I-Ebstein anomaly

I-Ebstein anomaly ukukhubazeka kwenhliziyo okungavamile lapho izingxenye ze-valve ye-tricuspid zingavamile. I-valve engu-tricuspid yehlukanisa ikamelo elingezansi lezinhliziyo (i-ventricle yangakwesokudla) negumbi eliphezulu lenhliziyo (i-atrium yangakwesokudla). Ku-Ebstein anomaly, ukubekwa kwe-valve ye-tricuspid nokuthi isebenza kanjani ukuhlukanisa amakamelo womabili akujwayelekile.

Isimo singesokuzalwa, okusho ukuthi sikhona ngesikhathi sokuzalwa.

I-tricuspid valve imvamisa yenziwe ngezingxenye ezintathu, ezibizwa ngokuthi amapheshana noma amacwecwe. Amapheshana avulekile ukuvumela igazi ukuba lisuke e-atrium yangakwesokudla (ikamelo eliphezulu) liye ku-ventricle efanele (ikamelo elingezansi) ngenkathi inhliziyo isakhululekile. Bavala ukuvimbela igazi ekuhambeni kusuka ku-ventricle efanele liye e-atrium efanele ngenkathi inhliziyo ipompa.

Kubantu abane-Ebstein anomaly, amapheshana afakwa ajule kwi-ventricle efanele esikhundleni sesikhundla esijwayelekile. Amapheshana ngokuvamile makhulu kunokujwayelekile. Ukukhubazeka kuvame ukudala ukuthi i-valve ingasebenzi kahle, futhi igazi lingahamba ngendlela engafanele. Esikhundleni sokugelezela emaphashini, igazi ligeleza libuyele e-atrium efanele. Ukulondolozwa kokugeleza kwegazi kungaholela ekwandisweni kwenhliziyo nokwakheka kuketshezi emzimbeni. Kungase kube nokuncipha kwe-valve eholela emaphashini (i-valve yamaphaphu).

Ezimweni eziningi, abantu futhi banembobo odongeni ehlukanisa amakamelo amabili aphezulu enhliziyo (i-atrial septal defect) nokugeleza kwegazi kulo mgodi kungadala ukuthi igazi elingenayo i-oxygen liye emzimbeni. Lokhu kungadala i-cyanosis, okuluhlaza okwesibhakabhaka esikhunjeni okubangelwa igazi elingenayo i-oxygen.

I-Ebstein anomaly yenzeka njengoba ingane ikhula esibelethweni. Imbangela ngqo ayikaziwa. Ukusetshenziswa kwemithi ethile (njenge-lithium noma i-benzodiazepines) ngesikhathi sokukhulelwa kungadlala indima. Isimo asivamile. Kuvame kakhulu kubantu abamhlophe.

Ukungajwayelekile kungaba kuncane noma kube nzima kakhulu. Ngakho-ke, izimpawu zingabuye zihluke kusukela kumnene kuze kube nzima kakhulu. Izimpawu zingakhula ngemuva nje kokuzalwa, futhi zingafaka izindebe nezinzipho ezinombala oluhlaza okwesibhakabhaka ngenxa yamazinga aphansi e-oxygen egazini. Ezimweni ezinzima, ingane ibonakala igula kakhulu futhi inenkinga yokuphefumula. Ezimweni ezimnene, umuntu othintekile angabonakaliswa iminyaka eminingi, kwesinye isikhathi abe unomphela.

Izimpawu ezinganeni ezindala zingabandakanya:

• Khwehlela
• Ukwehluleka ukukhula
• Ukukhathala
• Ukuphefumula ngokushesha
• Ukuphelelwa umoya
• Ukushaya kwenhliziyo okusheshayo

Izingane ezisanda kuzalwa ezinokuvuza okukhulu kuvalse ye-tricuspid zizoba nezinga eliphansi kakhulu lomoya-mpilo egazini lazo kanye nokwandiswa kwenhliziyo okuphawulekayo. Umhlinzeki wezokunakekelwa kwempilo angezwa imisindo yenhliziyo engajwayelekile, njengokukhononda, lapho elalele isifuba nge-stethoscope.

Ukuhlolwa okungasiza ukuxilonga lesi simo kufaka:

• I-x-ray yesifuba
• I-Magnetic resonance imaging (MRI) yenhliziyo
• Ukulinganiswa komsebenzi kagesi wenhliziyo (ECG)
• I-Ultrasound yenhliziyo (i-echocardiogram)

Ukwelashwa kuya ngobukhulu besici kanye nezimpawu ezithile. Ukunakekelwa kwezokwelapha kungafaka:

• Imithi yokusiza ngokuhluleka kwenhliziyo, njenge-diuretics.
• Oxygen nokunye ukwesekwa kokuphefumula.
• Ukuhlinzwa ukulungisa i-valve.
• Ukufaka esikhundleni se-tricuspid valve. Lokhu kungadingeka ezinganeni eziqhubeka nokuba zimbi noma ezinezinkinga ezinkulu kakhulu.

Ngokuvamile, izimpawu zokuqala ziyakhula, isifo siba sibi ngokwengeziwe.

Abanye abantu kungenzeka bangabi nazimpawu noma izimpawu ezincane kakhulu. Abanye bangase babe babi ngokuhamba kwesikhathi, bakhulise umbala ohlaza okwesibhakabhaka (i-cyanosis), ukwehluleka kwenhliziyo, ukuvimba kwenhliziyo, noma isigqi senhliziyo esiyingozi.

Ukuvuza okukhulu kungaholela ekuvuvukaleni kwenhliziyo nesibindi, kanye nokwehluleka kwenhliziyo ukuminyana.

Ezinye izinkinga zingafaka:

• Isigqi senhliziyo esingajwayelekile (arrhythmias), kufaka phakathi isigqi esisheshayo ngokungajwayelekile (tachyarrhythmias) kanye nesigqi esisheshayo ngokungajwayelekile (bradyarrhythmias kanye nenhliziyo block)
• Igazi liyaqina kusuka enhliziyweni liye kwezinye izitho zomzimba
• Ithumba lobuchopho

Shayela umhlinzeki wakho uma ingane yakho iqala izimpawu zalesi simo. Thola usizo lwezokwelapha ngokushesha uma kuvela izinkinga zokuphefumula.

Akukho ukuvimbela okwaziwayo, ngaphandle kokukhuluma nomhlinzeki wakho ngaphambi kokukhulelwa uma uthatha imithi okucatshangwa ukuthi ihlobene nokwakha lesi sifo. Ungakwazi ukuvimbela ezinye zezinkinga zesifo. Isibonelo, ukuthatha ama-antibiotic ngaphambi kokuhlinzwa kwamazinyo kungasiza ekuvimbeleni i-endocarditis.

Okungahambi kahle kuka-Ebstein; Ukungalungi kuka-Ebstein; Ukukhubazeka kwenhliziyo okuzalwa - u-Ebstein; Inhliziyo yokukhubazeka kokuzalwa - u-Ebstein; Isifo senhliziyo seCyanotic - Ebstein

• Okungahambi kahle kuka-Ebstein

UBhatt AB, Foster E, Kuehl K, et al. Isifo senhliziyo esizelwe kumuntu omdala osekhulile: isitatimende sesayensi esivela ku-American Heart Association. Ukujikeleza. 2015; 131 (21): 1884-1931. I-PMID: 25896865 pubmed.ncbi.nlm.nih.gov/25896865/.

IKliegman RM, iSt. Geme JW, iBlum NJ, i-Shah SS, iTasker RC, iWilson KM. Izilonda zenhliziyo ezi-Cyanotic: izilonda ezihambisana nokwehla kwegazi le-pulmonary. Ku: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Incwadi kaNelson Yezingane. Umhlaka 21. IPhiladelphia, PA: Elsevier; 2020: isahluko 457.

UStout KK, uDaniels CJ, u-Aboulhosn JA, et al. Umhlahlandlela we-2018 AHA / ACC wokuphathwa kwabantu abadala abanezifo zenhliziyo ezelwe: umbiko we-American College of Cardiology / American Heart Association Task Force on Guidelines Practice Guidelines. Ukujikeleza. 2019; 139: e698-e800. I-PMID: 30121239 pubmed.ncbi.nlm.nih.gov/30121239/.

I-Webb GD, iSmallhorn JF, uTherrien J, uRedington AN. Isifo senhliziyo esizalwa naso esigulini esidala nesingane. 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 75.