Improving Outcomes in Pulmonary Vein Stenosis

Columbus, OH — June 2018

Depending on the publication, the mortality rate for primary infant pulmonary vein stenosis could be anywhere from 30 percent to 80 percent. This uncertainty in outcomes makes it difficult to know when conservative management or aggressive intervention makes the most sense – or even how to talk to patient families about prognoses.

So a group of doctors and researchers at Nationwide Children’s Hospital, in what appears to be the first review of its kind, pooled data from 48 studies over the last 45 years to determine what could definitively be said about this disease’s outcomes, and what is still left to know. The study was published in The Journal of Pediatrics.

There are some clear trends, says Carl H. Backes, MD, lead author of the study and a member of both the Division of Neonatology and The Heart Center at Nationwide Children’s:

  • The more veins that are involved, the worse the prognosis is. Mortality incidence in infants with one to two vein stenoses was 36.1 percent across the studies, while the mortality incidence in infants with 3 to 4 vein stenoses was 83.3 percent.
  • Most stenoses are diagnosed and monitored through echocardiography, but computed tomography and magnetic resonance imaging likely provide a better overall picture of the condition.
  • The outcomes for patients haven’t changed much in 40 years.

But beyond those key points, it can be difficult to draw conclusions about pulmonary vein stenosis.

“In some ways, I feel energized having done this, because there are clear gaps in our knowledge and in the literature that we can work to fill,” says Dr. Backes, who is also an assistant professor of Pediatrics at The Ohio State University College of Medicine. “At the same time, this review was sobering. The data is so heterogeneous that it can be hard to find something to hold on to.”

Pulmonary vein stenosis as a post-surgical complication is somewhat more common and better understood, according to the authors. This Nationwide Children’s review focused on patients younger than 1 year of age who developed stenosis unrelated to a procedure. The majority of studies included in the review had fewer than five participants who fit those parameters.  

“These are single-center studies, and most institutions see just a few cases per year,” says Dr. Backes. “Nationally and internationally, we really need to get on the same page about how we measure this disease and how we report outcomes. Only then can we start working together to advance the field.”

A step in that direction is the multicenter Pulmonary Vein Stenosis Network registry study, led by The Hospital for Sick Children in Toronto, with a goal of enrolling 500 children. Nationwide Children’s is a network site.

Nationwide Children’s has also begun a multidisciplinary pulmonary vein stenosis program to better implement risk stratification and other care protocols for patients. Brian Boe, MD, an interventional cardiologist at Nationwide Children’s, is a creator of the program.

“Our Nationwide Children’s Hospital team is working diligently with these patients and collaborating with other pediatric institutions to improve the care of this rare but serious disease process,” says Dr. Boe.

Backes CH, Nealon E, Armstrong AK, Cua CL, Mitchell C, Krishnan U, Vanderlaan RD, Song MK, Viola N, Smith CV, McConnell PI, Rivera BK, Bridge J. Pulmonary vein stenosis in infants: a systematic review, meta-analysis, and meta-regression. The Journal of Pediatrics.  2018 Apr 9. [Epub ahead of print]