Abstract |
A 5-year-old girl with right atrial isomerism, complete atrioventricular septal defect, hypoplastic left ventricle, double outlet right ventricle, and mixed-type total anomalous pulmonary venous connection with totally occluded left pulmonary veins presented at our center for fenestrated total cavo-pulmonary connection with an extra cardiac conduit at the age of 3 years. Eleven months after the Fontan completion, she developed protein-losing enteropathy (PLE). Spontaneously closed fenestration was thought to be the cause of the PLE, and she underwent revision of fenestration at the age of 5 years. After the operation, PLE did not improve, and newly developed hypoxemia impaired her systemic ventricular function, leading to the initiation of veno-arterial extracorporeal membrane oxygenation (ECMO) with the Endumo(®) system 18 days after the operation to treat her hemodynamic instability. Although the ECMO circuit was changed three times during the first 8 days, the fourth circuit could be used for 74 days without hemolysis and serum leakage, until the patient unfortunately died 82 days after the operation due to multi-organ failure.
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Authors | Kunio Kusajima, Takaya Hoashi, Koji Kagisaki, Kotaro Yoshida, Takayuki Nishigaki, Teruyuki Hayashi, Hajime Ichikawa |
Journal | Journal of artificial organs : the official journal of the Japanese Society for Artificial Organs
(J Artif Organs)
Vol. 17
Issue 1
Pg. 99-102
(Mar 2014)
ISSN: 1619-0904 [Electronic] Japan |
PMID | 24379041
(Publication Type: Case Reports, Journal Article)
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Topics |
- Child, Preschool
- Extracorporeal Membrane Oxygenation
(instrumentation)
- Fatal Outcome
- Female
- Heart Defects, Congenital
(complications, surgery)
- Humans
- Postoperative Complications
(therapy)
- Protein-Losing Enteropathies
(complications)
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