Clinical practice variability among paediatric interventional cardiologists assessing pulmonary arteriovenous malformations
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Updated: Jul 30, 2026
Comprehensive Endovascular and Open Surgical Management of Cerebral Arteriovenous Malformations
Published on: October 20, 2017
Joshua Fields1, Jared Boon2, Osama Aldoss1,3
1Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Children's Wisconsin Herma Heart Institute, Milwaukee, WI, USA.
Insights
Diagnosing single ventricle pulmonary arteriovenous malformations (PVAMs) lacks standardized practices among pediatric cardiologists. This variability highlights the need for uniform approaches and new diagnostic tools for better patient care.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Single ventricle pulmonary arteriovenous malformations (PVAMs) are poorly understood and inconsistently assessed.
- Standardized clinical practice patterns are crucial for multi-center studies and improved understanding of PVAMs.
Purpose of the Study:
- To evaluate the clinical perspectives and practice patterns of pediatric interventional cardiologists in diagnosing single ventricle PVAMs.
Main Methods:
- A survey was electronically distributed to pediatric interventional cardiologists via the Congenital Cardiovascular Interventional Consortium listserv.
- Anonymous survey responses were collected electronically, with two reminder emails sent to encourage participation.
Main Results:
- Of 55 respondents, 98% agreed PVAMs result from absent hepatic vein flow to the lungs, but practice patterns varied significantly.
- A minority (20%) frequently used bubble studies pre-Fontan, while many (31%) rarely did, citing limited impact on clinical decisions.
- Nearly half (49%) lacked a standard volume for agitated saline injection during bubble studies.
Conclusions:
- Significant variation exists in clinical practice patterns among pediatric cardiology interventionalists regarding PVAM diagnosis.
- There is a clear need for a standardized clinical approach and potentially novel diagnostic tools to improve the diagnosis, study, and treatment of PVAMs.
Background:
Single ventricle pulmonary arteriovenous malformations are poorly understood and variably assessed in published literature. To improve our understanding of single ventricle pulmonary arteriovenous malformations and facilitate multi-centre studies, it will be necessary to have uniform clinical practice patterns among paediatric heart institutions.
Objectives:
The aim of this study was to assess paediatric interventional cardiologists' clinical perspectives and practice patterns for diagnosing single ventricle pulmonary arteriovenous malformations.
Methods:
We surveyed paediatric interventional cardiologists using the Congenital Cardiovascular Interventional Consortium listserv. A single survey was distributed electronically with two subsequent reminder emails. Voluntary participants completed the anonymous survey electronically via RedCap.
Results:
Among 253 Congenital Cardiovascular Interventional Consortium members, a total of 55 (21.7%) paediatric cardiology interventional attending physicians completed the survey. There was near unanimity (98%) that pulmonary arteriovenous malformations develop due to lack of hepatic vein blood flow to the lungs; however, there was wide variation among practice patterns. A minority (20%) of respondents perform bubble contrast echocardiograms (bubble studies) more than half the time pre-Fontan, whereas many (31%) almost never (< 5% of cases) perform bubble studies pre-Fontan. Most respondents reported that they did not perform bubble studies because results do not impact clinical decision making pre-Fontan (56%) or post-Fontan (60%). Many respondents (49%) do not have a typical volume of agitated saline that they inject for bubble studies.
Conclusions:
Clinical practice patterns vary widely among paediatric cardiology interventionalists. A standardised clinical approach, new diagnostic tools, or both are needed to standardise our field's approach to diagnosing, studying, and treating single ventricle pulmonary arteriovenous malformations.
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