Pulmonary Vein Stenosis (PVS) Requires Ongoing Intervention and Maintenance

Pulmonary vein stenosis (PVS) is cardiovascular condition in which oxygenated blood from the lungs cannot easily return to the left side of the heart to be pumped out into the body. The right side of the heart begins to struggle to pump blood into the lungs and the lungs become congested, which can lead children with PVS to experience heart failure. PVS, which is a progressive disease, is life-threatening if left untreated.

PVS affects mostly infants born prematurely. It may develop in the neonatal ICU shortly after birth or may be detected when babies are laboring to breathe or failing to gain weight.

“This is a rare disease that we are just beginning to understand better,” said Christopher Petit, MD, pediatric cardiologist and leader of the Children’s PVS Program team. “Who will get it and why are still poorly understood.”

PVS is a disease of inflammation that begins in the immature air spaces of a premature baby’s lungs, causing a narrowing of the pulmonary veins. The inflammation process can also be kicked off post-surgically after a patient is placed on a breathing machine. When infants develop PVS, it is often seen in only one of four to five veins of the heart. However, PVS is a progressive disease that can worsen in the affected veins over time and spread to other veins that were previously normal, making it particularly challenging to treat and manage.

Unlike many other cardiac conditions, PVS cannot be treated with a single therapy and then patients go on with their lives,” Dr. Petit said. “PVS requires chronic therapy and diligent follow-up to keep the veins open and to maintain the health of the lungs.”

1. Medical therapy: Medications may include diuretics, which can help relieve congestion in the lungs and ease symptoms of PVS, or 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 partnered with oncology doctors to study medications with anti-inflammatory agents, which may help slow the progression of PVS.
2. Direct vein therapy: Patients 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. Catheterizations are often 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 once or twice a year.

“Our goal in the PVS program is to keep all four veins as open as possible, preserving their ability to function,” Dr. Petit said.

Dr. Petit says he discovered early in his career that when a multidisciplinary team of experts is brought together to focus on a specific disease, outcomes improve. He envisioned such a team focusing on PVS and launched the Children’s PVS program in 2017, which now has one of the largest populations of PVS patients in the country. The PVS program team consists of pediatric cardiologists, an oncologist, a pulmonologist, a nutritionist and a program nurse to oversee patient surveillance, labs and therapies.

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The PVS Program team, led by Dr. Petit, 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.