Pulmonary Vein Stenosis (PVS)

Pulmonary vein stenosis remains a challenging disease. A new study led by David Kalfa, MD, PhD, Assistant Professor of Surgery at Columbia, evaluates current management and outcomes for PVS at several large medical centers treating adult congenital heart disease in Europe and North America. 

A promising approach to PVS repair, called the sutureless technique, was introduced two decades ago but long-term outcomes are still not optimal and prognosis still seems to depend on the initial severity of the disease.   

“We focused on patients operated on since the year 2000 to find out if diffuse PVO and peri-operative pulmonary hypertension are associated with worse outcomes, and if the sutureless technique produces better outcomes,” says Dr. Kalfa.

The study assessed 107 patients with native or acquired PVS who underwent a total of 141 pulmonary vein procedures.  Sixty percent of these procedures were sutureless repairs. 

Overall, there was no significant difference in mortality between sutureless and non-sutureless repair. Nevertheless, the sutureless technique seems to be more efficient in patients with acquired PVS, compared to those with congenital PVS. 

The study also offered a new severity score based on the echographic aspect of the pulmonary veins. This score may help guide the clinical decision-making process and allow surgeons to better predict the outcomes of this serious disease. The study also demonstrated that the mid-term and long-term prognosis was highly impacted by the initial severity of PVS and perioperative pulmonary hypertension.

 “Our analysis reveals that patients with both congenital and acquired PVS still have a poor prognosis,” says Dr. Kalfa.  “Restenosis, PV reoperation and mortality remain high.  However, we do have a new PVS severity score, based on easily adaptable imaging techniques, that allows us to predict outcomes.  A high severity score, pre-operative and post-operative pulmonary hypertension, are three independent risk factors associated with worse outcomes.”

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