Isizathu sokufa ngokukhawuleza kubadlali abancinci
I-Arrhythmogenic ye-ventricular cardiomyopathy (AVRC) yimizimba yezofuzo apho i-muscle ye-cardiaac ishintshintshwa ibe yindawo enomzimba onamafutha, ikakhulukazi kwi-ventricle efanelekileyo. Isizathu esibalulekileyo sokuba i-AVRC ibalulekileyo kukuba inokuvelisa i- arrhythmias yengozi yengozi. (AVRC igama "elitsha" le meko, elalibizwa ngokuthi "i-arrhymmogenic ventricular dysplasia.")
I-AVRC ayiqhelekanga, kodwa ayinqabile. Inokufumaneka kwisinye se-2000 ukuya kuma-5000 abadala ukuba uyayibheka. Nangona kunjalo, ixesha eliqhelekileyo uluntu ngokubanzi eliva malunga ne-AVRC kukuba xa umdlali omncinci efa ngokukhawuleza, ekubeni i-AVRC yenye yeemeko zengqondo ezinxulumene nokufa ngokukhawuleza kubadlali abancinci .
Ziziphi iimpawu ze-AVRC?
Ngelixa i-AVRC yintliziyo ye-cardiomyopathy-oko kukuthi, isifo sesisu senhliziyo - asiqhelanga nje ukuba iingxaki zeengcambu ezaneleyo ukuvelisa ukungaphumeleli kwentliziyo . Kunoko, ukubaluleka kwekliniki kukuba kungabangela i-cardiac arrhythmias - ngokukodwa, i- ventricular complexes , i- tricycardia ye-ventric , kunye namaxesha athile ama- fibrillation .
Iimpawu ezibangelwa yi-AVRC zivame ukuhambelana nama-arrhythmias anokuvelisa. Abantu abane-AVRC baya kubonisa ngokuqhelekileyo iipasiti zeepilpitations , i-headhead, okanye i- syncope . Ngelishwa, ukufa ngokukhawuleza kuya kwenzeka, kwaye nangona ngelanga, ukufa ngokukhawuleza kunokuba ngumqondiso wokuqala wokuba kukho nayiphi na ingxaki yenhliziyo.
Nangona i-AVRC ibangela ukufa ngokukhawuleza kunokwenzeka nanini na, lesi siganeko siyakwenzeka ngexesha lweziganeko zokuzikhandla. Yingakho i-AVRC yenye yeemeko ezivelisa ukufa ngokukhawuleza ngokubonakala ngokusemgangathweni, abadlali abanomdla.
Abantu abaninzi abane-AVRC - ukuya kwi-40% - abayi kuba neyodwa impawu, kwaye bafunyaniswa kuphela xa behlolwe ingxaki ngenxa yokuba ilungu lentsapho lifumaniswe ukuba nalo.
I-AVRC ichongwa njani?
Ukufumanisa i-AVRC ifezekiswa ngokuhlola i- electrocardiogram (edla ngokubonisa ukucwangciswa okukodwa kwe-QRS complex), kunye ne- echocardiogram (esoloko ibonisa ukungaqhelekanga kwimizimba yenhliziyo ye-ventricle efanelekileyo - kunye ngamanye ama-ventricle asekhohlo).
Ukuba ukuxilongwa kukuhlala kungathandabuzeki, ngamanye amaxesha i- MRI yentswelo inokukunceda ukuncedisa izinto. Ukuvavanya i-genetic kunokuba luncedo ekwenzeni ukuxilongwa, kwaye kunconywa kubo bonke abantu abaneli meko.
Nangona uvavanyo lwe-electrophysiologic lunokuthi luncedo ngokukhawuleza ekubonakaliseni i-tachycardia ye-ventricular ngenxa ye-AVRC kwi-tachycardia ye-ventricy ebangelwa ezinye iimeko zentliziyo, olo vavanyo aluncedo rhoqo, kwaye alufaneki.
Emva kokuxilongwa, ukuhlolwa kwezofuzo kuyaphakanyiswa ukuba izihlobo ezide. Malunga neyesithathu kwiidanga ezintathu zokuqala zomntu one-AVRC uya kugqiba ekuhlaleni le meko.
Ukunyanga i-AVRC
Injongo ephambili ekunakekeleni i-AVRC kukukhusela ukufa kwegazi ngokukhawuleza kwi-tachycardia ye-ventricular okanye i-fibrillation.
Ngenxa yokuba ukufa ngokukhawuleza kudla ngokudibanisa nokusebenza kulo mqathango, abadlali abavame ukuba ne-AVRC bafanele babalekele kwimidlalo yonke yokukhuphisana, kunye neentlobo ezibonakalayo ezinobunzima bezinto ezifana negalufu okanye isithsaba.
Ngaphezulu, kufuneka babalekele nayiphi na imisebenzi eyenza ukuba iipelpitations zibalulekileyo.
Ama-arhythmias anxulumene ne-AVRC avela ukuba aveliswe ngovuselelo oluvakalayo-inxalenye yenkqubo ye- nerveous autonomic eyandisa amazinga e-adrenaline, kwaye ixanduva lokulwa okanye ukuphendula kwendiza. Yingakho ukuziqhelanisa kuyinkinga nge-AVRC. Ngoko ke, abaninzi beeprodologists batusa ukusetyenziswa kwe- blocka blockers kule mqathango, ukuphazamisa umphumo we-adrenaline entliziyweni.
Abantu abaxhamlileyo baxhaswa ngokunyanisekileyo kubantu abaninzi abane-AVRC, ngokukodwa, nakubani na oye wafumana isiganeko sokubanjwa kwintliziyo, isiqwenga se-tachycardia esesigxina, isiqhelo se-syncope engachazwanga, okanye i-echocardiogram ibonisa ukubandakanyeka okukhulu kwintliziyo ye-heart.
Kuba abantu abane-AVRC abangenayo le miqathango, umngcipheko wokufa ngokukhawuleza kubonakala ubonakala uphantsi - xa nje balandela imimiselo yokusebenza kwaye bathathe i-blockers yabo.
Kubantu abano-AVRC abaye baxhasa ama-arrhythmias, i-long-term prognosis ibonakala ibonakala kakuhle ukuba bayakuphepha ukuzivocavoca, bathathe i-beta blockers, bafumane i-defibrillator, kwaye (kwezinye iimeko) bathathe i- antiarrthymic drug .
ILizwi
I-Arrhythmogenic ye-ventricular cardiomyopathy yimizimba yezofuzo ezinokuthi zivelise i-arrhythmias yokubulala i-cardiac arrhythmias, kwaye enye yezona zinto ezibangela ukufa kwabantu ngokukhawuleza kubadlali abancinci. Ngonyango olunzima, abantu abaneli meko bahlala benza kakuhle.
> Imithombo:
> Al-Khatib SM, uStevenson WG, uAckerman MJ, et al. 2017 Isikhokelo se-AHA / ACC / i-HRS yokuLawula iziguli ezine-Arrhythmias ze-Ventricular kunye nokuKhuselwa koKhuseleko lweCaracac ngokukhawuleza: Isishwankathelo seSigqeba: Ingxelo ye-American College of Cardiology / American Heart Association Task Force kwi-Clinical Practice Guidelines kunye ne-Heart Rhythm Society. Isibilini senhliziyo 2017.
> Ruwald AC, Marcus F, Estes NA 3rd, et al. Umbutho Wokhuphiswano kunye neMidlalo Yokuzonwabisa Ukuthatha Inxaxheba Ngeziganeko Zengqondo Kwizigulane Nge-Arrhythmogenic I-Ventricular Cardiomyopathy: Iziphumo Ezivela Kwi-North American Multidisciplinary Study of Arrhythmogenic Right Ventricular Cardiomyopathy. I-Eur Heart J 2015; 36: 1735.