@Article{chd.12570,
AUTHOR = {Laura S. Kverneland, Peter Kramer, Stanislav Ovroutski},
TITLE = {Five decades of the Fontan operation: A systematic review of international reports on outcomes after univentricular palliation},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {13},
YEAR = {2018},
NUMBER = {2},
PAGES = {181--193},
URL = {http://www.techscience.com/schd/v13n2/38976},
ISSN = {3071-1738},
ABSTRACT = {Almost fifty years after its first clinical application, the modified Fontan operation is among the
most frequently performed procedures in congenital heart disease surgery in children today. The
objective of this review is to systematically summarize the international evolution of outcomes in
regard to morbidity and mortality of patients with Fontan palliation. All studies published over the
past five decades with more than 100 Fontan patients included were screened. In eligible studies,
information concerning preoperative patients’ characteristics, Fontan modifications employed,
early mortality, long-term survival and frequency of relevant complications was extracted. Ultimately, thirty-one studies published by the largest surgical centers with an overall number of 9390
patients were included in this review. The extracardiac total cavopulmonary anastomosis is the
most frequently used Fontan modification. Hemodynamic data demonstrate a rigorous overall
adherence to suggested Fontan selection criteria. The analysis showed a clear trend toward
improved early and long-term survival over the time period covered. Although inconsistently
reported, severe complications such as arrhythmias, thromboembolic events and protein-losing
enteropathy as well as reoperations and reinterventions were frequent. In conclusion, patients palliated for complex univentricular heart malformations nowadays benefit from the experience and
technical developments of the past decades and have a significantly improved long-term prognosis.
However, important issues concerning postoperative long-term morbidity and mortality are still
unsolved and clear intrinsic limitations of the Fontan circulation are becoming evident as the population of Fontan patients ages.},
DOI = {10.1111/chd.12570}
}