I-Tricuspid atresia

I-Tricuspid atresia wuhlobo lwesifo senhliziyo esikhona lapho kuzalwa (isifo senhliziyo esibelethwe), lapho i-valve yenhliziyo ye-tricuspid ilahlekile noma ithuthukiswe ngokungajwayelekile. Iphutha livimba ukugeleza kwegazi kusuka ku-atrium engakwesokudla kuya ku-ventricle efanele. Okunye ukukhubazeka kwenhliziyo noma kwesitsha kuvame ukuba khona ngasikhathi sinye.

I-Tricuspid atresia iyindlela engajwayelekile yesifo senhliziyo esizelwe. Kuthinta cishe ama-5 kokuzalwa okuzenzakalelayo okuyi-100,000. Umuntu oyedwa kwabahlanu onalesi sifo naye uzoba nezinye izinkinga zenhliziyo.

Imvamisa, igazi ligeleza lisuke emzimbeni liye ku-atrium elungile, bese lidlula ku-valve enetricuspid iye ku-ventricle efanele bese iya emaphashini. Uma i-valve ye-tricuspid ingavuli, igazi alikwazi ukugeleza lisuka ku-atrium elifanele liye ku-ventricle efanele. Ngenxa yenkinga nge-tricuspid valve, ekugcineni igazi alikwazi ukungena emaphashini. Yilapho kufanele iye khona ukuyolanda i-oxygen (iba oxygenated).

Esikhundleni salokho, igazi lidlula emgodini ophakathi kwe-atrium yangakwesokudla nangakwesobunxele. Ku-atrium yangakwesobunxele, ixubeka negazi elicebile le-oxygen elibuya emaphashini. Le nhlanganisela yegazi elicebile nge-oxygen ne-oxygen engenampilo iyakhishwa emzimbeni isuke ku-ventricle yesobunxele. Lokhu kubangela ukuthi izinga le-oxygen egazini libe ngaphansi kunokujwayelekile.

Kubantu abane-tricuspid atresia, amaphaphu athola igazi ngomgodi ophakathi kwama-ventricles angakwesokudla nangakwesobunxele (okuchazwe ngenhla), noma ngokulondolozwa komkhumbi wombungu obizwa nge-ductus arteriosus. I-ductus arteriosus ixhuma umthambo we-pulmonary (umthambo emaphashini) ne-aorta (umthambo oyinhloko emzimbeni). Sikhona lapho kuzalwa ingane, kepha ngokujwayelekile sizivalela sodwa ngemuva nje kokuzalwa.

Izimpawu zifaka:

• Umbala we-Bluish esikhumbeni (i-cyanosis) ngenxa yezinga eliphansi le-oxygen egazini
• Ukuphefumula ngokushesha
• Ukukhathala
• Ukukhula okubi
• Ukuphelelwa umoya

Lesi simo singatholakala ngesikhathi sokucabanga kwe-ultrasound ngaphambi kokubeletha noma lapho ingane ihlolwa ngemuva kokuzalwa. Isikhumba se-Bluish sikhona lapho kuzalwa. Ukububula kwenhliziyo kuvame ukubakhona lapho kuzalwa futhi kungakhuphuka ngokuzwakalayo ngaphezu kwezinyanga ezimbalwa.

Ukuhlolwa kungafaka okulandelayo:

• ECG
• I-Echocardiogram
• I-x-ray yesifuba
• I-catheterization yenhliziyo
• I-MRI yenhliziyo
• Ukuhlolwa kwe-CT kwenhliziyo

Lapho kutholakala ukuxilongwa, ingane izovunyelwa ukuvunyelwa egumbini lokunakekelwa kwabasanda kuzalwa (NICU). Umuthi obizwa nge-prostaglandin E1 ungasetshenziswa ukugcina i-ductus arteriosis ivulekile ukuze igazi ligijime liye emaphashini.

Ngokuvamile, iziguli ezikulesi simo zidinga ukuhlinzwa. Uma inhliziyo ingakwazi ukupompa igazi elanele liye emaphashini nasemzimbeni wonke, ukuhlinzwa kokuqala kuvame ukwenzeka ezinsukwini ezimbalwa zokuqala zokuphila. Kule nqubo, kufakwa i-shunt yokufakelwa ukugcina igazi ligelezela emaphashini. Kwezinye izimo, lokhu kuhlinzwa kokuqala akudingeki.

Ngemuva kwalokho, ingane iya ekhaya ezimweni eziningi. Ingane izodinga ukuthatha umuthi owodwa noma eminingi yansuku zonke futhi ilandelwe eduze nodokotela wenhliziyo wabantwana. Lo dokotela uzonquma ukuthi isigaba sesibili sokuhlinzwa kufanele senziwe nini.

Isigaba esilandelayo sokuhlinzwa sibizwa ngokuthi inqubo kaGlenn shunt noma inqubo ye-hemi-Fontan. Le nqubo ixhuma uhhafu wemithambo ethwala igazi elingenayo i-oxygen kusuka engxenyeni engenhla yomzimba ngqo emthanjeni wamaphaphu. Ukuhlinzwa kuvame ukwenziwa lapho ingane iphakathi kwezinyanga ezine kuya kwezingu-6 ubudala.

Ngesikhathi sesigaba I no-II, ingane isengabukeka iluhlaza okwesibhakabhaka (i-cyanotic).

Isigaba III, isinyathelo sokugcina, sibizwa ngokuthi inqubo ye-Fontan. Eminye imithambo ethwala igazi elingenayo i-oxygen emzimbeni isuke ixhunywe ngqo emthanjeni wamaphaphu oholela emaphashini. I-ventricle yangakwesobunxele manje kufanele ipompe emzimbeni kuphela, hhayi amaphaphu. Lokhu kuhlinzwa kuvame ukwenziwa lapho ingane inezinyanga eziyi-18 kuye eminyakeni emithathu. Ngemuva kwalesi sinyathelo sokugcina, isikhumba sengane asisahlaza okwesibhakabhaka.

Ezimweni eziningi, ukuhlinzwa kuzothuthukisa isimo.

Izinkinga zingafaka:

• Isigqi senhliziyo esingajwayelekile, esisheshayo (arrhythmias)
• Isifo sohudo esingapheli (esivela kwisifo esibizwa nge-protein-losing enteropathy)
• Ukwehluleka kwenhliziyo
• Uketshezi esiswini (ascites) nasemaphashini (i-pleural effusion)
• Ukuvalwa kwe-shunt yokufakelwa
• Ukushaywa unhlangothi nezinye izinkinga zesistimu yezinzwa
• Ukufa okungazelelwe

Xhumana nomhlinzeki wakho wezempilo ngokushesha uma usana lwakho lune:

• Izinguquko ezintsha emaphethini okuphefumula
• Izinkinga zokudla
• Isikhumba esiphenduka siluhlaza okwesibhakabhaka

Ayikho indlela eyaziwayo yokuvimbela i-tricuspid atresia.

Tri atresia; I-Valve disorder - i-tricuspid atresia; Inhliziyo ezelwe - tricuspid atresia; Isifo senhliziyo seCyanotic - tricuspid atresia

• Inhliziyo - isigaba phakathi nendawo
• I-Tricuspid atresia

I-Fraser CD, uKane LC. Isifo senhliziyo esizalwa naso. Ku: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. ISabiston Incwadi Yokuhlinzwa. Umhla ka-20. IPhiladelphia, PA: Elsevier; 2017: isahluko 58.

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.