How is CPB generally conducted for hypoplastic left heart syndrome?

In the management of hypoplastic left heart syndrome (HLHS) during cardiopulmonary bypass (CPB), the approach typically involves the use of a single venous cannula combined with an arterial cannula placed in the pulmonary artery (PA). This technique is crucial for addressing the unique anatomical and physiological challenges presented by HLHS.

In cases of HLHS, the left side of the heart is underdeveloped, leading to inadequate systemic blood flow. The use of a venous cannula allows for the efficient drainage of deoxygenated blood from the right atrium into the bypass circuit. By positioning the arterial cannula in the pulmonary artery, the perfusionist can provide oxygenated blood directly to the pulmonary circuit while minimizing excess volume loading to the underdeveloped left ventricle, which is critical since it cannot effectively pump blood to the systemic circulation.

This method is particularly beneficial as it facilitates adequate perfusion of both the systemic and pulmonary circulations without overloading the compromised structures of the heart. It is standard practice during the staged surgical approach to HLHS, especially during the Norwood procedure and subsequent interventions.