Hepatic fibrosis is universal following Fontan operation, and severity is associated with time from surgery: a liver biopsy and hemodynamic study.. Liver biopsy and cardiac catheterizati
Trang 1significant increase in exercise capacity in those treated with Bosentan for 14 weeks There were
no important side effects The accompanying
editorial highlights the difficulty in designing trials for this population, where deterioration is slow and insidious and hard end points relatively infrequent
Goldberg DJ, Surrey LF, Glatz AC, et al Hepatic fibrosis is universal following Fontan operation, and severity is associated with time from
surgery: a liver biopsy and hemodynamic study
J Am Heart Assoc 2017;6(5).
Liver biopsy and cardiac catheterization were
offered as routine screening in Fontan patients.
At a mean age of 17 years and 15 years from the Fontan operation, all had hepatic fibrosis The degree of fibrosis did not correlate with serum liver function tests or hemodynamic findings or ventricular morphology but increased with time from the Fontan operation
Hilscher MB, Johnson JN, Cetta F, et al
Surveillance for liver complications after the
Fontan procedure Congenit Heart Dis.
2017;12(2):124–132
This excellent review summarizes the current
knowledge base and provides sensible guidance for the surveillance of liver abnormalities,
Trang 2John AS, Johnson JA, Khan M, et al Clinical
outcomes and improved survival in patients with protein-losing enteropathy after the Fontan
operation J Am Coll Cardiol 2014;64(1):54–62 Protein-losing enteropathy is associated with a
significant mortality risk, previously reported to
be approximately 50% within 5 years of
development of the disease In this report the risk of failure was substantially lower, with 88% surviving 5 years from diagnosis—a difference the authors attribute to improvements in
treatment Worse functional status, ventricular dysfunction, lower cardiac output, elevated
pulmonary artery pressure, and pulmonary
vascular resistance were associated with a poor outcome
Cunningham JW, Nathan AS, Rhodes J, et al
Decline in peak oxygen consumption over time predicts death or transplantation in adults with a
Fontan circulation Am Heart J 2017;189:184–
192
Serial exercise testing in a group of adult patients with a Fontan circulation demonstrated a
decline in peak oxygen consumption in those
who subsequently died or underwent cardiac transplantation NYHA functional class was not
Trang 3usefulness of objective indices of functional
capacity
Gouton M, Nizard J, Patel M, et al Maternal and fetal outcomes of pregnancy with Fontan
circulation: a multicentric observational study
Int J Cardiol 2015;187:84–89.
There has been a general reluctance to recommend pregnancy in women with a Fontan circulation, because of maternal and fetal/neonatal risk In this multicenter retrospective review, cardiac events occurred in 10% of women There was no maternal mortality There was a high incidence
of miscarriage, prematurity, and intrauterine growth retardation
Book WM, Gerardin J, Saraf A, Marie Valente A, Rodriguez F 3rd Clinical phenotypes of Fontan
failure: implications for management Congenit Heart Dis 2016;11(4):296–308.
This article reviews the various types of Fontan failure and provides a useful construct for their identification and for targeted treatment