I fate thinlung veilam chu a lo thanglian tha lo em ni? (Hypoplastic Left Heart Syndrome - HLHS) Hemi chungchang hi han sawi ila!

Vawiin hian thinlung natna tlem a buaithlak, leh hmuh tur awm lo tak, mahse pawimawh tak mai kan sawi dawn a ni. Hei hi Hypoplastic Left Heart Syndrome , emaw a tawi zawngin (HLHS) an tih natna a ni. He thu i hriat hian hlauhna i nei mai thei, mahse lungngai suh. Hemi chungchang hi awlsam takin, i hriatthiam theih dan tur angin sawi ila. A chhan chu, hetiang thil hi fimkhur a pawimawh hle a ni.

Hypoplastic left heart syndrome (HLHS) tih hi eng nge ni?

A awlsam zawngin, (HLHS) hi pianpui thinlung natna a ni a, nau piang laiin a awm thin . Hemiah hian naute thinlung veilam hmun thenkhat chu a lo thang tha lo. Pump ang maiin han ngaihtuah teh. He pump veilam hi taksa pum puia oxygen tamna thisen thawn chhuaktu a ni. Chuvangin, he veilam hi a lo thang tha lo a nih chuan buaina a lo chhuak thin. Chutiang a nih chuan thinlung veilam hmunte hi a nghawng ber a ni:

• Left ventricle : Hei hi thinlung veilam hnuai lam a main chamber a ni. Oxygenated thisen chu aorta ah a pump lut thin . HLHS-ah chuan a te lutuk a, a thawk tha thei lo fo.
• Aorta : Hei hi kan taksaa thisen kalna kawng lian ber a ni. Thinlung a\angin taksa pum puiah thisen a phur a. (HLHS) ah chuan hei pawh hi a lo thang tha lo mai thei.
• Mitral leh aortic valve: Hengte hi kawngkhar ang a ni. Thisen chu kawng khata luang chhuak chauh an phalsak a ni. Aortic valve hian veilam ventricle atanga thisen aorta chhungah a luang lut thei a ni. Mitral valve hian thinlung veilam sir upper chamber (atrium) atanga lower chamber (mitral valve) ah thisen a luang chhuak thei a ni . HLHS-ah chuan he valve hian hna a thawk tha lo thei a, a te lutuk thei bawk.

A châng chuan HLHS vei naute hian thinlung chunglam chamber pahnih inkarah hian bangah khur te tak te an nei thei (atrial septal defect) . A tlangpuiin hei hi bang thuk tak a ni tur a ni.

Thinlung pangngai leh (HLHS) nei thinlung hi eng nge an danglamna?

Kan thinlung pangngai hian kil hnih a nei a. A dinglam hian taksa atanga thisen deoxygenated chu lungah a pump lut a, oxygen a la khawm thin. Tichuan, oxygen nei thisen chu veilam lamah a kal a, taksa peng dang zawng zawngah pump chhuah a ni. Mahse, HLHS vei nauteah chuan thinlung veilam chu a te lutuk a, taksa peng dang zawng zawngah thisen a pump tling thei lo. Tichuan, ventricle dinglam chuan a luah ta a ni. Lung leh taksa dang zawng zawngah thisen pump chhuah a tum a ni. Hei hi ductus arteriosus kaltlangin tih a ni.Thisen kalna kawng bik ductus arteriosus an tih hmangin. Hei (ductus arteriosus) hi nausen pum chhunga thisen kalna kawng khat a ni. A pian hnu ni engemaw zat hnuah a khar tlangpui. Mahse (HLHS) vei nauteah chuan he thisen kalna hi a khar a nih chuan, enkawl loh chuan nunna atana hlauhawm a ni thei. A veilam a thawh loh avangin taksaa thisen a thlen theihna kawng awmchhun chu a block a ni.

Engtiang chiahin nge (HLHS) hi a hluar?

(HLHS) hi a tlem hle a ni. United States-a chhinchhiah danin kum tin nausen piang 3,800 zinga pakhat vel a nghawng a ni. (HLHS) hi pianpui thinlung natna (CHD) zawng zawng zinga 2% leh 3% inkar a ni. Hmeichhia aiin mipaah ​​a tam zawk.

Naute (HLHS) vei te hian eng symptoms nge a neih?

Naute (HLHS) vei symptoms chu a lang nghal lo mai thei. Pian hnu darkar tlemte emaw, ni engemaw zat emaw chhungin an lo lang thei a ni. A lan chhuah dan ber chu:

• Cyanosis : Vun, hmui leh kut zungtang te hi a sen hring (bluish-gray) a ni. Hei hi naute vun dum zawkah chuan greyish color angin a lang thei. Naute vun hring zawkah chuan blue color angin a lang thei. Hei hi taksain oxygen a hmuh tlem vang a ni.
• Thâwk harsa : Naute chuan thawk harsa ang maiin a inhria thei.
• Bawnghnute in harsat: Naute chuan hnute a in duh lo mai thei a, bawnghnute a in laiin a chil a chhak thei bawk.
• Lethargy : Naute chu muthilh leh chau angin a lang thei.
• Thinlung thawk chak tak.
• Sweating , vun hrual, emaw, khaw lum emaw.
• Pulse chak lo tak .

Heng symptoms te hi i hriat chian chuan damdawi lam thurawn lak nghal a pawimawh. Hlauthawng suh la, hma la thuai rawh.

(HLHS) awm chhante chu engte nge ni?

A tam zawkah chuan HLHS hi a chhan bik a awm lo . A chang chuan genetic factors vang pawh a ni thei. Naute gene mutations thenkhat , GJA1 emaw NKX2-5 emaw nei te chu HLHS vei theihna a sang hle. Tin, naute genetic condition thenkhat, Turner syndrome emaw Trisomy 18 emaw nei te pawhin HLHS an nei thei bawk.

Engtin nge HLHS hi hriat chhuah a nih?

Doctor-te chuan non-invasive imaging test hmangin HLHS hi an hre thei a ni. Hei hi naupai lai emaw, naute pian hnuah emaw tih theih a ni. Naupai lai hian:

• Prenatal ultrasound : Hei hi naupai laiin scan an tih tlangpui a ni.
• Fetal echocardiogram: Hei hi special scan a ni a, naute chu nausen pum chhunga a la awm laiin thinlung lama harsatna a neih leh neih loh enfiah theih a ni.

Naute a pian hnuah: Doctor-te chuan a natna lan chhuah dan enfiah a, test result enin an enfiah thin. A châng chuan, naute thinlung chu stethoscope hmanga i ngaihthlâk hian, thinlung mur ri i hre mai thei . Chumi awmzia chu thisen a luang tha lo tihna a ni.

Eng test nge diagnose (HLHS) atan hman thin?

• Chest X-ray: Hei hian naute thinlung leh lungphun lian leh te, a pianzia a enfiah thei a ni.
• Echocardiogram: Hei hi ultrasound test a ni bawk. Thinlung chhungril lam insiam dante chu chiang takin a lang thei a.
• Electrocardiogram (EKG/ ECG ): Hei hi thinlung thawk laia electric inthlak danglamna tehna atan hman a ni.
• Pulse oximetry screening: Hei hian naute thisena oxygen awm zat a hriat theih.

(HLHS) enkawlna tur a awm em?

Ni e, enkawlna a awm. A hmasain, naute chu damdawi prostaglandin , pek a ngai a ni. Hei hian ductus arteriosus chu a inhawng reng a ni. Hei hian taksa chhunga thisen kalna tur kawng a siam a ni. Chu bâkah, naute thinlung hnathawh ṭha zâwk tûrin damdawi dang pawh pêk a ni thei bawk. Naute chu thawk turin tanpui a ngai mai thei bawk. Tichuan, surgery eng emaw zat tih theih a ni. Heng surgery te hi lung leh taksa lama thisen kal dan tur redirect nan an hmang thin. Heng surgery an neih hnu hian thinlung hnathawh chu a pum pui deuhthaw chu right ventricle-ah a transfer vek a ni. Taksa pumpui thisen pump chhuaktu a ni.

Eng surgery nge tih (HLHS)?

Surgeon-te chuan tih tur pawimawh pathum an ti a: Norwood procedure , Glenn procedure , leh Fontan procedure . Heng kalphungte hi a hnuaia mi ang hian tih a ni. 1. Norwood procedure: He procedure hi HLHS vei nausen dam chhung kar hnih chhungin tih thin a ni. Hetiang tih danah hian surgeon-te chuan:

• Naute aorta lo thanglian lo chu taksaah thisen a kal theih nan siam danglam a ni.
• Shunt , tube te tak te chu thisen ventricle dinglam atanga thisen, aorta atanga emaw, lung lam panna pulmonary arteries-a kal theihna turin dah a ni.
• Thinlung chunglam (atria) inkarah inzawmna a siam a ni. Hei hian taksa chu lung atanga oxygen tamna thisen supply turin a pui a ni.

2. Bidirectional Glenn shunt operation: Naute tanSurgery vawi hnihna chu thla 4 atanga thla 6 vel ah tih tur a ni. Glenn-a surgery neih lai hian:

• Shunt hlui chu lakchhuah mek a ni.
• Shunt thar dahin naute superior vena cava (SVC) (taksa chunglam atanga thinlung thlenga oxygen tlachham thisen phurtu) chu pulmonary artery nen a thlunzawm a ni.
• He shunt hian right ventricle-a load a tihziaawm a, a chhan chu thisen chu lungah direct-in a kal theih avangin.

3. Fontan procedure: A tawp ber chu thla 18 leh kum 4 inkar a ni . Hetiang tih dan hian taksa atanga thisen lo kir zawng zawng chu lungah a divert a, thinlung chu a bypass a ni. Fontan kalphung chhung hian:

• Naute inferior vena cava (IVC) (taksa hnuai lam atanga thinlung thlenga oxygen tlachham thisen phurtu) chu pulmonary artery nen a inzawm a.

Enkawlnaah hian harsatna a awm em?

Ni e, naute thenkhat chuan surgery pakhat atanga operation dang an kal laiin an nunna pawh an hloh thei a ni. Tin, operation neih apiangin harsatna engemaw zat a awm thei bawk. Entir nan:

• Ventricle dinglam hnathawh that loh avanga harsatna awm.
• Aortic valve a leak a ni.
• Liver natna.
• Thinlung rhythm pangngai lo.
• Thisen a chhuak thin.
• Kaichhawng.
• Chaw ei harsat.
• Seizures a awm thin.
• Kidney lama harsatna nei.
• Lung natna (Cardiac arrest) a awm.

Hengte hi hriat tur hlauhawm tak tak a ni a, mahse doctor-te chuan heng hlauhawmnate hi an sawipui ang che a, enkawlna tha ber pek che an tum ang.

Heart transplant hi enkawlna tha tak a ni em?

A châng chuan, surgeon-te chuan chu surgery pathumte tih ai chuan heart transplant an rawt mai thei. Amaherawhchu, naute thinlung thlak tawhte chuan an dam chhung zawng damdawi (immune suppressant drugs) an ei a ngai dawn a ni.

HLHS hi pian hmain enkawl theih a ni em?

Tunah hian naupai laiin operation hmanga HLHS hi tihdam kim theih a la ni lo. Mahse, nausen surgeon-te chuan HLHS vei nausen lo piang chhuak (e.g., aortic valve te tak te)-a thil \ha lo awm thei ṭhenkhat ven nan intervention engemaw zat an ti thei \hin. Mahse hei hi thil bik takah chauh a ni.

(HLHS) hlauhawmna hi tihziaawm theih a ni em?

HLHS vei tam zawk chhan hi hriat chian a nih loh avangin a taka ven dan tur sawi a harsa hle. Mahse, naupai lai hian nungchang hrisel zawm hi a pawimawh reng a ni:

• Zu leh zu in loh nan.
• Damdawi dang Diabetes Mellitus ang chi control .
• Ei leh in hrisel ei.
• Nau neih hmaa vitamin nitin folic acid microgram 400 (400 mcg) tal ei .

Nangmah emaw, i kawppui emaw hian chhungkaw history-ah HLHS i nei a nih chuan naupai hmain genetic counselor nen inbiakna neih hmasak phawt a tha.

Ka fain (HLHS) a neih chuan eng nge ka beisei ang?

HLHS enkawl hnuah i fa chu a dam chhungin kum khatah vawi khat tal cardiologist a hmuh a ngai dawn a ni. Chu chuan a thinlung, a lung leh a taksa peng dangte chu a thawk tha hle tih a chiang ang. I fa chu a lo upat deuh deuh chuan puitling pianpui thinlung natna chungchanga specialist a hmuh a ngai ang . HLHS vei naupang tam tak chuan an dam chhungin thinlung damdawi an ei a ngai dawn a ni. Eng surgery pawh an neih hmain antibiotics an ei a ngai dawn bawk a, ha surgery pawh an ei hmasak phawt a ngai dawn a ni. Hei hian endocarditis (thinlung chhunga natna hrik) vei theihna a ti tlem thei a ni.

(HLHS) dinhmun thlirletna chu eng nge ni?

Enkawl loh chuan HLHS hi nau piang atanga ni emaw kar emaw chhungin thihna thlen thei a ni. Enkawlna nen chuan hmalam hun chu naute thinlung chhiatna (heart defect) a buaithlak danah a innghat a ni. I naute doctor hnenah surgery neih apianga a hlauhawm dan zawt thin ang che. Naupang thenkhat chuan dam chhung zawnga exercise lak theihna an tlahniam thei bawk. Doctor-te chuan inelna infiamna ang chi taksa tihchakna hahthlak tak takte tihtlem an rawt tlangpui.

(HLHS) vei naute damchhuah zat chu engzat nge ni?

Hei hi thil lungchhiatthlak deuh a ni a, mahse hriat a pawimawh hle. Nausen (HLHS) vei zinga 20% leh 60% inkar chu an dam kum khatnaah an dam thei a ni. Chumi hnuah chuan kum nga, kum sawm leh kum sawm leh panga lo awm tur atana damchhuah zat chu 40% vel a ni. Nausen piang rit pangngaia piang, piang hmaa piang lo piangte hian nau piang rit lo piangte aiin outcome tha zawk an nei thin. Zirchianna pakhatah chuan kum khatna naute dam chhuak tam zawk chu kum 18-ah an la dam tih hmuhchhuah a ni.

Heng statistics te hi hmuh a har khawp mai. Mahse, medical science hi nitin a tha chho zel tih hre reng ang che. Enkawlna thar, technology thar a lo thleng ta. Chuvangin beiseina nung reng a pawimawh.

Engtin nge ka fa ka enkawl ang?

Hetiang natna neia piang naupangte hian cardiologist hnen atanga hun rei tak enkawlna dawngin nun hrisel tak an hmang thei a ni. I fa chu hetiang hian i enkawl thei ang:

• I fa chu kum tin influenza (flu) vaccine leh COVID-19 vaccine pe thin ang che.
• I fa chu thla ruk danah emaw kum khatah vawi khat emaw cardiologist hnenah hruai thin ang che .
• I fa hnenah damdawi pek chu a hun takah pe rawh .
• I fate taksa tihchakna nasa tak chu tihtlem rawh , i doctor-in a rawt angin.
• I fain zirlai harsatna a neih chuan , pui rawh.

Doctor hnenah eng zawhna nge ka zawh ang?

I naute hian HLHS a vei a nih chuan doctor hnenah zawhna hetiang hi i zawt thei ang:

• Surgery neih hian eng hlauhawmna nge a neih?
• Operation hnuah pawh complication fimkhur tur a awm em?
• Ka fa hian eng damdawi nge a mamawh? Chutiang damdawi te hian side effect a nei em?
• Engtin nge he dinhmun (HLHS) hian ka fa nun a nghawng ang?
• Ka fa hian operation hnuah eng follow-up care nge a mamawh?
• Naute dang ka neih chuan chu naute pawhin (HLHS) a neih theihna tur risk a awm em?

HLHS vei naupang neih hi rilru hahna leh mahni chauha awm thei tak a ni . I fate thinlung natna avanga lungkhamna leh rinhlelhna i hmachhawn hian, emotional support hmuhna tur support group emaw hmun dang emaw zawn a pawimawh hle. Rilru chak taka awm reng hian i fa hriselna sang leh hniam te chu i hmachhawn thei ang.

Take-Home Thuchah a ni

(HLHS) hi thinlung natna khirh tak, pianpui atanga lo chhuak a ni. Amaherawhchu, a hmaa hriatchhuah a nih a, uluk taka enkawl a nih chuan naupangte hian nun \ha tak an nei thei a ni. Hei hian operation buaithlak tak tak eng emaw zat leh hun rei tak chhung damdawi lam enkawlna a ngai a ni. A pawimawh ber chu nangmah chauh i ni lo tih hriat hi a ni. Doctor, nurse leh health worker dangte chu nang leh i fate tanpui turin an awm reng a ni. Chutiang bawka thil tawn tawh nu leh pa dangte hnen atanga tanpuina lak a pawimawh hle bawk. Huaisenna hloh suh. I fa tana i inpeknate hi a hlu a ni.