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Interrupted Aortic Arch (IAA): sebuah laporan kasus

  • Yori Primanda ,
  • Pande Putu Yuli Anandasari ,
  • I Made Dwija Putra Ayusta ,

Abstract

Background: Interrupted aortic arch (IAA) is a congenital abnormality in which there is a discontinuity between the lumen of the ascending and descending aorta. This disorder is classified as a rare congenital disorder with an incidence of 0.003 out of 1000 live births. It is important to detect this disorder early because of the high mortality rate of untreated patients with the interrupted aortic arch in the first year of life, reaching 90%. This case report aims to report the sign and symptoms of interrupted aortic arch abnormalities, other accompanying congenital abnormalities, and the radiological feature of this disorder.

Case Presentation: In this case, a baby boy was born at 37 weeks 6 days of gestation with a birth weight of 2100 grams. At birth, the patient did not cry immediately, with an Apgar score of 6-8. It was found that there were differences in oxygen saturation rates of the upper and lower extremities. Initially, the patient underwent echocardiography and cardiac catheterization and the results were severe coarctation of the aorta and bidirectional large tubular patent ductus arteriosus. Then when the patient was 9 months old, a CT angiography examination was performed. A separation between the ascending and descending aortic lumen and several other congenital heart defects was obtained.

Conclusion: Although an interrupted aortic arch is a rare congenital abnormality, it is important to detect early signs and symptoms, other accompanying congenital abnormalities, and the radiological feature of this disorder. CT angiography is an imaging modality that plays an important role in establishing the diagnosis and determining the management of this case to prevent patient morbidity and mortality.

 

Latar Belakang: Interrupted aortic arch adalah kelainan kongenital dimana terdapat diskontinuitas antara lumen aorta ascenden dan descenden. Kelainan ini tergolong kelainan kongenital yang jarang terjadi dengan insiden 0.003 dari 1000 bayi yang lahir hidup. Penting untuk mendeteksi kelainan ini secara dini karena tingginya angka mortalitas pasien interrupted aortic arch yang tidak di treatment dalam satu tahun pertama kehidupan mencapai 90%. Laporan kasus ini bertujuan untuk melaporkan gejala dan tanda kelainan interrupted aortic arch, kelainan kongenital lain yang menyertai, serta bagaimana gambaran radiologis dari kelainan ini.

Presentasi Kasus :  Dalam kasus ini dilaporkan bayi laki-laki yang lahir pada umur kehamilan 37 minggu 6 hari dengan berat badan lahir 2100 gram. Pada saat lahir pasien tidak segera menangis dengan apgar score 6-8. Saat dilakukan pengecekan saturasi oksigen, didapatkan perbedaan angka saturasi oksigen ekstremitas atas dan bawah. Awalnya pasien menjalani pemeriksaan echocardiografi dan kateterisasi jantung didapatkan hasil severe coarctatio aorta dan large tubular patent ductus arteriosus bidirectional. Kemudian saat pasien berusia 9 bulan dilakukan pemeriksaan CT angiography dan didapatkan hasil pemisahan antara lumen aorta ascenden dan descenden serta beberapa kelainan jantung bawaan yang lain.

Simpulan: Walaupun interrupted aortic arch merupakan kelainan kongenital yang jarang terjadi, namun penting untuk dapat mendeteksi gejala dan tanda awal, kelainan kongenital lain yang menyertai, serta bagaimana gambaran radiologis dari kelainan ini. Modalitas pencitraan CT angiography memegang peranan penting dalam penegakkan diagnosis dan penentuan tatalaksana kasus ini untuk mencegah morbiditas dan mortalitas pasien.

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How to Cite

Primanda, Y., Anandasari, P. P. Y., & Ayusta, I. M. D. P. (2022). Interrupted Aortic Arch (IAA): sebuah laporan kasus . Intisari Sains Medis, 13(3), 778–781. https://doi.org/10.15562/ism.v13i3.1491

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Yori Primanda
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Pande Putu Yuli Anandasari
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I Made Dwija Putra Ayusta
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