700 Children's Blog

Pulmonary Vein Stenosis in Children: Multi-Disciplinary Care for a Complex Condition

Jul 09, 2018
image of young boy in a hospital bed

Pulmonary vein stenosis is a rare and sometimes devastating heart condition that doctors and researchers are still working to understand. Some studies show that children who have the disease have a good chance of survival, while other studies show that only 20 percent of affected children will live.

This uncertainty about outcomes has made it difficult for specialists to know what the best treatment is for each patient, or even how to talk to patient families about prognoses.

What is PVS?

A child may be diagnosed with PVS when the blood vessels that bring oxygen-rich blood from their lungs back to the heart are narrowed (stenosis). This can happen in one or multiple veins. If left unmonitored and untreated, PVS is a condition that can block the vein completely.

What are the symptoms of PVS?

Symptoms can come on very quickly, or the condition can progress more slowly with symptoms becoming more severe over time. Warning signs in both cases might include:

  • Shortness of breath
  • Rapid and shallow breathing
  • Rapid heartbeat
  • Fatigue
  • Poor appetite
  • Pale or “washed-out” skin hue
  • Blue-tinged appearance, called cyanosis, of the skin, lips or nail beds

What types of specialists help to treat PVS?

Because of the complexity of PVS, the typical treatment team has many different clinicians involved. This multi-disciplinary team includes cardiologists, cardiac surgeons and catheterization experts, radiologists, pulmonologists, neonatologists, nurse practitioners and researchers.

What is next for the treatment of PVS?

There is still much to learn about PVS in children. Though outcomes have not changed much for the last 40 years in pediatric cases, many centers including Nationwide Children’s are collaborating to learn as much as possible about the disease.

A recent study from The Heart Center at Nationwide Children’s has helped specialists understand that a child with single-vein stenosis can do well with minimal treatment and “watchful waiting,” while a child with four-vein stenosis may need aggressive intervention. For more information on how our cardiology and neonatology teams are working together address this disease in the youngest patients, visit NationwideChildrens.org/Heart or NationwideChildrens.org/Neonatology.

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Carl H. Backes, MD

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700 Children’s features the most current pediatric health care information and research from our pediatric experts – physicians and specialists who have seen it all. Many of them are parents and bring a special understanding to what our patients and families experience. If you have a child – or care for a child – 700 Children’s was created especially for you.