Pediatric Pulmonary Vein Stenosis: A Shift in Perspective

Pediatric intraluminal pulmonary vein stenosis (PVS) is a rare and challenging disease characterized by wall thickening from neo-intimal proliferation leading to luminal narrowing of one or more pulmonary veins. Once considered a universally fatal disease, with the development of novel treatment strategies, current data indicates PVS is a chronic disease with improving survival rates.¹ Vulnerable populations include those with genetic comorbidities, premature infants with lung disease, and patients with congenital heart disease, particularly those with anomalous pulmonary venous connections.^1,2 Clinical presentation varies based on the severity of the disease with symptoms that include failure to thrive, feeding intolerance, respiratory failure and/or low cardiac output. Further evaluation with non-invasive imaging by echocardiogram, nuclear lung perfusion scan and/or cross-sectional imaging can provide early indication of PVS when such symptoms present in vulnerable populations. The diagnosis is confirmed with cardiac catheterization using angiography, intravascular ultrasound, and assessment of hemodynamics.³

Over the past decade, outcomes for patients with PVS have markedly improved due to advancements in both interventional and therapeutic strategies. An important factor in these improved outcomes is the growing recognition of the need for a multi-disciplinary approach with a dedicated team to care for this delicate patient population. This collaborative approach allows for early diagnosis, prompt intervention, and personalized treatment plans tailored to the individual needs of each patient.^5,6 Here is a proposed framework for establishing an effective PVS program within an institution:

1. Assemble a core team of committed professionals

Identify colleagues that have expressed interest in PVS. Invite physicians, advanced practice providers, interventional cardiologists, cardiothoracic surgeons, radiologists. etc. to commit to a regularly scheduled meeting to review patient data, clinical progress, and creation of individualized plans of care. Participants with expertise in pulmonary hypertension are particularly helpful. This dedicated time will allow for the development of your program.

2. Build collaborative relationships

Seek opportunities to communicate with team members outside of your specialty. For example, utilize clinical pharmacists for medical therapy management, dieticians for optimizing nutrition, and social work for supportive services. Be inclusive of supplemental team members and value feedback to aid in decision making.

3. Create educational opportunities

Be visible; attend inpatient rounds and establish frequent contact with care teams to foster relationships that encourage others to ask questions. This will also allow you to reinforce the individualized plan of care with each contact.

Be specific in your documentation regarding risk factors as well as clinical signs and symptoms to be aware of. Create educational documents that can be accessed for easy reference. Offer to lecture on PVS in local and regional educational series. All these efforts help to maintain continuity, promote visibility of treatment plans, and empower medical providers to care for PVS patients.

4. Track program development

The way to drive your program forward is to track patient data. A simple spreadsheet or workbook can house your number of new patients, initiation of medical therapy, interventions performed, etc. Tracking data will allow for analysis of program growth, highlight gaps or needs in patient populations, and provide ideas for future research and planning. In turn, this will allow transparency of the program among senior leaders and management and provide easily accessible data when advocating for future resources.

The complexity of this disease and the challenges it presents in terms of both diagnosis and treatment underline the importance of ongoing collaboration. With a dedicated team, we can continue to improve our understanding of PVS and be hopeful that the future holds even greater advancements, further reducing morbidity and mortality, and ultimately offering affected children a brighter and healthier future.

1. Callahan, R., Morray, B. H., Hirsch, R., & Petit, C. J. (2022). Management of Pediatric Pulmonary Vein Stenosis.Journal of the Society for Cardiovascular Angiography & Interventions, 1(5), 100391. https://doi.org/10.1016/j.jscai.2022.100391
2. Swier, N. L., Richards, B., Cua, C. L., Lynch, S. K., Yin, H., Nelin, L. D., Smith, C. V., & Backes, C. H. (2016). Pulmonary Vein Stenosis in Neonates with Severe Bronchopulmonary Dysplasia.American journal of perinatology, 33(7), 671–677. https://doi.org/10.1055/s-0035-1571201
3. Callahan R, Gauthier Z, Toba S, Sanders SP, Porras D, Vargas SO. Correlation of Intravascular Ultrasound with Histology in Pediatric Pulmonary Vein Stenosis. Children (Basel). 2021 Mar 4;8(3):193. doi: 10.3390/children8030193.
4. Cory, M. J., Ooi, Y. K., Kelleman, M. S., Vincent, R. N., Kim, D. W., & Petit, C. J. (2017). Reintervention Is Associated With Improved Survival in Pediatric Patients With Pulmonary Vein Stenosis. Cardiovascular interventions, 10(17), 1788–1798. https://doi.org/10.1016/j.jcin.2017.05.052
5. Rachel D. Vanderlaan, Osami Honjo, Commentary: It takes a village: Changing the trajectory of pulmonary vein stenosis outcomes, The Journal of Thoracic and Cardiovascular Surgery, Volume 159, Issue 3,n 2020, Pages 1037-1038, ISSN 0022-5223, https://doi.org/10.1016/j.jtcvs.2019.09.100
6. Ireland, C. M., Callahan, R., & Jenkins, K. J. (2021). Patient and Family-Centered Care for Pediatric Intraluminal Pulmonary Vein Stenosis: Case of a 3 Year Old Patient with Focus on Nurse Practitioner Role.Children, 8(7), 567. https://doi.org/10.3390/children8070567