In This Recording
- Ami Bhatt, MD, director of the Adult Congenital Heart Disease Program, discusses a memorable ACHD patient
- The number of U.S. hospitalizations for adult congenital heart disease has increased for coronary artery disease
- Dr. Bhatt presents three reasons why this is happening—challenges with awareness, communication and measurement—and potential solutions for each
Ami Bhatt, MD, director of the Adult Congenital Heart Disease Program at Massachusetts General Hospital, discusses the prevention of atherosclerotic cardiovascular disease in adults with congenital heart disease.
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"You can't possibly shock me."
"It says I got shock you, man."
"I'm awake. You can't shock me."
"Look, I know you've been coming here for years, and you don't talk about it, but we all see the scar. You practically fell off the elliptical. I can't find a pulse, and the AED here is saying shock."
This is a conversation that my soon-to-be-patient, a 41-year-old man with a Tetralogy of Fallot status post right BT shunt and complete repair, lost a follow-up at age 18 when his doting mother no longer dragged him to visits, was having while laying on the floor of his local gym. He had been on the elliptical and felt a bit light-headed, and as he walked out the door to the gym, his trainer noticed that he was woozy and grabbed his arm for a pulse. His left arm.
"Buddy, lay down. I'm just going to check something."
The prior EMT now trainer, planned to use the device to assess his rhythm, but the device immediately began to bark orders, "Deliver shock." That brought us to the conversation today. Two minutes before EMS arrived, my patient appeared presyncopal again and was shocked by his trainer, while awake. In the ambulance he had several additional episodes of ventricular tachycardia at 180 beats a minute now treated with medications, but he was lucid. The typical ACHD survivor. He was a father of two, recently started his own law practice, and his exam, while revealing the short early diastolic murmur of free PR that was his downfall on palpation, did not allow assessment of his right ventricle through a muscular chest that made one wonder about the size of his aorta.
It was a long hospitalization and a harsh reintroduction to the health care system. With a bioprosthetic valve pulmonic valve replacement, right ventricular outflow tract reconstruction, cinching of his moderate to severely tricuspid valve as research now confirms provides benefit in ACHD surgeries. Then, he waited the requisite period for his own ICD. He returned to a different life. Finding clients who are okay going to the lawyer who almost died. Now hesitant on the elliptical as a well-meaning cardiac fellow had told him all about the research on inappropriate shock in ACHD status post ICD. I later saw this different man when he presented with chest pain, likely anxiety a year later. His profusion imaging was normal, but he was overweight, sluggish, stressed. He shared a room with a 30-year-old with total anomalous voluminous pulmonary venous return status post-surgical repair shortly after birth who also had chest pain. His stress was positive for inferior ischemia.
"I could really use a cigarette," he told my Tet patient when he heard the news.
His demographic is changing. With transition programs, by bringing those lost to follow-up back into the fold, and using echocardiography, MRI, cardio pulmonary exercise testing. Parameters to predict and intervene on patients in a timely manner we can change this landscape. We know how to care for adults with congenital heart disease. Increase awareness, build teams and study disease. But as our population ages, as cardiologists, we are failing them. The number of U.S. hospitalizations for adult congenital heart disease has increased for coronary artery disease compared to any other disease process by nearly 120% from 1998 to 2005. A Canadian population-based cohort study reported a 7% rate of myocardial infarction in older adults with congenital heart disease as compared to an age matched prevalence of 5% in non-ACHD subjects. Up to 14% of adults with congenital heart disease with angiographically confirmed CAD are younger than my 41-year-old Tetralogy patient.
Overall, more than 80% of adults with CHD have greater than one cardiovascular risk factor. In those individuals with adult congenital heart disease and obstructive CAD, most had greater than one cardiovascular risk factor, and the modifiable factors of hypertension and hyperlipidemia were the most predominant. Obesity is present in 30% of adults with moderate and complex CHD who require additional surgery. And in the Netherlands, it was demonstrated that there was a 3.4% prevalence of diabetes in the congenital heart disease group compared with only a little of 2% in a control group. Lastly, abnormal glucose metabolism and lower HDL levels are prevalent in congenital heart disease and in fact are related to associated morbidity and mortality.
So for individuals who are increasingly spending their entire life in cardiac care, how can this be happening? We will discuss three problems and their potential solutions today. The first is awareness. As a pediatric obesity crisis is being addressed, our congenital pediatric patients need to reap the benefits of programs, counseling and empowerment. In the established adult congenital heart disease population, we need to create these programs. Health and wellness programs, stress management, formal referrals to nutritionists, physical prescriptions for exercise that they can take home with them, enrollment in cardiac rehab for ASCVD prevention. Colleagues have published excellent studies in cardiac rehab demonstrating the benefit of exercise in this population. And hopefully, moving forward these trials will have extensions which look at the benefit for arteriosclerotic cardiovascular risk in those who stick with their cardiac rehab programs or pursue continued physical activity.
ASCVD risk factors must be given a seat at the quality improvement table along with congenitally experienced imagining, routine liver assessment and arrhythmia monitoring. There are ASCVD guidelines. In fact, they recommend that if there is a greater than 7.5% chance of having an event in 10 years, individuals should be maintained on a statin. Our patients need this ASCVD risk calculation. In one cohort over age 40 years, 44% of individuals with adult congenital heart disease had a statin indication. Hmm, think about that. Nearly 50% of your ACHD clinic's older adults might need to be on a statin. One single center studied their ACHD population and found that only that only 43% of their cases with an indication for statin were appropriately prescribed therapy as compared to nearly 20% more of controls. We are similarly deficient in prescribing aspirin. And this study did not include individuals with incidentally diagnosed coronary atherosclerosis on CT or cardiac cath. I suspect those numbers are coming and are equally concerning. We need to raise awareness.
This second issue is communication. Who is responsible for preventative cardiology? In congenital heart disease clinic, we have a multidisciplinary team taking care of the patient, but there are so many other issues that often need to be discussed. When we address general cardiac risk factors, does the patient find this irrelevant at the current time, therefore, the impact of those discussions is diminished? Or, is there a slight fatalistic approach amongst some of our patients that I may just not be around that long, therefore that is not where I will focus my energy. Who manages lipids? An adult congenital office, a referring cardiologist, or is it the primary care's job? If it is someone else's job, and you get a result, is there a warm handoff? And what about borderline elevated blood pressure that the primary care nurse practitioner noted at your request? Now what? Who should he contact with that result?
And as our patient's friends who are all of a certain age are more likely to be prescribed a statin start to complain about the muscle aches and the side-effects, whose responsibility is it to follow up and ensure that there is medication compliance in our ACHD community? Well, the straightforward solution is communicate better. Far easier said than done. Establishing the infrastructure for that communication, creating networks of care where primary care doctors, referring general cardiologists in the community and the advanced tertiary centers have methods to share data and community rapidly. Those are departmental, divisional and institutional level measures that we need to promote and ask for. And as we move towards a more patient centered model of cardiac care in the United States, there's perhaps no place where shared decision making will be as important as in the adult congenital heart disease population, especially when it comes to atherosclerotic disease prevention and treatment.
Our last challenge is our biggest window of opportunity--Measurement. Our population is only now reaching the age where atherosclerosis is becoming visible. Acute MI during adult congenital heart disease hospitalization increases mortality. It is not surprising news, but it is the first sign that we can now, we must now, start to measure risk factors and outcomes. We know how to do in congenital heart disease, and the adult cardiovascular disease world surely knows. Yes, adult congenital heart disease is heterogenous for reasons that could fill a TEDx conference, but the end game is we don't know until we measure. We don't know about the effects of coronary surgical manipulation, anomalous coronaries, if cyanosis is protective, or if endothelial dysfunction in coarctation is destructive.
I am glad that my 41-year-old patient with Tetralogy of Fallot is now back in care, as are many others. And I'm hopeful as I see that many more now come straight to me from pediatrics rather than being lost to follow-up. And our own efforts in risk factor education and modification are enjoying successes. As I look to the future, I clearly see the need to implement systems-based care, coordinated communication and shared decision making in this population to ensure that atherosclerotic cardiovascular disease does not become rampant under our watch. As our risk prediction metrics improve, I have faith that there will be far fewer presentations of ventricular arrhythmia in my TOF patients at the gym, but at the same time I worry that there will be more presentations of ventricular arrhythmias from coronary artery disease.
And despite how sturdy we were in our young years, with age I do not know that the outcome will look the same. We cannot allow this trend to progress. We do not want to see where increased risk factor burden leads us. And if we measure and seek to understand the evolution of atherosclerosis and congenital heart disease as individual institutions, we will miss the forest through the trees. And so I leave you with a phrase today and hope you will join us in your practice, in your advocacy and your research. To prevent the continued rise of acquired cardiac risk factors and disease in the congenital heart disease population. Atherosclerotic cardiovascular disease cannot happen, not in our house. Thank you.
Learn more about Ami Bhatt, MD
Learn about the Adult Congenital Heart Disease Program