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Updated: Jun 24, 2025
Three-Dimensional Echocardiographic Method for the Visualization and Assessment of Specific Parameters of the Pulmonary Veins
Published on: October 28, 2020
Multimodal Assessment and Intramodal Comparison of Imaging Techniques for Pediatric Pulmonary Vein Stenosis with
Victor Kieu1,2,3, Stephanie S Handler4,5, Michael Mitchell4,5
1Division of Cardiology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, USA. victor.ht.kieu@gmail.com.
Insights
Non-invasive imaging like echocardiography (echo) and computed tomography angiography (CTA) show reliable correlation with cardiac catheterization for diagnosing pediatric pulmonary vein stenosis (PVS) and pulmonary hypertension (PH). Early diagnosis is crucial for preventing disease progression.
Area of Science:
- Pediatric Cardiology
- Medical Imaging
- Cardiovascular Diagnostics
Background:
- Pulmonary vein stenosis (PVS) is a rare, progressive pediatric disease requiring early diagnosis and treatment.
- Multimodality imaging is complex for PVS evaluation, with limited pediatric data on imaging modality correlation.
- Accurate diagnosis is essential to prevent progressive pulmonary hypertension and right ventricular dysfunction.
Purpose of the Study:
- To determine the reliability of computed tomography angiography (CTA), echocardiography (echo), lung perfusion scan (LPS), and cardiac catheterization (cath) in measuring pediatric pulmonary vein stenosis (PVS) and pulmonary hypertension (PH).
- To compare the diagnostic accuracy of non-invasive imaging modalities against cardiac catheterization in pediatric patients.
Main Methods:
- A single-center retrospective pilot study involving 15 pediatric patients.
- Inclusion criteria required echo, CTA, and cath within a 1-month period; LPS data was included if available.
- PVS defined as >2 mmHg gradient (echo/cath) or 50% diameter reduction (CTA); PH diagnosis based on cath and non-invasive imaging sensitivity/specificity.
Main Results:
- Fifteen patients enrolled, with 87% diagnosed with primary PVS.
- CTA showed better agreement with cath than echo for PVS identification, except for the left lower pulmonary vein.
- Echocardiography and CTA demonstrated excellent sensitivity (91%) and specificity (100%) for diagnosing pulmonary hypertension compared to cardiac catheterization.
Conclusions:
- Non-invasive imaging, specifically echocardiography and CTA, demonstrates acceptable correlation with cardiac catheterization for the initial evaluation and screening of pediatric PVS.
- These non-invasive modalities are valuable tools for assessing associated pulmonary hypertension in pediatric PVS cases.
- Further research is warranted to fully establish the role of these imaging techniques in managing pediatric PVS.
Abstract:
Pulmonary vein stenosis (PVS) is a rare, serious, and progressive disease in the pediatric population. Evaluation is complex and involves multimodality imaging. Diagnosis is important as early treatment to prevent progressive pulmonary hypertension and right ventricular dysfunction is essential. Adult studies have shown good correlation between various imaging modalities; however, there are limited data in children. This is a single-center retrospective pilot study to determine the reliability of measurement of pulmonary vein stenosis and pulmonary hypertension across different imaging modalities-computed tomography angiography (CTA), echocardiography (echo), lung perfusion scan (LPS), and cardiac catheterization (cath). PVS was defined as > 2 mmHg by echo and cath and/or 50% reduction in diameter by CTA. Patients had to have an echo, CTA and cath performed within a 1-month timeframe of one another to be included in the study, with LPS data included if testing was completed at initial evaluation. Fifteen total patients were enrolled; 87% were categorized as primary PVS; a condition not directly related to direct injury or prior surgical intervention. Twenty-seven total stenotic pulmonary veins were identified (mean 1.8, range 1-4). CTA had a slightly better agreement with cath than echo in identifying PVS in different vein locations except in the LLPV. Additionally, echo and CTA had excellent sensitivity (91%) and specificity (100%) compared to cath for diagnosis of PH. We conclude that non-invasive imaging of echo and CTA has an acceptable correlation to cardiac catheterization for screening and initial evaluation of PVS and PH, as directly related to PVS, in pediatrics.
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