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Congenit Heart Dis. 2012 Jul-Aug;7(4):378-86. doi: 10.1111/j.1747-0803.2012.00647.x. Epub 2012 Apr 2.

Bilateral disease and early age at presentation are associated with shorter survival in patients with congenital heart disease and intraluminal pulmonary vein stenosis.

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Department of Cardiology, Children's Hospital Boston, Boston, MA 02115, USA.



Pulmonary vein stenosis (PVS) is a progressive disease that is frequently lethal. We have previously identified neoproliferation of myofibroblasts as the mechanism for progressive intraluminal PVS. PVS occurs in association with other congenital heart diseases (CHD) and in structurally normal hearts. This study sought to describe the spectrum of CHD seen with PVS and explore risk factors associated with mortality.


All patients diagnosed over a 12-year period with a combination of PVS involving ≥2 vessels and CHD were identified. Cases were categorized according to major anatomic and physiologic categories. Patient and disease characteristics associated with time to death were explored.


Eighty-two cases followed longitudinally at our institution were analyzed. Anatomic diagnoses included nonheterotaxy + anomalous pulmonary venous return (29%), heterotaxy + anomalous veins (20%), two ventricles + normal veins (22%), and single ventricle + normal veins (29%). Median age at diagnosis was 5.3 months (0-24 years). Despite multiple treatments, there were 35 (43%) deaths in the group with an estimated survival of 71%, 64%, and 44% at 1, 2, and 5 years, respectively. Bilateral disease at diagnosis (hazard ratio [HR] 3.9 [1.7, 9.2], P= .002), age <5 months at diagnosis (HR 3.4 [1.6, 7.6], P= .002), and involvement of >2 pulmonary veins at diagnosis (HR 3.7 [1.6, 8.8], P= .003) were associated with shorter time to death in univariate analysis. In multivariable analysis, both bilateral disease (HR 2.9 [1.2, 7.1]P= .02) and age <5 months at diagnosis (HR 2.4 [1.1, 5.6]P= .03) were independently associated with time to death.


Bilateral disease and earlier age at diagnosis are independent predictors of poor survival in patients with CHD and PVS, while patients with unilateral disease presenting at an older age have a better prognosis. These findings are helpful in risk stratification of patients with CHD and multivessel PVS.

[Indexed for MEDLINE]

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