Challenges of univentricular physiology in heterotaxy

Patients with heterotaxy syndrome exhibit an extensive constellation of congenital cardiac malformations, making these patients a challenging group to manage surgically. Many of these patients' hearts do not lend themselves to separation of the pulmonary and systemic circulations except by some modification of the Fontan procedure. Palliative procedures early in life are directed at creating a satisfactory balance of pulmonary and systemic blood flow and at the same time ensuring unobstructed pulmonary venous return. Early conversion from parallel pulmonary and systemic circulations to superior cavopulmonary connections is important, to reduce volume work of the systemic ventricle. Heterotaxy patients are generally considered a high-risk population with respect to eventual Fontan procedure. It is important to appreciate the unique and variable anatomy of the sinus node and conduction system and the potential for occult pulmonary venous obstruction, atrioventricular valve regurgitation, and recurrent cyanosis, which may be related to the development of pulmonary arteriovenous malformations.