Surgeons presented a tutorial describing the techniques involved in bilateral branch pulmonary arterial reconstruction in a 10-month-old male infant with Alagille syndrome (ALGS) and systemic/suprasystemic right ventricular pressure.

The case was written up in the Multimedia Manual of Cardio-Thoracic Surgery. 

Clinical evaluations of patients with ALGS can sometimes reveal elevated right ventricular pressure due to central branch pulmonary arterial stenosis and hypoplasia. In these cases, transcatheter interventions are usually inappropriate due to the complexity and multiplicity of the obstruction. 

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“In infants, we prefer surgical augmentation of these branches, which provides adequate decompression of the right ventricle and improves the patient’s haemodynamics,” the authors wrote. “The surgical procedure is usually extensive and complex and needs a longer cardiopulmonary bypass run.” 

Read more about Alagille syndrome etiology 

The surgeons presented a 10-step tutorial on how to conduct this surgery by recording a video of their performing this procedure on a 10-month-old boy with Alagille syndrome and systemic/suprasystemic right ventricular pressure, as well as advanced liver disease. First, a median sternotomy is performed, allowing access for a thymectomy and a pericardiotomy. The right main branch pulmonary artery is then dissected, followed by the left. At this point, a cardiopulmonary bypass should be initiated. 

The next step in this tutorial is the transection of the main pulmonary artery, which allows distal branches to be more easily exposed. Surgeons are then advised to create a plan for pulmonary artery branch reconstruction using data from preoperative computed tomography scans and intraoperative assessments. Once a plan is formulated, both the left and right pulmonary arteries are to be reconstructed. 

After these steps have been completed, a new pulmonary arterial confluence should be created. If any intracranial repair is needed, it should be performed at this point. The last few steps of the surgery are the reconstruction of the pulmonary artery and weaning the infant off of cardiopulmonary bypass. 

By completing these 10 steps, the child was discharged 6 days later and continued to do well upon follow-up. 


Said SM, Marey G. Bilateral branch pulmonary arterial reconstruction in the Alagille syndrome: the technique and its pitfallsMultimed Man Cardiothorac Surg. 2022;2022:10.1510/mmcts.2022.088. doi:10.1510/mmcts.2022.088