I naute hian a thinlungah khandaih ang maia vein a nei em? He natna hmuh tur awm lo (Scimitar Syndrome) hi i zir dawn teh ang!

"Aw, kan tê tê hian thawk a harsat hmel hle a, nau hnute a pek lai pawhin a thawk a chhuak vek... a chang chuan a vun chu a sen ang maiin a lang..." Hetiang thil hi i tawng tawh em? A nih loh leh i hriat mi naute chungah hetiang thil hi i hmu tawh em? A châng chuan heng symptoms te hi naute pianpui thinlung lama harsatna engemaw zat avanga lo awm thei a ni. Vawiin hian chutiang thinlung natna hmuh tur awm lo, mahse pawimawh tak pakhat chu kan sawi dawn a ni.

Scimitar Syndrome hi eng nge ni?

Hei hi Scimitar Syndrome an ti a . A hming hi a mak deuhin a ri a ni lawm ni? ‘Scimitar’ tih hian khandaih tip hrual, hmanlai Middle East-a hman thin kha a kawk a ni. Hetiang dinhmunah hian a chang chuan lung dinglam atanga thinlung thlenga thisen phurtu pulmonary vein chu X-ray-ah khandaih curved ang maiin a lang thin. He hming hi engtin nge a lo awm tih hi a ngaihnawm khawp mai lo maw?

A awlsam zawngin, hei hi pianpui thinlung natna a ni . Chu chu naute pum chhunga a la awm laia lo awm (condition) tihna a ni. Doctor-te chuan hei hi natna chi khat, Partial Anomalous Pulmonary Venous Return (PAPVR) an ti a. Chu chu lung atanga thisen kalna kawngte thinlung nena inzawm danah chhiatna a awm tihna a ni.

Hetiang dinhmunah hian symptom lian tak tak hmuh tur a awm a:

• Lung dinglam hi a lo thang kim lo (hypoplastic right lung). Chumi awmzia chu a lehlamah lung aiin a te zawk a, a thawk tha lo zawk tihna a ni.
• Lung dinglam (right pulmonary artery)-a thisen phurtu main artery chu a lo thang tha lo hle (hypoplastic right pulmonary artery). Hei hian lung dinglam thlenga thisen kal chu a ti tlem thei bawk.

Kan thinlung hnathawh mak tak takte hi tlem tal kan hrethiam thei ang em?

Chuvangin, Scimitar Syndrome chungchanga thil thleng dik tak hriat chian nan thinlung hrisel hnathawh dan hi han en hmasa ila, a tha em? Kan thinlung hi pump chak tak, rintlak tak angin han ngaihtuah teh. Kan taksa pum puiah thisen a pump chhuak a, zan leh zan a ni.

Hei hi a thleng tlangpui chu a ni:

1. Hman, oxygen- tlachham thisen taksa hmun hrang hrang atanga lakkhawm a, thinlung dinglamah a kir leh thin.

2. He thisen oxygen tlachham hi thinlung dinglam atanga lungah a thawn a, chu chu pulmonary arteries kaltlangin a kal a ni .

3. Lung chhungah hian he thisen hi `oxygenated` a ni a, tui bawlhhlawh chu filter a, tihthianghlim a nih ang bawkin.

4. Tichuan, he oxygen tamna thisen hi lung veilam lamah chuan pulmonary veins kaltlangin a kir leh a, chu chu lung atanga thinlunga oxygen phurtu vein a ni

5. Thinlung veilam chuan he oxygen tamna thisen hi taksa pum puiah a pump lut a, thisen kalna kawng lian ber, aorta kaltlangin a pump lut a ni.

Hei hi kan taksaa cell tin hian oxygen kan hmuh dan a ni. Hei hi thil kalphung buaithlak leh mak tak a ni lo em ni?

Chuti a nih chuan Scimitar Syndrome-ah hian eng nge thleng?

Tunah chuan thinlung hrisel hnathawh dan chu i hrethiam tawh mai thei. Scimitar Syndrome vei chunga thil thleng chu, thisen lo chhuak tur chu lung atanga oxygen lakkhawm a, thinlung veilam lam pan tur, chutiang lam pan ai chuan , kawng dik lo zawhin thinlung dinglamah a kal thin.

In pakhata tui rawn thlentu pipe chu hmun dik lovah, tui dahna hman tawhah pawh i connect leh mai thei ang tih han ngaihtuah teh. Tichuan thil thleng tur chu a thleng dik lo ang. Tui thianghlim leh tui bawlhhlawh inzawmkhawmna dinhmun a lo awm thei a ni.

Hei hian vein pakhat emaw a aia tam emaw (pulmonary veins), a tlangpuiin lung dinglam atanga oxygenated thisen phur thin chu thinlung veilam ni lovin thinlung dinglamah a inzawm a ni. Hei hian oxygen nei thisen thenkhat chu lungah a luang kir leh thei a ni. Tin, thinlung dinglam chu a thawkrim zawk bawk.

A châng chuan, lung dinglam hian pulmonary arteries aṭangin thisen a hmu lo va, aorta aṭangin direct-in thisen a dawng ṭhin. Aorta hi a takah chuan taksa pum puia thisen phurtu thisen kalna bulpui ber a ni. Chuvangin chutah pawh chuan harsatna tlem a awm. Hei hian thinlungah a tul lovah harsatna a siam thei a ni.

Hetiang dinhmun hi engtiang chiahin nge a awm? A tul lovah kan hlau tur em ni?

Hei hi i tan chuan hlauhawm tak angin a lang thei a, mahse Scimitar Syndrome hi a awm lo hle. Statistics atanga a lan dan chuan naute 100,000 zingah pakhat atanga pathum chauh a vei a ni. Hmeichhiaah pawh mipa aiin a let hnih zetin a tam zawk tih hmuhchhuah a ni bawk.

Chuvangin, a tul lovah hlau suh, hei hi a tlem hle a ni. Amaherawhchu hei hi hriat chian a pawimawh hle.

Hei hi eng chhan nge ni? Engvangin nge hetiang hi a lo awm?

Vawi tam tak chu doctor-te pawhin Scimitar Syndrome thlen chhan dik tak hriat harsa an ti fo thin. Mahse, nausen pianpui thinlung natna a neih theihna tur risk factor thenkhat a awm a. Chung zingah chuan:

• Genetics: A châng chuan thlah kal zelah genetic condition kal zel hian nghawng a nei thei a.
• Naupai laia damdawi ei \henkhat: Entirnan, hmai vun damdawi \henkhat `(Isotretinoin - Accutane®)` te hi a hlauhawm thei hle. Chuvang chuan doctor thurawn tel lova naupai laiin damdawi engmah ei loh tur a ni.
• Environment factors: Naupai laiin nuin zu a in emaw, zu a in emaw a nih chuan, chutiang thil chuan naute thinlung hmasawnna a nghawng thei a ni.
• Nua natna thenkhat: Entirnan, nuin naupai laiin rubella ang chi natna a vei chuan naute thinlung hmasawnna a nghawng thei a ni.
• Nu natna khirh tak, thunun theih loh: Naupai laiin zunthlum ang chi natna te hi uluk taka thunun a nih loh chuan naute pawh a nghawng thei a ni.

A pawimawh ber chu, a tam zawkah chuan hei hi tumah sualna a ni lo. Chuvangin nangmah leh nangmah in dem suh.

Eng symptom nge awm?

Scimitar Syndrome symptoms hi mi zawng zawng tan a inang vek lo. Naute thenkhat chu an pian hnu lawkah symptoms an nei thei . Chutiang huna fimkhur tur thilte chu:

• Nau hnute pek laiin thawk harsa leh chok.
• Vun leh hmui rawng blue/gray (cyanosis): Hei hi taksain oxygen a dawn tlem vang a ni.
• Thâwk rang tak.
• Ke, pum leh mit vel a hring (edema) a awm.

Amaherawhchu, naute \henkhat chuan an upat hma chuan natna lanchhuahna an nei lo mai thei. Ṭhenkhat chu puitlin thlengin chhinchhiahna nei lovin an awm thei. Mahse, puitlin hunah natna lan chhuah dan a lo lang a nih chuan:

• Lung infection awm fo thin.
• Exercise emaw exercise emaw tih laiin thawk harsa (dyspnea).

Engtin nge doctor-te hian hei hi an hriat chian? (Diagnosis) tih a ni.

Doctor-te chuan Scimitar Syndrome hriat theihna turin test eng emaw zat an nei a. Chungte chuan naute emaw puitling emaw lung dinglam lian leh te leh lung dinglam thisen kalna dinhmun te a en thei a ni.

• Chest X-ray: Hei hian thinlung, lung, thawkna kawng leh thisen kalna kawng hrang hrangte chu a rough idea a pe thei a ni. Hemi chungchangah hian ‘Scimitar’ lem i hmu mai thei.
• CT scan: Hei hian angle hrang hrang atanga X-ray lak a, taksa chhung lam chipchiar taka three-dimensional image siam a ni.
• MR angiography: Hei hi magnetic wave leh computer hmanga thisen kalna kawng chipchiar taka en theihna test a ni.
• Transthoracic echocardiogram (TTE): Hei hi ultrasound scan ang a ni. Thinlung hnathawh dan te, chamber te, valve te, thisen kal dan te ngun takin a en thei a ni.
• Cardiac catheterization: Hetiang tih danah hian thisen kalna kawng hrang hrangah dye bik inject a ni a, X-ray hmangin thisen kal dan tur enfiah a ni. Thinlung leh lung chhunga pressure tehna atan pawh hman a ni.

Heng test chungchang hi zawh hreh suh. Doctor-te chuan hengte hi a natna dik taka hriat theih nan leh enkawlna tha ber pek nan an ti thin.

Hei hi enkawl dan tur a awm em? (Enkawl dan tur) .

Ni e, Scimitar Syndrome enkawlna ber chu operation a ni.

• Naute tan chuan doctor-te chuan he natna hi an hriat chuan, an dam chhung thla hnih chhungin operation nei turin an thlem tlangpui . A chhan chu he tlin lohna hi siam that a hma chuan naute thanlenna leh nakin hun hriselna atan a tha zawk a ni.
• Puitling tan chuan .Scimitar Syndrome vei zawng zawng hian natna lan chhuah dan na tak (e.g., lung infection tam tak, thawk hahna nasa tak) an neih loh chuan operation an mamawh vek dawn lo. Mahse, symptoms a nasat chuan surgery a ngai mai thei.

Cardiac surgeon-in operation a neih chhunga a tih theih tur thil engemaw zat a awm a:

• Pulmonary vein, hmun dik lo taka inzawm , chu thinlung veilam lamah rerouted a ni. A chang chuan hei atan hian special patch an hmang thin.
• Chutiang a nih loh chuan vein chu thinlung veilam lamah direct-in a insiam lut a ni.
• A tlem hle a, pneumonectomy hi dinglam lung, a lo thang lo, a thawk tha lo chu paih vek turin tih theih a ni. Mahse, hei hi duhthlan tur dang a awm loh hunah chauh tih a ni.

Hei hi kan ven theih dan tur a awm em?

Dik tak chuan Scimitar Syndrome ang chi congenital heart defect hi a pum puia ven theih a ni lo a, a chhan chu genetic factors pawhin a nghawng a ni.

Mahse, naupai laiin nuin naute hrisel a neih theihna tur a tih theih tur thil a awm a:

• Naupai laiin zu, cigarette leh ruihhlo dangte chu pumpelh vek tur a ni.
• Damdawi eng pawh i ei hmain i doctor nen a hlauhawmna leh a thatna chungchang sawi hmasa phawt ang che.
• Rubella vaccine i la dawng lo a nih chuan naupai hmaa lak a pawimawh a, naupai laiin rubella kai hian naute thinlung a nghawng thei a ni.
• Diabetes ang chi natna khirh tak tak i neih chuan naupai laiin control tha rawh.
• Doctor-in a tih angin folic acid leh nau neih hmaa vitamin dangte ei thin ang che.

Hetiang dinhmun nen hian nun chu eng ang nge ni ang? (Hmalam hun thlirna)

Scimitar Syndrome vanga operation nei thinte hian hun rei tak chhunga result tha tak tak an nei tlangpui . Mi tam zawk chuan operation hnuah symptom an nei leh tawh lo.

Mahse, a chang chuan a bik takin naute leh naupang tê têah chuan rerouted pulmonary vein chu a tawi thei hle. Chutiang a nih chuan enkawl belh a ngai mai thei.

Scimitar Syndrome vei puitling thenkhat chuan natna lanchhuahna nei lovin nun hrisel takin an hmang thei a. Mahse, puitling dangte tan chuan he natna hian thinlung lam harsatna dang a thlen thei a ni. Chutiang hunah chuan quality of life a nghawng thei a ni. Symptoms a lo awm chuan (thaw hah lutuk, lung infection ang chi) surgery neih a ngai mai thei. I lung chhunga thisen kalna kawng (arteries)-a thisen sang a awm leh awm loh enfiah nan test bik i neih a ngai thei bawk.

Scimitar Syndrome nena inzawm thinlung natna dang a awm em?

Ni e, Scimitar Syndrome vei tam tak chuan rilru lam harsatna dang an nei thei bawk. Ṭhenkhat chu thinlung lama harsatna hrang hrang neia piang an ni. A nih loh leh, Scimitar Syndrome hian harsatna dang a thlen thei bawk.

Thil awmdan dang inzawm thei chu:

• Atrial Septal Defect (ASD): Thinlung chunglam pindan pahnih inkar khur a awm.
• Horseshoe lung: Lung hmun hnih inzawmna dik lo.
• Pulmonary hypertension: Pulmonary hypertension: Chuap thisen kalna kawng (arteries) a pressure sang.
• Pulmonary sequestration (accessory lung): Lung tissue atanga chhuak, thawk thei lo, thisen supply mumal lo, inthen darh.
• Tetralogy of Fallot: Naute lung lama thisen pump chhuah nan enkawl ngai, thinlung natna tlanglawn tak a ni.
• Ventricular Septal Defect (VSD): Thinlung hnuai lam pumping chamber pahnih inkar khur a awm.

Chûng thu hriat chu a hlauhawm lehzual thei a ni. Mahse, heng zawng zawng hi damdawi lam enkawlna a awm vek a ni. A pawimawh ber chu i doctor nen inbia a, i dinhmun dik tak hriatthiam a, i mamawh enkawlna i hmuh hi a ni.

Doctor zawh tur zawhna pawimawh

Nangmah emaw i fa emaw hian Scimitar Syndrome i nei tih i hriat chuan i doctor hnenah hetiang zawhna hi i zawt thei ang:

• Scimitar Syndrome thlentu awm thei ber chu eng nge ni?
• Kei/ka fa hian operation a mamawh em?
• Surgery i neih loh chuan eng nge thleng?
• Scimitar Syndrome nei hian thinlung hriselna hi engtin nge kan enkawl ang?
• Hetiang dinhmun hi ka neih chuan ka fa pawhin a vei theihna chance engzat nge awm?
• Scimitar Syndrome nei fa hmasa ber ka neih chuan ka fa pahnihna pawhin a vei theihna chance engzat nge awm?

Heng zawhnate hi zawh hlau leh zak ngai suh. I rilrua rinhlelhna awm zawng zawng tihfai a pawimawh hle.

A tawp berah chuan thil hriat reng tur (Take-Home Message) .

Scimitar Syndrome hi heart defect awm lo, pianpui atanga lo chhuak a ni a, a bul berah chuan lung dinglam leh lung nena inzawm thisen kalna kawngte a nghawng ber a ni.

Naute thenkhat chuan natna lanchhuahna engmah an lantir lo thei a, thenkhat erawh chuan an dam chhung thla hnih chhungin zai a ngai thei bawk.

Naute operation hmasa lama mamawhte chuan a hnuah enkawl tam zawk an mamawh mai thei a, a chhan chu thisen kalna kawng dang (diverted blood vessel) chu a zim vang a ni. Amaherawhchu, Scimitar Syndrome vei puitling leh naupang tam tak surgery nei te chu hriselna tha tak an nei a, nun pangngaiin an khawsa thin.

He thu hi i tan a \angkai ngei kan beisei. Hemi chungchanga zawhna dang i neih chuan i doctor nen inbia ngei ang che. Hrisel takin awm rawh!

`Scimitar Syndrome, lung natna, nau piangsual, lung, thisen kal dan, nausen natna lan chhuah dan, zai dan te