Primary Pulmonary Vein Stenosis: Outcomes, Risk Factors, and Severity Score in a Multicentric Study

Area of Science:

• Cardiovascular Surgery
• Thoracic Surgery
• Medical Research

Background:

• Primary pulmonary vein stenosis (PPVS) presents a significant challenge with a historically poor prognosis.
• Identifying reliable prognostic factors for PPVS repair outcomes remains crucial for current treatment strategies.

Purpose of the Study:

• To evaluate the outcomes of primary pulmonary vein stenosis repair in the contemporary era.
• To identify independent prognostic factors influencing patient survival and disease recurrence after PPVS repair.

Main Methods:

• Retrospective analysis of 30 patients with PPVS and normal pulmonary vein connection from 10 centers (2000-2012).
• Development and application of a specific pulmonary vein stenosis (PVS) severity score.
• Univariate and multivariate analyses to determine risk factors for death, reoperation, and restenosis.

Main Results:

• Overall rates of PV restenosis, reoperation, and mortality were 50%, 40%, and 30%, respectively.
• At 8 years, freedom from mortality was 70%, freedom from reoperation was 62%, and freedom from restenosis was 47%.
• Postoperative pulmonary hypertension and a high PVS severity score were independent predictors of adverse outcomes.

Conclusions:

• Primary pulmonary vein stenosis continues to have a guarded prognosis despite advancements like the sutureless technique.
• Postoperative pulmonary hypertension and the severity of the disease, assessed by a novel score, are critical independent prognostic factors.