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Clinical practice variability among paediatric interventional cardiologists assessing pulmonary arteriovenous malformations

Published online by Cambridge University Press:  10 October 2025

Joshua Fields Open the ORCID record for Joshua Fields [Opens in a new window] ,
Jared Boon ,
Osama Aldoss ,
Susan R. Foerster ,
Todd M. Gudausky ,
Stephen B. Spurgin Open the ORCID record for Stephen B. Spurgin [Opens in a new window]  and
Andrew D. Spearman Open the ORCID record for Andrew D. Spearman [Opens in a new window]
Joshua Fields
Affiliation:
Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Children’s Wisconsin Herma Heart Institute, Milwaukee, WI, USA
Jared Boon
Affiliation:
Department of Pediatrics, Division of Quantitative Health Sciences, Medical College of Wisconsin, Milwaukee, WI, USA
Osama Aldoss
Affiliation:
Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Children’s Wisconsin Herma Heart Institute, Milwaukee, WI, USA Cardiovascular Research Center, Medical College of Wisconsin, Milwaukee, WI, USA
Susan R. Foerster
Affiliation:
Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Children’s Wisconsin Herma Heart Institute, Milwaukee, WI, USA Cardiovascular Research Center, Medical College of Wisconsin, Milwaukee, WI, USA
Todd M. Gudausky
Affiliation:
Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Children’s Wisconsin Herma Heart Institute, Milwaukee, WI, USA Cardiovascular Research Center, Medical College of Wisconsin, Milwaukee, WI, USA
Stephen B. Spurgin
Affiliation:
Department of Pediatrics, Division of Cardiology, University of Texas Southwestern Medical Center, Dallas, TX, USA
Andrew D. Spearman*
Affiliation:
Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Children’s Wisconsin Herma Heart Institute, Milwaukee, WI, USA Cardiovascular Research Center, Medical College of Wisconsin, Milwaukee, WI, USA
*
Corresponding author: Andrew D. Spearman; Email: aspearman@mcw.edu
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Abstract

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.

Keywords

pulmonary arteriovenous malformationsingle ventricleGlennFontanhepatic factorCHDechocardiography

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Type
Original Article
Information
Cardiology in the Young , First View , pp. 1 - 6
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Creative Common License - CCCreative Common License - BY
This is an Open Access article, distributed under the terms of the Creative Commons Attribution licence (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted re-use, distribution and reproduction, provided the original article is properly cited.
Copyright
© The Author(s), 2025. Published by Cambridge University Press

Introduction

Pulmonary arteriovenous malformations are vascular malformations that universally develop in patients with palliated single ventricle CHD. Reference Srivastava, Preminger and Lock1-Reference Spearman and Ginde5 Single ventricle pulmonary arteriovenous malformations were first reported more than 50 years ago when they were observed to develop after Glenn palliation. Reference Mathur and Glenn6,Reference McFaul, Tajik, Mair, Danielson and Seward7 Despite the long-standing clinical recognition, single ventricle pulmonary arteriovenous malformations remain poorly understood with no known medical treatments.

Contrast echocardiograms with agitated saline (bubble echos or bubble studies) are a commonly used clinical tool to assess for pulmonary arteriovenous malformations in single ventricle circulation and in hereditary forms of AVMs, such as hereditary haemorrhagic telangiectasia. Reference Chang, Alejos and Atkinson8–Reference Phimister, Bushee and Merbach13 Clinical practice guidelines for hereditary haemorrhagic telangiectasia were updated in 2020 and recommended screening for pulmonary arteriovenous malformations with bubble echocardiograms, but no such guidelines currently exist for single ventricle pulmonary arteriovenous malformations. Reference Faughnan, Palda and Garcia-Tsao14,Reference Faughnan, Mager and Hetts15 In fact, existing guidelines for hereditary haemorrhagic telangiectasia cannot be directly adapted for use in patients with single ventricle circulation due to variable and complex single ventricle circulation. For example, peripheral vein injections used in hereditary haemorrhagic telangiectasia can lead to false positive or false negative findings in single ventricle circulation due to veno-venous collaterals or streaming/unequal pulmonary blood flow, respectively. Ultimately, uniform clinical practice patterns in single ventricle CHD are necessary to facilitate multi-centre studies of this relatively small and heterogenous patient population. Thus, the objective of this study was to determine the clinical perspectives and practice patterns among paediatric interventional cardiologists who directly diagnose single ventricle pulmonary arteriovenous malformations.

Methods

Survey

We electronically surveyed paediatric interventional cardiologists using the Congenital Cardiovascular Interventional Consortium listserv. The Congenital Cardiovascular Interventional Consortium listserv is an email communication for Congenital Cardiovascular Interventional Consortium members to collaborate on clinical and research topics related to CHD. Congenital Cardiovascular Interventional Consortium members include paediatric interventional cardiologists predominantly from North America but also from South America and Europe. The Congenital Cardiovascular Interventional Consortium database is currently housed at Joe DiMaggio Children’s Hospital in Hollywood, Florida, USA. A single survey was distributed electronically with two subsequent reminder emails. Voluntary participants completed the anonymous survey electronically via RedCap. This survey study was reviewed and approved by the Institutional Review Board at the Medical College of Wisconsin. An Institutional Review Board-approved informational letter was included in the listserv emails and approved by the Institutional Review Board in place of a documented informed consent.

To identify the experience level of survey respondents, we collected demographic data on clinical experience and clinical practice location. To identify perspectives about the aetiology of single ventricle pulmonary arteriovenous malformations, we collected data on specific variables involved in single ventricle pulmonary arteriovenous malformation pathogenesis. To identify overall conceptual approaches to single ventricle pulmonary arteriovenous malformations (i.e., whom and when to test), we collected data on the frequency of bubble study testing pre- and post-Fontan, as well as the rationale for performing or not performing bubble studies. Lastly, to identify potential variability in the technical aspects of assessing single ventricle pulmonary arteriovenous malformations, we collected data on technical aspects of bubble study testing and pulmonary vein oximetry testing.

Statistical analysis

Data are expressed as median and interquartile range for continuous data and n (%) for categorical data unless otherwise stated. Analyses were performed using GraphPad Prism 10 (GraphPad Software, San Diego, CA).

Results

Respondent demographics

A total of 55 respondents completed the voluntary survey, which was sent to a list of 253 registered listserv members for the Congenital Cardiovascular Interventional Consortium, yielding a 21.7% response rate. Demographics, including years of experience as an attending paediatric interventional cardiologist, number of Glenn or Fontan catheterisations performed annually, and current practice location, are summarised in Table 1.

Table 1. Respondent demographics

N= number of respondents (percent).

Aetiology of single ventricle pulmonary arteriovenous malformations

Survey respondents overwhelmingly (54/55, 98.2%) believe that single ventricle pulmonary arteriovenous malformations develop due to a lack of hepatic vein blood flow to the lungs (i.e., lack of hepatic factor) (Table 2). The single respondent who did not select the hepatic factor response selected non-pulsatile flow as the causative factor in single ventricle pulmonary arteriovenous malformations. Multiple answers were allowed with this question, yet only a minority (8/55, 14.5%) selected more than one causative factor. Of the potential additional factors, non-pulsatile flow was the most common answer (7/55, 12.7%). A small minority (6/55, 10.9%) selected both lack of hepatic factor and non-pulsatile flow. The single respondent who selected “other” identified lack of hepatic venous blood flow and self-reported that pulmonary arteriovenous malformations form due to “other unknown factors.”

Table 2. Survey responses—aetiology of single ventricle PAVMs

N= number of respondents (percent).

* 64 total responses from 55 survey respondents with 8/55 respondents (14.5%) checking more than one row (i.e., multifactorial aetiology).

Conceptual considerations for assessing single ventricle pulmonary arteriovenous malformations

There was significant variability among respondents in various aspects of their conceptual approaches for assessing single ventricle pulmonary arteriovenous malformations (Table 3). In pre-Fontan catheterisations, a minority of respondents routinely perform bubble studies in over 50% of cases (11/55, 20.0%). In contrast, a larger proportion (17/55, 30.9%) perform pre-Fontan bubble studies in < 5% of cases. Among those who perform studies in < 5% of cases pre-Fontan, most responded (9/16, 56.3%) that bubble studies pre-Fontan do not impact clinical making. An additional open response commented that “AVMs large enough to be clinically significant are generally evident by angio. If small enough to need a bubble study, they don't impact decisions.” No respondents reported safety concerns about performing bubble echos.

Table 3. Survey responses—conceptual considerations for assessing single ventricle PAVMs

N= number of respondents (percent).

Similar to pre-Fontan catheterisations, a minority of respondents routinely perform bubble studies in over 50% of cases post-Fontan (8/55, 14.6%). A similar proportion (10/55, 18.2%) perform post-Fontan bubble studies < 5% of cases. Surprisingly, though, a larger proportion of respondents (22/55, 40.0%) perform bubble studies in 5–24% of pre-Fontan cases. Among those who perform studies in < 5% of cases post-Fontan, most respondents (6/10, 60.0%) similarly responded that bubble studies post-Fontan do not impact clinical decision making. An open response commented that “sometimes [perform bubble studies] to assess if Fontan has helped AVMs regress, but for the most part rely on other data to determine pulmonary arteriovenous malformations.” Similar to pre-Fontan, no respondents reported safety concerns about performing bubble echos.

Technical considerations for assessing single ventricle pulmonary arteriovenous malformations

When performing bubble studies in patients with single ventricle circulation, most, but not all, respondents (45/53, 84.9%) inject agitated saline separately into each branch of the pulmonary artery (Table 4). There was pronounced variation among respondents in the use of agitated saline injection for bubble studies (Table 4, Figure 1). A minority of respondents (15/53, 28.3%) reported using the same volume of air and saline for bubble study injections in all tests, but there was still variability among this group in the specific volumes used (Figure 1). Almost half of the respondents (26/43, 49.1%) reported that the volume of agitated saline they use for bubble studies varies from test to test. Factors influencing the variable amount of agitated saline included expected factors such as patient age and patient size; however, there were also unexpected factors influencing the volume of agitated saline, such as “available equipment” and “enough to make sure it’s not a false negative.”

Figure 1. Volumes of air and saline used by respondents who self-reported using the same volume of air and saline for all bubble studies. Scatter plot showing each response with median and interquartile ranges.

Table 4. Survey responses—echnical considerations for assessing single ventricle PAVMs

N= number of respondents (percent).

Beyond technical considerations of bubble studies, we also assessed technical considerations for measuring pulmonary vein oxygenation (Table 4). Nearly all respondents measure pulmonary vein saturations pre-Fontan (50/55, 90.9%), but there is variability in where and how. More than half (28/50, 56.0%) report collecting upper and lower pulmonary vein samples pre-Fontan, whereas a large proportion (21/50, 42.0%) report collecting whichever vein is most easily accessible. Lastly, most respondents (30/50, 60.0%) only collect pulmonary vein samples under baseline FiO2 conditions.

Discussion

In this survey of paediatric cardiology interventionalists, there is strong consensus about the aetiology of single ventricle pulmonary arteriovenous malformations, but there are pronounced differences in the conceptual and technical approaches for diagnosing single ventricle pulmonary arteriovenous malformations. These diagnostic differences highlight potential challenges in performing multi-institutional studies to improve our understanding of single ventricle pulmonary arteriovenous malformations or potential future clinical trials treating single ventricle pulmonary arteriovenous malformations.

Previous publications have hypothesised that single ventricle pulmonary arteriovenous malformations develop from lack of hepatic vein blood flow to the pulmonary vasculature (the so-called hepatic factor hypothesis), lack of pulsatile flow, or a combination of the two physiologic variables. Reference Srivastava, Preminger and Lock1,Reference Vettukattil, Slavik and Monro3,Reference Spearman and Ginde5,Reference Jonas16–Reference Field-Ridley, Heljasvaara and Pihlajaniemi22 While these variables have been given relatively equal weight in previously published literature, there was strong consensus among our respondents (98.2%) that single ventricle pulmonary arteriovenous malformations develop due to a lack of hepatic factor perfusion to the lungs, and only a minority (10.9%) selected both hepatic factor and non-pulsatile flow. Thus, the hepatic factor hypothesis is the current era consensus for single ventricle pulmonary arteriovenous malformation aetiology.

There are numerous guidelines in our field for follow-up and testing recommendations for CHD; however, our field does not yet have guidelines for when and how to assess single ventricle pulmonary arteriovenous malformations. In contrast, there are clear recommendations to screen and re-screen for pulmonary arteriovenous malformations with bubble echocardiography in patients with hereditary AVMs (i.e., hereditary haemorrhagic telangiectasia) and even detailed methodology for how to technically perform bubble echocardiograms in this patient population. Reference Velthuis, Buscarini and Gossage11,Reference Faughnan, Palda and Garcia-Tsao14,Reference Faughnan, Mager and Hetts15,Reference Beslow, Kim and Hetts23 Specifically, a review published in Journal of American Society of Echocardiography in 2015 recommended to perform bubble echos using 8 ml saline, 1 ml air,1 ml blood, and then subsequently inject 5 ml of this freshly agitated saline within 3 seconds in the antecubital vein. Reference Velthuis, Buscarini and Gossage11 This approach differs slightly from the 2014 guidelines for cardiac sonographers published in the same journal with recommendations to use 8 ml saline agitated with 0.5 ml room air injected through a forearm or hand vein (no specification of blood, volume of injection, or rate of injection). Reference Porter, Abdelmoneim and Belcik24 Importantly, these recommendations differ from single ventricle pulmonary arteriovenous malformation assessment where most assessments are performed in the catheterisation lab with direct injection into branch pulmonary arteries. We propose, based on our survey results and previously published protocols, to perform single ventricle pulmonary arteriovenous malformation bubble echos in each lung by agitating 9 ml saline with 1 ml air and injecting 5–10 ml of agitated saline within 3 seconds with a catheter positioned in the proximal aspect of each branch pulmonary artery.

Many respondents in our survey indicated that their resistance to diagnostic testing was because bubble studies do not impact clinical decision making. In other words, it may be currently futile to diagnose single ventricle pulmonary arteriovenous malformations because we lack medical therapies for treating single ventricle pulmonary arteriovenous malformations. Despite this perspective, recent studies have identified potential molecular pathways that may be involved in single ventricle pulmonary arteriovenous malformation pathogenesis. Reference Bartoli, Hennessy-Strahs, Dowling, Gaynor and Glatz25,Reference Bartoli, Hennessy-Strahs, Dowling, Gaynor and Glatz26 Previous and current research groups have also developed animal models that effectively phenocopy single ventricle pulmonary arteriovenous malformations. Reference Malhotra, Riemer and Thelitz27–Reference Wan, Rousseau and Mattern33 Thus, research into single ventricle pulmonary arteriovenous malformations is progressing, identification of therapeutic targets in animal models is feasible, and clinical trials may realistically begin in the near future.

This cross-sectional survey has several limitations that are inherent with electronic survey studies. Foremost, we are limited by our small sample size with potential for selection bias. Our respondents are all paediatric cardiology interventional physicians, with most respondents having > 15 years of clinical experience as a cardiac interventional attending physician; however, responses may differ among paediatric cardiology sub-specialists who may refer patients for cardiac catheterisation. Additionally, despite providing opportunities for free-text responses, our survey is at risk for response bias.

In conclusion, our survey demonstrates that clinical practice patterns vary widely among paediatric interventional cardiologists. A standardised clinical approach, new diagnostic tools, Reference Spurgin, Arar and Zellers34 or both are needed to advance our field’s approach to diagnosing, studying, and potentially treating single ventricle pulmonary arteriovenous malformations.

Acknowledgements

This study was supported by the National Institutes of Health from the National Heart, Lung, and Blood Institute (K08HL157510 - ADS), the Medical College of Wisconsin Department of Pediatrics, and the Herma Heart Institute Innovation Funds. The authors would like to acknowledge Nancy Sullivan (Memorial Healthcare System, Joe DiMaggio Children’s Hospital) for her help in facilitating communication with the Congenital Cardiovascular Interventional Consortium listserv.

Author contribution

Designing a research study—JF, JB, TMG, SRF, ADS

Acquiring data—JF, JB, ADS

Analysing data—JF, JB, TMG, SRF, SBS, ADS

Writing and editing the manuscript—JF, TMG, SRF, SBS, ADS

All authors approve the final version of this manuscript.

Disclosures

The authors have no relationships with industry and no conflicts of interest.

Study approval

This study was approved by the Medical College of Wisconsin Institutional Review Board (#49458).

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Figure 0

Table 1. Respondent demographics

Figure 1

Table 2. Survey responses—aetiology of single ventricle PAVMs

Figure 2

Table 3. Survey responses—conceptual considerations for assessing single ventricle PAVMs

Figure 3

Figure 1. Volumes of air and saline used by respondents who self-reported using the same volume of air and saline for all bubble studies. Scatter plot showing each response with median and interquartile ranges.

Figure 4

Table 4. Survey responses—echnical considerations for assessing single ventricle PAVMs