Clinical Journal of Gastroenterology

, Volume 12, Issue 1, pp 20–24 | Cite as

Successful treatment of esophageal bleeding due to rupture of major aortopulmonary collateral arteries by transcatheter arterial embolization

  • Takanori Ito
  • Masatoshi IshigamiEmail author
  • Yoji Ishizu
  • Teiji Kuzuya
  • Takashi Honda
  • Masaya Matsushima
  • Taichi Kato
  • Yoshiki Hirooka
Case Report


Major aortopulmonary collateral arteries (MAPCAs) are unique vessels associated with hypoxia induced by congenital heart disease (CHD). Although MAPCAs are essential to supply blood to the lungs, their development and proliferation can induce life-threatening complications, such as rupture into the lung. Here, we describe a rare case of esophageal bleeding from MAPCAs in a CHD patient, which was successfully treated by transcatheter arterial embolization (TAE). A 16-year-old male with CHD experienced a hematemesis and melena after the Bentall procedure to treat valvular heart disease. Emergent esophagogastroduodenoscopy revealed spurting bleeding from the middle esophageal vessels; accordingly, endoscopic variceal ligation (EVL) was performed. However, he had a hematemesis again after 2 weeks of EVL. The arterial phase of dynamic computed tomography indicated that a MAPCA associated with CHD was the origin of bleeding. Hence, TAE of this MAPCA with a mixture of n-butyl-2-cyanoacrylate and ethiodized oil was performed to prevent re-bleeding. Color Doppler mode in endoscopic ultrasonography via the esophagus revealed mosaic-like signals in MAPCAs located in the esophageal wall. This finding was consistent with tortuous MAPCAs accompanied by turbulent blood flow. When clinicians encounter CHD patients with unexpected massive esophageal bleeding, bleeding related to MAPCAs should be considered.


Major aortopulmonary collateral artery Congenital heart disease Esophageal bleeding Transcatheter arterial embolization 



Major aortopulmonary collateral artery


Congenital heart disease


Transcatheter arterial embolization


Endoscopic variceal ligation


Computed tomography






Endoscopic ultrasonography




Celiac artery


Superior mesenteric artery


Compliance with ethical standards

Conflict of interest

The authors who have taken part in this study declared that they do not have anything to disclose regarding funding or conflict of interest with respect to this manuscript.

Human rights

All procedures were performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

Informed consent

Informed consent for being included in this report was obtained from family of this patient.


  1. 1.
    Boshoff D, Gewillig M. A review of the options for treatment of major aortopulmonary collateral arteries in the setting of tetralogy of Fallot with pulmonary atresia. Cardiol Young. 2006;16:212–20.CrossRefGoogle Scholar
  2. 2.
    Tchervenkov CI, Roy N. Congenital heart surgery nomenclature and database project: pulmonary atresia—ventricular septal defect. Ann Thorac Surg. 2000;69:97–105.CrossRefGoogle Scholar
  3. 3.
    Reinhartz O, Reddy VM, Petrossian E, et al. Unifocalization of major aortopulmonary collaterals in single-ventricle patients. Ann Thorac Surg. 2006;82:934–8 (discussion 8–9).CrossRefGoogle Scholar
  4. 4.
    Cheng J, Fan CM, Tang M, et al. Initial research on postoperative management of tetralogy of fallot with major aortopulmonary collaterals. Cardiology. 2016;134:406–10.CrossRefGoogle Scholar
  5. 5.
    Lanjewar C, Shiradkar S, Agrawal A, et al. Aneurysmally dilated major aorto-pulmonary collateral in tetralogy of Fallot. Indian Heart J. 2012;64:196–7.CrossRefGoogle Scholar
  6. 6.
    Sharma A, Kumar S, Priya S. Ruptured aneurysm of major aortopulmonary collateral artery: management using amplatzer vascular plug. Cardiovasc Diagn Ther. 2016;6:274–7.CrossRefGoogle Scholar
  7. 7.
    Carotti A, Albanese SB, Filippelli S, et al. Determinants of outcome after surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg. 2010;140:1092–103.CrossRefGoogle Scholar
  8. 8.
    Mainwaring RD, Patrick WL, Carrillo SA, et al. Prevalence and anatomy of retroesophageal major aortopulmonary collateral arteries. Ann Thorac Surg. 2016;102:877–82.CrossRefGoogle Scholar
  9. 9.
    Cheng J, Fan C, Tang M, et al. Initial research on postoperative management of tetralogy of Fallot with major aortopulmonary collaterals. Cardiology. 2016;134:406–10.CrossRefGoogle Scholar
  10. 10.
    Miyazaki O, Nosaka S, Hayakawa M, et al. Sudden death due to rupture of major aortopulmonary collateral arteries in a patient with tetralogy of Fallot and pulmonary atresia. Emerg Radiol. 2001;8:293–6.CrossRefGoogle Scholar
  11. 11.
    Kish JW, Katz MD, Marx MV, et al. N-butyl cyanoacrylate embolization for control of acute arterial hemorrhage. J Vasc Interv Radiol. 2004;15:689–95.CrossRefGoogle Scholar
  12. 12.
    Pollak JS, White RI. The use of cyanoacrylate adhesives in peripheral embolization. J Vasc Interv Radiol. 2001;12:907–13.CrossRefGoogle Scholar
  13. 13.
    Toyoda H, Nakano S, Kumada T, et al. Estimation of usefulness of N-butyl-2-cyanoacrylate-lipiodol mixture in transcatheter arterial embolization for urgent control of life-threatening massive bleeding from gastric or duodenal ulcer. J Gastroenterol Hepatol. 1996;11:252–8.CrossRefGoogle Scholar
  14. 14.
    Frodsham A, Berkmen T, Ananian C, et al. Initial experience using N-butyl cyanoacrylate for embolization of lower gastrointestinal hemorrhage. J Vasc Interv Radiol. 2009;20:1312–9.CrossRefGoogle Scholar
  15. 15.
    Marelli AJ, Mackie AS, Ionescu-Ittu R, et al. Congenital heart disease in the general population: changing prevalence and age distribution. Circulation. 2007;115:163–72.CrossRefGoogle Scholar
  16. 16.
    Khairy P, Ionescu-Ittu R, Mackie AS, et al. Changing mortality in congenital heart disease. J Am Coll Cardiol. 2010;56:1149–57.CrossRefGoogle Scholar

Copyright information

© Japanese Society of Gastroenterology 2018

Authors and Affiliations

  • Takanori Ito
    • 1
  • Masatoshi Ishigami
    • 1
    Email author
  • Yoji Ishizu
    • 1
  • Teiji Kuzuya
    • 1
  • Takashi Honda
    • 1
  • Masaya Matsushima
    • 2
  • Taichi Kato
    • 3
  • Yoshiki Hirooka
    • 1
  1. 1.Department of Gastroenterology and HepatologyNagoya University Graduate School of MedicineNagoyaJapan
  2. 2.Department of RadiologyNagoya University Graduate School of MedicineNagoyaJapan
  3. 3.Department of PediatricsNagoya University Graduate School of MedicineNagoyaJapan

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