Type B Interrupted Aortic Arch with a Patent Ductus Arteriosus in an Adult Presenting with Secondary Polycythaemia
  • Jonan Chun Yin Lee
    Department of Radiology & Imaging, Queen Elizabeth Hospital, Hong Kong, Hong Kong
  • Jeanie Betsy Chiang
    Department of Radiology & Imaging, Queen Elizabeth Hospital, Hong Kong, Hong Kong
  • Boris Chun Kei Chow
    Department of Radiology & Imaging, Queen Elizabeth Hospital, Hong Kong, Hong Kong

Keywords

Interrupted aortic arch, congenital heart disease, secondary polycythaemia, computed tomography, magnetic resonance imaging

Abstract

Interrupted aortic arch (IAA) is an extremely rare congenital cyanotic heart disease characterized by complete disruption between the ascending and descending aorta. A patent ductus arteriosus (PDA) or other collateral pathways provide blood flow to the distal descending aorta. Mortality is extremely high at early infancy, particularly after the closure of ductus arteriosus. Survival and presentation in adulthood are extremely rare. Here we illustrate a rare case of type B interrupted aortic arch in an adult who presented with secondary polycythaemia. The blood supply to descending aorta and beyond is almost solely by a patent ductus arteriosus. The case demonstrates the value of multimodality imaging including CT and MRI for diagnosis and treatment planning in these patients.

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References

  • Edwards P, Prosser R, Wells C. Chorea, polycythaemis, and cyanotic heart disease. J Neurol Neurosurg Psychiatry 1975;38(8):729–739.

  • Celoria GC, Patton RB. Congenital absence of the aortic arch. Am Heart J 1959;58:407–413.

  • Oppenheimer-Dekker A, Gittenberger-de Groot AC, Roozendaal H. The ductus arterious and associated cardiac anomalies in interruption of the aortic arch. Pediatr Cardiol 1982;2(3):185–193.

  • Gordon E, Person T, Kavarana M, Ikonomidis J. Interrupted aortic arch in the adult. J Card Surg 2011;26(4):405–409.

  • Sakellaridis T, Argiriou M, Panagiotakopoulos V, Krassas A, Argiriou O, Charitos C. Latent congenital defect: interrupted aortic arch in an adult – case report and literature review. Vasc Endovascular Surg 2010;44(5):402–406.

  • Yildirim N, Aydin M, Hekimoglu K, Gungorduk A. Isolated interrupted aortic arch, a rare cause of hypertension in adults. Int J Cardiol 2008;127(2):e52–53.

  • Sharratt GP, Carson P, Sanderson JM. Complete interruption of aortic arch, without persistent ductus arteriosus, in an adult. Br Heart J 1975;37(2):221–224.

  • Dillman JR, Yarram SG, D'Amico AR, Hernandez RJ. Interrupted aortic arch: spectrum of MRI findings. AJR Am J Roentgenol 2008;190(6):1467–1474.

  • Thorne S. Management of polycythaemia in adults with cyanotic congenital heart disease. Heart 1998;79:315–316.

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    Published: 2020-02-26
    Issue: 2020: Vol 7 No 4 (view)

    How to cite:
    1.
    Lee JCY, Chiang JB, Chow BCK. Type B Interrupted Aortic Arch with a Patent Ductus Arteriosus in an Adult Presenting with Secondary Polycythaemia. EJCRIM 2020;7 doi:10.12890/2020_001511.