Interorgan Communication-Pulmonary Vein Stenosis in Children-A Review of Epidemiology, Pathophysiology, and Current

Usha S Krishnan1, Mary P Mullen2,3

  • 1Department of Pediatrics (Pediatric Cardiology), Vagelos College of Physicians and Surgeons of Columbia University Irving Medical Center, Morgan Stanley Children's Hospital of New York Presbyterian, New York, New York, USA.

Comprehensive Physiology
|December 20, 2025
PubMed

Insights

Pulmonary vein stenosis (PVS) is a multi-organ disease impacting infants and adults. Early surveillance and understanding organ system communication aid in prompt diagnosis and tailored therapies for PVS.

Area of Science:

  • Cardiovascular Science
  • Pediatric Pulmonology
  • Pathophysiology

Background:

  • Pulmonary vein stenosis (PVS) is a complex condition affecting multiple organ systems, often linked to bronchopulmonary dysplasia (BPD) in preterm infants and congenital heart disease (CHD).
  • Pathophysiology involves inflammation in organs like the lungs and gut, alongside blood flow perturbations from intracardiac shunts or thoracic structures.
  • Genetic factors, flow dynamics, shear stress, and other proliferative triggers contribute to PVS development, particularly in CHD patients.

Purpose of the Study:

  • To elucidate the multi-organ communication pathways involved in the pathophysiology of pulmonary vein stenosis.
  • To highlight the importance of understanding these interactions for developing effective therapeutic strategies.
  • To emphasize the role of early surveillance and diagnostic protocols in managing PVS.

Main Methods:

  • Review of existing literature on PVS pathophysiology and multi-organ involvement.
  • Analysis of factors contributing to PVS, including inflammation, blood flow dynamics, and genetic predispositions.
  • Evaluation of surveillance protocols and their impact on diagnosis and intervention.

Main Results:

  • Understanding inter-organ communication is crucial for comprehending PVS pathophysiology and guiding therapy.
  • Early surveillance protocols enable prompt diagnosis and targeted interventions.
  • Development of tailored drug therapies is facilitated by a deeper understanding of the disease process.

Conclusions:

  • Effective management of PVS requires a comprehensive approach considering its multi-organ nature.
  • Early detection through enhanced surveillance improves patient outcomes.
  • Continued research into the underlying mechanisms of PVS will drive further therapeutic advancements.

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