NECCA 2016 Annual Meeting Summary

by Lucy Arnold

This year’s meeting was attended by about 60 participants and took place at the Portland Regency Hotel and Spa located in the heart of beautiful downtown Portland.  After breakfast and a video highlighting aortopathies prepared by Steve Sanders , we were welcomed by our Maine host Mike Epstein, who also highlighted the history, beauty and length of Maine’ coastline and other boasts.

NECCA president Naomi Gauthier began with a reminder that we all have implicit biases which hinder our ability to see people for who they truly are. One of our organizational goals is to continue to break down incorrect assumptions (implicit bias) through empathy, collaboration. and the pursuit of evidence based research aimed to educate ourselves and others. (For those interested, the implicit Association Test that was mentioned can be found at There were updates on NECCA governance including an open invitation for people to get involved, a big thank you to the three outgoing board members (Audrey Marshall, Jeremy Asnes, and Michael Willers) as well as a thanks to Jim Lock and Bill Hellenbrand for their contributions to the inception of NECCA and NECCRF.  We also welcomed three new members: Rahul Rathod, Sara Ford, and Rob Elder. President-Elect David Kane was introduced. This year he will work with Naomi in preparation for next year when she will step down after 9 years of untiring leadership.

We enjoyed excellent food, drink, and warm collegiality. This year’s meeting again demonstrated that we are a group with the stamina, energy, and growing friendships to foster and develop important regional projects that improve the care of our patients with potentially life threatening illness.  We learned from our newest NECCA recruit Duke Cameron that novel ideas that revolutionize care such as Helen Taussig’s BT shunt are often fostered in environments like NECCA that encourage creativity, foster listening, and minimize assumptions (implicit bias). Our charge is to use the tools of genetics and newer technologies to define mechanisms of illness which can guide new evidence based therapies for our patients.  It is also critical for us to not allow our patients to be labeled as “defective.”  Instead, we hope to help them define and achieve their personal goals and dreams from childhood into adulthood.

We look forward to having you join us for next year’s meeting at the Equinox Golf Resort and Spa in Manchester Vermont on October 20-22, 2017!

NECCA COMMITTEE REPORTS: A report from the NECCA committees began with an introduction from Lucy Arnold (NECCA meeting planning committee chair), a committee developed largely because of the tremendous growth we have  seen as an organization with an increasing number of new NECCRF grants and NECCA projects. This was followed by updates from representatives from the advocacy committee (Pip Hidestrand) and the QI committee (Yvonne Paris) The Advocacy committee has chosen to focus their first project on better preparing adolescents as they make the transition to adult care by developing tools/guidelines for them to understand their condition, and be better able to manage their own care with help from qualified congenital heart specialists in their region. The QI committee continues to be busy and productive.  Work continues on the Syncope SCAMP (including re-design), prolonged QT SCAMP, and a new Pacemaker Project.  A highlight of the year was publication of a paper entitled “Regional Implementation of Pediatric Cardiology Syncope Algorithm using SCAMPs Methodology (Journal of AHA).

THEME TOPIC: Size Really Matters: Optimizing Management of the Enlarged Aorta in Patients with Aortopathies and Congenital Heart Disease. Mark Lindsay spoke first on “Aortic Disease Utilizing Genetics to Uncover Pathogenesis.” This was followed by Ron Lacro ‘s talk ”Aortic Enlargement: Clinical Presentations, Diagnostic Evaluations, and Medical Management,” which highlighted clinical applications of those lessons learned in the lab. Both Mark and Ron’s talks emphasized that although we worry about dissection and rupture, the risk for aortic dissection in young people is fortunately rare.  Absolute aortic size, however, may not actually translate into absolute risk for dissection depending on phenotype and family history, with Marfan’s syndrome for instance having higher risk than patients with congenital heart disease. Mark presented elegant research showing how losartan reversed aortic changes in knock out mice. The magnitude of this effect did not seem to translate to humans, however, as evidenced by the large trial led by Ron Lacro comparing beta blockers and losartan head to head. Although the losartan effect was not found to be superior, both were effective, and Ron recommended using adequate doses if an agent is to be used. Ongoing work in Mark’s lab has now turned to finding other modulating factors, including promising new intra- and extra-cellular agents that may suggest a new target for medical therapy.

Duke Cameron, began his talk “Surgical Management of the Dilated Aorta in Congenital Heart Disease” with a priceless taped interview of Helen Taussig discussing how the BT shunt came to be, which can now be viewed on the NECCCA website. He then provided historical perspective andchanging approaches to aortopathy surgery, where the decision to intervene at all is as important as the choice of operation. In patients with MFS, the area of aortic dilation is usually at the aortic root. If the aneurysm is greater than 5.0 cm dissection is more likely. Patients with bicuspid aortic valve are more likely to develop ascending aortic aneurysm which require attention. The highest risk groups are those with Loeys-Dietz and Ehler Danlos and possibly Turners as they can rupture at dimensions less than 5.0 cm. In contrast, those with Arterial Switch, after Norwood, or Ross procedures rarely develop aortic dissection or rupture. Dissections are reported in those s/p TOF repair with highest risk being those greater than 20 yrs post-op, pulmonary atresia, later repair and male gender. He concluded that further work is needed to establish more evidence based guidelines for intervention.

The moderated panel, led by Adrian Moran and including Ron, Mark, Duke Cameron, and Anne Marie Valente reviewed cases and identified significant gaps in knowledge in imaging, exercise, pregnancy, and lifestyle, and surgical and medical considerations. The breakout sessions explored these further and thought that exploring practice variation across NECCA could be the first step in developing consensus about approach.

After the final group discussion, plans were made to form a new Aortopathy Working Group with an aim to develop a regional management guideline for aortopathies regarding consistency of imaging, medical/surgical management, and exercise and lifestyle factors.  We hope some of you will consider joining this group, which already includes Ron Lacro and Mark Lindsay.

NECCA PROJECTS: We heard an update from Dominic Abrams on the Sudden Cardiac Death Initiative, which provides a process to systemically assess patients who have suffered sudden cardiac death as well as their living relatives. This organized system links all New England medical examiners, regional cardiologists, and a core lab in Boston to assess pathology. The goal is to have as seamless process for families that will phenotype relatives and assess risk, as well as have a thoughtful approach to those who deserve a molecular autopsy. This is underwritten by a generous private donation that funds the pathology review and, when appropriate, further gene testing.

Doug Mah presented their work on the NECCA Pacemaker/ICD Quality Improvement Project, which aims to develop a system to optimize follow up for all Cardiac Rhythm Medical Device (CRMD) patients within the NECCA framework. With the assistance of the project coordinator, Elsie Helou, as well as representatives from each state, 312 patients have been tracked to date. They have already found that 30% of patients have missed their annual clinic visit, and over 50% have missed telephone transmissions. Next steps include finalizing patient entry into the database, creation of educational materials, and site specific optimization plans. Funding was provided by a grant from NECCRF.

Rob Elder presented updates from the Fontan Working Group that was formed after last year’s meeting. Crucial to developing better Fontan management guidelines would be to identify characteristics of a patient with a “good” Fontan repair. The group has thus chosen to conduct a prospective regional study utilizing the “surprise question” to providers of: “Would you be surprised if your patient had a significant adverse event in the next year?“ The primary outcome measure would be to see if a “no” answer correctly identifies “good” Fontan repairs, and secondary outcomes would include whether any differences can be identified in clinical characteristics of those who were classified as a “good Fontan” vs those all others (the “at-risk” category).

In addition, a survey targeting pediatric and adult congenital cardiac providers in NE focuses on practice variation in surveillance and screening of Fontan patients is being circulated. If you haven’t completed it, the link to the Fontan Survey is

NECCRF RESEARCH: Scott Yeager, President of NECCRF, summarized some of the opportunities for grants, and introduced several of the present grant recipients, including updates from research by Brooke Davey, Laura Mansfield, and Sarah de Ferranti. As always, donations particularly by grateful parents looking to support congenital heart disease research is greatly appreciated.


Life Expectancy and Causes of Death in Marfan Syndrome (NEJM August 1972)
Aortic registry Trial (Januzzi et al Am J Card 2004)
Bicuspid Aortic Valve and Associated Aortic Dilation in Young (Fernandez et al, Heart 2012)Atenolol vs Losartan in Children and Young Adults with Marfan’s Syndrome (Lacro et al NEJM 2015)
Beta Blocker and Losartan (Brooke et al NEJM 2008)
Recent Clinical Drug Trials (Can J. Cardiol 2016 Singh and Lacro)
Causes of Sudden Cardiac Death (Maron JACC 2014)
Tabulation of Exercise Related Aortic Dissection (Elefteriades, Cardiology, 2006)


NECCA 2015 Annual Meeting Summary

The annual NECCA meeting took place this year at Wentworth by the Sea, in New Castle NH. The venue and the beautiful fall weather did not disappoint.

The meeting began with Steve Sanders narrating a live video, taking us through the history of the Fontan operation and its modifications over time using surgical diagrams and specimen demonstration.  After a welcome from Lucy Arnold on behalf of this year’s planning committee (Tom Johnson host chairman, Chris Clarke, Naomi Gauthier, Erin Horan, and Lucy Arnold NECCA planning committee chair), NECCA President Naomi Gauthier inspired us with clips of our patients who are happier and feel less alone when they meet and play with other children who also have congenital heart disease.

The committee reports demonstrate how much NECCA has grown in just six years. There is a new fledgling Advocacy committee that has begun to formulate its charge, and is looking for member input on a survey to refine its goals. If you have not already participated, please click on the following link to give your input: New members to this committee are welcome. If interested, please contact Sharon O’Brien at  The Communications Committee has done a lot of work completely redesigning and upgrading the website and also have  launched a new discussion forum that is just waiting for members to use.

Our QI group has made even further strides.  Chairman Yvonne Paris reviewed the Syncope SCAMP. 1317 patients have been enrolled, with 88% having mild typical syncope that was readily definable. A draft manuscript discussing this very common problem is close to completion. If published, this would be the second NECCA-based manuscript, the first being a product of work from the Chest Pain SCAMP.  The chest pain SCAMP was also reviewed by Dave Fulton. That particular SCAMP has been “retired” and is now moving into the primary care office setting. Mike Epstein reviewed the newly launched NECCA SCAMP on prolonged QT, the first SCAMP written and developed uniquely by the NECCA organization.

We then saw how a project launched through discussions just one year ago in Newport can gain tremendous momentum. The Sudden Cardiac Death project headed up by Dominic Abrams is now funded and NECCA is a key agent in providing the infrastructure to move toward actuation. Dominic has also applied for an NIH grant (also involving NECCA) that, if awarded, would provide significant additional resources to address this important problem.  We had a special guest, Dr. Jon Skinner, who has developed and runs New Zealand’s SCD registry. He presented an overview of their program and then stayed to assist the working group in developing a model for New England.

This seventh meeting’s central theme focused on management of the patient with Fontan physiology. The concept of developing a Fontan Health Maintenance Guideline was introduced by Tom Johnson.  Invited speaker talks, panel discussions, and breakout sessions prepared for a final group discussion on how to best implement a project to improve the care of our Fontan patients.

A talk by Ed Walsh on Electrophysiologic Considerations in the Fontan Heart demonstrated the great strides the EP world has made in its ability to understand mechanisms, identify, and treat common arrhythmias in our Fontan patients, but also illustrated the sobering link between aging Fontan physiology and poor hemodynamics and arrhythmias.

Keynote speaker Jack Rychik’s address entitled Not as Good as We think: the State of Affairs for the Patient with Single Ventricle after Fontan Operation further served to show that there is much we do not know, and much we need to study, in order to benefit our Fontan patients as they reach adulthood. He also presented their flagship Fontan survivorship program at CHOP.

After a great lunch, the moderated panel fielded questions/discussed cases presented, and breakout groups met to ask the question “How can we improve the care and monitoring of our patients with Fontan Physiology?” Fred Wu then summarized some of the recent events at the ACC/Heart House Aging Fontan Stakeholders meeting.

A well deserved cocktail reception sparked even further discussion in a more social setting and extended well into the evening for many participants.

Sunday morning started with a hearty breakfast for early risers, and we heard back from Dominic Abrams with an update from their working group on Sudden Cardiac Death. We also saw an impressive iPhone app developed by our very own Kevin Hall to recruit patients for a cardiomyopathy registry and project, utilizing a new medical research software library released by Apple. This opens up intriguing possibilities for NECCA as well.

Scott Yeager had reminded us about utilizing NECCRF grants to help fund projects, and encouraged people to find donors so more can be funded. NECCRF updates included a slide presentation with updates on Sarah DeFerranti’s lipid project which has shown significant progress in recruitment of patients and sites, is well on its way for data collection, and remains within budget. Brooke Davey then presented her NECCRF grant award for a fascinating project studying T-Cell Receptor Excision Circles (TREC) and the finding of low TREC levels in many patients with congenital heart disease. She is collaborating with colleagues at Connecticut Children’s and Yale to ask the question: Do lower TREC levels measured on newborn screens correlate with more infection related hospital stays due to relative immunodeficiency in patients with congenital heart disease?

John Fahey and Rob Elder tag-teamed the final lecture outlining the basic physiologic consequences of the Fontan circulation. The most striking aspect of their talks was provocative argument that some interventions we think are beneficial may actually cause more harm than good in our patients (ex: fenestration closure, or medications such as diuretics or beta blockers).

This set the stage for the liveliest discussion of the meeting, which was a group discussion about a possible Health Maintenance Guideline for Fontan patients introduced earlier by Fred Wu and Tom Johnson. Initial discussions focused on identifying high risk factors for poor outcome in Fontan, and then defining surveillance/intervention strategies as a guideline based on high vs low risk. Others turned the question around and asked what makes a “Good Fontan” good as they enter adulthood, and what modifiable risk factors might we find that would drive our surveillance?  The conversation continued with enthusiasm around getting a committee together to further this project over the coming year and explore the possibility of pursuing this as a formal SCAMP.

NECCA take home message: Collaboration empowers us to study uncommon and common problems with people who have crucial skills and knowledge across generations. We now have advanced communication applications to gather important data sets to rapidly implement change (often at a distance from each other and even from our patients). But we cannot forget the value of face to face relationships with our patients and with each other. We still have to ask the right questions, admit that what we thought was right may have been wrong, and that lab values and isolated images do not define our patients. Ultimately, if we can capture the best of rapidly changing technologies and still remember the necessity to listen and the value of human touch, we will help our patients be happy, active and healthy enough to embrace their futures.