(IV-3A) Adult patient after Fontan procedure with heterotaxy syndrome, vena cava inferior – pulmonary artery tunnel narrowing Tomkiewicz-Pająk L., Miszalski-Jamka T., Krupiński M., Banyś P., Olszowska M., Podolec M., Drabik L., Podolec P.
Experts: Janusz Skalski J., Jacek Kołcz J., Trojnarska O., Kopeć G., Miszalski- Jamka T.
Background Heterotaxy syndrome is a rare defect that involves the heart and other organs. It is a disorder that results in certain organs forming on the opposite side of the body. The incidence of typical forms is 2,5/100 000 births. Heterotaxy coming from greek word “heteros” meaning different and “taxis” meaning arrangement. These malformations are always associated with additional cardiac lesions such as univentricular heart (UV). The Fontan operation was introduced in 1968 and has become the definitive treatment for …