Pulmonary Vein Stenosis Program

What is pulmonary vein stenosis (PVS)?

Pulmonary vein stenosis (PVS) is a rare and serious cardiovascular condition in which oxygenated blood from the lungs cannot easily return to the left side of the heart to be pumped out of the body. The affected areas of the lung become congested and the right side of the heart may have difficulty pumping blood to the lungs. This situation causes children with PVS to experience heart failure. If the disease worsens and goes untreated, PVS can be life-threatening.

There are two main forms of PVS diagnosed in children:

  1. Primary, also known as idiopathic, is often seen in infants who are born prematurely and may have prematurity-associated lung disease called chronic lung disease.
  2. Secondary, also known as post-surgical, can develop following surgical vein repair like in total anomalous pulmonary venous return (TAPVR).

When infants develop PVS, it is often seen in only one of four to five veins of the heart. However, PVS is a disease that can worsen in the affected veins over time and spread to other veins that were previously normal. Progression of PVS is one of the unique aspects of this disease, and progression to other veins and previously healthy areas makes PVS particularly challenging and dangerous.

What are symptoms of PVS?

In most infants and children, PVS causes heart failure and breathing problems. In addition, some children may have difficulty gaining weight or experience delayed growth and development.

How is PVS treated?

PVS is a treatable disease that requires diligence, close follow-up and close attention in case the condition worsens.

There are two main forms of treatment:

  1. Medical therapy: Medications are an important aspect of treating PVS. This may include diuretics, which can help relieve congestion in the lungs and ease symptoms of PVS. In some cases, children are also treated with medications for pulmonary hypertension, a type of high blood pressure that affects the arteries in the lungs and right side of the heart. In addition, the Children's Healthcare of Atlanta PVS Program has begun trials with novel medication, including immunosuppression medications, such as rapamycin and Gleevec. These medications show early promise in slowing or diminishing the worsening of PVS.
  2. Direct vein therapy: Infants and children with PVS will likely undergo surgical or catheter-based intervention to treat affected veins. A child with PVS will typically undergo regular cardiac catheterizations so that the affected pulmonary veins can undergo balloon dilation or stenting to maintain the veins and keep them from closing off. Oftentimes, catheterizations are performed frequently—almost monthly—for the first two years of a child's life. However, as the disease begins to slow down, usually by age 2, the need for this type of treatment slows, with many older children undergoing a catheterization only one to two times each year.

What makes the PVS Program at Children's unique?

The Children’s PVS Program includes a multidisciplinary team within the Children's Heart Center that was started in 2017. We have one of the largest populations of patients with PVS in the country, with more than 130 children treated in our program over the past five years.

The PVS Program team, led by Medical Director, James Kuo, MD, includes Michael Briones, DO, Pediatric Hematologist and Oncologist; Shannon Jones, MD, Pediatric Cardiologist; Divya Suthar, MD, Pediatric Cardiologist; and Timothy Slesnick, MD, Pediatric Cardiologist; as well as Rosemary Gray, RN, Clinical Nurse Coordinator.

Our PVS Program offers advanced medical therapy to our patients and our team treats the most severe cases of PVS. We also collaborate with an international group, the Pulmonary Vein Stenosis Network, which is run by The Hospital for Sick Children in Toronto, Ontario.

The Children’s PVS Program will be hosting the first-ever Pulmonary Vein Stenosis Symposium in April 2021 in Atlanta. A faculty of 40 specialists with backgrounds in cardiac surgery, catheterization, neonatology, pulmonology and cardiology will bring their expertise for this two-day conference.