What is the Stage I surgical repair method for hypoplastic left heart syndrome?

The correct answer is the Norwood procedure, which is the primary surgical intervention used in the management of hypoplastic left heart syndrome (HLHS) during Stage I of treatment. This complex surgery is designed to reconstruct the heart so that it can adequately supply blood to the body despite the underdevelopment of the left side of the heart.

The Norwood procedure typically involves several key steps: creating a new aorta from the small, hypoplastic left ventricle and connecting it to both the right ventricle and the pulmonary arteries. This enables the right ventricle to pump blood to both the lungs and the body, compensating for the inadequate left-sided structures. Additionally, a shunt is often placed to enhance pulmonary blood flow. The Norwood procedure is crucial in establishing systemic and pulmonary circulation in HLHS patients.

This procedure is part of a staged surgical approach, which also includes the Glenn procedure and the Fontan procedure in later stages, aimed at progressively improving the function of the heart as the child grows. However, the Norwood is specifically designated as the first step in this palliative surgical management of HLHS.