Peripheral Pulmonary Artery Growth in Alagille Syndrome after Central Pulmonary Artery Enlargement: A Case

Harada T1*, Fukae K1, Matsuo O2 and Ando Y1

1Departments of Pediatric Cardiac Surgery, Kumamoto City Hospital, Japan
2Departments of Pediatric Cardiology, Kumamoto City Hospital, Japan

^*Correspondance to: Takeaki Harada 

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Abstract:

Background: Peripheral pulmonary artery stenosis is a rare congenital defect that is frequently associated with Alagille syndrome and Williams syndrome. However, the optimal treatment of peripheral pulmonary artery stenosis is controversial. No reports have described central pulmonary artery enlargement in a patient with Alagille syndrome and peripheral pulmonary artery stenosis. We herein report a case in which central pulmonary artery enlargement enabled the peripheral pulmonary arteries, including the lobar and segmental arteries, to adequately grow. Case Report: We treated an infant with Alagille syndrome, tetralogy of Fallot, and peripheral pulmonary artery stenosis. At 54 days of age, a central shunt was placed to encourage development of the pulmonary artery. However, no pulmonary artery growth was achieved. Because of worsening hypoxemia, enlargement of the central pulmonary arteries as well as total correction of the tetralogy of Fallot were performed. Although the peripheral pulmonary artery stenosis was not reconstructed at the segmental and lobar levels, growth of the diameter of the right and left pulmonary arteries and their first branches, and improvement of the Nakata index were seen at 5 years postoperatively. Conclusion: Despite the difficulty of successfully surgically enlarging the peripheral pulmonary arteries in patients with Alagille syndrome, reconstruction of the central pulmonary arteries achieves sufficient enlargement of even the peripheral pulmonary arteries

Keywords:

Alagille syndrome; Peripheral pulmonary artery stenosis; Central pulmonary artery reconstruction

Cite the Article:

Harada T, Fukae K, Matsuo O, Ando Y. Peripheral Pulmonary Artery Growth in Alagille Syndrome after Central Pulmonary Artery Enlargement: A Case Report. Ann Clin Case Rep. 2024; 9: 2555..