So wo ba kumaa no koma nifa nnyin yiye? (Hypoplastic Koma Nifa mu Yare) .

Na sɛ wɔwoo wo ba kumaa no a ɔwɔ komayare nso ɛ? Wo de susuw sɛnea ɛno bɛyɛ awerɛhow no ho hwɛ. Wɔwo nkokoaa binom a wɔn koma nifa nnyin yiye. Nnuruyɛfo frɛ eyi Hypoplastic Right Heart Syndrome. Eyi ma mframa pa dodow a akokoaa no nya no so tew. Momma yɛnka eyi ho asɛm nkɔ akyiri, ɛnte saa?

Dɛn ne Hypoplastic Koma Nifa mu Yare?

Sɛ yɛbɛka no tiawa a, Hypoplastic Right Heart Syndrome yɛ komayare a ɛntaa nsi a wɔwo nkokoaa binom . Nea ɛba wɔ eyi mu ne sɛ akokoaa no koma nifa no afã horow bi ntumi nnyin koraa. Fa no sɛ ɛte sɛ pɔmpɛ ketewaa bi. Koma no nifa ne nea egye mogya a mframa pa nnim no fi nipadua no mu na ɛde kɔ ahurututu mu ma enya mframa pa. Enti, sɛ saa nifa yi ankɔ so yiye a, ɛrenyɛ n’adwuma yiye.

Wɔ saa tebea yi mu no, koma no afã horow a edidi so yi betumi aka:

• Tricuspid valve: Eyi te sɛ ɔpon a ɛda koma no nifa so ahurututu ne nifa ahurututu ntam. Sɛ ɛnyɛ adwuma yiye a, esiw mogya a ɛbɛkɔ no kwan.
• Ahurututu mu ntini: Eyi ne ahurututu mu ntini a ɛde mogya fi ahurututu nifa mu kɔ ahurututu mu no fi ase.
• Right ventricle: Eyi ne ɔdan titiriw a wɔde mogya gu mu wɔ koma no nifa so. Sɛ ɛbɛyɛ ketewaa a, ɛremma mogya kakraa bi.
• Ahurututu mu ntini: Eyi ne ntini kɛse a ɛde mogya fi ahurututu nifa mu kɔ ahurututu mu.

Ɛdenam sɛnea tebea no mu yɛ den anaasɛ emu nyɛ den so no, ebia koma no afã horow yi dwumadi so bɛtew anaasɛ akɔ soro.

Ɛtɔ mmere bi a, nkokoaa a wɔwɔ HRHS nso betumi anya komayare afoforo. Nhwɛsoɔ:

• Atrial septal defect (ASD): Tokuru a ɛwɔ ɔfasu no mu wɔ koma no atifi adan abien (atria) ntam.
• Ventricular septal defect (VSD): Tokuru a ɛwɔ ɔfasu no mu wɔ koma no ase adan abien (ventricles) ntam.
• Atrioventricular canal defect: Tokuru kɛse bi a ɛwɔ koma no mfinimfini a ɔhaw afoforo pii ka ho.

Tebea yi yɛ aniberesɛm dɛn?

Hypoplastic right heart syndrome (HRHS) betumi ayɛ nea anibere wom kɛse ama nnipa binom sen afoforo . Nnipa a wɔwɔ tebea horow a emu yɛ den te sɛ oxygen dodow a ɛba fam koraa , hia ayaresa ntɛm ara . Nanso, ebia nnipa binom rennya bi a emu yɛ den saa, na ɔhaw nketenkete nkutoo na ɛwɔ hɔ. Ebia wɔrenhu nnipa a wɔte saa no kosi sɛ wobenyin.

Saa tebea yi abu so dɛn?

Eyi yɛ tebea a ɛntaa nsi koraa ampa.Nhwehwɛmufo bu akontaa sɛ nnipa a wɔwɔ United States no nnu 50,000 na wɔwɔ HRHS. Ɛntaa nsi sen Hypoplastic Left Heart Syndrome, tebea a ɛma koma no benkum ntumi nkɔ yiye.

Dɛn ne hypoplastic right heart syndrome (HRHS) ho sɛnkyerɛnne ahorow?

Nea edidi so yi yɛ tebea yi ho sɛnkyerɛnne:

• Nsa ne anan honam ani kɔla a ɛsakra bruu. Wɔfrɛ eyi cyanosis . Ɛkyerɛ sɛ nipadua no nnya mframa pa a ɛdɔɔso.
• Ɔhome a ɛyɛ den. Wɔfrɛ eyi sɛ ɔhome a ɛyɛ den .
• Ahome a wɔhome ntɛmntɛm. Wɔfrɛ eyi tachypnea .
• Akokoaa no nnom nufusu yiye.
• Ɔbrɛ a ɛba ntɛmntɛm, nna a ɔte nka bere nyinaa.

Sɛ wo ba no wɔ saa sɛnkyerɛnne ahorow yi mu biako anaa nea ɛboro saa a, ɛbɛyɛ papa sɛ wobɛkɔ oduruyɛfo nkyɛn ntɛm ara.

Dɛn na ɛde eyi ba?

Nokwarem no, wonnya nhuu nea ɛde koma nifa a ɛma mogya tu (HRHS) ba ankasa .

Nanso, nhwehwɛmufo gye di sɛ ebia awosu na ɛka ho , efisɛ wɔabɔ amanneɛ sɛ abusua koro mufo pii wɔ HRHS bere a wɔwoo wɔn no. Enti, nhwehwɛmu foforo ho hia.

Nsɛnnennen afoforo bɛn na eyi betumi de aba?

Hypoplastic Right Heart Syndrome (HRHS) betumi de nsɛnnennen te sɛ:

• Oxygen dodow a ɛba fam wɔ nipadua no mu. Wɔfrɛ eyi hypoxemia .
• Koma a ɛyɛ adwuma yiye wɔ nifa so.
• Koma a ɛbɔ a ɛnteɛ. Wɔfrɛ eyi arrhythmia .

Wɔ mmofraase, wɔ wɔn a HRHS nka wɔn kɛse saa mpo fam no, nyinsɛn betumi atu esiane oxygen a ɛba nipadua no mu a ɛba fam nti.

Ɔkwan bɛn so na wohu yare yi?

Ɛtɔ mmere bi a, akwahosan ho ɔyaresafo betumi ahu HRHS bere a akokoaa no da so ara wɔ awotwaa mu (akokoaa no nkɔso) . Wɔde ultrasound scan di dwuma de hu akokoaa no koma. Sɛ wopɛ sɛ wohwehwɛ mu yiye a, wubetumi nso ayɛ awotwaa mu echocardiogram . Saa sɔhwɛ abien yi nyinaa nyɛ nea wɔde hyɛ nipadua mu.

Nhwehwɛmu bɛn na wɔyɛ bere a wɔawo akokoaa no akyi?

Sɛ wɔwo akokoaa no wie a, wobetumi ayɛ nhwehwɛmu ahorow a ɛnyɛ nea wɔde hyɛ nipadua mu yi de ahu sɛ akokoaa no wɔ HRHS anaa:

• Pulse oximeter (pulse ox): Eyi yɛ afiri ketewaa bi a ɛte sɛ clip a ɛkyerɛ oxygen dodow denam nea ɛde bata wo nsateaa anaa w’aso ho no so.
• Electrocardiogram (EKG/ECG): Sɔhwɛ a ɛkyerɛ sɛnea koma no mu anyinam ahoɔden yɛ adwuma.
• Echocardiogram (Echocardiogram - echo): Eyi yɛ sɔhwɛ a wɔde nnyigyei asorɔkye a ɛte sɛ ultrasound di dwuma de twa koma no mfonini. Ebetumi akyerɛ sɛnea koma no te, sɛnea ntini no yɛ adwuma, ne sɛnea mogya no sen.
• Koma MRI (Magnetic Resonance Imaging): Ɛde magnetic field ne radio asorɔkye di dwuma de yɛ koma no ho mfonini a ɛkɔ akyiri.

Dɛn ne ayaresa ahorow a wɔde sa eyi?

Hypoplastic right heart syndrome (HRHS) ayaresa betumi ayɛ nnuru, ayaresa a ɛnyɛ den pii, anaa oprehyɛn . Botae titiriw a ɛwɔ saa ayaresa ahorow yi nyinaa mu ne sɛ ɛbɛboa akokoaa no ahurututu ma wanya mogya ne mframa pa a ɛdɔɔso .

Aduro

• Wɔde prostaglandin di dwuma de boa ma akokoaa no ductus arteriosus no bue. Saa ductus arteriosus yi yɛ afiri bi a akokoaa no de nya mogya a oxygen wom fi ɛna no hɔ bere a ɔwɔ awotwaa mu no. Bere a akokoaa no fi ase home fa ahurututu no mu wɔ awo akyi no, mpɛn pii no, saa afiri yi to mu wɔ nna kakraa bi mu. Nanso, wɔ akokoaa a ɔwɔ HRHS mu no, esiane sɛ koma no fã nifa ntumi nyɛ adwuma yiye nti, sɛ wɔma saa afiri yi bue a, ɛboa ma mogya kɔ ahurututu no mu.

Akwan a wɔfa so yɛ nneɛma a ɛnyɛ den koraa

• Oduruyɛfo betumi de afiri teateaa bi a wɔfrɛ no catheter adi dwuma de stent (afiri ketewaa bi a ɛte sɛ dade ahama) ahyɛ akokoaa no ductus arteriosus mu. Eyi ma ntini no bue na ɛma mogya kɔ ahurututu no mu yiye.
• Foforo nso ne ahurututu mu ntini a ɛtow . Wɔ eyi mu no, wɔde catheter bi fa mogya ntini bi mu kɔ ahurututu mu ntini a enbue yiye no mu, na ɛma tokuru bi ba ahurututu no mu. Eyi ma mogya kɔ ahurututu mu ntini no mu. Nnuruyɛfo binom nso de ɔkwan bi a wɔfrɛ no ablation di dwuma ma eyi.

Sɛɛgyiri

HRHS a emu yɛ den no hia oprehyɛn de sa. Wɔtaa yɛ eyinom wɔ akwan horow pii so.

• Blalock-Taussig-Thomas (BTT) shunt: Wɔde eyi di dwuma de ma mogya san kɔ akokoaa a ɔwɔ HRHS ahurututu mu. Ɛde ntama a wɔayɛ di dwuma de ma abusuabɔ bi ba subclavian artery (ntini bi a ɛwɔ kɔla dompe mu) ne ahurututu mu ntini no ntam.
• Asram anaa mfe kakraa bi akyi no, oduruyɛfo a ɔyɛ oprehyɛn betumi ayɛ Glenn oprehyɛn ne/anaasɛ Fontan oprehyɛn . Saa akwan yi mu biara ma wonya ɔkwan foforo a mogya fa so twa ahurututu nifa a entumi nyɛ adwuma yiye no ho hyia. Mpɛn pii no, nifa so ahurututu no de mogya kɔ ahurututu mu ntini no mu.

Glenn ne Fontan akwan horow no ma mogya fi ntini kɛse bi mu kɔ ahurututu mu ntini no mu tẽẽ, na efi hɔ kɔ ahurututu no mu kɔfa mframa pa.

So nsɛnnennen bi wɔ ayaresa no mu?

Sɛnea ɛte wɔ oprehyɛn nyinaa ho no, nsɛnnennen bi wɔ hɔ a ebetumi de saa ayaresa ahorow yi aba. Nsɛnnennen no betumi ayɛ soronko a egyina ayaresa no so. Nhwɛsoɔ:

• Ebia stent no befi baabi a na ɛwɔ kan no.
• BTT shunt oprehyɛn betumi ama mogya ntini no ayɛ ketewaa anaasɛ asɛe ntini bi.
• Ahurututu mu ntini a ɛtow betumi ama koma no abɔ a ɛnteɛ (arrhythmia) anaasɛ asɛe mogya ntini bi.
• Oprehyɛn akɛse betumi ama mogya mmoroso, ahurututu mu nsu a ɛretu, koma atɔ, asaabo anaa mmerɛbo mu yare, anaa koma a ɛbɔ a ɛnteɛ.

Nanso mma ɛnhaw wo. Nnuruyɛfo no bɛbɔ wo amanneɛ wɔ eyinom nyinaa ho na wɔayɛ nneɛma a ɛho hia.

Bere tenten ahe na egye ansa na asa?

Bere a wɔde ma obi ho tɔ no gu ahorow a egyina ayaresa no so. Oprehyɛn akɛse gye bere tenten sen biara. Ebia wo ba no bɛtra ayaresabea nna pii, ebia bɛboro dapɛn biako. Ɛho behia sɛ wɔhwɛ wɔn yiye bere a wɔasan aba fie no.

Sɛ me ba no wɔ tebea yi mu a, dɛn na metumi ahwɛ kwan?

Esiane sɛ oxygen dodow sua wɔ wɔn nkwa nna mfiase nti, nnipa a wɔwɔ hypoplastic right heart syndrome (HRHS) no betumi ahyia nsɛnnennen te sɛ:

• Kasa ho nsɛm.
• Nsɛm a ɛfa nkae ho.
• Asiane a ɛkɔ soro sɛ obi benya koma, mmerɛbo, anaa asaabo mu yare bere a obi anyin no.
• Adwene mu haw anaa dadwen mu haw.
• Ɛyɛ den sɛ wobɛyɛ biribi ho nhyehyɛe anaa wobedi ɔhaw bi ho dwuma.

Nanso, abenfo wɔ hɔ a wɔbɛboa wɔ eyinom nyinaa mu. Enti mma ɛnhaw wo.

Dɛn ne tebea yi ho nkɔmhyɛ?

Ɛntaa mma koraa sɛ nnipa a wɔwɔ koma nifa a ɛma mogya tu (HRHS) a emu yɛ den no bɛtra ase akosi wɔn mpanyin afe so a wɔnyɛ wɔn oprehyɛn . Nanso, ebia nnipa a wɔwɔ HRHS a emu nyɛ den pii no renhu mpo sɛ wɔwɔ tebea no kosi sɛ wɔbɛyɛ mpanyimfo.

Wobɛyɛ dɛn ahwɛ abofra a ɔwɔ HRHS?

Ɔhwɛ a wo ba no hia no begyina sɛnea ne tebea no mu yɛ den no so . Sɛ wɔyɛɛ no ​​oprehyɛn wɔ awo akyi pɛɛ a, ebehia sɛ wɔhwɛ no yiye paa bere a wadu fie no. Wo duruyɛfo anaa awogyefo bɛma wo afotu pa wɔ sɛnea wobɛhwɛ wo ba no ho.

Bere bɛn na ɛsɛ sɛ wokɔ oduruyɛfo nkyɛn?

Ɛho behia sɛ akokoaa a wɔayɛ no oprehyɛn kɔ oduruyɛfo nkyɛn kɔhwɛ no . Wɔbɛkyerɛ wo nea ɛsɛ sɛ wohwɛ yiye ne nea ɛsɛ sɛ woka kyerɛ oduruyɛfo no ntɛm ara sɛ wuhu biribi a. Sɛ wowɔ nsɛmmisa anaa dadwen bi wɔ wo ba no tebea ho a, frɛ oduruyɛfo no ntɛm ara.

Sɛ wo ba no wɔ ɔhaw ahorow wɔ ne nkate horow a obesua anaasɛ ɔbɛhwɛ so a, wubetumi nso anya nnuruyɛfo afoforo a wobetumi aboa wɔ saa mmeae no. Wo duruyɛfo de akwankyerɛ a ɛho hia bɛma wo.

Nsɛm bɛn na ɛsɛ sɛ mibisa oduruyɛfo no?

Nsɛm bi a wubetumi abisa wo ba no oduruyɛfo ni:

• So ɛsɛ sɛ wɔyɛ me ba oprehyɛn?
• Ɛho hia sɛ wɔyɛ wo oprehyɛn no ntɛm dɛn?
• Oprehyɛn dodow ahe na ebehia, na bere bɛn na wɔbɛyɛ?
• Dɛn ne anidaso a ɛwɔ hɔ ma me ba a ɔwɔ tebea pɔtee yi?

Mete sɛnea ɛyɛ den sɛ wubehu sɛ wo ba no wɔ komayare bi ase. Nanso, mmofra a wɔwo wɔn nnɛ no wɔ ayaresa a na awo ntoatoaso a atwam no ntumi nnya. Wo ne onipa a ne ho yɛ den sen biara a wubetumi ayɛ ama wo ba saa bere yi. Sua HRHS ho ade. Bisa wo duruyɛfo no biribiara a emu nna hɔ ho asɛm. Wo ne wo duruyɛfo nkasa na si nea eye ma wo ba no ho gyinae.

Awiei koraa no, kae eyi (Take-Home Message) .

Hypoplastic Right Heart Syndrome (HRHS) yɛ tebea a ɛntaa nsi a wɔde wo nkokoaa, a koma no fã nifa nnyin yiye. Eyi ma mframa pa dodow a nipadua no nya no so tew. Sɛ wohu abofra no ntɛm na wɔsa no yare a ɛfata (nnuru, oprehyɛn ketewaa, anaa oprehyɛn kɛse) a ebetumi ama abofra no asetra atu mpɔn . Wɔ tebea a ɛte saa mu no, ɛho hia paa sɛ wo sɛ ɔwofo sɛ wobɔ wo amanneɛ na wo ne wo nnuruyɛfo bom yɛ adwuma. Kae sɛ ɛnyɛ wo nko ara. Wubetumi anya mmoa a wuhia.

` Hypoplastic Right Heart Syndrome, HRHS, Nkokoaa Koma Yare, Koma a Wɔde Awo Wɔn, Cyanosis, Ɔhome a Ɛyɛ Den, Koma Oprehyɛn