Fate of Patients With Single Ventricles Who Do Not Undergo the Fontan Procedure

Background The Fontan procedure, the last of a series of palliative operations for patients born with single ventricles, is associated with a significant late burden of complications. There are other strategies for patients who are suboptimal candidates for Fontan completion; however, the long-term outcomes of these different surgical options have not been clearly elucidated. We performed a systematic literature review to establish the current role of other treatment approaches besides the Fontan procedure. Methods The MEDLINE and Embase databases were systematically searched for articles describing the long-term outcomes of patients with single ventricles who have not received the Fontan procedure. Results A total of 36 articles met all inclusion criteria. There is a scarcity of contemporary data on the non-Fontan cohort. Historical studies provided a significant contribution. Conclusions Long-term survival of unoperated patients with single ventricles is possible under the rare conditions of having balanced hemodynamics. As many as half of patients may survive on only a systemic-to-pulmonary artery shunt or bidirectional cavopulmonary shunt for more than 20 years with reasonable functional status. In patients with a failing single ventricle, the bidirectional cavopulmonary shunt is an excellent bridge to heart transplantation and may provide better posttransplant survival than patients with a Fontan circulation. Currently, the Fontan procedure continues to be the best definitive palliation for patients born with single ventricle lesions. However, for those with borderline indications, other strategies should be carefully considered.