Ngaba uyazazi ezi zinto malunga ne-ventricle yasekhohlo yentliziyo yakho? (I-Ventricular Non-Compaction yasekhohlo - LVNC) Masithethe!

Namhlanje siza kuthetha ngemeko yentliziyo eyinkimbinkimbi kancinci, kodwa enokuba yinto ebaluleke kakhulu. Usenokuba awuzange uve ngale nto ngaphambili. Ibizwa ngokuba yi-Left Ventricular Non-Compaction (LVNC) njengoko oogqirha beyibiza njalo. Eli gama lisenokuvakala ngathi yinto enkulu, kodwa ungakhathazeki, masiyigcine ilula.

Ngoko ke yintoni i-left ventricular noncongestive heart failure (LVNC)?

Ngamafutshane, le yimeko eyenzeka xa igumbi elingaphantsi kwicala lasekhohlo lentliziyo yethu, iLeft Ventricle, lingakhuli kakuhle. Cinga ngayo, ngesiqhelo le ventricle yasekhohlo yentliziyo yethu yinyama enamandla kakhulu, egudileyo. Yinxalenye ephambili epompa igazi emzimbeni wethu wonke. Kodwa kumntu one-(LVNC), i-ventricle yasekhohlo ifana nesiponji, ityebile kancinci, kwaye icwangciswe njengomnatha. Inendalo enamagquma, njengangaphakathi kwesonka.

Oku kudla ngokuthathwa njengengxaki yentliziyo yokuzalwa. Oku kuthetha ukuba usenokuba nale meko entliziyweni yakho ukususela ngexesha lokuzalwa kwakho . Ukuba ayinyangwa kakuhle, le (LVNC) ingakhokelela kwiingxaki ezinkulu zentliziyo, oko kukuthi, isifo sentliziyo.

Le meko ye-LVNC iyichaphazela njani indlela esebenza ngayo intliziyo?

Ngoku khangela, i-ventricle yasekhohlo yentliziyo yethu inomsebenzi obaluleke kakhulu. Yile ipompa igazi elinomoya-mpilo omninzi ukusuka emiphungeni ukuya emzimbeni wonke. Njengenjini yamanzi. Nangona kunjalo, xa le meko (LVNC) isenzeka, i-ventricle yasekhohlo, efana nesiponji, ayinakukwazi ukufinyela okanye ukuphumla ngokufanelekileyo. Oko kukuthi, inobunzima bokuqinisa nokutyhala igazi phambili, okanye ukuphumla kwakhona ukuze ilizalise ngegazi. Ngoko ke, isiphumo kukuba umsebenzi wokupompa igazi emzimbeni wonke awuwenzi ngokufanelekileyo . Yiyo loo nto iingxaki ezahlukeneyo ziqala ukuvela.

Ingaba le meko ingaqhelekanga kangakanani ebizwa ngokuba yi-LVNC?

Esi sisifo esingaqhelekanga ngokwenene. Ngokutsho kweengcali zonyango, ngabantu abamalunga neshumi elinesibini kuphela kwisigidi abafunyaniswa benesi sifo minyaka le. Nangona kunjalo, inani lokwenyani lisenokuba phezulu kakhulu . Isizathu kukuba abanye abantu ababonisi zimpawu. Emva koko abayi kugqirha, ngoko ke abafumani zimpawu.

Zithini iimpawu ze-LVNC?

Le yeyona nto ibalulekileyo. Abanye abantu basenokungabonakalisi zimpawu . Nangona kunjalo, abanye banokuba neengxaki ezinkulu zentliziyo. Umzekelo, ukufa ngesiquphe kwentliziyo kunokwenzeka. Malunga nesibini kwisithathu sabantu abane-LVNC basengozini yokuhlaselwa sisifo sentliziyo .

Kulungile, makhe sibone ukuba zeziphi iimpawu umntu one-(LVNC) anokubonisa:

Iimpawu eziqhelekileyo :

• Amahlwili egazi angenzakala. La ayingozi kakhulu, kuba ukuba la mahlwili aya engqondweni okanye emiphungeni, inokuba yingxaki enkulu.
• Usenokuba nobunzima bokuphefumla xa ulele ebhedini.
• Ukuquleka .
• Ukubetha kwentliziyo - Oku kunokuvakala ngathi yidrama ibetha ngaphakathi esifubeni, okanye ngathi intliziyo iyabetha ngamandla.
• Ukungakwazi ukwenza umthambo, ukuphefumla kancinci nokuba ungazibhokoxa kancinci (Dyspnea) .
• Isingqisho sentliziyo esingaqhelekanga (i-Arrhythmia) .
• Ukudumba kwemilenze (iLymphedema) - ingakumbi emaqatheni.
• Ukuphefumla kancinci/Ukuphelelwa ngamandla - Ukuziva ngathi uphelelwa ngamandla nokuba ungenza umgudu omncinci.

Kutheni le meko ye-LVNC isenzeka? Zithini izizathu?

Ixesha elininzi, le meko (LVNC) ibangelwa lutshintsho lwemfuza (i-Gene mutation) olwenzeka ngaphambi kokuba uzalwe. Le yinto enzima kakhulu. Olu tshintsho lwemfuza lunokwenzeka ngokungacwangciswanga, oko kuthetha ukuba akukho sizathu, okanye lunokuzuzwa kubazali bakho. Oko kuthetha ukuba lunokuba yinto eyenzeka kwiintsapho. Cinga nje, ezinye iintsapho zinabantu abaninzi abanesifo sentliziyo.

Kwakhona, ipesenti encinci kakhulu yabasetyhini inokuphuhlisa i-LVNC okokuqala ngexesha lokukhulelwa ngaphandle kwesizathu esicacileyo. Oogqirha bacinga ukuba oku kusenokuba kungenxa yoxinzelelo olongezelelekileyo kwimisipha yentliziyo ngexesha lokukhulelwa. Kwabanye abafazi, ezi mpawu ziyaphela emva kokubeleka. Kodwa kwabanye, i-LVNC inokuba nemiphumo yexesha elide.

Oogqirha bayixilonga njani le meko ye-LVNC?

Ugqirha uza kwenza uvavanyo oluninzi ukuze aqinisekise ukuba unayo na le (LVNC) okanye awunayo. Makhe sijonge ukuba zeziphi ezi vavanyo:

• I-Echocardiogram: Oku kujonga indlela intliziyo yakho epompa ngayo igazi, indlela esebenza ngayo iivalvu, kunye nokuba kukho naziphi na iingxaki ngeevalvu. Kufana ne-ultrasound scan yentliziyo. Kulapho ubona khona okokuqala imeko ye-ventricle yasekhohlo enjenge-spongy.
• I-Electrocardiogram (ECG): Le ilinganisa umsebenzi wombane wentliziyo yakho. Ingabona naziphi na iingxaki ngesingqisho sentliziyo yakho.
• Uvavanyo olukhethekileyo lokujonga imifanekiso: Olu luquka ii-cardiac CT scans kunye ne-magnetic resonance imaging (MRI). Ezi zinokubonisa ulwakhiwo lwentliziyo, njengokuba intliziyo yenziwe njani kunye nobukhulu beendonga zayo. Kwi-LVNC, ukubonakala okufana nesiponji kubonakala ngokucacileyo kwezi scans.
• I-Coronary angiogram: Oku kuvavanya indlela igazi elihamba ngayo kwimithambo yegazi ehambisa igazi entliziyweni, kunye nokuba kukho naziphi na izithintelo. Ngamanye amaxesha olu vavanyo lunokufuneka ukuze kuthintelwe ezinye izifo zentliziyo.

Ziziphi iindlela zonyango zale meko ye-LVNC?

Unyango lwe-LVNC luyahluka ngokuxhomekeke kwiimpawu zakho. Asinguye wonke umntu ophathwa ngendlela efanayo. Umzekelo, ukuba unesifo sentliziyo, ugqirha wakho unokukunika amayeza anjengala:

• Ii-Angiotensin-converting enzyme inhibitors (ACE inhibitors): Umzekelo, i-benazepril (Lotensin®) okanye i-lisinopril (Prinivil®, Zestril®) okanye i-sacubitril-valsartan (Entresto®) (ii-angiotensin-neprilysin inhibitors). Ezi zisebenza ngokuphumla nokuvula imithambo yegazi yakho (imithambo yegazi kunye nemithambo yegazi) ukuze kube lula ukuba igazi lihambe kuzo. Oku kunciphisa umthwalo womsebenzi entliziyweni.
• Ii-Beta-blockers: Umzekelo, i-metoprolol (Lopressor®, Toprol XL®) okanye i-atenolol (Tenormin®). Ezi zithintela iihomoni ezithile (umz. i-adrenaline), zehlisa uxinzelelo lwegazi kwaye zilawula isantya sentliziyo.
• I-Digoxin (Digoxin®, Lanoxin®): Oku kulawula isantya sentliziyo yakho, kwandisa kancinci amandla okupompa kwentliziyo yakho, kwaye kuphucula ukujikeleza kwegazi.
• I-Diuretics okanye "iipilisi zamanzi": Ezi zisebenza ngokubangela ukuba umzimba ukhuphe ityuwa namanzi amaninzi ngomchamo. Oku kunciphisa ukudumba kwaye kunciphisa umthwalo womsebenzi entliziyweni.

Ngamanye amaxesha, ukuba kukho umngcipheko wokuma kwentliziyo ngequbuliso, ugqirha unokucebisa ukuba kufakwe isixhobo esibizwa ngokuba yi-Implantable Cardioverter Defibrillator (ICD) phantsi kwesikhumba sentliziyo. Esi sisixhobo esincinci esisebenza ngebhetri. Sibona isigqi esikhawulezayo nesisongela ubomi entliziyweni kwaye sibangele ukubetheka kombane entliziyweni ukuze kubuyiselwe isigqi esiqhelekileyo. Kwiimeko ezinzima kakhulu, ukuba ezinye iindlela zonyango aziphumelelanga, ukufakelwa intliziyo kunokufuneka.

Ngaba le meko ye-LVNC inganyangeka ngokupheleleyo? Ngaba ingaguqulwa?

Hayi bo. Okwangoku akukho nyango lwe-LVNC . Oko kukuthi, ayinakuguqulwa. Kodwa ungakhathazeki. Unyango lunokuphucula umgangatho wobomi bakho kwaye lunciphise iingxaki ezinokubakho ngenxa yesifo sentliziyo . Kwaye, njengoko benditshilo ngaphambili, kwiimeko ezinzima kakhulu, ukufakelwa intliziyo kunokufuneka.

Singayiphepha njani le meko ye-LVNC?

Akukho ndlela yokuthintela i-LVNC. Oku kungenxa yokuba idla ngokubangelwa zizinto zemfuza. Nangona kunjalo, ukuba une-gene mutation ebangela le meko, okanye ukuba ucinga ukuba unokuba nayo (umzekelo, ukuba kukho umntu kusapho lwakho onayo le meko), uvavanyo lwemfuza lunokuba luncedo kakhulu.Umcebisi ngezemfuza uza kukuchazela wena nosapho lwakho iziphumo zovavanyo lwakho. Baza kukunceda uqonde ukuba kuthetha ukuthini ukuba ne-LVNC kunye nomngcipheko wakho wokudlulisela oku kubantwana bakho.

Ngaba kukho ezinye iimeko zempilo ezinxulumene nale meko ye-LVNC?

Ewe. Ngamanye amaxesha le meko ibizwa ngokuba yi-(LVNC) inokubonwa njengenxalenye yezinye iimeko zemfuza (ii-syndromes). Ezi ziyinkimbinkimbi ngakumbi, kodwa oogqirha bayaziqwalasela ezi meko xa bexilonga i-(LVNC). Nazi imizekelo embalwa:

• Isifo sikaBarth
• Isifo sikaNoonan
• Isifo sikaRoifman
• Isifo sikaToriello Carey

Liyintoni ikamva lomntu one-LVNC? Lingakanani ixesha lokuphila?

Imbono yomntu one-LVNC inokwahluka. Iyahluka kumntu nomntu. Xa unyango lulungile, iimpawu zinokulawulwa , kwaye abanye abantu baphila ubomi obuqhelekileyo.

I-LVNC yimeko enokubangela ukufa, kodwa ayisoloko injalo . Uninzi lwabantu abane-LVNC abafi ngenxa yesi sifo. Eyona nto ibalulekileyo kukubona ugqirha wentliziyo rhoqo uze uvavanywe . Unokukunceda ulawule impilo yentliziyo yakho kwaye anciphise umngcipheko weengxaki.

Ukuba mna okanye umntwana wam une-LVNC, yeyiphi imibuzo endifanele ndiyibuze ugqirha?

Ukuba wena okanye umntwana wakho unayo (i-LVNC), okanye ukuba uneenkxalabo, kungcono ukubuza ugqirha wakho imibuzo efana nale:

• Yintoni enokubangela ukuba umntwana wam/wam abe ne-LVNC? (oko kukuthi, ingaba yimfuza okanye enye into)
• Ngaba ucebisa uvavanyo lwemfuza lwe-LVNC? Ukuba kunjalo, ngaba lungabalulekanga kwamanye amalungu osapho?
• Zeziphi iimvavanyo ekufuneka mna/umntwana wam ndizifumane ukuze ndixilonge i-LVNC? Yintoni endinokuyilindela kuzo?
• Ziziphi iindlela zonyango lwe-LVNC? Loluphi unyango olulungele mna/umntwana wam? Ngaba kukho naziphi na iziphumo ebezingalindelekanga kolu nyango?
• Ukuba mna okanye umntwana wam sinesifo sentliziyo ngequbuliso, zithini iimpawu? Ndingenza ntoni ngoko nangoko?

Le mibuzo iza kukunceda uxoxe ngayo nogqirha wakho kwaye uqonde ngcono imeko yakho.

Ngoko ke, zeziphi izinto ezibalulekileyo ekufuneka sizikhumbule kweli bali?

Intliziyo engasebenziyo ye-ventricular yasekhohlo (LVNC) sisifo sentliziyo esingaqhelekanga, esihlala sizalwa naso. Oku kwenzeka xa i-ventricle yasekhohlo yentliziyo ingakhuli kakuhle. Endaweni yokuba ibe sisihlunu esigudileyo nesiqinileyo, iba nkulu kwaye ibe si-sponge. Oku kubangela ukuba intliziyo ingapompi igazi ngokufanelekileyo.

Ukuba unayo (LVNC),Eyona nto ibalulekileyo kukubona ugqirha wentliziyo rhoqo uze ulandele iingcebiso zakhe. Nangona kungekho nyango lokunyanga oku, unyango olunikwa ngugqirha wakho lunokunciphisa umngcipheko weengxaki kwaye luphucule umgangatho wobomi bakho. Kubalulekile ukuba ungoyiki kwaye ufumane unyango olufanelekileyo . Khumbula, awuwedwa, kwaye oogqirha bakhona ukukunceda.

Isifo sentliziyo, i-ventricle yasekhohlo, i-LVNC, iimpawu zesifo sentliziyo, ukusilela kwentliziyo, izifo zemfuza, unyango lwesifo sentliziyo