011; 124: 2761.