I-amyloidosis yenhliziyo

I-amyloidosis yenhliziyo yisifo esidalwa ukufakwa kweprotheni engavamile (i-amyloid) ezicutshini zenhliziyo. Lezi zimali zenza kube nzima ukuthi inhliziyo isebenze kahle.

I-Amyloidosis yiqembu lezifo lapho ama-clumps amaprotheni abizwa ngama-amyloid akha khona ezicutshini zomzimba. Ngokuhamba kwesikhathi, lawa maprotheni angena esikhundleni sezicubu ezijwayelekile, okuholela ekwehlulekeni kwesitho esithintekayo. Kunezinhlobo eziningi ze-amyloidosis.

I-amyloidosis yenhliziyo ("i-stiff heart syndrome") ivela lapho amadiphozithi e-amyloid ethatha indawo yemisipha yenhliziyo ejwayelekile. Luhlobo olujwayelekile kakhulu lwe-cardiomyopathy evimbelayo. I-amyloidosis yenhliziyo ingathinta indlela amasiginali kagesi ahamba ngayo enhliziyweni (uhlelo lokuqhuba). Lokhu kungaholela ekushayeni kwenhliziyo okungajwayelekile (arrhythmias) nasezimpawu zesimo senhliziyo ezingalungile (block block).

Isimo singazuzwa njengefa. Lokhu kubizwa ngomndeni i-amyloidosis yenhliziyo. Ingakhula futhi njengomphumela wesinye isifo esifana nohlobo lomdlavuza wamathambo nowegazi, noma njengomphumela wenye inkinga yezokwelapha edala ukuvuvukala. I-amyloidosis yenhliziyo ivame kakhulu emadodeni kunabesifazane. Lesi sifo asivamile kubantu abangaphansi kweminyaka engama-40.

Abanye abantu kungenzeka bangabi nazimpawu. Uma zikhona, izimpawu zingafaka:

• Ukuchama ngokweqile ebusuku
• Ukukhathala, ukunciphisa ikhono lokuzivocavoca umzimba
• Ukushaya inhliziyo (ukuzwa kokushaya kwenhliziyo)
• Ukuphefumula ngomsebenzi
• Ukuvuvukala kwesisu, imilenze, amaqakala, noma enye ingxenye yomzimba
• Inkinga yokuphefumula ngenkathi ilele

Izimpawu ze-amyloidosis yenhliziyo zingahlotshaniswa nezimo eziningi ezahlukahlukene. Lokhu kungenza inkinga kube nzima ukuyithola.

Izimpawu zingafaka:

• Imisindo engajwayelekile emaphashini (ukuqhuma kwamaphaphu) noma ukububula kwenhliziyo
• Umfutho wegazi ophansi noma owehlayo uma usukuma
• Imithambo yentamo ekhulisiwe
• Sivuvukile isibindi

Ukuhlolwa okulandelayo kungenziwa:

• Isifuba noma isisu se-CT scan (kubhekwa "njengegolide elijwayelekile" ukusiza ukuxilonga lesi simo)
• I-Coronary angiography
• I-Electrocardiogram (ECG)
• I-Echocardiogram
• Ukucabanga kwe-Magnetic resonance (MRI)
• Ukuskena kwenhliziyo yenukliya (MUGA, RNV)
• IPositron emission tomography (PET)

I-ECG ingakhombisa izinkinga ngokushaya kwenhliziyo noma ngezimpawu zenhliziyo. Kungakhombisa nezimpawu eziphansi (ezibizwa nge- "voltage low").

I-biopsy yenhliziyo isetshenziselwa ukuqinisekisa ukuxilongwa. I-biopsy yenye indawo, njengesisu, izinso, noma umnkantsha wamathambo, ivame ukwenziwa futhi.

Umhlinzeki wakho wezokunakekelwa kwempilo angakutshela ukuthi wenze izinguquko ekudleni kwakho, kufaka phakathi ukunciphisa usawoti noketshezi.

Ungahle udinge ukuthatha amaphilisi amanzi (isisu) ukusiza umzimba wakho ukuqeda uketshezi oluningi. Umhlinzeki angakutshela ukuthi uzilinganise nsuku zonke. Ukuzuza kwesisindo samakhilogremu ama-3 noma ngaphezulu (ikhilogremu elilodwa noma ngaphezulu) ngaphezulu kwezinsuku ezi-1 kuye kwezi-2 kungasho ukuthi kunoketshezi oluningi emzimbeni.

Imithi efaka i-digoxin, i-calcium-channel blockers, ne-beta-blockers ingasetshenziswa kubantu abane-atrial fibrillation. Kodwa-ke, izidakamizwa kufanele zisetshenziswe ngokuqapha, futhi umthamo kumele ubhekwe ngokucophelela. Abantu abane-amyloidosis yenhliziyo bangazwela ngokwengeziwe kwimiphumela emibi yale mithi.

Ezinye izindlela zokwelashwa zingafaka:

• Ukwelashwa ngamakhemikhali
• I-cardioverter-defibrillator engatshala (AICD)
• I-Pacemaker, uma kunezinkinga ngezimpawu zenhliziyo
• I-Prednisone, umuthi olwa nokuvuvukala

Ukufakelwa kwenhliziyo kungacatshangelwa kubantu abanezinhlobo ezithile ze-amyloidosis abanenkinga yokusebenza kwenhliziyo embi kakhulu. Abantu abane-amyloidosis yefa bangadinga ukufakelwa kwesibindi.

Esikhathini esedlule, i-cardiac amyloidosis bekucatshangwa ukuthi yisifo esingelapheki nesibulala ngokushesha. Noma kunjalo, insimu ishintsha ngokushesha. Izinhlobo ezahlukene ze-amyloidosis zingathinta inhliziyo ngezindlela ezihlukile. Ezinye izinhlobo zinzima kakhulu kunezinye. Abantu abaningi manje bangalindela ukusinda futhi bathole ikhwalithi enhle yokuphila iminyaka eminingana ngemuva kokuxilongwa.

Izinkinga zingafaka:

• I-Atrial fibrillation noma i-ventricular arrhythmias
• Ukwehluleka kwenhliziyo yokubopha
• Ukwakhela uketshezi esiswini (ascites)
• Ukuzwela okwandisiwe ku-digoxin
• Umfutho wegazi ophansi nesiyezi kusuka ekuchameni ngokweqile (ngenxa yemithi)
• Isifo se-sinus syndrome
• Isifo sohlelo lokuqhutshwa kwenhliziyo (i-arrhythmias ehlobene nokuqhutshwa okungavamile kwemithambo ngemisipha yenhliziyo)

Shayela umhlinzeki wakho uma unalesi sifo futhi uthuthukise izimpawu ezintsha njenge:

• Isizungu lapho ushintsha isikhundla
• Ukuzuza ngokweqile (uketshezi) ukuzuza
• Ukwehla kwesisindo ngokweqile
• Ukuphelelwa amandla
• Izinkinga zokuphefumula ezinzima

I-Amyloidosis - inhliziyo; I-amyloidosis yenhliziyo eyinhloko - uhlobo lwe-AL; I-amyloidosis yenhliziyo yesibili - uhlobo lwe-AA; I-Stiff heart syndrome; Isenile amyloidosis

• Inhliziyo - isigaba phakathi nendawo
• Ukuqina kwenhliziyo
• Ipayipi le-biopsy

UFalk RH, uHershberger RE. Ama-cardiomyopathies avulekile, abekelwe imingcele, futhi angena ngaphakathi. Ku: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, ama-eds. Isifo Senhliziyo SikaBraunwald: Incwadi Yemithi Yezinhliziyo Nemithambo. Umhla ka-11. IPhiladelphia, PA: Elsevier; 2019: isahluko 77.

UMcKenna WJ, u-Elliott PM. Izifo ze-myocardium ne-endocardium. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 54.