Researchers discovered that patients with severe pulmonary arterial hypertension (PAH) who are at risk of giant pulmonary artery aneurysms (PAAs) should undergo lung transplantation and reconstruction of the pulmonary artery unless contraindicated, as published in the Journal of Cardiac Surgery.

Due to the availability of better therapies, physicians are less likely to recommend lung transplantation as a therapeutic strategy for patients with severe PAH. As for PAA, it is an extremely rare disease but counts PAH among its causes. Patients with PAH who also have a large PAA are at a high risk of complications, such as dissection or rupture.

There is debate around the best surgical strategy to adopt for patients with severe PAH and a giant PAA. Doi and colleagues sought to examine existing medical literature on the best surgical course of action to take. Two surgical strategies stand out: heart-lung transplantation, as well as lung transplantation with pulmonary artery reconstruction.


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“Heart-lung transplantation is usually performed in patients with severe left ventricular dysfunction or underlying complex congenital heart disease and severe PAH with PAA,” Doi and colleagues wrote.

At the Alfred Hospital in Melbourne, Australia, heart-lung transplantations are performed through a clamshell incision. The advantage of this method is that the donor hila are positioned anterior to the phrenic nerve, providing easy access to the posterior mediastinum should complications (such as bleeding) occur.

As for lung transplantation with pulmonary artery reconstruction, Doi et al reported that the procedure “is being increasingly recognized as an alternative in experienced lung transplantation centers.” When performing this procedure, it is vital that pulmonary arterial and right ventricular pressures are monitored, especially when extensive reconstruction is needed.

“PAA > 60 mm and symptoms associated with PAA should be considered for early listing for transplantation,” the authors concluded. “Unless there is a mandatory indication to replace the heart, we recommend [bilateral lung transplantation] and reconstruction of the pulmonary artery in patients with PAH and PAAs.”

Reference

Doi A, Gajera J, Niewodowski D, et al. Surgical management of giant pulmonary artery aneurysms in patients with severe pulmonary arterial hypertensionJ Card Surg. Published online January 18, 2022. doi:10.1111/jocs.16235