Matenda a mtima otsalira

Matenda a mtima wamanzere kumanzere amapezeka pamene mbali zakumanzere za mtima (mitral valve, ventricle yakumanzere, valavu ya aortic, ndi aorta) sizikula kwathunthu. Vutoli limakhalapo pakubadwa (kobadwa nako).

Mtima wamanzere wotsalira ndimatenda achilendo amtundu wobadwa nawo. Amakonda kwambiri amuna kuposa akazi.

Monga momwe zimakhalira ndi zofooka zamtima zobadwa, palibe chifukwa chodziwikiratu. Pafupifupi 10% ya ana omwe ali ndi hypoplastic left heart syndrome amakhalanso ndi zovuta zina zobadwa. Amagwirizananso ndi matenda ena amtundu wamtundu monga Turner syndrome, Jacobsen syndrome, trisomy 13 ndi 18.

Vutoli limayamba asanabadwe pomwe mpweya wamanzere ndi zina sizimakula bwino, kuphatikiza:

• Aorta (chotengera chamagazi chomwe chimanyamula magazi olemera okosijeni kuchokera kumtunda kumanzere kupita kumthupi lonse)
• Kulowera ndi kutuluka kwa ventricle
• Mitral ndi aortic valves

Izi zimapangitsa kuti ventricle yakumanzere ndi aorta zisakule bwino, kapena hypoplastic. Nthawi zambiri, ventricle yakumanzere ndi aorta ndizocheperako kuposa zachilendo.

Mwa makanda omwe ali ndi vutoli, mbali yakumanzere ya mtima imalephera kutumiza magazi okwanira mthupi. Zotsatira zake, mbali yakumanja ya mtima iyenera kuyendetsabe kufalikira kwamapapu ndi thupi. Mpweya wabwino ungathandizire kufalikira kwa mapapo ndi thupi kwakanthawi, koma ntchito yowonjezera iyi pamapeto pake imapangitsa mbali yakumanja yamtima kulephera.

Kuthekera kokhako kopulumuka ndikulumikizana pakati pa dzanja lamanja ndi lamanzere la mtima, kapena pakati pamitsempha ndi mitsempha ya m'mapapo (mitsempha yamagazi yomwe imanyamula magazi kupita m'mapapu). Ana nthawi zambiri amabadwa ndi izi:

• Foramen ovale (dzenje pakati pa dzanja lamanja ndi lamanzere)
• Ductus arteriosus (chotengera chaching'ono chamagazi chomwe chimalumikiza aorta ndi mtsempha wamagazi)

Malumikizidwe onsewa nthawi zambiri amatseka okha patangopita masiku ochepa kuchokera pobadwa.

Kwa makanda omwe ali ndi hypoplastic left heart syndrome, magazi amachoka mbali yakumanja yamtima kudzera mumitsempha yam'mapapo amayenda kudzera mu ductus arteriosus kupita ku aorta. Imeneyi ndi njira yokhayo yoti magazi afikire thupi. Ngati ductus arteriosus aloledwa kutseka mwa mwana yemwe ali ndi hypoplastic left heart syndrome, mwanayo amatha kufa msanga chifukwa palibe magazi omwe adzaponyedwe mthupi. Ana omwe amadziwika kuti ndi hypoplastic left heart syndrome nthawi zambiri amayamba ndi mankhwala kuti ductus arteriosus ikhale yotseguka.

Chifukwa pali kutuluka pang'ono kapena kutuluka mumtima wakumanzere, magazi obwerera kumtima kuchokera m'mapapu amafunika kudutsa pa foramen ovale kapena atrial septal defect (dzenje lolumikiza zipinda zosonkhanitsira kumanzere ndi kumanja kwamtima) kubwerera kumanja kwamtima. Ngati palibe foramen ovale, kapena ngati ndi yaying'ono kwambiri, mwanayo akhoza kufa. Ana omwe ali ndi vutoli amatseguka pakati pa atria, mwina ndi opaleshoni kapena kugwiritsa ntchito chubu chowonda, chosinthasintha mtima (catheterization yamtima).

Poyamba, mwana wakhanda yemwe ali ndi hypoplastic left heart amatha kuwoneka wabwinobwino. Zizindikiro zimatha kupezeka m'maola ochepa oyamba, ngakhale zingatenge masiku ochepa kuti zizindikilo. Zizindikirozi zitha kuphatikiza:

• Bluish (cyanosis) kapena khungu loyipa
• Manja ozizira ndi mapazi (malekezero)
• Kukonda
• Kutaya koyipa
• Osauka oyamwa ndi kudyetsa
• Mtima wogunda
• Kupuma mofulumira
• Kupuma pang'ono

M'mwana wakhanda wathanzi, mtundu wabuluu m'manja ndi m'miyendo umayankha kuzizira (izi zimadziwika kuti zotumphukira za cyanosis).

Mtundu wabuluu m'chifuwa kapena m'mimba, milomo, ndi lilime ndizachilendo (wotchedwa central cyanosis). Ndi chizindikiro kuti mulibe mpweya wokwanira m'magazi. Central cyanosis nthawi zambiri imakula ndikulira.

Kuyezetsa thupi kumatha kuwonetsa zizindikilo za kulephera kwa mtima:

• Mofulumira kuposa kugunda kwamtima
• Kukonda
• Kukulitsa chiwindi
• Kupuma mofulumira

Komanso kugunda m'malo osiyanasiyana (dzanja, kubuula, ndi ena) kumatha kukhala kofooka kwambiri. Nthawi zambiri pamakhala phokoso (koma osati nthawi zonse) pamtima pomwe mumamvera pachifuwa.

Mayeso atha kuphatikiza:

• Catheterization yamtima
• ECG (electrocardiogram)
• Zojambulajambula
• X-ray ya chifuwa

Akazindikira kuti mtima wamanzere wam'magazi wapangika, mwanayo adzagonekedwa m'chipinda cha ana osamalidwa bwino. Makina opumira (othandizira mpweya) angafunike kuthandiza mwana kupuma. Mankhwala otchedwa prostaglandin E1 amagwiritsidwa ntchito kupangitsa kuti magazi azizungulira mthupi posunga ductus arteriosus.

Izi sizithetsa vutoli. Vutoli limafuna kuchitidwa opaleshoni nthawi zonse.

Opaleshoni yoyamba, yotchedwa opareshoni ya Norwood, imachitika m'masiku ochepa oyamba a mwanayo. Ndondomeko ya Norwood ili ndi kupanga aorta yatsopano mwa:

• Kugwiritsa ntchito valavu yamapapo ndi mtsempha wamagazi
• Kulumikiza minyewa yakale ya hypoplastic ndi mitsempha yamtima ku aorta yatsopano
• Kuchotsa khoma pakati pa atria (atrial septum)
• Kupanga kulumikizana kwapangidwe kuchokera pamitsempha yolondola kapena mtsempha wamagulu onse pamitsempha yam'mapapo kuti magazi azitha kuyenda m'mapapu (otchedwa shunt)

Njira zosiyanasiyana za Norwood, zotchedwa Sano, zitha kugwiritsidwa ntchito. Njirayi imapangitsa kuti pakhale ma ventricle oyenera olumikizana ndi mtsempha wamagazi.

Pambuyo pake, mwana amapita kunyumba nthawi zambiri. Mwanayo adzafunika kumwa mankhwala tsiku lililonse ndikutsatiridwa ndi dokotala wamtima wa ana, yemwe adzawone gawo lachiwiri la opaleshoni liyenera kuchitidwa.

Gawo lachiwiri la ntchitoyi limatchedwa njira ya Glenn shunt kapena hemi-Fontan. Amatchulidwanso kuti shunt cavopulmonary shunt. Njirayi imalumikiza mitsempha yayikulu yonyamula magazi amtambo kuchokera kumtunda wapamwamba wa thupi (vena cava wapamwamba) molunjika kumitsempha yamagazi m'mapapu (mitsempha yam'mapapo) kuti ipeze mpweya. Kuchita opaleshoni kumachitika nthawi zambiri mwana ali ndi miyezi 4 mpaka 6 yakubadwa.

Munthawi yoyamba I ndi II, mwanayo amatha kuwoneka wabuluu (cyanotic).

Gawo lachitatu, gawo lomaliza, limatchedwa njira ya Fontan. Mitsempha yotsala yomwe imanyamula magazi amtambo kuchokera mthupi (malo otsika a vena cava) amalumikizidwa molunjika kumitsempha yamagazi kumapapu. Vuto loyenera tsopano limangokhala ngati chipinda chopopera thupi (sipakhalanso mapapu ndi thupi). Opaleshoni imeneyi imachitika nthawi zambiri mwana akakhala miyezi 18 mpaka 4 wazaka. Pambuyo pa gawo lomalizirali, mwanayo salinso wowonjezera ndipo ali ndi mpweya wabwino wamagazi m'magazi.

Anthu ena angafunike maopaleshoni ochulukirapo m'zaka zawo za m'ma 20 kapena 30 ngati atayamba kulimbana ndi zovuta zina kapena zovuta zina zaku Fontan.

Madokotala ena amaganiza kuti kuziika mtima ndi njira ina yochiritsira m'malo mochita opaleshoni yachitatu. Koma pali mitima yochepa yoperekedwa kwa makanda ang'onoang'ono.

Ngati sanalandire chithandizo, hypoplastic left heart syndrome imapha. Kuchuluka kwa opulumuka pakukonzanso komwe kumachitikabe kukuwonjezeka chifukwa cha njira zopangira opaleshoni ndi chisamaliro pambuyo poti opaleshoni ikuyenda bwino. Kupulumuka pambuyo pa gawo loyamba ndikoposa 75%. Ana omwe amapulumuka chaka choyamba amakhala ndi mwayi wopulumuka kwanthawi yayitali.

Zotsatira za mwana atachitidwa opaleshoni zimatengera kukula ndi magwiridwe antchito a ventricle woyenera.

Zovuta zimaphatikizapo:

• Kutsekedwa kwa shunt yokumba
• Kuundana kwamagazi komwe kumatha kubweretsa sitiroko kapena kuphatikizika kwamapapu
• Kutsekula m'mimba kwa nthawi yayitali (kuchokera ku matenda otchedwa enteropathy yotaya mapuloteni)
• Madzimadzi m'mimba (ascites) komanso m'mapapu (kupuma kwamphamvu)
• Mtima kulephera
• Nyimbo zosasinthasintha, zamtima (arrhythmias)
• Sitiroko ndi zovuta zina zamanjenje
• Kuwonongeka kwa minyewa
• Imfa mwadzidzidzi

Lumikizanani ndi omwe akukuthandizani nthawi yomweyo ngati khanda lanu:

• Amadya pang'ono (kuchepa kudya)
• Ili ndi khungu labuluu (cyanotic)
• Ikusintha kwatsopano m'mapweya

Palibe choletsa kudziwika cha hypoplastic left heart syndrome. Mofanana ndi matenda ambiri obadwa nawo, zomwe zimayambitsa matenda otupa mtima kumanzere sizikudziwika ndipo sizinagwirizane ndi matenda kapena machitidwe a mayi.

HLHS; Kobadwa nako mtima - hypoplastic lamanzere mtima; Matenda a mtima wa Cyanotic - mtima wamanzere wotsalira

• Mtima - gawo kupyola pakati
• Mtima - kuwonera kutsogolo
• Matenda a mtima otsalira

CD ya Fraser, Kane LC. Matenda amtima obadwa nawo. Mu: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery: Maziko Achilengedwe a Njira Zamakono Zopangira Opaleshoni. Wolemba 20th. Philadelphia, PA: Elsevier; 2017: chap 58.

Webb GD, Smallhorn JF, Therrien J, Redington AN.Matenda obadwa nawo mumtima mwa wamkulu komanso wodwala. Mu: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, olemba. Matenda a Mtima a Braunwald: Buku Lophunzitsira la Mankhwala Amtima. 11th ed. Philadelphia, PA: Elsevier; 2019: mutu 75.