A Complete Mind Shift!
Interview with ACHD Professor Tanja Raedle-Hurst
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- At the Doctor's Appointment with Tanja Raedle-Hurst
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- Researcher Talk with Prof. Dr. Dr. med Gerhard-Paul Diller
- Researcher Talk with Prof. Dr. Marc-Phillip Hitz
Homburg, Saarland University Hospital, pediatric cardiology. It is peak period in the colorful hospital hallway in front of Tanja Raedle-Hurst's consulting office. Parents with their babies and children suffering from heart disease are waiting for the examination before or after surgery. In-between, there are patients who have long been through with this successfully: adults with congenital heart disease.
Some of the patients cover distances of several hundred kilometers for the routine follow-up examination. Tanja Raedle-Hurst takes her time for everybody. She has just cordially said good-bye to her last patient for today. She has switched off the monitor of her ultrasound machine and just quickly entered some more data into the computer. Now she is sitting towards us on her doctor's chair in the consulting room.
The Heart-Explorer: Dr. Raedle-Hurst, how was your day?
Tanja Raedle-Hurst: Rich and nice! All the patients were doing fine today. This is when work is fun.
The Heart-Explorer: You are an ACHD cardiologist. How does one become such a doctor?
Tanja Raedle-Hurst: I started with a conventional residency in internal medicine and adult cardiology at the Gießen University Hospital. Already back then I looked after adult patients with congenital heart disease. I have always taken a great interest in this subject. And when I had to move to Homburg for family reasons the department of pediatric cardiology at the Saarland University was just looking for medical employees for residency. That was a lucky coincidence. I turned up at their doorstep immediately and asked if I, as a cardiologist, could also do a residency in ACHD.
Adults with congenital heart disease are getting increasingly older nowadays.
The Heart-Explorer: For a cardiologist, such a lateral entry into pediatric cardiology sounds uncommon, how did they react?
Tanja Raedle-Hurst: You are right, this is an uncommon approach. The pediatric cardiologists in Homburg, however, were enthusiastic about it at once. Luckily, children with congenital heart disease increasingly reach adult age nowadays, and also adults with congenital heart disease get increasingly older. Naturally, this also means that they can develop typical adults' diseases too, such as, for example, high blood pressure, diabetes, cardiac insufficiency, a deteriorating kidney function or liver problems. Pediatricians are familiar with specific heart defects, but less so with adults' diseases. An exchange between different medical fields has therefore become hugely important.
We did not have to deal with congenital heart defects previously.
The Heart-Explorer: As an adult cardiologist, could you use your knowledge about acquired heart disease?
Tanja Raedle-Hurst: Only to a limited extent. I had to change my thinking completely. But that was what I found particularly interesting. When dealing with congenital heart disease, you are suddenly confronted with completely different, sometimes highly complex anatomical structures and entirely different hemodynamics. Thus, in congenital malformations, the blood flow in the vessels, as well as anything influencing the fluid mechanics, develops differently as compared with acquired heart disease which we adult cardiologists are familiar with and are able to assess accordingly. You see, during our residency we were not confronted with this type of cardiac disease.
The Heart-Explorer: How large is the gap regarding diagnostics and treatment?
Tanja Raedle-Hurst: I had known in advance that I would have to double down on my knowledge. That is why I had been doing research from the beginning and investigated different biomarkers in my patients within the scope of clinical studies. For instance, diagnostic threshold values in patients with congenital heart disease differ from those we know from adult cardiology. In many fields, we have to carefully approach this patient group that is still largely unknown.
You do have to be trained in this for things to be going in the right direction for patients.
The Heart-Explorer: What is the point here?
Tanja Raedle-Hurst: Let me give you an example by referring to biomarkers. These are biological substances in the blood that allow us a better assessment of the course of a disease. The hormone NT-proBNP is one of these substances. It is produced mainly in the left heart chamber. When a doctor detects a NT-proBNP value of 400 in a patient with left heart failure that can be perfectly alright. However, if this occurs in a Fontan patient, things are completely different. In this case it can hint at a poor functioning of the Fontan circulation. It might even be the case that such a patient is on the brink of Fontan failure. It does therefore make sense that doctors specializing in ACHD examine these patients, simply because this is such a sensitive subject that requires being specifically trained in order to assess findings accurately. This is the only way to make sure these patients are doing well.
In a Nutshell: Fontan Hearts - Life With Just a Single Heart Chamber
Leben mit einer einzigen Herzkammer
Usually, blood flows through the heart valve into the pulmonary artery in order to supply the body with oxygen. There is a number of congenital heart defects in which the blood cannot be transported to the pulmonary artery without hindrance. In these cases, often one of the two heart chambers has not developed properly. This means that the one functioning heart chamber has to pump the blood to the systemic circulation alone.
How do I know? If, for example, the right atrioventricular valve - the tricuspid valve – between the right atrium and the right heart chamber does not work, the body does not get sufficient oxygen. As a result, lips, toes and fingertips turn blue. In former times, children died of such a tricuspid atresia. Today they can benefit from a particular operation that was invented by the French cardiac surgeon François M. F. Fontan in 1971 to specifically treat tricuspid atresia.
The procedure was named after him and has meanwhile been refined. During the procedure, the superior and the inferior caval vein are connected to the pulmonary artery in such a way as to enable the deoxygenated blood to flow directly into the pulmonary artery, where it is saturated with oxygen. For patients with malformed heart valves and heart chambers to lead a life as good as possible, early detection, a timely Fontan-procedure and a lifelong medical follow-up afterwards are vital.collapse
The patients in questions hardly have any symptoms.
The Heart-Explorer: What can the single patient do?
Tanja Raedle-Hurst: The patients should regularly see an ACHD specialist for follow-up examinations. This is really important because they might hardly or even not at all have any health problems despite there being relevant findings.
The Heart-Explorer: So the patients themselves are not aware of changes in their heart?
Tanja Raedle-Hurst: Not necessarily. In the past ten years I have often made the experience that especially patients with right heart disease such as tetralogy of Fallot or pulmonary atresia present without or with hardly any symptoms even though there are severe findings such as, for example, a leaking pulmonary valve.
The Heart-Explorer: Are you saying that, in some cases, the routine examination can become a matter of life and death?
Tanja Raedle-Hurst: Exactly. The patients require continuous monitoring and follow-up because a belated repair of residual findings such as a severely leaking pulmonary valve can result in damage to the heart chambers in the long run. Evaluating the ultrasound findings, the cardiac MRI or laboratory findings we can detect potential deterioration at an early stage. And heart failure, that is, a deteriorating pump function of the heart, should be prevented at all costs.
It frustrates me to this day that there are still heart defects that go unnoticed.
The Heart-Explorer: Is there anything that surprised you, the adult cardiologist, during your ACHD training?
Tanja Raedle-Hurst: Only when I started here did I really realize what a great role the surgeon plays during childhood. Having received timely and good surgery during childhood is tantamount to a good prognosis and a good course of the disease. I am saddened by the fact that there are still patients with uncorrected heart defects who should, in fact, have had surgery in their childhood. Their quality of life and prognosis are much poorer. That frustrates me to this very day. However, we still see such cases. And I should have thought that we have come much further in the advanced era of today.
There is still a high need for full-scale research for this special patient group.
The Heart-Explorer: In the meantime you have become one of Germany's first female ACHD professors, funded by Deutsche Herzstiftung (German Heart Foundation). What's next?
Tanja Raedle-Hurst: My main concern is to establish this subject at universities and to make sure that full-scale research for this special patient group continues to be conducted. That is my major concern.
One single center does not have enough data to do research.
The Heart-Explorer: From your perspective: which role does the Competence Network with its National Register play here?
Tanja Raedle-Hurst: A vital role. Because one single center has only few patients and accordingly few data for research. And the more data you have, the better you can identify certain trends. The Competence Network with its Register is of major importance for us to be able to gather further knowledge and to offer the patients treatment that is accordingly good. For instance, we know today, thanks to the Register's data, that patients whose heart defects have been corrected early in childhood have a much better prognosis.
Interdisciplinary collaboration is the essential thing here.
The Heart-Explorer: How do research results from the Competence Network enter the actual health care?
Tanja Raedle-Hurst: I think it is important that we all work together on an interdisciplinary basis. That is the essential thing for us to be able to help our patients. Of course, the pediatric colleagues have to establish an accurate diagnosis during childhood and refer the patients to me early enough, so that I can continue offering them good treatment during adulthood. Our aim is for the patients to reach old age and to also reach it well. That is what makes our research so important. And thus, I like the fact that so many people participate in the Register and as such help us doctors to gain further knowledge which we can then, in turn, use in clinical practice.
The Heart-Explorer: Professor Dr. Raedle-Hurst, thank you for the interview.