Pulmonary Vein Stenosis in Children: Multi-Disciplinary Care for a Complex Condition
Jul 09, 2018
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
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 possibleabout 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.
Carl H. Backes, Jr, MD, is a member of the Center for Perinatal Research at The Research Institute at Nationwide Children's Hospital. He is an assistant professor of pediatrics at The Ohio State University College of Medicine, attending neonatologist and attending cardiologist at Nationwide Children's Hospital.
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