[An experience of the modified Norwood’s operation for hypoplastic left heart syndrome with aberrant origin of right subclavian artery and persistent left superior vena cava–the procedure without total circulatory arrest and cardiac arrest].

The research discusses a successfully performed modified Norwood operation on a 21-day-old neonate diagnosed with hypoplastic left heart syndrome, which was further complicated by an aberrant right subclavian artery and a persistent left superior vena cava. The surgery was performed without inducing total circulatory or cardiac arrest, employing a new method that led to uneventful postoperative recovery.

Patient Profile and Diagnosis

• The patient was a 21-day-old neonate diagnosed with a rare congenital heart defect called hypoplastic left heart syndrome (HLHS) with additional vascular abnormalities – aberrant (irregular) origin of the right subclavian artery and a persistent left superior vena cava.
• HLHS is a condition where the left section of the heart is critically underdeveloped. The persistent left superior vena cava is a congenital anomaly in which the vein draining the left part of the head and upper part of the body into the heart remains persistent. An aberrant right subclavian artery occurs when the artery that typically supplies blood to the right arm deviates from its normal course.

Modified Norwood Operation

• The researchers described a new approach to the modified Norwood operation, a common treatment for HLHS, which was conducted without triggering full circulatory or cardiac arrest.
• During the aortic arch reconstruction portion of the surgery, a 4 mm Gore-Tex graft was used to maintain cerebral perfusion. This graft was attached between the right carotid artery and the right pulmonary artery for a systemic-pulmonary shunt, facilitating blood flow to the brain.
• Coronary perfusion, which maintains blood flow to the heart, was achieved by placing a small cannula on the relatively large ascending aorta during the anastomosis (connection) between the main pulmonary artery and the ascending aorta.

Aortic Arch Reconstruction and Results

• Equine pericardial patch was used in the reconstruction of the aortic arch and the ascending aorta was directly attached to a part of the main pulmonary artery (PA).
• The postoperative recovery was uneventful, demonstrating no complications or adverse events during recovery.
• Postoperative MRI findings confirmed successful aortic arch and pulmonary artery reconstruction, revealing no stenosis (narrowing) of the aortic arch and pulmonary artery.
• The successful recovery highlights the potential of the modified approach in the Norwood operation without total circulatory arrest and cardiac arrest, even in patients with compounding anomalies.