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Intraluminal Pulmonary Vein Stenosis in Children: A "New" Lesion
Viviane G Nasr1, Ryan Callahan2, Zachary Wichner1
1From the Department of Anesthesiology, Critical Care and Pain Medicine, Division of Cardiac Anesthesia, Boston Children's Hospital, Boston, Massachusetts.
Insights
Pulmonary vein stenosis (PVS) is a rare childhood disorder causing vein narrowing, leading to severe complications. Early diagnosis and multidisciplinary treatment are crucial for managing this condition.
Area of Science:
- Pediatric Cardiology
- Vascular Biology
- Rare Diseases
Background:
- Pulmonary vein stenosis (PVS) is a rare, progressive narrowing of extrapulmonary veins, affecting both children and adults.
- In children, intraluminal PVS is associated with prematurity, bronchopulmonary dysplasia, and genetic syndromes, carrying a significant mortality risk.
- The condition can lead to pulmonary hypertension, right ventricular failure, and death if untreated.
Purpose of the Study:
- To review the current understanding of intraluminal pulmonary vein stenosis in children.
- To discuss diagnostic modalities, treatment strategies, and prognostic factors for pediatric PVS.
- To highlight the challenges and outcomes associated with managing this rare disorder.
Main Methods:
- Review of existing literature on pediatric pulmonary vein stenosis.
- Analysis of diagnostic techniques including cardiac catheterization, pulmonary vein angiography, MRI, CT, and echocardiography.
- Evaluation of therapeutic interventions such as catheter-based procedures, surgery, medical therapy, and lung transplantation.
Main Results:
- The incidence of intraluminal PVS in children is estimated between 0.0017% and 0.03%.
- Mortality is approximately 50%, with predictors including extensive vein involvement, early onset, and hemodynamic compromise.
- Multiple treatment options exist, but morbidity and mortality remain high, emphasizing the need for ongoing monitoring and tailored interventions.
Conclusions:
- Pulmonary vein stenosis in children is a severe condition requiring a comprehensive management approach.
- Early diagnosis and timely, aggressive treatment involving a combination of interventions are essential.
- Despite advances, outcomes remain challenging, underscoring the need for further research and improved therapeutic strategies.
Abstract:
Pulmonary vein stenosis (PVS) is a rare disorder that leads to progressive narrowing of the extrapulmonary veins. PVS has been reported in both children and adults and in its worse iteration leads to pulmonary hypertension, right ventricular failure, and death. Multiple etiologies of PVS have been described in children and adults. This review will focus on intraluminal PVS in children. Intraluminal PVS has an estimated incidence ranging from 0.0017% to 0.03%. It is associated with conditions such as prematurity, bronchopulmonary dysplasia, necrotizing enterocolitis, Smith-Lemli-Opitz syndrome, and Down syndrome. Cardiac catheterization and pulmonary vein angiography are the gold standard for diagnosis and anatomic delineation. Other imaging modalities including magnetic resonance imaging, chest tomography, and transesophageal echocardiography are increasingly being used. Mortality of PVS in children is approximately 50%. Predictors of mortality include involvement of ≥3 pulmonary veins, bilateral pulmonary vein involvement, onset of PVS in infancy, elevated pulmonary artery pressure or systolic pulmonary artery-to-aortic pressure ratio, right ventricular dysfunction, restenosis after surgery, distal/upstream disease, and disease progression to previously uninvolved pulmonary veins. Treatment includes catheter-based pulmonary vein dilations with or without stenting, surgical interventions, medical therapy, and in some instances, lung transplantation. Cardiac catheterization for PVS involves a comprehensive hemodynamic and anatomic assessment of the pulmonary veins as well as therapeutic transcatheter interventions. Several surgical strategies have been used. Sutureless repair is currently most commonly used, but patch venoplasty, endarterectomy, ostial resection, and reimplantation are used in select circumstances as well. Medical therapies such as imatinib mesylate and bevacizumab are increasingly being used in an effort to suppress the myofibroblastic proliferation seen in PVS patients. Lung transplantation has been used as an alternative treatment strategy for end-stage, refractory PVS. Nonetheless, despite the different innovative approaches used, morbidity and mortality remain high. At present, the preferred treatment strategy is frequent reassessment of disease progression to guide use of catheter-based and surgical interventions in conjunction with medical therapy.