This post was authored by Gerard R. Martin, MD, FACC, immediate past chair of the ACC’s Adult Congenital and Pediatric Cardiology Council.

“For the first 20 years of my son’s life, I felt as though I was driving with my eyes closed. My son was born with CHD, but during those first 20 years there was very little information available and very few support groups,” said Barbara DeMaria, a parent ambassador for the Adult Congenital Heart Association (ACHA), during the recent Congenital Heart Advocacy Day reception held at the ACC’s Heart House.

DeMaria is one of more than 100 patients, family members and health care stakeholders who attended the 7th Annual Congenital Heart Advocacy Day in Washington, DC, on March 18 and 19. The two-day event is designed to bring together congenital heart disease survivors, their families and health care providers to help educate Congress about the need for increased research and programmatic funding to help better understand the disease.  

This year, in particular, attendees met with lawmakers seeking support for National Institutes of Health research funding and Center for Disease Control and Prevention surveillance efforts. In addition, they sought to recruit additional members of Congress to join the Congressional Congenital Heart Caucus.  I had the honor of speaking to and hearing from DeMaria and others the day prior to them heading to Capitol Hill.

This lobby day, of which the ACC has been a long-time partner, is one of the key ways we continue to keep up the drum beat for continued advances in treatment and care for children and adults living with the disease. Not only is congenital heart disease still the nation’s number one birth defect, there are currently more than one million adults living with congenital heart disease thanks to advances that have already been made.

It’s events like this that showcase just how far we have come, that make me encouraged about the future of care for adults and children with congenital heart disease. For me, this lobby day symbolizes the coming together of physicians, patients and families in recognition of the need for collaboration with and among each other. It is this collaboration that I believe members of Congress and others are now beginning to appreciate, understand, and reward through programs, research and initiatives that can (and in some cases already are) not only save lives, but help those with the disease live longer, healthier and productive lives.

There is still much work to do – and more Advocacy Days to be had – but working together I am confident that we can continue to make progress in the treatment of congenital heart disease patients, like DeMaria’s son, all along the care continuum.

This post was authored by Robert Vincent, MD, CM, FACC, member of the ACC’s Adult Congenital and Pediatric Cardiology (ACPC) council and membership section.

Congenital Heart Awareness Week is an opportune time to raise awareness of the world's #1 birth defect, as well as acknowledge all that the ACC and its member volunteers and staff have done, and continue to do, to help cardiologists care for patients with congenital heart disease (CHD). While we’ve had many “wins” this past year (check out Dr. Jenkins’ previous blog post), there is still much to be done in the name of CHD patients and their families.

As the number of adult patients with CHD (ACHD) is only increasing, last week JACC published results of a survey of general adult cardiologists that looked at the prevalence of general adult cardiologists who care for ACHD patients, and identified patterns of consulting with ACHD specialists, and awareness of ACHD national clinical guidelines. Among other findings, a lack of access to ACHD specialists appears to be a significant gap in care, as 38.5 percent of respondents reported not having access.

In an effort to address these gaps, the ACC’s Adult Congenital and Pediatric Cardiology (ACPC) council and membership section is working with ACC's Chapters and the patient advocacy group, the Adult Congenital Heart Association, to educate general cardiologists through the Provider Action for Treatment of Congenital Heart disease (PATCH) program. The PATCH program also focuses on developing networking programs between ACHD subspecialty cardiologists and general cardiologists through grand rounds, visits to ACHD clinics, and more. In addition, webinars on various aspects of ACHD care are available for ACC's members. To view the archives webinars, click here.

In addition, every year the ACC teams up with the Adult Congenital Heart Association and Mended Little Hearts for a National CHD Lobby Day, and this year’s activities will take place on March 18 and March 19. The College will be hosting a welcome reception at Heart House on March 18.  To get involved contact Stephanie Mitchell at smitchel[at]acc.org.   

Finally, the newly revamped CardioSmart.org has a congenital heart defects condition center, and includes an overview, questions to ask a doctor, the latest news and research on the topic, how to get support through our partner organizations like Mended Hearts or Mended Little Hearts, and more. I encourage you to share these resources and information with your patients.

For those attending ACC.13, there are several pediatric cardiology and ACHD-focused sessions, including the 2013 Dan G. McNamara Lecture with Aldo Castaneda, MD, FACC, who will be discussing the historical development of pediatric cardiac surgery.  Interested attendees should also check out the congenital cardiology solutions learning pathway.

As always, ACC members interested in congenital heart disease issues are welcome to join the College’s Adult Congenital and Pediatric Cardiology membership section. Contact Stephanie Mitchell at smitchel@acc.org if interested.

This post is authored by Kathy Jenkins, MD, MPH, FACC, chair of the Adult Congenital and Pediatric Cardiology Council and Section.

In our profession it is always pleasing when years of efforts and advocacy pay off and we finally get a “win.”  In this case, the win is for congenital heart disease (CHD) providers and patients since the American Board of Medical Specialties (ABMS) has announced it will create a physician certification in the Adult Congenital Heart Disease (ACHD) subspecialty (read more about the announcement here).

CHD is the number one birth defect in the U.S., affecting nearly 40,000 of the nearly 4 million live births each year. Thanks to advances in treatment in care, the number of children with CHD living into adulthood is rapidly growing. Adult CHD patients, who have surpassed the number of pediatric patients, generally require life-long specialized care due to their complex anatomic and physiologic outcomes.  As the US  ACHD population is estimated to exceed one million adult congenital heart disease (CHD) patients, it is crucial to ensure these patients receive appropriate care from adult congenital cardiologists as they transition out of pediatric care.

For more than a decade, the ACC has been spearheading a multi-society effort to establish a subspecialty certification in ACHD. At the ACC’s 32nd Bethesda Conference in Oct. 2000, the development of an exam and corresponding certification in ACHD administered through the ABIM became a goal and participants outlined a need for a comprehensive strategy for ensuring an adequate and appropriately-trained ACHD workforce given the rapidly increasing ACHD patient population.

Establishing a nationally recognized training pathway and a certification will ensure ACHD patients seeking ACHD care will be able to seek out care from a specialty trained cardiologist. Likewise, by standardizing the training, knowledge and competencies needed, cardiologists specializing in ACHD will have a defined training curriculum and a nationally recognized board examination to validate this certification.

Moving forward, the Accreditation Council for Graduate Medical Education (ACGME) is in the process of developing accreditation standards for training programs with the expectation that the certification exam will be available within the next three years.

This is great news for the College and the CHD community at large and we should all be proud of the role we have played to date and should be excited about what future opportunities will hold.  On behalf of the Council I am grateful to the many ACC members and the multi-society stakeholder partners who contributed countless hours to this effort. We especially thank pediatric and adult congenital cardiology leaders Gerard Martin, MD, FACC, who spearheaded the effort to petition ABIM and ABP, and Michael Landzberg, MD, FACC, Curt Daniels, MD, FACC and Thomas Graham, MD, FACC who co-chaired the petition writing groups and were true stewards of this effort.

For more information about ACC’s CHD activities and efforts, visit CardioSource.org/ACPC. Also be sure to check out the recent JACC President’s Page “The ACC: Taking on Congenital Heart Disease and Winning!” which I co-wrote with ACC President William Zoghbi, MD, FACC.

This post is authored by Silvana M. Lawrence, MD, FACC, member of the ACC’s Sports and Exercise Section.

Although summer has come and gone, fall brings with it the excitement of the start of school and American-loving competitive sports like football. However, with the start of competitive sports also comes the fear of the unexpected event of a young athlete collapsing on the field.

Towards the beginning of fall, cardiologist’s offices become overwhelmed with young athletes needing sports clearance, and many questions surface that were not taught during training nor were considered in the daily practice of pediatric cardiology. Fortunately, parents pay more attention to complaints reported at the time of physical exams at the primary care physician’s office prior to sports participation. However, there is an undulating comfort level to clear young athletes with any positive answers to history questions or unusual findings on a cardiac exam.

To participate in sports in the US, a young athlete must first complete a screening, which includes a history and physical exam currently based on the AHA recommendations. However, those who undergo a screening at their cardiologist’s office will, undoubtedly, receive at least one test, i.e., an ECG, and not infrequently, additional testing that might include an echocardiogram, Holter and/or a stress exercise test.

Several screening programs exist in different parts of the country aiming at early detection of conditions associated with sudden cardiac death. Presently, an ongoing large screening program of high school athletes is occurring in the state of Texas: The Texas Adolescent Athlete Heart Screening Registry – TAAHSRTM. This program has screened over 6,000 athletes to date, ages 14-18 years and utilizing history, 12 lead ECG and limited echocardiogram. Initial analyses of data have demonstrated a referral rate of about 9 percent for abnormalities detected either by ECG or an echocardiogram. We are currently putting together a paper on our findings, which I think will be a good piece of information. I also want to follow these kids longitudinally and see what happens with their natural history.

There is also a multifaceted group of young adults with congenital heart disease, repaired and palliated, that poses challenges. The present Bethesda guidelines have provided guidance for many years. However, many in the field agree it might be time to revise our data and perhaps dare to cautiously, but yet firmly, expand the type of physical activities these patients can (and ought to) engage in.

Challenges are posed daily by the growing population of inactive children that in addition to underlying heart disease, have added risk factors such as obesity, hypertension and abnormal lipid levels. Our inability, to some extent, to better define the level of physical inactivity necessary in order to protect the diseased heart, is hurting us by not promoting physical activity perhaps in the most needed segment of our pediatric population.

Exciting times are ahead of us and I truly believe we are making a difference in kids’ lives. We may not have the perfect model and, in fact, I think we do need to improve our understanding of the process and results/data to reassess the best mode of screening. Regardless, the face of sports and exercise cardiology continues to reach new horizons and, undoubtedly, will contribute to change the world of athletes with a healthy or a diseased heart.  

This post was authored by Kathy Jenkins, MD, FACC, Chair of the Adult Congenital and Pediatric Cardiology Council.

In the news this week, Max Page, the seven year old boy who played “Little Darth Vader” in the popular car commercial that debuted at the 2011 Superbowl, underwent open-heart surgery to repair a congenital heart defect.  He underwent surgery to repair a hole in his heart and replace his pulmonary valve.

A few days before the surgery in an interview on The Today Show, Max’s mom “discussed Max’s fear of his upcoming surgery and described how his family has been helping him prepare for the procedure.” She shared how days before the surgery he said, “Mom I don’t have a choice. I have to go through it. I don’t like it and it’s still scary — but I have to. So I think I might as well go through it with a good attitude.”

Max is a brave little boy well beyond his years, but unfortunately Max’s situation is not unique. In fact, congenital heart disease (CHD) is our nation’s number one birth defect, and nearly 40,000 infants in the U.S. are born each year with CHDs. Fortunately as medical care treatments have advanced, children born with CHD are living longer and healthier lives, even into adulthood.

The latest issue of Cardiology magazine focuses on CHD and the successes we have seen along the continuum of care. Congenital heart disease requires lifelong care, and the College’s Adult Congenital Pediatric Cardiology (ACPC) Section has been working to ensure patients with congenital heart disease have continuous cardiovascular care throughout their lives. There are now more adults living with CHD than children.

In the cover story, Gerard Martin, MD, FACC notes how there is a need for collaboration among adult CHD specialists, general cardiologists and pediatric cardiologists to educate each other, along with patients, on how to manage care over the course of a patient’s life. Moving forward, the College is fortunate to have leading national patient advocacy groups such as the Adult Congenital Heart Association, Children’s Heart Foundation and Mended Little Hearts as involved partners in providing patient and physician resources.

I also discuss how the ACPC Section has evolved over the years, what we have accomplished thus far, and what our plans are moving forward to improve care for CHD patients. If you haven’t done so already, I urge you to flip through the latest issue and learn about what the ACPC Council is up to.

We should all take Max’s word of advice for kids who have CHD: “‘If you use your FORCE and dream big, you can achieve anything. We may be small — but we’re mighty!’”

According to the Centers for Disease Control and Prevention, it has been estimated that at least 280 infants with unrecognized critical congenital heart defects (CCHD) are discharged each year from newborn nurseries in the U.S. Pulse oximetry newborn screening, a simple bedside test to determine the amount of oxygen in a newborn’s blood and the pulse rate, can identify some infants with a CCHD before they show any signs.

The ACC has long advocated for the universal coverage of CCHD screenings for newborns. Studies have shown that this approach to early detection of more subtle forms of congenital heart disease can prevent related complications and promote early diagnosis and treatment.

In September 2011, HHS Secretary Sebelius approved adding screening for CCHDs to the Recommended Uniform Screening Panel, which has been invaluable information as states pursue this policy. Members of the ACC’s Adult Congenital and Pediatric Cardiology Section including Gerard Martin, MD, FACC, worked with HHS to develop recommendations for the implementation of the screenings.

At the state level, there has been a push by several of ACC’s chapters working with other pediatric and cardiovascular societies to introduce policies for the newborn screenings.

Just last week Virginia Gov. Bob McDonnell originally vetoed a bill supporting legislation that would establish a public-private work group to develop a program for screening newborns for critical congenital cyanotic heart disease using pulse oximetry monitoring and providing appropriate early intervention services to infants identified as having the disease. But upon further review and discussions with key stakeholders led by ACC Staff including Delegate Patrick Hope, the ACC Virginia Chapter and others including Mended Little Hearts and AHA, McDonnell decided to implement an executive order that would put the congenital heart disease screening policy in place and officially signed the order today. This is a great example of how a strong grassroots push can be effective and instrumental.

To date Indiana, Maryland, New Jersey, Tennessee, and West Virginia have approved plans that will assure universal testing of newborns for congenital heart disease. The New Hampshire legislature recently passed a CCHD screening bill, which will be reviewed by an oversight committee prior to the Governor’s evaluation and signature, and the Connecticut Senate recently passed a bill to require CCHD screening. The health departments of Michigan, Ohio and Alabama are also currently crafting regulations to require the screening.

The ACC’s State Government Relations team is currently strategizing with stakeholders to advance the screening requirement in several other states. We’ve come a long way over the past few years as the implementation of policies for CCHD screening becomes more widespread.

This post is written by Kathy Jenkins, MD, FACC, Chair of the Adult Congenital and Pediatric Cardiology Council

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Congenital Heart Defects Awareness Week is Feb. 7-14. According to the Center for Disease Control and Prevention (CDC), “congenital heart defects affect nearly 1 percent of infants born in the U.S.” As medical care and treatments have advanced, infants with congenital heart defects are living longer and healthier lives and over the past 10 years we have seen a 25 percent reduction of congenital heart disease (CHD) morbidity and mortality. It is estimated that approximately two million people of all ages are living with congenital heart defects in the U.S.

There is still a lot that can be done to help those who are living with congenital heart defects and the physicians who treat them. Since its inception in 2005, the College’s Adult Congenital and Pediatric Cardiology (ACPC) Section has been working to bring quality care to children and adults with CHD through education, quality and advocacy efforts.

The ACPC section has identified several tools for CHD patients as they transition between a pediatric cardiologist and an adult congenital cardiologist, including the Adult Congenital Heart Association’s Personal Health Passport and other tools like Follow My Heart, an electronic based personal health record (PHR). These types of tools are important as CHD patients transition into an adult congenital clinical setting. A CHD patient’s ability to access electrocardiograms and echocardiogram, heart catheterization, and operative reports and other important medical information will help his or her various healthcare providers understand the patient’s unique health care needs.

On the quality side, the College’s continued efforts with NCDR’s IMPACT Registry™ (IMproving Pediatric and Adult Congenital Treatment) is an enormous step in measuring outcomes and care for CHD patients undergoing a diagnostic or treatment cath. Additionally, the ACPC Section has other quality effort underway to develop quality metrics in CHD which may ultimately be used in local or national Quality Improvement initiatives.

On March 1, the ACPC Section will join the Adult Congenital Heart Association and Mended Little Hearts for National Congenital Heart Lobby Day. Together we will lobby Congress for continued funding for the Congenital Heart Futures Act, (included in the ACA and passed into law in 2012). The law established a population-based surveillance registry (through the CDC's existing National Center on Birth Defects and Disabilities). This registry will allow for increased research to better understand congenital heart disease incidence, prevalence and disease burden, as well as a CHD public health impact assessment.  Congenital Heart advocates will also promote the recently established Congenital Heart Caucus chaired by Representative Bilirakis.

A welcome reception for National Congenital Heart Lobby Day will be held February 29 at Heart House. The reception is generously sponsored by the Texas Chapter of the ACC. Thank you also to ACC’s Iowa, Georgia, and Louisiana Chapters who have provided funding to benefit this advocacy effort for ACC members, as well as congenital heart disease patients and family members.

To join us or for more information about the National Congenital Heart Lobby Day click here.  For more information about the College’s ACPC Section visit www.cardiosource.org/ACPC.

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For some time now, the lack of continuous care for congenital heart disease (CHD) patients who are transitioning from the pediatric to adult care settings has been a concern for adult cardiology practices.  Although many CHD patients require specialized care, many more still end up in adult cardiology clinics. I experienced two scenarios when seeing CHD patients in my nonurban practice; a basic layman’s history is provided or several storage boxes of records are presented that must be reviewed and summarized.  One is inadequate; the other is cumbersome and subject to errors of oversight.  A complete, organized patient history is difficult to come by and ultimately can threaten the quality of care as these cases continue to increase in volume. Enhancing collaboration between a coordinated care team, to include congenital heart specialists, and CHD patients would open the doors to consistent and efficient treatment.

I have come up with the acronym DOORRS to sum up the essential information patients should easily be able to provide their cardiologist upon an initial visit. 

D  Initial Diagnosis 

O  Operations Chronologically with institution and surgeon 

O  All Operative Notes 

R  Most Recent diagnostic studies and results 

R  Recommended follow up 

S  Special considerations or previous complicating factors unique to this patient 

I have received positive and encouraging feedback from colleagues and the College on this issue, which affects us all but to which there is no easy solution.  I know the ACC’s Adult Congenital and Pediatric Cardiology (ACPC) Section is on top of this issue and is dedicated to collaborating with patient advocacy groups, such as the Adult Congenital Heart Association, on promoting professional and patient resources for CHD patient care.  For the College’s part, several ACC chapters have supported past National Congenital Heart Lobby Day activities, which have focused primarily on advocating for national CHD surveillance tools.  

The Congenital Heart Futures Act (included in the ACA) authorizes establishing a population-based surveillance registry (through the CDC's existing National Center on Birth Defects and Disabilities). However, the funding for this expansion has not been appropriated.  

To compound the issues related to care of the CHD patients, limited outcomes data regarding CHD treatment is available. The NCDR’s IMPACT Registry™ (IMproving Pediatric and Adult Congenital Treatment) has been established to assess the prevalence, demographics, management and outcomes of pediatric and adult patients with CHDs who are undergoing diagnostic catheterizations and catheter-based interventions. The collection and analysis of this data facilitates performance measurement, benchmarking and quality improvement initiatives.  The IMPACT Registry, which has collected over 3,000 patient records so far, will provide significant contributions to the knowledge base and outcomes associated with CHD. While this is exciting and a big step forward, gaps in data collection and outcomes reporting remain in other CHD care settings, most notably the ambulatory setting.  

I look forward to hearing your thoughts on ensuring congenital heart disease patients receive appropriate care as they age and move out of the pediatric setting.  What have you done in your practice to increase efficiency for adult CHD patients? What resources and educational tools would be helpful to your care of adult CHD patients?  

Make sure to check out the Nov./Dec. issue of Cardiology Magazine for an article on ACHD.

ACC’s pediatric and congenital heart disease cardiologists and nurses have long been lobbying for universal screening for congenital heart disease, including the very inexpensive ‘pulse oximetry’ test for newborns. This approach to early detection of more subtle forms of congenital heart disease can prevent related serious complications and promote early diagnosis and treatment. The ACC has endorsed the recommendations of an HHS working group on pulse oximetry screening for all newborns -- something we helped become a priority for them. In September, an HHS advisory committee finally recommended that pulse ox be added to the list of screenings for 30 core conditions that HHS recommends for all newborns, but requested a special working group look into how it might be implemented. The working group’s recommendations are published in Pediatrics. Former chair of ACC’s Adult Congenital and Pediatric Cardiology (ACPC) Council Gerard Martin, MD, FACC, is one of the authors of the recommendations. Geoffrey Rosenthal, MD, FACC, also deserves special recognition in these efforts. 

Implementation of pulse ox screening helps detect the most common congenital heart defects by measuring blood oxygen levels. The recommendations state that if pulse ox detects low oxygen levels in a newborn, he or she will receive further evaluation, including first being evaluated for other conditions that might cause low oxygen levels (like infection) and would receive an ECG. A pediatric cardiologist would then interpret the ECG. The recommendations note that the biggest concern is the threat of false positives, which would expose the newborn to unnecessary testing, and perhaps unnecessary hospital transfer. As such, the recommendations include strategies to reduce false positives, such as waiting 24 hours after birth to screen and making sure the baby is alert rather than asleep when the test is administered. Following the release of the recommendations, HHS said that a “plan of action” is expected very soon. The recommendations are available online. The ACC is working with Mended Little Hearts on a CardioSource forum to discuss challenges with pulse oximetry screening – check it out here.

Deaths from congenital heart defects in both children and adults have declined over the last 7 years for which complete data is available, according to new research. From 1999 to 2006, we saw a 24% decrease in death rates resulting from congenital heart disease among all ages, says researcher Suzanne Gilboa, PhD, an epidemiologist at the National Center on Birth Defects and Developmental Disabilities at the CDC. Thank you pediatric and adult CHD cardiologists and clinical teams!

ACC’s Gerard Martin, MD, FACC (chair of the Adult Congenital and Pediatric Cardiology Council), was quoted in the article. He said: "It's exciting news for parents who have a child with congenital heart disease and it's exciting news for all the professionals who take care of children with congenital heart disease.”

We’ll be able to track further progress in CHD much better now, given the ACC recently launched the IMPACT Registry, which assesses the prevalence, demographics, management and outcomes of pediatric and adult patients with congenital heart disease who are undergoing diagnostic catheterizations and catheter-based interventions. Not surprisingly, Martin’s Children’s National Medical Center is our first participant! The collection and analysis of the IMPACT Registry’s will provide significant contributions to the knowledge base and outcomes associated with congenital heart disease. Learn more and register: https://www.ncdr.com/IMPACT/default.aspx.