I-Common Genetic Heart Disorder
I-cardiomyopathy ye-Hypertrophic (HCM) yinto engavamile yokuvelisa i-genetic (intliziyo engama-1 kwabangama-500), leyo ingabangela iingxaki ezininzi, kuquka ukuhluleka kwentliziyo kunye nokufa ngokukhawuleza. Nangona kunjalo, ubunzima be-HCM buyatshintsha ukusuka kumntu kumntu, kwaye abantu abaninzi abane-HCM bangakhokelela ubomi obuqhelekileyo.
Izizathu
I-HCM ibangelwa enye okanye enye yeenguqu ezininzi zokuguquka kwemvelo ezivelisa ingxaki ekukhuleni kwentambo ye-muscle fibers.
I-HCM idluliselwa njengendlela "yokuzimela", oko kuthetha ukuba ukuba i-gene engavamile izuze kumzali omnye, umntwana uya kuba nesifo.
Nangona kunjalo, malunga nesiqingatha sezigulane ezine-HCM, i-disorder disorder ayifumaneli ifa, kodwa iyenzeka njengenguqu yokuguqula izityalo - kwimeko apho, abazali kunye nabantakwabo besigulane abayi kuba yingozi kwiHCM. Nangona kunjalo, lo "tshintsho" olutshintsho lunokudluliselwa kwisizukulwana esilandelayo.
Iziphumo zeCardiac
Kwi-HCM, iindonga ze-muscular ze-ventricles (amacumbi angaphantsi entliziyo) zenziwe ngokungaqhelekanga-imeko ebizwa ngokuthi "hypertrophy." Ukunyusa kubangela ukuba imisipha yenhliziyo isebenze ngokungavamile, ubuncinane ubuncinane. Ukuba kunzima, i-hypertrophy inokukhokelela ekuphelelweni kwentliziyo kunye neemeyimu ze- cardiac arrhythmias .
Ukongezelela, ukuba kuba yingozi kakhulu i-hypertrophy inokubangela ukuphazamiseka kwiimvolumu, ezingaphazamisana nomsebenzi we-valve ye-aorti kunye ne-valve mitral, ukuphazamisa ukuhamba kwegazi ngentliziyo.
I-HCM inokubangela ubuncinane iintlobo ezintlanu zeengxaki ezinzulu zesifo:
1) I-HCM inokubangela ukungasebenzi kwe-diastolic. "Ukukhubazeka kwe-Diastolic" kubhekisela ku "bunzima" obungaqhelekanga bomzimba we-ventricular muscle, okwenza kube nzima ukuba iifriricle zizalise igazi phakathi kwentsimbi nganye.
Kwi-HCM, i-hypertrophy ngokwayo ivelisa ubuncinane i-diastolic ukungasebenzi. Ukuba kunzima ngokwaneleyo, le ntsholongwane ye-diastolic ingakhokelela ekufeni kwe-diastolic intliziyo, kunye neempawu ezinzima ze- dyspnea (ukuphefumula okufutshane) kunye nokukhathala. Nangona i-diastolic enobudlelwane obunzima yenza kube nzima kwizigulane nge-HCM ukunyamezela i-arrhythmias ye-cardiac, ngakumbi i-fibrillation ye -rial .
2) I-HCM inokubangela "ukukhutshwa kwe-ventricular outflow. (I-LVOT). " Kwi-LVOT, kunomqobo wokunciphisa inxalenye eyenza kube nzima kwi-ventricle yokushiya ukulahla igazi layo ngentliziyo nganye. Le ngxaki iyenzeka ne- aortic valve stenosis , apho i-valve ye-aortic iyaqina kwaye ihluleka ukuvula ngokuqhelekileyo. Nangona kunjalo, ngelixa i-aortic stenosis ibangelwa zizifo kwintliziyo ye-valve ngokwayo, i-LVOT kunye ne-HCM ibangelwa ukunyanyiswa kwesisu senhliziyo phantsi kwe-valve ye-aortic. Le meko ibhekiselwa "njenge-stenosis ye-subvalvular." Njengokuba kunjalo ne-aortic stenosis, i-LVOT ebangelwa yi-HCM inokukhokelela ekuphelelweni kwentliziyo.
3) I-HCM inokubangela ukubuyiswa kwe-mitral. Kwi- mitral regurgation , i-mitral valve ihluleka ukuvala ngokuqhelekileyo xa i-ventricle ihamba, ivumela igazi ukuba lihlele phambili ("regurgitate") kwi-atrium yekhohlo.
I-mitral regurgement ebonwayo ne-HCM ayibangelwa yinkinga ye-valve yenhliziyo, kodwa kunoko, iveliswa yimpembelelo kwindlela iivumelwano ze-ventricle, ezibangelwa kukunyuka kwemisipha ye-ventricular. Ukubuyiswa kwe-Mitral yindlela enye apho abantu abane-HCM abanokuphuhliswa kwentliziyo.
4) I-HCM inokubangela i-ischemia yesisu senhliziyo. I-Ischemia - i-oxygen deprivation - ibonakala kakhulu kwizigulane ezine- coronary artery disease (CAD) , apho ukucinywa kwintsimbi ye-coronary kuphazamisa ukuhamba kwegazi kwindawo ethile yentliziyo yesisu. Nge-HCM, i-heart muscle inokuba yomelele kangangokuba ezinye iindawo zesisipha azifumani ukuhamba kwegazi okwaneleyo, nangona iimpawu ze-coronary ziqhelekile.
Xa oko kwenzeka, i- angina ingenzeka (ngokukodwa ngokuzikhandla), kunye ne- myocardial infarction (ukufa kwesisu senhliziyo) kunokwenzeka.
5) I-HCM ingabangela ukufa ngokukhawuleza. Ukufa ngokukhawuleza kwi-HCM kubangelwa ngenxa ye-tachycardia ye-ventricular okanye i- fibrillation ye-ventricular , kwaye ngokuqhelekileyo ihambelana nokuzikhandla okukhulu. Kusenokwenzeka ukuthi i-ischemia yentliziyo ye-muscle ivelisa amaninzi ukuba ayininzi i-arrhythmias ekhokelela ekufeni ngokukhawuleza kwizigulane ezine-HCM. Ngenxa yoko, ezininzi izigulane ezineHCM zifuna ukunciphisa umsebenzi wazo.
Iimpawu
Iimpawu ezibangelwa ngabantu abane-HCM ziyahluka. Kuqhelekileyo kwizigulane ezinezifo ezinzima ukuba zingenayo nayiphi na impawu. Nangona kunjalo, ukuba kukho nayiphi na ingxaki yentliziyo ekhankanywe apha, ubuncinane iimpawu ezithile. Iimpawu eziqhelekileyo ezifunyenwe zizigulane ezine-HCM zi-dyspnea kunye nokuzivocavoca, i- orthopnea , i-dyspnea yomsondeza ebusuku , i- palpitations , iziqulatho zesikhumba esiphathekayo , intlungu yesifuba, ukukhathala okanye ukuvuvukala kweenyawo. I-Syncope (ukulahlekelwa kwengqondo) kunoma ubani onokuHCM, ingakumbi ukuba inxulumene nokuzilolonga, yinto ebalulekileyo kakhulu, kwaye ingabonisa ingozi enkulu kakhulu yokufa ngokukhawuleza. Naliphina isiqhelo se syncope sifanele sihlolwe ngokukhawuleza ngugqirha.
Ukuxilongwa
Ngokubanzi, i- echocardiogram yindlela efanelekileyo yokufumanisa i-HCM. I-echocardiogram ivumela ukulinganisa okuchanekileyo kobunzima beendonga zomoya, kwaye iyakwazi ukubona i-LVOT kunye nokuphindaphinda kwe-mitral.
I- electrocardiogram (ECG) ingabonakalisa i-hypertrophy ye-ventricular hypertrophy, kwaye isetyenziswe njengesixhobo sokuhlola ukujonga i-HCM kubadlali abancinci.
Bobabini i-ECG kunye ne-echocardiogram kufuneka iqhutywe kwizihlobo eziseduze kwanoma ubani ofunyanwe ne-HCM, kunye ne-echocardiogram kufuneka yenziwe kunoma yimuphi umntu apho i-ECG okanye uvavanyo lwangaphakathi lubonisa ukuxhaswa kwe-ventricular hypertrophy.
Unyango
I-HCM ayikwazi ukuphiliswa, kodwa kwiimeko ezininzi ulawulo lwezokwelapha lunokulawula iimpawu kunye nokuphucula iziphumo zeklinikhi. Nangona kunjalo, ukulawulwa kwe-HCM kunokuba nzima kakhulu, kwaye nabani na abaneempawu ngenxa ye-HCM kufuneka ilandelwe ngumfundi we-cardiologist.
B block blockers kunye ne- calcium blockers banokunceda ukunciphisa "ukuqina" kwintliziyo yomzimba. Ukuphepha ukukhulelwa kwamanzi kubalulekile kwizigulane ezine-HCM ekunciphiseni iimpawu ezinxulumene ne-LVOT. Kwezinye izigulane zonyango ukususa iinxalenye zeentliziyo zomeleleyo ziyimfuneko ukukhupha i-LVOT.
I-fibrillation yase-Atrial, xa ivela, ihlala idala iimpawu ezinzima kunye neemfuno ezilawulwa ngokugqithiseleyo kwizigulane ezineHCM kunabantu bonke.
Ukuthintela Ukufa Ngokukhawuleza
I-HCM iyona nto ibangela ukufa ngokukhawuleza kuma-athlete amancinci Nangona ukufa ngokukhawuleza kuhlala kuyinkathazo eyingozi, kubaluleke kakhulu xa kwenzeka kubantu abatsha. Ngenxa yalesi sizathu, ukunyamezela ngokunyanisekileyo kunye nokukhuphisana komsebenzi kufuneka kukhutshwe kwizigulane ezineHCM.
Zininzi iindlela eziye zazama ukunciphisa umngcipheko wokufa ngokukhawuleza kwizigulane ezineHCM - kubandakanywa ukusebenzisa i-beta blockers kunye ne-calcium blockers, kunye neziyobisi ezichasayo . Nangona kunjalo, ezi ndlela azibonakalanga ngokufanelekileyo. Ngoku kubonakala kucacile ukuba, kwizigulane ezineHCM ezinobungozi bokufa ngokukhawuleza zibonakala ziphezulu, i- defibrillator enokugxininiswa kufuneka iqwalaselwe ngamandla.
Imithombo:
UMassie, uBom "Inkohlakalo Yentliziyo" e: Goldman L kunye noAusiello D (Eds). I-Cecil Book of Medicine, WB Saunders, 2003.
Nishimura RA, Holmes DR Jr. Ukwenziwa kwezonyango. I-cardiomyopathy ye-hypertrophic obstructive. N Engl J Med 2004; 350: 1320.
UGersh BJ, uMaron BJ, uBonow RO, et al. Ngo-2011 i-ACCF / i-AHA izikhokelo zokuxilongwa kunye nokunyangwa kwe-cardiomyopathy: i-summary management: ingxelo ye-American College of Cardiology Foundation / i-American Heart Association Task Force kwi-Practice Guidelines. Ukuhamba ngo-2011; 124: 2761.