Bilateral Pulmonary Arterial Branches Rehabilitation in Alagille Syndrome

The patient is a fifteen-month-old, 9kg girl with Alagille syndrome and liver dysfunction who presented with suprasystemic right ventricular pressure due to severe bilateral branch pulmonary arterial stenoses. She underwent multiple transcatheter balloon dilation of her branch pulmonary arteries. Her preoperative liver function tests were abnormally elevated. Also, the preoperative computed tomography (CT) scan showed severe bilateral pulmonary arterial branches stenoses and hypoplasia with the right main branches affected more than the left.  

An echocardiogram showed a bicuspid aortic valve that was functioning normally with right ventricular hypertrophy. Cardiac catheterization showed multiple bilateral branch pulmonary arterial stenoses. The right and left main branch pulmonary arteries had a z score of -2.8 and -2.3 respectively, and the right ventricular pressure was suprasystemic at baseline. 

Through a median sternotomy, on a beating heart with mild hypothermia, the patient underwent bilateral branch pulmonary arterial augmentation with decellularized pulmonary homograft patches. Three patches were used on the left main pulmonary arterial branches, and four patches were used on the right side. The cardiopulmonary bypass time was 316 minutes. 

Postaugmentation right ventricular pressure was less than 50 percent of the systemic pressure. The chest was closed on the second postoperative day, and the patient was extubated twenty-four hours later. She was discharged on the eleventh postoperative day. 

References

1. Luong R, Feinstein JA, Ma M, Ebel NH, Wise-Faberowski L, Zhang Y, Peng LF, Yarlagadda VV, Shek J, Hanley FL, McElhinney DB. Outcomes in Patients with Alagille Syndrome and Complex Pulmonary Artery Disease. J Pediatr. 2021 Feb;229:86-94.e4
2. Mainwaring RD, Hanley FL. Surgical Techniques for Repair of Peripheral Pulmonary Artery Stenosis. Semin Thorac Cardiovasc Surg. 2017 Summer;29(2):198-205
3. Monge MC, Mainwaring RD, Sheikh AY, Punn R, Reddy VM, Hanley FL. Surgical reconstruction of peripheral pulmonary artery stenosis in Williams and Alagille syndromes. J Thorac Cardiovasc Surg. 2013 Feb;145(2):476-81

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