Congenital – Original Submission
Pulmonary Vein Stenosis: Outcomes in Children With Congenital Heart Disease and Prematurity

https://doi.org/10.1053/j.semtcvs.2018.09.027Get rights and content

Pulmonary vein stenosis (PVS) is a rare condition that has been linked to prematurity and congenital heart disease (CHD). Despite these associations, treatment options are limited and outcomes are guarded. We investigated differences in PVS outcomes based on the presence of CHD and prematurity, and risk factors for mortality or lung transplantation in PVS. Single-center retrospective cohort study of patients diagnosed with PVS between January 2005 and May 2016 and identified by ICD codes with chart validation. Cox proportional hazard models assessed risk factors for the composite outcome of mortality or lung transplantation. Ninety-three patients with PVS were identified: 65 (70%) had significant CHD, 32 (34%) were premature, and 14 (15%) were premature with CHD. Sixty-five (70%) underwent a PVS intervention and 42 (46%) underwent ≥2 interventions. Twenty-five subjects (27%) died or underwent lung transplant 5.8 months (interquartile range [IQR] 1.1, 15.3) after diagnosis. There was no difference in age at diagnosis or mortality based on presence of CHD or prematurity. PVS diagnosis before age 6 months and greater than 1 pulmonary vein affected at diagnosis were associated with higher mortality (hazards ratio [HR] 3.4 (95% confidence interval 1.5, 7.5), P = 0.003, and HR 2.1 per additional vein affected (95% confidence interval 1.3, 3.4), P = 0.004, respectively). Survival in children with PVS is poor, independent of underlying CHD or prematurity. Younger age and greater number of veins affected at diagnosis are risk factors for worse outcome. Understanding causal mechanisms and development of treatment strategies are necessary to improve outcomes.

Section snippets

Background

Pulmonary vein stenosis (PVS) is a rare entity, with a previously estimated prevalence of 0.03% in patients with congenital heart disease (CHD).1, 2 While PVS can be present after intervention for primary pulmonary vein anomalies,3 such as total and partial anomalous pulmonary venous return, in recent years, there have been numerous reports of isolated PVS in patients with structurally normal hearts.4, 5, 6, 7, 8, 10 In addition, PVS has been described in association with prematurity,

Methods

We conducted a retrospective cohort study of patients with confirmed diagnosis of PVS followed at the Children's Hospital of Philadelphia between January 2005 and May 2016. Subjects were first identified by database inquiry for ICD-9 codes 747.4 (anomalies of pulmonary veins) or 747.49 (stenosis of pulmonary veins). Diagnosis of PVS was confirmed by manual review of charts, including echocardiographic, catheterization, and MRI/CT reports, as available. Subjects met inclusion criteria if they

Study Cohort

Ninety-three subjects were included in this study. Sixty-five subjects (70%) had significant CHD and 32 (34%) were premature. Fourteen (15%) had CHD and were premature. There were no significant differences in gender, race, ethnicity, age of diagnosis, or presence of genetic syndromes based on the presence of CHD or prematurity (Table 1). Pulmonary hypertension was more common among subjects without CHD (P = 0.04) and among premature subjects (P = 0.003).

CHD characteristics are listed in Table 2

Discussion

Outcomes for patients with PVS are generally poor. In this study, we report risk factors for mortality in a large group of patients with PVS. We found significant mortality within 1 year of diagnosis, independent of underlying diagnosis of CHD and/or prematurity. History of prematurity or CHD is not independently associated with poor survival. Younger age at PVS diagnosis and greater number of pulmonary veins involved were identified as independent risk factors for death or lung transplantation.

Conclusions

PVS is associated with poor outcomes independently of CHD, prematurity, or treatment strategy. Early age and greater than 1 pulmonary vein affected at diagnosis stratify patients toward a greater risk and should be considered in patient counseling and follow-up. Further study is needed to evaluate causal mechanisms of PVS in CHD and premature infants, with the long-term goal of developing treatment strategies that could lead to improved survival.

References (30)

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    Nevertheless, the sutureless technique in our current series was not significantly associated with improved results compared with the other 2 techniques. In this series, patient-related factors, including young age and low body weight at the initial repair, premature birth, and associated cardiac anomalies, were not found to increase the risk of adverse outcomes, which was consistent with the results of prior studies.4,5,24,25 Several single or multiinstitutional reports have pointed out the prognostic performance of PPVS severity scoring systems and yet showed the correlation between severity score and outcomes, including death and reintervention.2,5,6,20,21

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Funding: This work was supported by the National Institutes of Health [NIH grants T32 HL007915 (MPD), K01HL125521 (LMR)], and Pulmonary Hypertension Association supplement to K01HL125521 (LMR).

Conflicts of Interest: None.

The Children's Hospital of Philadelphia IRB: #16-013070, Approved 9/8/16.

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