Kua rerekē tō ngākau pēnei? Me kōrero tātou mō te Hypertrophic Cardiomyopathy (HCM)!

Kua rongo kē koe i tētahi mate manawa e matotoru haere ana te uaua o te manawa, he āhua uaua? I ētahi wā, kāore pea koe e mōhio kei a koe tēnei mate, nā te mea kāore ētahi tāngata e pāngia e tētahi tohu. Engari he mea nui kia mōhio koe ki tēnei mate nā te mea ka pā ki te manawa. I tēnei rā ka kōrero tātou mō tēnei mate e kiia nei ko te hypertrophic cardiomyopathy, ko te `HCM' rānei i waenga i ngā tākuta. Kaua e manukanuka, ka kōrerohia e tātou he mea ngāwari noa iho, i roto i tētahi huarahi e mārama ai koe.

Kia kite tātou i te tuatahi he aha te HCM?

Ki te whakarapopototanga, he mate uaua te hypertrophic cardiomyopathy (HCM) e pā ana ki ngā uaua o tō ngākau. E rua ngā take matua e pā ai:

• Te matotoru o te uaua o te ngākau: Ina koa ko ngā rūma o raro o te ngākau, e kīia nei e tātou ko ngā ventricles, he matotoru ake ngā pakitara.
• Te pakari o te pūkahukahu maui: Ka pakari te ruma matua i te taha maui o te ngākau (te pūkahukahu maui), ka kore e tino ngāwari, pērā i te rapa.
• Ngā huringa o te takirere mitral: Ka puta pea ngā huringa o te mahi a te takirere mitral, he takirere nui i roto i te ngākau.
• Ngā huringa pūtau: Ka puta ētahi āhuatanga rerekē i roto i ngā pūtau uaua o te ngākau (ngā huringa pūtau).

Ka taea e ēnei mea te whakararu i te mahi a te ngākau.

He aha te pānga o tēnei āhuatanga HCM ki tō tinana?

Āe, kia kite tātou he aha ngā pānga o ēnei huringa ki te tinana.

Te matotoru o te uaua o te ngākau

Koinei te tohu matua e kitea ana i roto i te HCM. I te nuinga o te wā, ka puta tēnei matotorutanga i te pakitara e wehe ana i te taha maui me te taha matau o te ngākau, e kiia nei ko te `(septum).` Whakaarohia, ina matotoru tēnei septum i waenganui i ngā rūma o raro o te ngākau `(ventricles)`, ka taea e ia te aukati i te rere o te toto mai i te ventricle maui ki te aorta, te oko toto matua e kawe ana i te toto puta noa i te tinana. Ka kiia tēnei ko te `(outflow tract obstruction)`. Kātahi ka kaha ake te mahi a te ngākau ki te pupuhi toto i tua atu i tēnei aukati. Ko te momo HCM e aukati ana i te rere o te toto e kiia ana ko te `(Hypertrophic Obstructive Cardiomyopathy - HOCM)`.

I ētahi wā, ka matotoru ake ētahi atu wāhanga o te ngākau, pērā i te pito, te ventricle matau, te ventricle maui katoa rānei. I ēnei wā, kāore e aukatia te rere o te toto. Heoi, ka whakaitihia te nui o te toto ka taea e te ventricle te whakahaere. Ka kiia tēnei ko te Hypertrophic Nonobstructive Cardiomyopathy.

Te pakari o te ventricle maui

Ina matotoru te uaua o te ngākau, ka kore e whakangā te ventricle maui, ka kī tonu i te toto nā ngā panonitanga o aua pūtau. He aha te mea ka tupu? Nā te mea he iti iho te toto i roto i te ruma, he iti iho te toto whai hāora e tukuna ana ki ētahi atu okana me ngā uaua o te tinana. Ka whakanuia hoki e tēnei whakapakeketanga te pēhanga i roto i te ngākau, ā, ka pāngia pea koe e ngā tohu pēnei i:

• Mamae o te uma
• Te poto o te manawa
• Pōuriuri, ngoikore rānei
• Te ahua kei te tere te pao o tō ngākau (palpitations)

Ngā Huringa o te Takirere Mitral

E ai ki te kōrero i mua ake nei, ina aukatia te ara rere o te ventricle maui, kāore pea te takirere mitral i roto i te ngākau e mahi tika. Ka ārai pea tēnei i te rere o te toto, ā, ka piki ake te pēhanga i roto i te ventricle maui.

Ko te take o tēnei aukati ko te pānga o te takirere mitral ki te septum matotoru. Ina tupu tēnei, kāore te takirere mitral e kati tika, ā, ka turuturu te toto ki roto i te atrium maui.

Ngā Huringa Pūtau

Ki te tirohia tata atu i raro i te karuārai, kāore ngā pūtau uaua o te ngākau o te tangata whai HCM i te whakarite tika, engari i roto i te āhua pōrearea. Ka taea e tēnei mate te whakarerekē i ngā tohu hiko e haere ana i roto i ngā rūma o raro o te ngākau, ka puta he manawataki ngākau rerekē e kiia nei ko te ventricular arrhythmia. He mea morearea tēnei.

He pēhea te noa o te ingoa o tēnei āhuatanga ko te HCM?

E ai ki te whakatau tata ki te 1.5 miriona tāngata i te Hononga o Amerika anake, arā, tata ki te kotahi i roto i te 500 tāngata, he HCM tō rātou. He mea noa ake tēnei i tētahi mate pēnei i te "(Multiple Sclerosis)". Nō reira, ehara i te mea onge.

I tehea tau ka whanake te HCM?

I te nuinga o te wā, ka puta tēnei mate manawa i roto i te hunga rangatahi, tata ki te tekau mā waru, rua tekau rānei tau te pakeke. Heoi, ka taea te tīmata i ngā tau katoa.

Mena kei a au te HCM, he tūponotanga kei hapu ahau?

Tērā pea me whai tiaki hauora motuhake koe, pērā i te echocardiography auau. Heoi, tera pea ka hauora tō hāputanga, ā, ka whānau noa hoki. Heoi, he mea nui kia kōrerohia tēnei ki tō tākuta i mua i tō hāputanga, me te kōrero anō hoki mō ngā mōrearea. Mā tō tākuta anō hoki koe e kōrero ko ēhea ngā rongoā mō te HCM ka taea tonu e koe te tango i te wā e hāpu ana. Me whakauru pea he pacemaker, he implantable cardioverter defibrillator (ICD) rānei mēnā e tika ana i te wā e hāpu ana.

He aha ngā take o te HCM?

He maha ngā āhuatanga ka pā ki te HCM:

• Ira: Koinei te tino take. He mate HCM ka taea te tuku iho mai i ngā mātua ki ngā tamariki. Ko te tikanga he hapa kei roto i ngā ira e whakahaere ana i ngā āhuatanga o te uaua ngākau. He maha ngā momo ira e puta ai te HCM. Ahakoa he hapa ira, ehara i te mea ka pāngia katoatia te whānau e te mate i te ara kotahi. Tērā pea kei ētahi tāngata te ira HCM engari kāore e pāngia e te mate.
• Te toto toto tiketike: Ko te toto toto tiketike kua roa nei e noho ana ka taea hoki te waiho hei take mō tēnei.
• Te koroheketanga: Tērā pea ka puta tēnei āhuatanga i a koe e pakeke haere ana.
• Ngā take kāore i te mōhiotia te take:I ētahi wā, kāore e kitea he take motuhake mō te HCM.

He aha ngā tohu ka pā ki te tangata whai HCM?

Tērā pea ka pāngia ētahi tāngata whai HCM e ngā tohu maha, ko ētahi ia kāore he tohu. Ko ngā tohu tino kitea ko:

• Mamae o te uma: Ka puta tēnei i te nuinga o te wā i te wā e whakakorikori tinana ana, engari ka puta pea i ētahi wā i a koe e tū noa ana, i muri rānei i te kai.
• Te poto o te manawa me te ngenge: He mea noa ēnei tohu i roto i ngā kaumātua, inā koa ka ngenge koe. Nā te pikinga o te pēhanga i roto i te atrium maui me ngā pukahukahu tēnei.
• Te ngoikore, te ngenge rānei (syncope): Ka puta pea tēnei i ngā manawataki o te ngākau (arrhythmias) i ngā urupare rerekē rānei o ngā oko toto i te wā e whakakorikori ana. I ētahi wā, kāore e kitea he take.
• Ngā Pāpātanga: Ka puta pea tēnei i te manawataki o te ngākau rerekē, pērā i te atrial fibrillation (atrial flutter) te ventricular tachycardia rānei (ventricular tachycardia). Tata ki te 25% o te hunga whai HCM he atrial fibrillation. Ka piki ake te mōrearea o te toto totoka me te ngoikore o te ngākau.
• Te pupuhi o ngā uaua i te taha raro o te tinana, i te kaki rānei.

He aha atu ngā raruraru hauora ka hono atu ki te HCM?

He tokomaha te hunga whai HCM e ora noa ana, ā, kāore he raruraru hauora nui. Ko ētahi kāore e hiahia ki te tango rongoā. Heoi, ahakoa kāore he tohu, ka tohutohu pea tō tākuta kia kaua e whakakorikori tinana.

Heoi, ko ētahi tāngata whai HCM ka pāngia pea e ngā mate ngakau ka whakapoto i ō rātou oranga, ka whakaiti rānei i te kounga o ō rātou oranga. Ko ngā mea matua ko:

Te Hopukia o te Manawa me te Matenga o te Manawa

Ko te hopukanga ohorere o te ngakau he ngaronga ohorere o te mahi a te ngakau nā te tere o te tere o te ngakau (hei tauira, te Ventricular Tachycardia). I tēnei wā, me whai maimoatanga ohorere, pērā i te CPR me te defibrillation, i te tīmatanga o ngā tohu. Ki te kore e hoatu tika tēnei maimoatanga, ka mate ohorere te ngakau.

He iti te mōrearea o te nuinga o te hunga whai HCM ki te mate ohorere o te ngakau. Heoi, ko te HCM te tino take o te mate ohorere o te ngakau i roto i te hunga kei raro iho i te 35 tau te pakeke. Kua rongo pea koe kua honoa hoki te HCM ki te matenga ohorere o ētahi kaitākaro ngaio taitamariki.

Te ngoikore o te ngākau

Ahakoa kei a koe te `(Heart Failure)` ehara i te mea kua "failure" tō ngākau, ā, kua mutu te mahi. Ko tōna tikanga kāore tō ngākau e pupuhi toto tika ana, e pai ana hoki. Ko ngā tohu o te `(Heart Failure)` ko:

• Te poto o te manawa.
• Te ngenge i te wā e mahi ana (te ngenge).
• Te pupuhi o ngā pona, ngā waewae, me te puku.
• Te hiahia ki te mimi i te pō i a koe e okioki ana.
• Pōuriuri, pōhēhē, uaua ki te arotahi, hemo te ngākau.
• Te āhua nei kei te tere te pao o tō ngākau (palpitations).
• He maremare maroke, he maremare tere.
• Te kī tonu, te pupuhi, te kore hiahia kai, te mamae rānei o te puku.

Me pēhea te tautuhi i te HCM? (Tautuhinga)

Ka tautuhia te HCM i runga i ngā mea e whai ake nei:

• Hītori hauora: Ka pātai atu tō tākuta ki a koe mō ō tohumate, me te mea kua pāngia tētahi o tō whānau e ngā mate ōrite.
• Whakamātautau tinana: Ka whakarongo te tākuta ki tō ngākau me ō pukahukahu. Tērā pea ka rongo te hunga whai HOCM i tētahi tangi ngākau rerekē (heart murmur).
• Ngā Whakamātautau: Ko te echocardiogram (echo) te whakamātautau matua e whakamahia ana hei tautuhi i te HCM. Ka āwhina tēnei ki te kite mēnā kua matotoru ngā pakitara o te ngākau.

Anei ētahi atu whakamātautau:

• Ngā whakamātautau toto.
• Te hiko o te manawa (ECG).
• X-ray o te uma.
• Whakamātautau ahotea whakakori tinana.
• Te whakaurunga o te kāteretere o te ngākau.
• Whakaahua aukume (MRI).

He aha ngā maimoatanga mō te HCM?

Mā tō tākuta e whakatau ko tēhea te maimoatanga e tika ana māu i runga i ngā āhuatanga e whai ake nei:

• He ārai kei te putanga atu o te toto i te ngākau (he whaiti te ara rere toto)?
• He pēhea te mahi a tō ngākau?
• Ō tohumate.
• Tō tau me te nui o tō kaha ki te mahi.
• He rerekē ngā manawataki o tō ngākau (arrhythmias)?

Ko te whāinga matua o te maimoatanga he whakaiti, he ārai rānei i ngā tohu, me te whakaiti i te mōrearea o ngā raruraru pēnei i te ngoikore o te ngakau me te matenga ohorere o te ngakau.

Hei maimoatanga, ka taea te mahi i ngā mahi e whai ake nei:

• Tāutuhia ngā mōrearea, ā, haere tonu ki te toro atu ki te tākuta.
• Ngā huringa o te āhua noho.
• Ngā rongoā.
• Ngā tikanga hauora motuhake.

Me pēhea te tirotiro mō te HCM?

I te mea he mate HCM ka taea te tuku iho i tētahi whakatipuranga ki tētahi whakatipuranga, mēnā kei a tētahi o ō mātua, o ō tuākana, o ō teina rānei, o ā rātou tamariki rānei (arā, he whanaunga tuatahi) te mate, he mea nui kia whakamātauria koe.

Ko te taahiraa tuatahi ko te tirotiro i tō ngākau mā te Electrocardiogram (ECG) me te Echocardiogram (echo). Mena ka whakaatuhia e ēnei whakamātautau ngā tohu o te HCM, me toro atu koe ki tētahi tākuta tohunga ki tēnei mate (he tākuta ngākau).

I ētahi wā, ahakoa he hītori whānau tōu mō te HCM, tera pea he noa ngā hua o ō whakamātautau. Mena koinā, ka tūtohu ngā tākuta kia whai i te echo me te ECG i ia toru tau tae noa ki te 30 tau, ā, i ia rima tau anō hoki.

Ko wai kei te nui ake te mōrearea o te mate ohorere o te ngakau nā te HCM?

He iti te mōrearea o te nuinga o te hunga whai HCM ki te mate ohorere o te ngākau. Heoi, he iti noa te hunga whai HCM e nui ake ana te mōrearea. He mea nui kia mōhio koe mēnā kei a koe tēnei mate. Ka taea e tō tākuta te kōrero ki a koe he aha tāu e taea ai te whakaiti i tō mōrearea.

Ko te hunga e nui ake ana te mōrearea ki te mate ohorere o te ngakau nā te HCM ko:

• Ngā tāngata whai hītori whānau mō te mate ohorere o te ngākau.
• Ngā tāngata kua maha ngā wā kua pāngia e te mate pāwera i tō rātou tamarikitanga.
• Ngā tāngata he rerekē te tere o te toto toto ki te whakakori tinana.
• Ngā tāngata kua pāngia e te tere ohorere o te ngakau (arrhythmia).
• Te hunga he nui ngā tohu, ā, he ngoikore te mahi a te ngākau.

Kōrero ki tō tākuta mō ō mōrearea whaiaro. Mena e rua, neke atu rānei ō āhuatanga mōrearea mō te matenga ohorere, ka tūtohu pea tō tākuta i ngā rongoā ārai-arrhythmic (ngā rongoā hei whakahaere i ngā manawataki ngakau rerekē) me te implantable cardioverter defibrillator (ICD) hei whakaiti i tō mōrearea.

He aha ngā rongoā e whakamahia ana hei whakahaere i te HCM?

Ka tohua e ngā tākuta ngā momo rongoā hei whakahaere i ō tohu me te ārai i ngā raruraru anō. Ko ngā rongoā pēnei i ngā "Beta blockers" me ngā "calcium channel blockers" ka whakangāwari i te uaua o te ngākau, ka āwhina i te whakakī i te toto me te pupuhi toto kia pai ake te mahi. Ka taea e ētahi atu rongoā te whakahaere i tō tere o te ngākau, te whakaiti rānei i te mōrearea o ngā manawataki ngākau rerekē (arrhythmias).

Tērā pea ka kīia atu ki a koe kia kaua e tango i ētahi rongoā, hei tauira, ko ngā nitrates (e whakaheke ana i te toto toto) ko te digoxin rānei (e whakanui ana i te kaha o te whakakorikori o te ngakau).

I ētahi wā, me tango e koe ngā paturopi me ētahi atu mahi ārai hei whakaiti i te mōrearea o te mate e pāngia ai te ora e kiia nei ko te bacterial endocarditis.

He aha ngā tikanga hauora motuhake e whakamahia ana hei rongoā i te HCM?

He maha ngā tikanga hei rongoā i te Hypertrophic Obstructive Cardiomyopathy (HOCM), he āhuatanga e aukati ai te rere o te toto:

Te tango i te septum

He tikanga pokanga tēnei. I roto i te tangohanga o te septal myectomy, ka tangohia e te taote pokanga tētahi wāhanga iti o te pakitara septal matotoru. Ka whānui ake te ara rere mai i te ventricle maui ki te aorta. Ka whakaarohia noa tēnei pokanga e ngā tākuta mēnā kāore he rongoā e āwhina. He maha ngā wā ka whakatauhia te raruraru o te mitral valve.

Te Whakakorenga o te Etanora

Ka kiia hoki tēnei ko te `(septal ablation)`. Ka whakamahia tēnei tikanga mō te hunga kāore e taea te mahi pokanga `(septal myectomy)` i whakahuatia ake nei. I roto i tēnei, ka kitea e te tākuta he uaua iti e tuku toto ana ki te taha o runga o tō septum. Kātahi ka whakauruhia e ia he catheter poihau ki roto i taua uaua ka pupuhi. Muri iho, ka werohia e ia he `(contrast agent)` tae hei kimi i te wāhi tika o te pakitara septal matotoru e aukati ana i te rere o te toto.

Kia kitea te wāhi, ka werohia e te tākuta he iti noa te waipiro parakore mā roto i te catheter. Ka patua e te waipiro ngā pūtau, ā, i roto i ngā marama torutoru e whai ake nei ka hoki te septum ki tōna rahi noa. Mā tēnei ka whānui ake te ara mō te rere o te toto.

Pūwhakaiti i te Fibrillator Cardioverter Whakatō (ICD)

Ka taea e te taputapu whakapūmau i te manawa o te ngakau (ICD) te āwhina i te hunga e pāngia ana e te mate ngakau e morearea ana ki te ora, e te mate ohorere rānei o te ngakau. He taputapu iti te ICD ka whakapūmautia ki raro i tō kiri. Ka honoa ki tō ngākau mā roto i ngā waea. Ka aroturuki tonu te ICD i te manawataki o tō ngākau. Ina kitea he tere rawa te manawa o te ngākau, he rerekē, ka tukuna he ru hiko iti engari kaha ki te ngākau, ka meinga kia patupatu noa anō. Ma tō tākuta koe e kī atu mēnā he tika te ICD māu.

Te Whakahaere i te Raru o te Manawa

He maha ngā huarahi hei whakahaere i te ngoikore o te ngākau, mai i ngā huringa momo noho ki ngā rongoā hei whakaiti i ngā tohu me te āwhina i te uaua o te ngākau kia mahi tika.

Me pēhea taku whakapai ake i te kounga o tōku oranga me te HCM?

Ahakoa he tohu kei a koe, ahakoa kua tāpaetia koe ki te HCM, mēnā rānei kei roto i tō whānau tēnei mate, he maha ngā mea ka taea e koe te mahi hei pupuri i te hauora o tō ngākau kia tino pai:

• Te whakawhāiti i ngā wai me te tote (konutai): Mena kei a koe ngā tohu o te ngoikore o te ngākau, me whakawhāiti pea koe i tō inu wai me te tote. Pātai atu ki tō tākuta mō ngā aratohu motuhake mō tō kai, tae atu ki te waipiro me te kawheine.
• Kia tūpato ki te whakakori tinana: Ka kōrero tō tākuta ki a koe mō te whakakori tinana e tika ana māu. He maha ngā tāngata whai HCM ka taea te mahi i ngā mahi aerobic kāore e whakataetae. Kāore ngā tākuta e taunaki ana i te hiki taumaha, i ngā hākinakina kaha rānei.
• Tirohia tō tākuta i ia wā: Mena he HCM tōu, me toro atu koe ki tō tākuta ngakau i ia wā kia aroturukihia ai tō āhua.
• Whakaitihia tō tupono ki te pangia: Mena kei a koe te HCM, ka tohutohu tō tākuta kia mahi koe i ngā mahi hei whakaiti i tō tupono ki te pangia e te mate e kiia nei ko te "infective endocarditis".

Me pēhea te whakaiti i te mōrearea o te whakawhanake i te Infective Endocarditis?

Ko te hunga whai HCM he nui ake te tūponotanga ka pāngia e te mate kitakita e kiia nei ko te "endocarditis bacterial" me te "endocarditis pangia".

Ko te mate pukupuku o te ngākau he mate pāwera ki ngā takirere o te ngākau, ki te arai rānei o te ngākau (endocardium). Ka puta tēnei ina uru ngā iroriki (ina koa ko ngā huakita, engari i ētahi wā ko ngā harore me ētahi atu moroiti) ki roto i tō toto ka whakaeke i te arai o tō ngākau, i ngā takirere rānei. Ka taea e tēnei te whakamātotoru, te poka, te whara rānei i ngā takirere. He maha ngā wā ka puta he takirere ngākau turuturu. Ki te kore e rongoatia tika, ka mate pea.

Ka taea e koe te mahi i ēnei mahi hei whakaiti i tēnei mōrearea:

• Tiakina ō niho me ō kapia: Tirohia te tākuta niho i ia ono marama, paraihia ō niho i ia rā, mirohia ō niho ki te miro, ā, tirohia mēnā kei te pai te urunga o tō paraihe niho.
• Tirohia te tākuta mēnā kei a koe ngā tohu o te mate urutā:
• He kirika neke atu i te 100 nekehanga Fahrenheit, he werawera, he makariri rānei.
• Te kiri mākūkū.
• Mamae, ngawari, whero, pupuhi rānei.
• He patunga, he motu rānei kāore e ora, he patunga whero, wera, e pupuhi ana te waikura.
• Korokoro mamae, korokoro kikī, mamae rānei i te wā e horomia ana.
• He rerenga mai i te sinus, he ihu pupuhi, he ānini, he mamae rānei i ngā paparinga o runga.
• He maremare maroke, he maremare rānei e roa ana mō ngā rā e rua.
• Ngā ira mā i te waha, i te arero rānei.
• Whakapairuaki, ruaki, korere rānei.

Kaua e whakaroa ki te rapu maimoatanga. Kāore te makariri noa me te rewharewha e hua ai te endocarditis. Heoi, ka taea e ētahi atu mate me ngā tohu rite te hua o te endocarditis. Nō reira, kia tūpato, me toro atu ki tō tākuta.

• Inumia ngā paturopi ārai i mua i ētahi tikanga hauora me ngā tikanga niho. Kōrero ki tō tākuta mō te momo paturopi me tango e koe, te nui, me ngā momo tikanga me tango e koe.
• Puritia he kāri tautuhi mate pukupuku pūkahukahu (bacterial endocarditis) mai i te American Heart Association.

Hei whakamutunga, koinei tāu e kōrero ai (Karere hei tango ki te kāinga)

Ko te nuinga o te hunga whai HCM he ora noa, he harikoa. He maha ngā maimoatanga e wātea ana mō te hunga whai tohu, mō te hunga rānei e tūpono ana ki te whakawhanake i ngā raruraru nui. Ko tō matapae ka whakawhirinaki ki te pai o te mahi a tō uaua ngākau, ō tohu, me te pai o tō urupare me te whai i tō mahere maimoatanga.

Pātai atu ki tō tākuta mō ō mōrearea me ngā mea ka taea e koe te mahi hei whakapai ake i tō kounga o te oranga me te ārai i ngā mate. Mena kua tautuhia koe he HCM, ā, ka puta ngā tohu, ka whakapae rānei koe he mate tōu, me toro atu ki tō tākuta tonu. Kia maumahara, ka taea e koe te ora pai me tēnei mate!

`Te ngakau nui rawa atu, te mate ngakau nui (HCM), te mate ngakau, te whakanui i te ngakau, te mamae o te uma, te poto o te manawa, te mate ohorere o te ngakau