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Published on: August 26, 2025
Primary pulmonary vein stenosis during infancy: state of the art review
David B Frank1,2, Philip T Levy3,4, Corey A Stiver5,6
1Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Insights
Primary pulmonary vein stenosis (PPVS) is an emerging infant condition. A multidisciplinary team framework is proposed for diagnosis, management, and treatment, emphasizing collaborative research for this rare disease.
Area of Science:
- Pediatric Cardiology
- Neonatology
- Medical Research
Background:
- Primary pulmonary vein stenosis (PPVS) is an increasingly recognized condition in infants, distinct from acquired stenosis.
- Optimal management strategies for PPVS remain poorly defined, despite growing insights into its pathophysiology and therapeutic options.
Purpose of the Study:
- To establish a unified framework for the diagnosis, management, and treatment of infant PPVS.
- To highlight the need for institutional and multi-institutional research to address knowledge gaps, particularly in ex-premature infants.
Main Methods:
- A multidisciplinary healthcare team convened to review existing evidence on PPVS.
- The team aimed to develop a common approach for clinical care and research.
Main Results:
- The review emphasizes the critical role of multidisciplinary teams in accelerating clinical learning and patient care.
- Multi-institutional collaborations are essential for generating sufficient statistical power to advance knowledge on rare diseases like PPVS.
Conclusions:
- Establishing dedicated, diverse healthcare teams within institutions is vital for improving care for infants with PPVS.
- Collaborative research networks are crucial for advancing understanding and treatment of rare pediatric cardiovascular conditions like PPVS.
Abstract:
Primary pulmonary vein stenosis (PPVS) is an emerging problem among infants. In contrast to acquired disease, PPVS is the development of stenosis in the absence of preceding intervention. While optimal care approaches remain poorly characterized, over the past decade, understanding of potential pathophysiological mechanisms and development of novel therapeutic strategies are increasing. A multidisciplinary team of health care providers was assembled to review the available evidence and provide a common framework for the diagnosis, management, and treatment of PPVS during infancy. To address knowledge gaps, institutional and multi-institutional approaches must be employed to generate knowledge specific to ex-premature infants with PPVS. Within individual institutions, creation of a team comprised of dedicated health care providers from diverse backgrounds is critical to accelerate clinical learning and provide care for infants with PPVS. Multi-institutional collaborations, such as the PVS Network, provide the infrastructure and statistical power to advance knowledge for this rare disease.
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