Ua haʻalele ʻo ʻAnomalona i ke aa kala korona mai ke aʻa aʻa

ʻO ke aniani i waiho ʻia mai ke aʻa kolonaʻi (ALCAPA) he kīnā kīnā ʻole. ʻO ke aʻa kolona hema (LCA), ka mea lawe i ke koko i ka puʻuwai puʻuwai, hoʻomaka mai ke aʻa pulmonary ma kahi o ka aorta.

Aia ʻo ALCAPA ma ka hānau ʻana (hānau hānau).

He pilikia ʻo ALCAPA i ka wā e ulu ana ka puʻuwai o ka pēpē i ka wanaʻao. ʻAʻole pili pono ke kīʻaha koko i ka puʻuwai puʻuwai.

I ka naʻau maʻamau, hoʻomaka ka LCA mai ka aorta. Hāʻawi ia i ke koko momona o ka oxygen i ka puʻuwai puʻuwai ma ka ʻaoʻao hema o ka puʻuwai a me ka kiwikā mitral (ka puʻuwai puʻuwai ma waena o nā keʻena luna a me lalo o ka puʻuwai ma ka ʻaoʻao hema). ʻO ke aorta ke kīʻaha koko nui e lawe i ke koko waiwai oxygen mai ka puʻuwai a i ke koena o ke kino.

I nā keiki me ALCAPA, hoʻomaka ka LCA mai ke aʻa aʻa. ʻO ke aʻa pulmonary ke kīʻaha koko nui e lawe i ke koko-maikaʻi ʻole mai ka puʻuwai a i nā akemāmā e kiʻi i ka oxygen.

Ke hiki mai kēia hemahema, lawe ʻia ke koko i nele i ka oxygen i ka ʻiʻo o ka puʻuwai ma ka ʻaoʻao hema o ka puʻuwai. No laila, ʻaʻole lawa ka oxygen o ka puʻuwai puʻuwai. Hoʻomaka ka make o ke kiko ma muli o ka nele o ka oxygen. Hiki i kēia ke kumu i ka hōʻeha puʻuwai i ka pēpē.

ʻO kahi kūlana i kapa ʻia ʻo "coronary steal" e hōʻino hou i ka puʻuwai me nā ALCAPA. ʻO ke kahe o ke koko haʻahaʻa i ke aʻa o ka pulmonary ke kumu o ke kahe o ke LCA pili ʻole i ke kahe pulmonary ma kahi o ka puʻuwai puʻuwai. ʻO kēia ka hopena i ka liʻiliʻi o ke koko a me ka oxygen i ka puʻuwai puʻuwai. Hiki i kēia pilikia ke alakaʻi i ka puʻuwai puʻuwai i kahi pēpē. Hoʻolālā ka ʻaihue coronary i ka manawa o nā pēpē me ALCAPA inā ʻaʻole mālama mua ʻia ke ʻano.

Nā ʻōuli o ALCAPA i loko o kahi pēpē:

• Ke uē a sweating paha i ka wā e hānai ana
• Huhū
• ʻIli pale
• ʻAi hānai maikaʻi ʻole
• Hanu wikiwiki
• Nā ʻōuli o ka ʻeha a me ke kaumaha i loko o ka pēpē (kuhi hewa pinepine ʻia no ka colic)

Hiki ke ʻike ʻia nā hōʻailona ma waena o nā mahina 2 mua o ke ola o ka pēpē.

ʻO ka maʻamau, ʻike ʻia ʻo ALCAPA i ka wā kamaliʻi. I nā hihia maʻamau, ʻaʻole ʻike ʻia kēia hemahema a hiki i ke keiki a makua paha o kekahi.

I ka wā o kahi hoʻokolohua, e loaʻa paha i ka mea mālama ola nā hōʻailona o ALCAPA, me:

• Rhythm puʻuwai maʻamau
• Puʻuwai hoʻonui ʻia
• ʻŌhumu ka naʻau (laha ʻole)
• Pākuʻi wikiwiki

ʻO nā hoʻokolohua i kauoha ʻia e komo pū:

• ʻO Echocardiogram, kahi ultrasound e ʻike i nā ʻano puʻuwai a me ke kahe o ke koko i loko o ka puʻuwai
• Electrocardiogram (ECG), kahi e ana ai i ka hana uila o ka puʻuwai
• Pākuʻi x-ray
• MRI, kahi e hāʻawi ai i kahi kiʻi kikoʻī o ka puʻuwai
• Cardiac catheterization, kahi hana e hoʻokomo ʻia ai kahi paipu lahilahi (catheter) i loko o ka puʻuwai e ʻike i ke kahe o ke koko a lawe i nā ana pono o ke kaomi koko a me nā pae oxygen.

Pono ka ʻoki kino e hoʻoponopono iā ALCAPA. Hoʻokahi wale nō ʻoki e pono ai i ka nui o nā hihia. Eia nō naʻe, e hilinaʻi ke kīʻaha i ke kūlana o ka pēpē a me ka nui o nā kīʻaha koko i pili.

Inā hōʻino nui ka puʻuwai puʻuwai e kākoʻo ana i ka mitral valve mai ka hoʻemi ʻia o ka oxygen, pono paha i ka pēpē ke ʻoki e hoʻoponopono a hoʻololi paha i ka pahu. Mālama ka pahu mitral i ke kahe o ke koko ma waena o nā keʻena ma ka ʻaoʻao hema o ka puʻuwai.

Hiki ke hana i kahi transplant puʻuwai inā pilikia nui ka puʻuwai o ka pēpē ma muli o ka nele o ka oxygen.

ʻO nā lāʻau i hoʻohana ʻia:

• Pila wai (diuretics)
• Nā lāʻau e hoʻonā i ka puʻuwai puʻuwai paʻakikī (inotropic agents)
• Nā lāʻau i hoʻohaʻahaʻa i ka ukana hana ma ka puʻuwai (beta-blockers, ACE inhibitors)

Me ka mālama ʻole ʻia, ʻaʻole ola ka hapa nui o nā pēpē i ko lākou makahiki mua. ʻO nā keiki i ola me ka ʻole o ka lapaʻau ʻana e loaʻa paha iā lākou nā pilikia puʻuwai koʻikoʻi. ʻO nā pēpē me kēia pilikia i mālama ʻole ʻia e hiki ke make koke i nā makahiki aʻe.

Me ka lapaʻau mua e like me ke kaha ʻana, hana maikaʻi ka hapa nui o nā pēpē a hiki ke kali i kahi ola maʻamau. Pono nā ukali maʻamau me kahi loea puʻuwai (cardiologist).

ʻO nā pilikia o ALCAPA me:

• Maʻi hohola
• Hewa puʻuwai
• Nā pilikia puʻuwai puʻuwai
• Poino paʻa i ka puʻuwai

Kāhea i kāu mea hāʻawi inā kāu pēpē:

• Ke hanu wikiwiki nei
• Keʻakeʻa loa ke nānā aku
• ʻIke kaumaha a uē pinepine

Ke kumu Anomalie o ke aʻa coronary hema e puka mai ana mai ke aʻa puʻupuʻu; ALCAPA; Maʻi maʻi ALCAPA; Bland-White-Garland maʻi maʻi; Hapa puʻuwai Congenital - ALCAPA; Hewa hānau - ALCAPA

• Ua haʻalele ʻo ʻAnomaloma i ke ʻalā kōpuna

ʻO nā hoahānau JA, Frommelt MA, Jaquiss RDB, Myerburg RJ, Fraser CD Jr, Tweddell JS. Nā lula ʻae kūkākūkā loea: ke kumu ʻaʻano anomalos o kahi aʻa coronary. ʻO J Thorac Cardiovasc Surg. 2017; 153 (6): 1440-1457. PMID: 28274557 pubmed.ncbi.nlm.nih.gov/28274557/.

Nā hoahānau JA, Gaynor JW. ʻO ke kaha no nā anomalies congenital o nā aʻa coronary. I: Sellke FW, del Nido PJ, Swanson SJ, eds. ʻO Sabiston lāua ʻo Spencer Surgery o ka umauma. 9th ed. Piladelapia, PA: Elsevier; 2016: chap 124.

Johnson JT, Harris M, Anderson RH, Spicer DE, Jacobs M, Tweddll JS, et al. ʻO nā anomal coronary coronary. I: Wernovsky G, Anderson RH, Kumar K, et al. Ka Pediatric Cardiology o Anderson. ʻEhā ed. Piladelapia, PA: Elsevier; 2020: chap 46.

ʻO Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM. ʻO nā puʻuwai congenital a me nā malformations vascular. I: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. ʻO Puka Textbook of Pediatrics ʻo Nelson. 21st ed. Piladelapia, PA: Elsevier; 2020: chap 459.