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Cirrhosis a Long Term Consequence of Congenital Tricuspid Atresia After Fontan Procedure: 2327

The American journal of gastroenterology, 2018-10, Vol.113 (Supplement), p.S1306-S1307 [Peer Reviewed Journal]

Copyright Wolters Kluwer Health Medical Research, Lippincott Williams & Wilkins Oct 2018 ;ISSN: 0002-9270 ;EISSN: 1572-0241 ;DOI: 10.14309/00000434-201810001-02326

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  • Title:
    Cirrhosis a Long Term Consequence of Congenital Tricuspid Atresia After Fontan Procedure: 2327
  • Author: Bommena, Shoma ; Joshi, Ishani ; Mehta, Shivang
  • Subjects: Congenital diseases ; Liver diseases
  • Is Part Of: The American journal of gastroenterology, 2018-10, Vol.113 (Supplement), p.S1306-S1307
  • Description: Congenital tricuspid valve atresia is a heart defect in which the tricuspid valve is not formed. Many of these children now live much longer due to surgical intervention. A 27-year-old female with tricuspid valve atresia that was repaired with single stage Fontan procedure at the age of 15 months presented to our ED at gestational age of 26 weeks 4 days with painful contractions for one week associated with gross rupture of membranes. She was admitted. On hospital day 5, she had more diffuse abdomen tenderness and amniocentesis performed, which showed chorioamnionitis. She was taken to operating room for primary low vertical caesarian section with spinal anesthesia. The procedure and her recovery were uncomplicated; she was discharged on post-op day 4 in a stable condition. At her three week postpartum visit she had complaints of persistent postoperative pain for which she was referred to ED. A CT abdomen pelvis non-contrast was done, hematoma and infection were ruled out. This also revealed cirrhotic appearance of liver with features of portal hypertension with splenomegaly. Her liver function tests were within normal limits except for a mildly elevated alkaline phosphatase. Her echocardiogram showed a severely hypoplastic right ventricle with depressed function. Fontan operation directs systemic venous return into pulmonary circulation which allows for passive venous flow into the lungs to achieve the goal of reduced intracardiac mixing. Post-procedure the systemic venous pressures are obligatorily elevated. Chronic venous congestion and chronic low cardiac output are hallmarks of Fontan circulation, which induces acute chronic injury, fibrosis and cirrhosis. The degree of fibrosis is related to duration of Fontan circulation. In most children post-Fontan, liver disease develops slowly without obvious clinical features. Elevation of liver enzymes is minimal and hepatic function is well preserved. Serum albumin is low due to protein losing enteropathy rather than hepatic dysfunction. INR is also mostly normal. Radiological assessment with standard US and CT can identify cirrhosis. Transient elastography and diffusion weighted MRI detect cirrhosis but cannot accurately distinguish early stages of fibrosis. Liver biopsy is the gold standard and has been proposed for all patients 10 years after Fontan operation. The most effective method of surveillance for liver disease in Fontan patients is uncertain, but early diagnosis will be beneficial.
  • Publisher: New York: Wolters Kluwer Health Medical Research, Lippincott Williams & Wilkins
  • Language: English
  • Identifier: ISSN: 0002-9270
    EISSN: 1572-0241
    DOI: 10.14309/00000434-201810001-02326
  • Source: AUTh Library subscriptions: ProQuest Central
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