Pediatrics

Pulmonary Vein Stenosis

A rare form of heart disease treated by world-renowned experts

Pulmonary vein stenosis (PVS) is a rare and progressive disease affecting newborns and children of all ages. Despite aggressive treatment, PVS is a potentially lethal disease with no known cause or cure. Children with PVS need specialized care like that found at NewYork-Presbyterian, where our multidisciplinary team of pediatric specialists focuses on treating children with this complex

What is PVS?

In pulmonary vein stenosis (PVS), the pulmonary veins – which return oxygen-rich blood from the lungs –become increasingly narrowed. The veins continue to narrow until they are completely closed off. As a result, the right side of the heart struggles to pump blood into the lungs and the lungs become congested, which can lead to right heart failure.

There are two types of PVS:

Primary PVS, or congenital PVS, occurs in children with no prior surgery on the heart or the pulmonary veins. Primary PVS often develops in infants born prematurely or with lung inflammation from reflux.
Secondary PVS occurs in infants and children after surgical repair of the pulmonary veins due to congenital heart disease (CHD).

Some children may only have PVS in one pulmonary vein. Others may have multiple veins affected, in either one or both lungs. Regardless of the number of veins involved, PVS usually will worsen in the affected veins over time and spread to other veins that were previously normal, making it difficult to manage and treat.

Symptoms of PVS

The symptoms of PVS can vary in children from no symptoms to a variety of symptoms, including:

Fast breathing (tachypnea)
Fatigue or lack of energy
Poor weight gain
Frequent colds or chest infections
Bleeding in the lungs (coughing up blood)

About Our Program

The NewYork-Presbyterian pediatric cardiology program, operating across NewYork-Presbyterian Morgan Stanley Children’s Hospital and NewYork-Presbyterian Komansky Children's Hospital, provides advanced, multidisciplinary care of children with PVS, beginning at diagnosis and continuing throughout childhood. Because PVS requires chronic therapy and diligent follow-up to keep the pulmonary veins open and maintain the health of the lungs, a dedicated team of pediatric specialists get to know your child’s specific condition and continue to closely monitor your child, providing him or her with the safest, most advanced treatments available.

How we diagnose PVS

Many children are discovered to have PVS when they have tests for other reasons. Children who are suspected of having PVS may undergo the following tests to examine blood flow in the pulmonary veins and the function of the right heart:

Echocardiogram
Imaging tests, such as CT scan or MRI
Cardiac catheterization
Lung perfusion scan

How we treat PVS

Medical therapy: Medications may include diuretics to relieve congestion in the lungs, medications for pulmonary hypertension, or anti-inflammatory agents to slow the progression of PVS. Our pediatric cardiologists have developed some of the latest effective therapies for PVS, and lead national research efforts to improve the care of the most severely affected patients with PVS.
Catheter-based interventions: Stent implantation and/or balloon angioplasty are used to open pulmonary veins. Our pediatric interventional cardiologists perform these procedures on newborns, infants, and older children with PVS to maintain veins and even re-open previously closed-off veins to improve the blood flow in these children.
Surgery: Relieves narrowing of the veins, allowing the blood to flow unobstructed through the veins. Our pediatric cardiac surgeons are world-renowned experts in PVS surgery and offer several different surgical approaches to open narrowed veins in children and infants with PVS.

Ongoing Monitoring

Depending on your child’s specific PVS, our team of dedicated pediatric specialists monitors your child regularly to make sure his or her pulmonary veins and lungs remain healthy.

Our Approach to Care

Multidisciplinary and Collaborative Care

Doctor examining a newborn's head PVS requires chronic therapy and diligent follow-up to keep the pulmonary veins open and maintain the health of the lungs. Our pediatric cardiologists closely collaborate with interventional cardiologists, pediatric cardiac surgeons, pediatric pulmonologists, pediatric anesthesiologists, pediatric electrophysiologists, and pediatric cardiac nurses to diagnose PVS and provide your child with treatment tailored to your child’s needs.

Thoughtful and Compassionate Care

We care for you promptly, thoroughly, and with the thoughtfulness and compassion that you and your child deserve. As a parent or caregiver, we consider you to be part of your child's team. We encourage you to ask any questions you may have and let us know how we can support you and your family during this experience.

Why Choose Us

World leaders in pediatric interventional cardiology

NewYork-Presbyterian has particular expertise in pediatric interventional cardiology, a unique specialty that involves the non-surgical treatment of congenital and acquired cardiovascular disorders for even the smallest hearts. In our state-of-the-art, dedicated congenital cardiac catheterization laboratories, our interventional cardiologists perform cardiac catheterization, a minimally invasive procedure in which a long, flexible catheter (tube) is inserted into a blood vessel and guided to the heart and surrounding blood vessels to examine the structures inside. From infants to young adults, patients of all ages benefit from a full range of diagnostic and therapeutic interventional cardiology procedures that require only a single day of hospitalization and eliminate the need for open-heart surgery.

Seamless access to highly specialized services

Patients at NewYork-Presbyterian have seamless access to the exceptional resources of NewYork-Presbyterian Komansky Children's Hospital and NewYork-Presbyterian Morgan Stanley Children’s Hospital, where our compassionate teams provide highly specialized sub services for any need that may arise:

Pediatric Cardiac Critical Care: The Pediatric Cardiac Intensive Care Unit serves the needs of critically ill infants, children, and adolescents in need of specialized heart care, including those recovering from a heart transplant. This specialized unit is staffed by a highly skilled team of intensivists from multiple subspecialties, including anesthesiology, pediatric cardiology, pediatric heart surgery, neonatology (newborn care), critical care, and pulmonology (lung care).
Program for Pediatric Cardiomyopathy, Heart Failure & Transplantation: Cardiomyopathy is the leading reason for heart transplantation in children. NewYork-Presbyterian is one of the largest and most successful pediatric heart transplant centers in North America and the world.
Extracorporeal Membrane Oxygenation (ECMO) Program: ECMO is a short-term device to support the heart and lungs in very young patients, or in children whose anatomy will not permit a ventricular assist device. NewYork-Presbyterian/Columbia has been repeatedly designated a Platinum Level Award of Excellence in Life Support by the Extracorporeal Life Support Organization (ELSO) since the first year the Platinum designation was awarded.