Congenital – Original SubmissionPulmonary Vein Stenosis: Outcomes in Children With Congenital Heart Disease and Prematurity
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.
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Cited by (30)
Cardiovascular computed tomography in pediatric congenital heart disease: A state of the art review
2022, Journal of Cardiovascular Computed TomographyCitation Excerpt :Primary pulmonary vein stenosis is a rare entity and has been described in association with prematurity, particularly in those with chronic lung disease. It has relatively poor outcomes despite interventions, with the development of pulmonary arterial hypertension with progressive disease.66,67 Secondary pulmonary vein stenosis in pediatric patients occurs most commonly postoperatively in ∼10% patients after repair of TAPVC.
Management of Pediatric Pulmonary Vein Stenosis
2022, Journal of the Society for Cardiovascular Angiography and InterventionsPredictive Factors for Postoperative Intensive Care Unit Admission and Mechanical Ventilation After Cardiac Catheterization for Pediatric Pulmonary Vein Stenosis
2022, Journal of Cardiothoracic and Vascular AnesthesiaCitation Excerpt :Male sex was more associated with ICU admission and postoperative MV. The increased prevalence of male sex also was reported by others;24,25 however, it is not clear how to explain this association. The anesthetic management of PVS can be challenging, particularly in patients with high RVSP and/or RV dysfunction.
Transcatheter Cardiac Interventions in the Newborn: JACC Focus Seminar
2022, Journal of the American College of CardiologyCitation Excerpt :Pulmonary vein stenosis is a rare and serious condition associated with prematurity and CHD. This disease is often progressive and severe, carrying a high risk of mortality, particularly for patients diagnosed at a younger age and with multivessel involvement.75 Although data regarding neonatal catheter-based intervention are limited, balloon angioplasty and stent placement are central tenets of treatment for infants with pulmonary vein stenosis.
Pulmonary vein stenosis: Anatomic considerations, surgical management, and outcomes
2022, Journal of Thoracic and Cardiovascular SurgeryClinical Outcomes, Predictors, and Surgical Management of Primary Pulmonary Vein Stenosis
2022, Annals of Thoracic SurgeryCitation Excerpt :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
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.