Congenital Heart Disease in Pediatric and Adult Patients by Ali Dabbagh Antonio Hernandez Conte & Lorraine Lubin

Author:Ali Dabbagh, Antonio Hernandez Conte & Lorraine Lubin
Language: eng
Format: epub
Publisher: Springer International Publishing, Cham

This anatomy complicates the neonatal presentation of TGA, as physiology more consistent with critically decreased pulmonary blood flow is present if the obstruction is severe, similar to patients with critical pulmonary stenosis or pulmonary atresia. Restoration of ductal patency is crucial to provide pulmonary blood flow. In cases of less severe obstruction, the typical TGA mixing physiology may be more obvious.

Palliative placement of a systemic to pulmonary shunt in the neonatal period allows delay of further surgical correction until the patient is an older infant. The most common surgical options for repair are the Rastelli procedure and the Nikaidoh reconstruction.

Rastelli palliation involves enlarging the VSD and patching it so that LV blood is ejected into the aorta. The potential for developing late subaortic stenosis exists if the intraventricular tunnel to the aorta is not adequately large, and with the patient’s growth, this becomes an increasing reason for reoperation. Optimal creation of the tunnel may be hampered by a small VSD, especially in the inlet position, or if atrioventricular valve tissue from either side straddles the VSD. The main pulmonary artery trunk is divided, the proximal stump oversewn, and a conduit is placed to create right ventricular to pulmonary artery continuity (Fig. 20.10). This also is a common cause of reintervention as the patient grows. As the RV to PA conduit functionally reduces the size of the RV, a hypoplastic RV is a relative contraindication to the procedure. Additionally, if there is an anomalous coronary artery crossing the RVOT, it may preclude placement of the conduit.

Fig. 20.10(a–c) Rastelli repair of TGA/VSD/LVOTO

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