More than the heart: Hepatic, renal, and cardiac dysfunction in adult Fontan patients

More than the heart: Hepatic, renal, and cardiac dysfunction in adult Fontan patients.

Byrne RD, Weingarten AJ, Clark DE, Huang S, Perri RE, Scanga AE, Menachem JN, Markham LW, Frischhertz BP.

Congenit Heart Dis. 2019 Jul 7. doi: 10.1111/chd.12820. [Epub ahead of print]

PMID: 31282062

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Take Home Points:

  • MELD and MELD-XI are scoring systems used to assess the severity of chronic liver disease.
  • Systolic ventricular dysfunction and decreased oxygen saturation were associated with hepatic and renal dysfunction which were shown by an increase in MELD and MELD-XI scores and creatinine level.

Dr. M.C. Leong


Commentary from Dr. M.C. Leong (Kuala Lumpur, Indonesia), section editor of ACHD Journal Watch: The Model for End-Stage Liver Disease (MELD) is a scoring system used to assess the severity of chronic liver disease by using the patient’s serum bilirubin, serum creatinine and the international normalized ratio (INR). It is used originally to predict survival of patients and to decide for liver transplant in patients post transjugular intrahepatic porto-systemic shunt (TIPS) procedure.


MELD = (3.78 × ln[total serum bilirubin (mg/dL)]) + (11.2 × ln[INR]) + (9.57 × ln[serum creatinine (mg/dL)]) + 6.43.


A modified scoring systemic named MELD-XI, which omits INR from its equation, has been used to correlate the impact of failing Fontan and hepatic fibrosis. The reason behind omitting INR from the equation was due to the high prevalence of Warfarin use in patients with Fontan.


MELD‐XI = (11.76 × ln[serum creatinine (mg/dL)]) + (5.11 × ln[total serum bilirubin(mg/dL)]) + 9.44.


This study aimed to investigate the association between MELD and MELD-XI; and Fontan circuit congestion and dysfunction in 98 patients post-Fontan (age 15 years or older). Of the hemodynamic variable analyzed, decreased oxygen saturation and moderate to severe reduction in ejection fraction, measured via echocardiography, were associated with increased MELD and MELD-XI scores as well as increased creatinine levels. Age at Fontan, systemic ventricular morphology, degree of atrio-ventricular valve regurgitation and Fontan pressures were not associated with elevated MELD, MELD-XI scores and creatinine levels.





MELD and MELD-XI scores still lack evidence to link its association with failing Fontan. Also, they may be useful as surrogate biochemical markers to monitor hepatic fibrosis patients with Fontan circulation but not as a tool to predict the risk of Fontan associated liver disease. Nevertheless, it may present as a good biochemical marker to complement the already available monitoring tools.