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Herz

, Volume 39, Issue 1, pp 98–104 | Cite as

Serial evaluation of hepatic function profile after Fontan operation

  • R. Kaulitz
  • P. Haber
  • E. Sturm
  • J. Schäfer
  • M. Hofbeck
Original article

Abstract

Moderate persistent elevation of the γ-glutamyltransferase (γGT) level is a frequent finding during long-term follow-up of patients with total cavopulmonary connection (TCPC) for palliation of functionally univentricular hearts. Serial intraindividual data revealed a significant increase in the γGT level within a minimum 4-year interval in more than 80 % of cases. The level of γGT elevation showed a significant correlation to hemodynamic parameters such as systemic ventricular end diastolic pressure and mean pulmonary artery pressure, but did not strongly correlate with duration of follow-up or other liver function parameters, which were less frequent and less impressively deranged. None of the patients had signs of synthetic dysfunction. With increasing postoperative follow-up, abnormalities of sonographic hepatic texture including increased echogenicity, inhomogeneity, or liver surface nodularity were found. All 17 patients with liver surface nodularity had a follow-up period of over 10 years. Structural abnormalities did not correlate with biochemical or hemodynamic parameters. Doppler evaluation revealed inspiratory dependence of hepatic vein flow in more than 90 % as a relevant finding after TCPC; a decrease in portal vein flow velocity was observed in many patients. Since long-term survivors after Fontan procedure are at an increased risk of cardiac hepatopathy and cirrhosis, detailed routine investigation and monitoring of hepatic morphology are needed.

Keywords

Univentricular heart Fontan operation Hepatic congestion Cardiac hepatopathy Doppler sonography 

Serielle Untersuchung der Leberfunktion nach Fontan-Operation

Zusammenfassung

Eine mäßige Erhöhung der y-Glutamyltransferase (yGT) findet sich häufig im Langzeitverlauf bei Patienten mit totaler kavopulmonaler Anastomose. Im intraindividuellen Verlauf zeigt sich innerhalb von mindestens 4 Jahren ein signifikanter Anstieg bei mehr als 80 %. Es besteht eine Korrelation zu hämodynamischen Parametern wie dem systemventrikulären enddiastolischen Druck und mittleren Pulmonalarteriendruck, nicht jedoch zur Nachbeobachtungsdauer oder anderen Leberfunktionsparametern, die insgesamt nur leichte Veränderungen aufwiesen. Eine Störung der Syntheseleistung bestand bei keinem Patienten. Mit zunehmender Nachbeobachtungsdauer zeigen sich Veränderungen der Lebertextur mit erhöhter Echogenität, Inhomogenität und unregelmäßiger, nodulärer Leberoberfläche. Alle 17 Patienten mit knotig veränderter Leberoberfläche wiesen eine Nachbeobachtungsdauer von über 10 Jahren auf. Strukturelle Leberveränderungen korrelierten nicht mit Leberfunktionstests oder hämodynamischen Parametern. Dopplersonographisch ist der Lebervenenrückstrom bei über 90 % durch eine deutliche Abhängigkeit von der Inspiration gekennzeichnet. Veränderungen des Pfortaderflusses und eine Abnahme der Strömungsgeschwindigkeit sind häufig. Da im Langzeitverlauf nach einer Fontan-Operation das Risiko der Entwicklung einer kardialen Hepatopathie und Zirrhose besteht, sind regelmäßige Untersuchungen zur Beurteilung der Leberfunktion und Struktur unerlässlich.

Schlüsselwörter

Univentrikuläres Herz Fontan-Operation Leberstauung Kardiale Hepatopathie Dopplersonographie 

Notes

Conflict of interest

On behalf of all authors, the corresponding author states that there are no conflicts of interest.

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Copyright information

© Urban & Vogel 2013

Authors and Affiliations

  • R. Kaulitz
    • 1
  • P. Haber
    • 2
  • E. Sturm
    • 1
  • J. Schäfer
    • 2
  • M. Hofbeck
    • 1
  1. 1.Division Pediatric Cardiology and Pediatric Intensive Care MedicineTuebingen University Children’s HospitalTuebingenGermany
  2. 2.Department of RadiologyTuebingen University HospitalTuebingenGermany

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