Fontan circulation is a well-established palliation in patients with functional single ventricles. Absence of a sub-pulmonary pumping chamber creates a unique physiology in which blood flow is mainly guided by negative intrathoracic and elevated central venous pressures. Various pulmonary anatomic or pathophysiologic changes can jeopardize optimal Fontan circulation. Long-term survival of patients who have undergone the contemporary total cavopulmonary connection is satisfactory. Thorough literature review in conjunction with accumulated clinical experience can lead clinicians to extract conclusions regarding Fontan and lung interactions indicating the purpose of this review.
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