Why Would a Child Need a Cardiologist?
True story, I swear it:
One day in the early 1990’s not long after I completed my fellowship, while I was waiting for a back elevator near the catheterization lab at what was then called The New York Hospital, I spied two men in suits waiting along with me. It was an odd sight because this was really not a public elevator and they clearly did not work in the cath lab. For some reason they were off the beaten path, avoiding the public elevators. One of the fellows bore, at first glance, a strong resemblance to the character actor Bruno Kirby. I sidled over to confirm the sighting and it became obvious to me that this was not the co-star of “City Slickers.” Just then, I was approached by the other gentleman. He was staring at my ID and and said “Excuse me for asking, but why is it that a child would need a Cardiologist?”
It was neither the first nor last time I have been asked that question, but it may have been the most memorable. It turns out that the gentleman was former President Richard Nixon. He stood there patiently, letting the elevator pass three or four times (to the chagrin of not-Bruno Kirby, who by the way was his bodyguard) while I explained my branch of medicine to him. He sought me out on another occasion when we passed each other in the hallway outside the gift shop. With riveting sincerity, as if he were about to be quizzed on it, he asked me to explain to him how it is that we could create a working circulation for a person who was missing half of the chambers of the heart. And there I was, explaining single ventricle physiology to the former leader of the free world, all because somewhere he had come across an article about the Fontan operation, and filed it away in case he ever passed by me again. We should all be that curious.
Side note: President Nixon died a year or so later at New York Hospital in the same room where Andy Warhol died, where Jackie Onassis received care before going home for hospice care, and where, indirectly, the Iran hostage crisis began. That sounds like a future Sunday Brunch!
While we have come to expect that if we all live long enough, we will develop some wear-and-tear on our heart that will require the input of a Cardiologist (or as we call them, an “Adult Cardiologist”), the Pediatric Cardiologist is not a standard member of a child’s patient care team. Our care is reserved for Pediatric patients with some symptom, sign, family history, or other circumstance that indicates investigation for possible heart disease.
It may relieve you to know in advance that the majority of new patients that we see - infants and children with heart murmurs, teenagers with chest pain, etc. - will eventually be proven to have normal hearts. But medicine isn’t about playing the percentages. Each of those conditions can be a sign of heart problems, so every workup is its own story.
Pediatric Cardiology is a sub-specialty of Pediatrics. This means that after medical school, we serve a three year residency in Pediatrics, and then add another three year fellowship studying only Pediatric Cardiology. So, yeah, a Pediatric Cardiologist is always at least 32 years old or so by the time they are able to join a faculty or start practicing, or really make any sort of a living at all.
Pediatric Cardiology, then, is not a subdivision of (Adult) Cardiology, as people sometimes assume. Those folks serve a three year residency in Internal Medicine and then a fellowship in Cardiology. Same time frame, but they have a different origin story.
There are about 2,000 board-certified Pediatric Cardiologists in the US, and nearly 40,000 Adult Cardiologists. We don’t overlap much, though we are often told “you must know my uncle: he was a Cardiologist in Minnesota.” (Probably not).
Folks who practice in free-standing children’s hospitals probably don’t really know any Adult Cardiologists, except maybe socially. I consider myself privileged to have spent almost 4 decades at Weill Cornell, which has a children’s hospital within a larger hospital, and therefore I have interacted with many Adult Cardiologists over the years, and they’ve been uniformly great.
Especially in New York, the community of Pediatric Cardiologists is nicely integrated. Even though I’ve been in one place for so long, I am very familiar with Pediatric Cardiologists at all of the institutions in the metropolitan area. This is partly due to the fact that we have trained so many of them as part of our combined Cornell/Columbia fellowship program at New York Presbyterian, and also because I spent twelve years as an officer (and two years as President) of the Pediatric Cardiology Society of Greater New York, a terrific group which has fostered a friendly and collaborative relationship between the institutions for almost 6 decades.
There are sub-sub-specialties in Pediatric Cardiology, too. For example, if a child develops an arrhythmia, we might enlist the care of a Pediatric Cardiac Electrophysiologist. This would be a person who spent another year or two after fellowship just learning about Pediatric arrhythmias. There are also subspecialties in heart failure, cardiac catheterization, advanced imaging (cardiac MRI) and a few other disciplines.
Pediatric Cardiologists and Pediatric Cardiothoracic Surgeons have a relationship unlike any other medical and surgical disciplines. The collaboration is so close that in most institutions, the Pediatric heart surgeon is found geographically in the Pediatric Cardiology office, and not in the Cardiothoracic Surgery Department, even though, of course, Pediatric heart surgery is a subspecialty of surgery and heart surgery, and not Cardiology. The surgeons grow off a separate tree than we do, but our branches intertwine in the care of children with congenital heart disease. And by the way, the Pediatric heart surgeons train forever. I’m sure they all grew up with grade school classmates who retired from a military or public service job before they themselves finally completed their training. Think about that.
Surgical cases are virtually always discussed formally at Pediatric Cardiac Catheterization conference, where the Pediatric Cardiologists and the Pediatric CT surgeons - as well as other people like radiologists, intensive care physicians, etc. - convene to discuss the case, confirm agreement on the necessity and type of operation, and plan the postoperative management. Cath conference is generally a weekly occurrence, except at Cornell we do it two mornings a week. The more communication, the better.
Uniquely, a Pediatric Cardiologist is present in the operating room for almost all open heart surgeries involving Pediatric patients. This is because we perform a transesophageal echocardiogram (a cardiac ultrasound performed through the throat, which runs right behind the heart) at the beginning and end of each surgery. The first echo is to document the anatomy one final time in order to confirm the surgical plan. The second is to evaluate the repair itself, and to document the function of the new circulation in order to guide the care over the postoperative period in the ICU. In adult heart surgery, those echocardiograms are done by the anesthesiologist.
Incidentally, the Pediatric heart surgeons have little to do with the postoperative care after the patient leaves the OR. This is handled by the Pediatric Critical Care specialists, in collaboration with the Pediatric Cardiologists. Nowadays there are also Pediatric Cardiac Intensivists, who take another fellowship after either their Cardiology fellowship or their Intensive Care fellowship.
Occasionally the Pediatric Cardiologist and the CT surgeon decide together that the repair needs to be improved upon. In that case, we can go right back on the bypass machine and revise the operation immediately. The Pediatric Cardiologist and the surgeon, then, really need to have a good relationship, and healthy trust in each others’ opinions. I am blessed to have been present in the OR a couple thousand times, and to work with Pediatric heart surgeons who I trust not only in their surgical skills but in their intellect and their motivation. Not all Pediatric Cardiologists can say that. I’ve had colleagues elsewhere who have been cursed at, threatened, pushed and - in one case - hit with a scalpel by a thin-skinned surgeon. In my time at NYP, we have all appreciated, together, that it’s vital to roll the patient out of the room with the best possible repair. Feelings can’t be hurt when we have to decide that the repair could be improved. I’m grateful to be continuing my participation in the Weill Cornell OR on Tuesdays, while spending the rest of the week here at Avalon. Membership on the OR team and one-on-one care of patients and families have become the two aspects of Pediatric Cardiology that bring me the most joy. More of both, I say!
Pediatric Cardiologists have, I would surmise, the lowest burnout rate in all of medicine. I think this is because we have the best of all worlds. We see people who have or at least might have a somewhat specific problem. We can focus on the one organ that is actually understood because we’ve watched it do its thing, and not because somebody damaged part of it in a laboratory animal and then figured out what the animal couldn’t do. We get tremendous long-term continuity with kids and families with heart disease. There are people out there who I diagnosed prenatally with heart disease, shepherded them and their families through 4 or 5 procedures, and then handed them over to our Adult Congenital Heart Disease colleagues (yes, that’s a thing now), after they graduated from college. It’s a great feeling. On the other hand, we also get to reassure in one visit parents whose children do not have heart disease. It’s so rewarding to look them in the eye and say “Promise me you’re never going to worry about this again,” and then step out of their lives forever.
In the 21st Century, for those of us who are affiliated with institutions with outstanding surgical care, even the patients with what would be considered severe disease generally have happy endings. We are responsible for caring for children with real heart disease, and get to experience the exhilarating acuity of the OR and the collaborative challenge of postoperative management, with overwhelmingly positive outcomes. Here at Cornell we have had the lowest risk-adjusted mortality rate for congenital heart disease surgery in the state of New York. Yup, the games are more fun when you win them all.
The Pediatric Cardiologist, then, usually retires when they come to work one day and their key (or their ID) doesn’t let them in the door. When they finally get the hint, they’ll usually say, “OK, I get it, but can I still come to cath conference?”
Most people choose Pediatric Cardiology because somewhere along the line they became fascinated with congenital heart disease. In fact, during fellowship most of the time is spent that way. But in practice, we spend more time caring for patients with normal hearts that are referred for innocent heart murmurs, or symptoms like chest pain, fainting and palpitations. It takes a long time to become comfortable with those issues and sometimes the hardest thing is making the firm determination that a person is normal and does not need to see the Cardiologist again. Over the decades I have evolved to find those encounters just as rewarding as the victories that come in the OR and the ICU. So whatever it is that your Pediatrician send you here to evaluate, we’re ready for it and happy to help.
As I said earlier, none of this means that we don’t find heart disease when we are not expecting it. Those symptoms that provoke referral can truly be a sign of significant diseases which carry significant risk. It’s important to sort that out. Life-threatening heart disease in healthy young people is a needle in the haystack. We are the metal detector.
Speaking of needles, it should be pointed out that when a child comes to see the Pediatric Cardiologist it’s important for them to know that there are not going to be any shots. We’re really not going to hurt them. Great to know.
They almost definitely will have an electrocardiogram, or ECG, which just collects and displays the electrical activity that comes off the body, almost all of which comes from the heart. There’s a tremendous amount of information in those squiggles. A normal ECG in anybody bigger than a breadbox is a great piece of information to have. The USA is behind most medically developed nations in terms of screening teenagers, or at least even just teen athletes, with ECGs to find those who may be at risk for catastrophic events during exercise. It’s a controversial topic. There’ll be a blog devoted to it before too long. Try to guess which side I’m on.
Incidentally, the waves shown on an ECG are named P, Q, R, S and T. Some people have another little wave after the T wave. It’s called - you guessed it - a U wave. There’s no formal explanation of why the lettering starts at P, but one explanation is that Einthoven, who received the Nobel Prize for inventing the ECG, preferred P because the pronunciation is similar across most languages. I like that explanation the best.
And by the way, an ECG is the same thing as an EKG. One of my mentors, Mary Allen Engle, who was one of the mothers of Pediatric Cardiology and a disciple of the great Helen Taussig, would gently admonish anyone who called it an EKG. “We are not in Germany, dear.” Maybe Einthoven was right.
They’ll probably also have an echocardiogram, which is an ultrasound of the heart. If you’ve ever had an ultrasound, and then you saw an echocardiogram, you’re exactly right, they’re the same machine. The echo machine is just an ultrasound machine that is adjusted to take moving pictures of the only organ that can’t stay still for a portrait. That fundamental difference is why echo belongs to Cardiologists and the other ultrasounds belong to Radiologists. Echo is a great tool for so many reasons, not the least of which is that while you have to lie down to have it, you don’t really need to stay still. A patient echo tech can get great images on a fussy baby, a squirming toddler or an impatient teen. I’m sure there’ll be a blog about echo some time, and it will mostly be about echo techs, who are the real backbone of any Pediatric Cardiology service.
So that’s our field in a nutshell. When I write a blog about Dr. Fontan, then you’ll know as much about it as President Nixon. I am grateful that it exists. It helps a lot of people, and provides everything I went to medical school hoping to find. What could be a better calling that to care for the hearts of children?
-Patrick Flynn
