Ukuthi yini i-Hypoplastic Left Heart Syndrome? Izimpawu, Izimbangela, kanye Nokwelashwa

I-Hypoplastic left heart syndrome (HLHS) iyisimo esingavamile kodwa esibucayi senhliziyo esizalwa neso. Kulesi simo, ihlangothi elingakwesobunxele lenhliziyo alikhuli kahle ngesikhathi sokukhulelwa, okwenza lingakwazi ukupompa igazi kahle emzimbeni.

Lokhu kusho ukuthi inhliziyo yengane yakho ayikwazi ukwenza umsebenzi wayo obaluleke kakhulu wokuthumela igazi eliphezulu ngokusika emzimbeni wayo. Nakuba lokhu kuzwakala kukhulu kakhulu, ngifuna ukwazi ukuthi amaqembu ezempilo akhiqize izindlela zokwelapha eziphumelelayo ezisiza abantwana abaningi abane-HLHS ukuba baphile impilo ezele, ephambili.

Ukuthi yini i-Hypoplastic Left Heart Syndrome?

I-Hypoplastic left heart syndrome kwenzeka lapho ihlangothi elingakwesobunxele lenhliziyo yengane lingathuthuki kahle. Igama elithi "hypoplastic" lisho nje ukuthi "alingathuthuki kahle" noma "lincane kakhulu."

Enhliziyweni enempilo, ihlangothi elingakwesobunxele lipompa igazi eliphezulu ngokusika emzimbeni wonke. Uma kwenzeka i-HLHS, izingxenye eziningi zenhliziyo elingakwesobunxele ziyaphulukana, zincinyane kakhulu, noma azisebenzi kahle. Lokhu kufaka i-left ventricle (igumbi elikhulu lokupompa lenhliziyo), i-mitral valve, i-aortic valve, futhi ngezinye izikhathi i-aorta ngokwayo.

Ngaphandle kwenhliziyo elingakwesobunxele esebenzayo, inhliziyo yengane yakho ithembela ngokuphelele ehlangothini elingakwesokudla ukwenza umsebenzi kabili. Lokhu kudala inselele ngokushesha edinga ukunakekelwa kwezokwelapha ngokushesha ngemuva kokuzalwa.

Ziyini izimpawu ze-Hypoplastic Left Heart Syndrome?

Izimpawu ze-HLHS zivame ukuvela emahoreni ambalwa noma ezinsukwini ezimbalwa ngemuva kokuzalwa. Lezi zimpawu zenzeka ngoba umzimba wengane yakho awutholi igazi eliphezulu ngokwanele ngokusika.

Nawa zimpawu ezivame kakhulu ongaziqaphela:

• Umbala wesikhumba oluhlaza okwesibhakabhaka noma oluhlaza okumnyama, ikakhulukazi eduze kwemilomo, iinzipho, noma iinzipho zezinyawo
• Ubunzima bokuqeda umoya noma ukuqeda umoya ngokushesha
• Ukudla okubi noma ukwenqaba ukudla
• Ukukhathala okukhulu noma ukulala
• I-pulse ebuthakathaka noma izinga lenhliziyo eliphakeme
• Izandla neenyawo ezibandayo
• Ukukhipha ukushisa okukhulu ngesikhathi sokudla

Lezi zimpawu zingathuthuka ngokushesha futhi zingaba zimbi kakhulu njengoba ukuxhumana kwemvelo kwenhliziyo okusiza izingane ukuba ziphile esizalweni kuqala ukuvala. Uma uqaphela noma yiziphi zalezi zimpawu, funa ukunakekelwa kwezokwelapha ngokushesha.

Yini ebangela i-Hypoplastic Left Heart Syndrome?

I-HLHS ithuthuka phakathi kwamasonto ayisishiyagalombili okuqala okukhulelwa lapho inhliziyo yengane yakho ikhahlela. Imbangela eqondile ayiqondakali ngokuphelele, kodwa akuyona into owenzile noma awenzanga ngesikhathi sokukhulelwa.

Izimo eziningi ze-HLHS zenzeka ngokungahleliwe ngaphandle komlando womndeni wezinkinga zenhliziyo. Noma kunjalo, i-genetics ingadlala indima encane emindenini eminye. Uma une-HLHS enye, kunethuba elikhulu kancane (cishe i-2-3%) lokuthi abantwana bakusasa bangaba nalezi zimo noma ezinye izimo zenhliziyo.

Izici zemvelo ngesikhathi sokukhulelwa kwasekuqaleni zingase zinikeze, kodwa abacwaningi basafunda ngalezi zixhumanisi. Okubaluleke kakhulu ukuqonda ukuthi i-HLHS ayikwazi ukuphepheka, futhi akuyona iphutha lakho.

Nini okufanele ubona udokotela nge-Hypoplastic Left Heart Syndrome?

I-HLHS ivame ukutholwa ngesikhathi se-ultrasounds eziqondile zokukhulelwa, ngokuvamile phakathi kwamasonto angu-18-24 okukhulelwa. Uma udokotela wakho ethola i-HLHS ngaphambi kokuzalwa, uzoqamba uhlelo oluqondile lokulethwa nokunakekelwa ngokushesha.

Kodwa-ke, uma i-HLHS ingatholakali ngaphambi kokuzalwa, kufanele ufuna ukunakekelwa kwezokwelapha okusheshayo uma ingane yakho esanda kuzalwa ibonisa noma yiziphi izimpawu ezinkulu. Lokhu kufaka umbala wesikhumba oluhlaza okwesibhakabhaka noma oluhlaza okumnyama, ubunzima bokuqeda umoya, ukudla okubi, noma ukulala okukhulu.

Ngisho noma awuqiniseki ukuthi kukhona okuphosakele, themba izinzwa zakho. Kuhlale kungcono ukuba ochwepheshe bezempilo bahlole ingane yakho uma ukhathazekile ngokuphefumula, ukudla, noma amazinga amandla.

Ziyini izici ezingozi ze-Hypoplastic Left Heart Syndrome?

Ngokungafani nezinye izimo zezempilo, i-HLHS ayinazo izici ezingozi ezicacile ezikhuphula amathuba akho okuba nengane ethintekayo. Izimo eziningi zenzeka ngokungahleliwe emindenini engenayo umlando wezinkinga zenhliziyo.

Kodwa-ke, kunezici ezimbalwa ezingase zikhuphule kancane amathuba:

• Ukuba nomlando womndeni we-HLHS noma ezinye izifo zenhliziyo ezingavamile
• Izimo ezithile ze-genetic, nakuba lezi zingavamile
• Ukukhulelwa indodana (i-HLHS ithinta abafana kancane kunamantombazane)

Kubalulekile ukukhumbula ukuthi lezi zici azisho ukuthi i-HLHS izoqala ngokuqinisekile. Izingane eziningi ezinalezi zici ezingozi zizalwa zineenhliziyo ezempilo, futhi izingane eziningi ezinale-HLHS azinazo lezi zici ezingozi.

Ziyini izinhlanganisela ezingaba khona ze-Hypoplastic Left Heart Syndrome?

Ngaphandle kokwelashwa, i-HLHS iyisongo elikhulu empilweni phakathi kwezinsuku ezimbalwa noma amasonto okuqala okuphila. Ngisho nokwelashwa, kungaba nezinselele eziqhubekayo iqembu lakho lezokwelapha lizokusiza ukuthi uziqonde.

Izinhlanganisela ezingaba khona zingafaka:

• Izinkinga zokulingana kwenhliziyo (arrhythmias)
• I-blood clots engaya kwezinye izingxenye zomzimba
• Ukunciphisa ukubekezelela komzimba njengoba ingane yakho ikhula
• I-stroke, nakuba lokhu kungavamile ngokunakekelwa kwezokwelapha okufanele
• Izinkinga zezinso noma isibindi ezivela ekunciphiseni ukugcwala kwegazi
• Ukulibaziseka kokuthuthuka, nakuba izingane eziningi zithuthuka ngokujwayelekile
• Ukudinga ukuhlinzwa okwengeziwe kwenhliziyo kamuva ekuphileni

Nakuba lo luhlu lungase luzwakale lukhulu, khumbula ukuthi iqembu lezokwelapha lengane yakho liqeqeshwe ekuphepheni nasekuphatheni lezi zinhlanganisela. Izingane eziningi ezinale-HLHS ziya esikoleni, zidlala imidlalo, futhi zihlanganyela emisebenzini evamile yobunye.

I-Hypoplastic Left Heart Syndrome ihlonishwa kanjani?

I-HLHS ivame ukuhlonishwa ngaphambi kokuzalwa ngesikhathi se-ultrasounds eziqondile zokukhulelwa. Udokotela wakho angabona ubukhulu benhliziyo futhi aqonde lapho ihlangothi elingakwesobunxele lingathuthuki kahle.

Uma i-HLHS ingatholakali ngaphambi kokuzalwa, odokotela bangayihlola ngemuva kokuzalwa besebenzisa ukuhlolwa okuningi. Lokhu kufaka i-echocardiogram (i-ultrasound yenhliziyo), ebonisa izithombe eziningi zobukhulu nomsebenzi wenhliziyo yengane yakho.

Ukuhlolwa okwengeziwe kungafaka i-X-rays yesifuba, i-electrocardiograms (EKGs), ne-pulse oximetry ukulinganisa amazinga oksijini egazini lengane yakho. Lokhu kuhlolwa kusiza odokotela ukuthi baqonde ukuthi inhliziyo yengane yakho isebenza kanjani futhi bahlele indlela yokwelapha engcono kakhulu.

Ukwelashwa kwe-Hypoplastic Left Heart Syndrome yini?

Ukwelashwa kwe-HLHS kuhilela uchungechunge lokuhlinzwa okuthethoniwe okukodwa, okuvame ukubizwa nge-Norwood procedure pathway. Lokhu kuhlinzwa kusiza ukuqondisa ukugcwala kwegazi ukuze inhliziyo yengane yakho isebenze ngegumbi lokupompa elilodwa kuphela.

Ukuhlinzwa kokuqala (i-Norwood procedure) kuvame ukwenzeka ngaphakathi nesonto lokuqala lokuphila. Ukuhlinzwa kwesibili (i-Glenn procedure) kwenzeka cishe ngenyanga engu-4-6 ubudala, futhi ukuhlinzwa kwesithathu (i-Fontan procedure) kuvame ukwenziwa phakathi kweminyaka engu-2-4.

Phakathi kokwelashwa, ingane yakho izothola ukunakekelwa okucophelelayo futhi ingase idinge imithi ezosiza inhliziyo yayo ukuba isebenze kahle. Imindeni eminye icabanga ngokutshalwa kwenhliziyo njengendlela yokwelapha ehlukile, nakuba le ndlela inezinselele zayo nezincazelo.

Iqembu lakho lezokwelapha lizokusebenza eduze nawe ukuze liqonde uhlelo lokwelapha oluhle kakhulu sesimo sakho esithile. Wonke umntwana uhlukile, futhi isikhathi singase sihluke kuye kokuthi ingane yakho ikhula kangcono kanjani futhi iphendula kanjani ekwelapheni.

Ukunakekela ikhaya lengane enale-Hypoplastic Left Heart Syndrome kunjani?

Ukunakekela ingane enale-HLHS ekhaya kudinga ukunakekelwa kwezidingo zayo ezikhethekile, kodwa imindeni eminingi iyathola ukuthi izindlela zabo zoba lula ngokuhamba kwesikhathi nokuqeqeshwa.

Ingane yakho izodinga imithi ejwayelekile ezosiza inhliziyo yayo ukuba isebenze kahle. Lokhu kungafaka ama-diuretics ukususa amanzi engeziwe, imithi ezosiza inhliziyo ukuba iphonse kahle, noma ama-blood thinners ukuvimbela ama-clots.

Ukudla kungaba inselele ngoba izingane ezinale-HLHS zivame ukukhathala kalula. Ungase udinge ukunikeza ukudla okuncane, okuvamile futhi usebenze nodokotela wezondlo ukuqinisekisa ukuthi ingane yakho ithola ama-calories anele ngokukhula okunempilo.

Qaphela izimpawu ezingase zibonise ukuthi ingane yakho idinga ukunakekelwa kwezokwelapha, njengobunzima obukhulu bokuqeda umoya, ukudla okubi, ukukhathazeka okukhulu, noma ukushintsha emazingeni abo ajwayelekile wemisebenzi. Iqembu lakho lezokwelapha lizokufundisa ukuthi yini okufanele uyibheke nokuthi nini okufanele ubhale.

Kufanele ulungiselele kanjani izikhathi zakho zokubona udokotela?

Ukulungiselela izikhathi zokubona udokotela kungakusiza ukuba wenze okuningi ngesikhathi sakho neqembu lezokwelapha lengane yakho. Gcina uhlu zemibuzo evela phakathi kwezikhathi zokubona udokotela, futhi ungangabazi ukubuza nganoma yini ekukhathazayo.

Letha irekhodi lemithi yengane yakho, kufaka phakathi ukuthi bayithatha nini nanoma yiziphi izimpawu ezibuhlungu oziqaphelayo. Futhi, gcina uqaphele izindlela zokudla zengane yakho, amazinga wemisebenzi, nanoma yiziphi izimpawu oziqaphelayo.

Uma kungenzeka, letha ilungu lomndeni noma umngani ezikhathini zokubona udokotela. Ukuba nokwesekwa kungakusiza ukuba ukhumbule ulwazi olubalulekile futhi uzizwe ungakhulu. Imindeni eminingi iyathola ukuthi kuwusizo ukuthatha amanothi ngesikhathi sezikhathi zokubona udokotela noma ukubuza uma bangarekhoda imiyalo ebalulekile.

Iyini into ebaluleke kakhulu nge-Hypoplastic Left Heart Syndrome?

I-HLHS ngokuqinisekile iyisimo esibucayi senhliziyo esidinga ukunakekelwa kwezokwelapha okuphila kwakho konke, kodwa kubalulekile ukwazi ukuthi izingane eziningi ezinale-HLHS zikhula zibe impilo ezele. Ukuthuthuka kwezindlela zokwelapha nokunakekelwa kwezokwelapha kuye kwathuthukisa kakhulu imiphumela eminyakeni embalwa edlule.

Iqembu lezokwelapha lengane yakho liqeqeshwe ukunakekela izingane ezinalezi zimo eziyinkimbinkimbi zenhliziyo, futhi bayoba abalingani bakho kulolu hambo. Nakuba kuzoba nezinselele, imindeni eminingi iyathola ukuthi izingane zabo ezinale-HLHS ziyizingane eziqinile, ezisebenzayo ezihlanganyela esikoleni, ezemidlalo, nasemisebenzini yomphakathi.

Khumbula ukuthi awuwedwa kulolu hambo. Kunezinkampani zokusekela, izikhungo eziku-inthanethi, kanye nezinto ezikhethekile zemindeni ethintekile yi-HLHS. Ukuhlangana nezinye imindeni kungakunikeza iseluleko esisebenzayo, ukwesekwa ngokomzwelo, nethemba ngekusasa.

Imibuzo evame ukubuzwa nge-Hypoplastic Left Heart Syndrome

Ingane yami enale-HLHS ingaphila impilo evamile?

Izingane eziningi ezinale-HLHS zikhula zibe impilo esebenzayo, ezele, nakuba zizodinga ukunakekelwa kwenhliziyo okuphila kwakho konke. Nakuba kungase kube nokuvinjelwa kwemisebenzi ethile, izingane eziningi zihlanganyela esikoleni, ezemidlalo, nasemisebenzini yomphakathi. Wonke umntwana uhlukile, futhi iqembu lakho lezokwelapha lizokusiza ukuqonda ukuthi yini efanele isimo sakho esithile.

Ingane yami izodinga ukutshalwa kwenhliziyo ekugcineni?

Akuyona ngempela. Indlela yokwelashwa okuhlinzwa okukodwa (i-Norwood pathway) yenzelwe ukusiza inhliziyo yengane yakho ukuba isebenze isikhathi eside ngegumbi lokupompa elilodwa. Nakuba ezinye izingane zingase zidinga ukutshalwa kwenhliziyo ekugcineni, eziningi ziphila amashumi eminyaka ngenhliziyo yazo eyenziwe kabusha ngokuhlinzwa. Iqembu lakho lezokwelapha lizokulandelela umsebenzi wenhliziyo yengane yakho futhi lixoxe ngezinketho uma kudingeka.

Ngingaba nabantwana abengeziwe ngemuva kokuba nengane enale-HLHS?

Yebo, imindeni eminingi iyaqhubeka nokuba nabantwana abengeziwe. Nakuba kunethuba elikhulu kancane (cishe i-2-3%) lokuba nengane enye enesifo senhliziyo, iningi labantwana abalandelayo lizala zineenhliziyo ezempilo. Udokotela wakho angaxoxa ngokwelulekwa kwe-genetic uma ufuna ulwazi oluqondile ngezingozi.

Ukuhlinzwa kuthatha isikhathi esingakanani futhi ukululama kunjani?

Ukuhlinzwa ngakunye kuvame ukuthatha amahora amaningana, futhi isikhathi sokululama sihluka kuye ekuhlinzweni nasekuphenduleni komuntu siqu kwengane yakho. Ukuhlinzwa kokuqala kuvame ukudinga ukulala esibhedlela isikhathi eside, ngokuvamile amasonto amaningana. Ukuhlinzwa kwamuva kungase kube nesikhathi esifushane sokululama. Iqembu lakho lokuhlinzwa lizokunikeza izikhathi ezithile nethemba ngenqubo ngayinye.

Yimiphi imisebenzi ingane yami ezokwazi ukuhlanganyela kuyo njengoba ikhula?

Izincomo zemisebenzi zihluka kuye kwengane ngayinye kuye kumsebenzi wenhliziyo yayo nempilo jikelele. Izingane eziningi ezinale-HLHS zingaba nezinye imisebenzi, imidlalo yesikole, nemicimbi yomphakathi. Udokotela wakho we-cardiologist uzosisebenza nawe ukuze uqonde amazinga afanele wemisebenzi futhi angase akhuthaze ukugwema ezinye imidlalo yokuncintisana ephakeme ngenkathi ekhuthaza ezinye izindlela zokuzivocavoca.