I fate thinlung dinglam chu a lo thanglian tha lo em ni? (Hypoplastic Lung dinglam natna) .

I fa tê tê chu thinlung lam harsatna neia piang ni ta se engtin nge ni ang? Chu chu a lungchhiatthlâk êm êm ang tih han ngaihtuah teh. Nausen thenkhat chu an thinlung dinglam a thang tha lova piang an ni. Hei hi Doctor ten Hypoplastic Right Heart Syndrome an ti a. Hei hian naute oxygen a hmuh zat a ti tlem a ni. Hemi chungchang hi chipchiar zawkin sawi ila, kan sawi dawn em ni?

Hypoplastic Right Heart Syndrome hi eng nge ni?

A awlsam zawngin, Hypoplastic Right Heart Syndrome hi thinlung natna hmuh tur awm lo tak a ni a, naute thenkhat chu an piang chhuak thin . Hemi a thil thleng chu naute thinlung dinglam hmun thenkhat chu a lo thanglian kim lo tih hi a ni. Pump tlemte ang maiin han ngaihtuah teh. Thinlung dinglam hi taksa atanga oxygen tlachham thisen la chhuakin lungah oxygen la turin a thawn chhuak thin a ni. Chuvangin, he dinglam hi a lo thang tha lo a nih chuan a hna a thawk tha lo ang.

Hetiang dinhmunah hian a hnuaia thinlung hmunte hi a nghawng thei a ni:

• Tricuspid valve: Hei hi thinlung dinglam atrium leh dinglam ventricle inkar gate ang a ni. A hnathawh that loh chuan thisen kalna tur a tikhawlo thin.
• Pulmonary valve: Hei hi ventricle dinglam atanga lung lama thisen phurtu pulmonary artery intanna valve a ni.
• Right ventricle: Hei hi thinlung dinglamah thisen pump chhuahna hmunpui ber a ni. A te deuh chuan thisen a pump tlem zawk ang.
• Pulmonary artery: Hei hi thisen dinglam ventricle atanga lung lam panna thisen lian tak a ni.

A natna a nasat emaw, a na lo emaw a zirin, heng thinlung hmun hrang hrangte hnathawh hi a tlahniam emaw, a tipung emaw thei a ni.

A chang chuan HRHS vei naute hian thinlung lam natna dang an nei thei bawk. Entir nan:

• Atrial septal defect (ASD): Thinlung chunglam chamber pahnih (atria) inkar bang chhunga khur awm.
• Ventricular septal defect (VSD): Thinlung hnuai lam chamber pahnih (ventricles) inkar bang chhunga khur awm.
• Atrioventricular canal defect: Thinlung lai takah khur lian tak a awm bakah harsatna dang engemaw zat a awm bawk.

He dinhmun hi engtiang chiahin nge a pawi?

Hypoplastic right heart syndrome (HRHS) hi mi thenkhat tan chuan midang aiin a na zawk thei . Mite chu natna khirh tak nei, oxygen level hniam tak tak nei te chuan enkawl vat an ngai a ni . Mahse, mi ṭhenkhat chuan chutiang chuan an nei lo mai thei a, harsatna tenau chauh an nei. Chutiang mite chu an kum upat hma loh chuan hriatchhuah an ni lo mai thei.

Hetiang dinhmun hi engtiang chiahin nge a awm?

Hei hi thil awmdan tlemte tak a ni tak zet a ni.Zirchiangtute chuan United States-ah hian mi 50,000 aia tlem loin HRHS an nei niin an chhut. Hypoplastic Left Heart Syndrome aiin a tlem zawk a, hei hi thinlung veilam a lo thang tha lo.

Hypoplastic right heart syndrome (HRHS) natna lan chhuahna chu engte nge ni?

A hnuaia mi hi he natna lan chhuah dan hi a ni:

• Kut leh ke vun rawng dum (blue discoloration) a awm. Hei hi cyanosis an ti a . Taksain oxygen a dawng tlem tihna a ni.
• Thâwk harsa. Hei hi dyspnea an ti a .
• Thâwk rang tak. Hei hi tachypnea an ti a .
• Naute hian bawnghnute a in mumal lo.
• Chau vat vat, mut theih reng reng.

I naute hian heng symptoms pakhat emaw a aia tam emaw a neih chuan doctor pan nghal a tha ber.

Eng thilin nge hei hi a thlen?

Dik tak chuan, hypoplastic right heart syndrome (HRHS) awm chhan dik tak hi hmuhchhuah a la ni lo .

Mahse, zirchiangtute chuan genetics hi a inrawlh thei niin an ngai , chhungkaw khata mi engemaw zatin an pian tirh atangin HRHS an nei tih an sawi a ni. Chuvangin zirchian belh a ngai a ni.

Hei hian eng harsatna dang nge a thlen theih?

Hypoplastic Right Heart Syndrome (HRHS) hian harsatna a thlen thei a, chungte chu:

• Taksaah oxygen level hniam. Hei hi hypoxemia an ti a .
• Dinglam atanga thinlung natna.
• Lungphun mumal lo. Hei hi arrhythmia an ti a .

Kum naupang takah chuan HRHS-in a nghawng nasa lutuk lo tan pawh taksaa oxygen level hniam vangin nau pai a awm thei a ni.

He natna hi engtin nge an hriat chhuah?

A châng chuan, healthcare provider chuan naute chu a pum chhunga a la awm laiin HRHS a hmu thei (fetal development) . Ultrasound scan hmangin naute thinlung an hmu thin. Ngun zawka i en duh chuan fetal echocardiogram pawh i ti thei bawk. Heng test pahnih hi non-invasive a ni.

Naute pian hnuah eng test nge tih thin?

Naute a pian hnuah heng non-invasive test te hian naute hian HRHS a nei em tih hriat theih a ni:

• Pulse oximeter (pulse ox): Hei hi clip ang chi hmanraw te tak te a ni a, i kut zungtang emaw beng emaw-ah oxygen level tehna a ni.
• Electrocardiogram (EKG/ECG): Thinlung electric hnathawh dan tehna test a ni.
• Echocardiogram (Echocardiogram - echo): Hei hi ultrasound ang chi sound wave hmanga thinlung thlalak lakna a ni. Thinlung insiam dan te, valve hnathawh dan te, thisen kal dan te a entir thei a ni.
• Heart MRI (Magnetic Resonance Imaging): Magnetic field leh radio wave hmangin thinlung thlalak chipchiar tak a siam thin.

Hei hi eng enkawlna nge ni?

Hypoplastic Right Heart Syndrome (HRHS) hi damdawi hmanga enkawl theih a ni a, minimally invasive procedure hmanga enkawl theih a ni a, surgery hmanga enkawl theih a ni bawk . Heng enkawlna zawng zawng hian a tum ber chu naute lungin thisen leh oxygen a neih theih nan tanpui hi a ni .

Damdawi

• Prostaglandin hi naute ductus arteriosus hawn reng turin hman a ni. He ductus arteriosus hi naute hian a pum chhunga a awm laiin nu hnen atanga oxygenated thisen a dawnna tur tube a ni. Naute chu a pian hnua lung hmanga thawk a chhuah tan avangin he tube hi ni engemaw zat chhungin a khar tlangpui. Mahse, HRHS vei nauteah chuan thinlung dinglam a thawk tha lo a nih avangin he tube hi hawn reng hian lungah thisen a kal theih nan a pui thin.

Minimally invasive tih dan tur a awm

• Doctor chuan naute ductus arteriosus-ah stent (thir mesh ang chi tube te tak te) a dah theih nan catheter an tih tube te tak te a hmang thei a. Hei hian duct chu a inhawng reng a, lungah thisen a kal tha thei bawk.
• A dang chu pulmonary valve perforation a ni . Chutah chuan thisen kalna kawng kaltlangin catheter chu pulmonary valve inhawng tha lo takah an lut a, chu chuan valve chhungah khur a siam a ni. Hei hian thisen chu pulmonary artery-ah a luang lut thei a ni. Doctor thenkhat chuan hei atan hian ablation an tih procedure an hmang bawk.

Inzai

HRHS vei na tak chu enkawl nan operation neih a ngai a ni. Hengte hi stage engemaw zatah tih a ni tlangpui.

• Blalock-Taussig-Thomas (BTT) shunt: Hei hi HRHS vei naute lung chhunga thisen kal lehna atana hman a ni. Synthetic fabric hmangin subclavian artery (collarbone-a artery pakhat) leh pulmonary artery inzawmna siam a ni.
• Thla engemaw zat emaw, kum engemaw zat emaw hnuah chuan surgeon chuan Glenn procedure leh/ emaw Fontan procedure a ti thei a ni . Heng tih dan tin hian thisen kalna tur kawng thar a siam a, chu chu right ventricle thawk tha lo tak vel a ni. A tlangpuiin right ventricle hian thisen chu pulmonary artery-ah a pump lut thin.

Glenn leh Fontan te tih dan hian thisen chu vein lian tak atanga pulmonary artery-ah direct-in a luang lut a, chuta tang chuan lung-ah oxygen la turin a kal a ni.

Enkawlnaah hian harsatna a awm em?

Surgery zawng zawng ang bawkin heng enkawlna hmang hian harsatna awm thei engemaw zat a awm. Enkawl dan azirin harsatna a awm thei. Entir nan:

• Stent hi a awmna hmun atang hian a kal thei a ni.
• BTT shunt surgery hian thisen kalna kawng a ti tawi emaw, nerve a tichhe thei a ni.
• Pulmonary valve perforation hian heartbeat mumal lo (arrhythmia) a thlen thei a, thisen kalna kawng a tichhe thei bawk.
• Surgery lian tak tak hian thisen sang, pleural effusions, cardiac arrest, kidney emaw liver natna emaw, heartbeat mumal lo emaw a thlen thei a ni.

Mahse, lungngai suh. Heng zawng zawng hi Doctor ten an hriattir ang che a, a tul angin hma an la ang.

Eng chen nge tihdam a ngaih?

Enkawl dan azirin dam leh hun a inang lo. Surgery lian tham tak takah hian hun rei ber a ni. I fa chu damdawi inah ni engemaw zat a awm thei a, kar khat aia tam pawh a awm thei bawk. An haw hnuah uluk taka enkawl an ngai dawn a ni.

Ka naute hi hetiang dinhmunah hian a awm a nih chuan eng nge ka beisei theih ang?

Naupan laia oxygen level tlahniam avangin hypoplastic right heart syndrome (HRHS) vei te hian harsatna an tawk thei a, chungte chu:

• Ṭawng chungchangah.
• Memory chungchangah harsatna a awm.
• Kum upa zawkah chuan thinlung, thin, kal natna vei theihna a sang.
• Mood disorder emaw anxiety disorder emaw a awm thei.
• Thil ruahman harsa emaw, harsatna chinfel emaw.

Mahse, heng zawng zawnga pui turin mithiam an awm. Chuvangin lungngai suh.

Hetiang dinhmun hi eng prognosis nge ni?

Severe hypoplastic right heart syndrome (HRHS) vei te hi puitling thlenga operation tel lova dam khawchhuah hi a tlem hle . Mahse, HRHS na lo zawk nei te chuan puitling an nih hma chuan he natna hi an nei tih pawh an hre lo mai thei.

HRHS nei naupang chu engtin nge i enkawl?

I naute enkawlna mamawh chu a dinhmun a nasat danah a innghat ang . A pian hnu lawka zai a nih chuan in a thlen hnuah uluk taka enkawl a ngai dawn a ni. I doctor emaw midwife emaw chuan i naute enkawl dan tur chungchangah thurawn tha tak tak a pe ang che.

Engtikah nge Doctor i hmuh a ngaih?

Naute operation nei tawh chuan doctor pan a ngai dawn a, follow-up appointment neih a ngai dawn a ni . Fimkhur tur leh thil i hriat chuan doctor hnena hrilh nghal tur te an hrilh ang che. I naute dinhmun chungchanga zawhna emaw, ngaihtuahna emaw i neih chuan doctor ko nghal rawh.

I fain a rilru put hmang zir emaw, tihreh emaw a harsatna a neih chuan, chutiang lama ṭanpui theitu specialist dang pawh i hmu thei bawk. I doctor chuan a tul angin kaihhruaina a pe ang.

Doctor hnenah eng zawhna nge ka zawh ang?

I fa doctor hnena zawhna i zawh theih ṭhenkhat chu hetiang hi a ni:

• Ka fa hian operation a mamawh em?
• Engtiang chiahin nge operation tih vat a ngai?
• Surgery engzat nge neih a ngai dawn a, engtikah nge tih a nih ang?
• Hetiang dinhmun bik nei ka fa tan hian eng nge a hmalam hun?

I fa hian thinlung natna a nei tih hriat a harsat zia ka hrethiam. Amaherawhchu, tunlaia nausen piangte hian thlah hmasa lama an la neih ngai loh enkawlna an nei a. Hetih lai hian i fa tana mi chak ber i ni. HRHS chungchang zir rawh. Thil chiang lo eng pawh doctor zawt rawh. I fa tana tha ber tur chu i doctor nen inbia ang che.

A tawp berah chuan hei hi hre reng ang che (Take-Home Message) .

Hypoplastic Right Heart Syndrome (HRHS) hi nausen piang chhuak, thinlung dinglam hi a lo thanglian tha tawk lo tihna a ni. Hei hian taksain oxygen a dawn zat a ti tlem a ni. A hmaa hriat chhuah leh enkawlna \ha (damdawi, operation tenau, a nih loh leh surgery lian) chuan naupang nun a ti \ha thei a ni . Chutiang dinhmunah chuan nu leh pa angin i doctor-te nena inhriattir leh thawhho a pawimawh hle. Nangmah chauh i ni lo tih hre reng ang che. I mamawh tanpuina i hmu thei a ni.

` Hypoplastic Right Heart Syndrome, HRHS, Nausen Lung natna, Pianpui Lung chhiatna, Cyanosis, Thâwk harsa, Lung zai