Pulmonary artery stenosis should be treated with surgery or other intervention in patients with Alagille syndrome (ALGS), according to a meta-analysis published in the journal Advances in International Cardiology.

The author of the review paper reported that treatment reduced the right ventricular to left ventricular pressure ratio by 25%. Researchers also found no difference in terms of recovery, reintervention, or death rates between patients treated with surgery or interventionally.

Pulmonary artery stenosis is the most common vascular complication of Alagille syndrome. 

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To clarify the clinical features, management, and outcomes of pulmonary artery stenosis in this disease, Shi-Min Yuan, MD, PhD, from the Department of Cardiothoracic Surgery, The First Hospital of Putian, Fujian Medical University, in Putian, China, conducted a meta-analysis of the literature. 

Read more about the complications of Alagille syndrome

Dr. Yuan identified 38 studies of ALGS with arteriohepatic dysplasia or pulmonary artery stenosis, atresia, or hypoplasia. The studies included a total of 401 ALGS patients with pulmonary artery pathologies. Of these, the majority (65.8%) did not receive an intervention for their pulmonary artery stenosis. Of patients who did receive an intervention, 76 had percutaneous interventions and 61 had pulmonary artery stenosis surgery.

There were no differences between the right ventricular pressures or the ratio of right ventricular pressure over left ventricular pressure before and after the operation. 

There were also no significant differences in the ratio of right ventricular pressure over left ventricular pressure between patients with no residual pulmonary artery stenosis treated interventionally, those with residual pulmonary artery stenosis who were treated interventionally, and those treated surgically.

The recovery rate, reintervention rate, or mortality rate did not differ between patients who were treated interventionally or those who underwent pulmonary artery stenosis surgery. 


Yuan S. Pulmonary artery pathologies in Alagille syndrome: a meta-analysis. Advances in Interventional Cardiology/Postępy w Kardiologii Interwencyjnej. 2022;18(2):111-117. doi:10.5114/aic.2022.118526