Surgically treating peripheral pulmonary artery stenosis reduces right ventricular pressure by 70%, according to a new study published in The Journal of Thoracic and Cardiovascular Surgery. Treatment also led to freedom from death in 94% of cases and the maintenance of low pressure in 90% of cases after 3 years.

“These results suggest that the surgical treatment of [peripheral pulmonary artery stenosis] is highly effective in most patients,” first author L. Mac Felmly, MD, and the coauthors of the study wrote.

Peripheral pulmonary artery stenosis, a type of congenital heart disease, is common among patients with Alagille syndrome (ALGS) as well as other diseases.


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In the present study, a team led by Frank L. Hanley, MD, from the Division of Pediatric Cardiac Surgery, Stanford University School of Medicine in California summarized their experience with the surgical treatment of peripheral pulmonary artery stenosis.

Read more about the comorbidities of ALGS

They analyzed 145 patients, 39 of whom had ALGS. All patients underwent surgery to treat their peripheral pulmonary artery stenosis.

The median right ventricle to aortic peak systolic pressure ratio for all patients before the operation was 1.01. This was reduced to 0.3 after the operation. Three patients, 2 of whom had ALGS, died in the hospital, while 4 died later. Eighty-two patients had subsequent catheterization and 74 had a pressure ratio below 0.5. 

Peripheral pulmonary stenosis is defined as a narrowing of 1 or more of the pulmonary artery branches. The stenosis causes an increase in right ventricular pressure to systemic levels. This can lead to right ventricular hypertrophy, an increase in myocardial oxygen consumption, and eventually right ventricular failure. 

Alagille syndrome is a rare genetic disease caused by mutations in the JAGGED1 gene and, in a small proportion of cases, the NOTCH2 gene, leading to abnormalities in the heart, face, skeleton, eyes, and liver.

Reference

Felmly LM, Mainwaring RD, Collins RT, et al. Surgical repair of peripheral pulmonary artery stenosis: a two-decade experience with 145 patients. J Thorac Cardiovasc Surg. Published online August 10, 2022. doi:10.1016/j.jtcvs.2022.07.037