There is great uncertainty. Important scientific findings are still pending, including issues such as the impact of Covid-19/SARS-CoV-2, the infection situation, secondary damage after a previous infection with the virus, immunization and, above all, how to fight it effectively. How to find ways of handling the pandemic in a relaxed as possible way, particularly as a risk patient with congenital heart disease? Is that even possible?

We tried to find out in a video talk with the specialist for adults with congenital heart disease, Prof. Dr. Dr. med. Gerhard-Paul Diller. Our editorial team was backed up by Eva Niggemeyer, a language teacher who herself has a complex congenital heart defect.

Heart-Explorer Magazine: Ms. Niggemeyer, how have you experienced the pandemic in the past seven weeks? What has changed in your daily routine, as well as in your professional life as a language teacher; which restrictions felt most drastic to you?

Eva Niggemeyer:  I have been at home since the beginning of the lockdown. I no longer go out shopping. My neighbors have been helping me out here. And, of course, I’m currently not working. Our educational institution is currently closed. We are not allowed to offer on-site language classes. That was the situation until two days ago. It was then that I mustered enough courage to go shopping again for the first time. And this was an almost traumatic experience to me. I won’t do this again for sure. Many people aren’t observing the regulations. I was also surprised to find that the checkout staff seems to be exempted from the mandatory mask wearing. I do understand why that is. However, the measures I’ve been advised to observe in order to protect myself from catching the virus only work if other people wear their mask correctly or wear one at all and if they keep that recommended distance. I rely on that. And, as it is, not everybody pays attention. This makes me feel very insecure.

Heart-Explorer Magazine: Just recently, a new framework has been specified regarding easing certain measures on the federal and state level. Now, we have to rely more on the citizens’ individual responsibility. Play schools and schools, shops and restaurants, as well as museums and concert venues are opening up again, although they do have to follow strict conditions. How do you, as an at-risk patient, feel about that?

Eva Niggemeyer: Well, yes, that’s difficult. I’m currently watching the institutions of continuing education reopen in an increasing number of federal states. And we are expecting our institution to reopen any day now, too. This being the case, I, for my part, cannot say to what extent it would even be advisable for me to return to my workplace. In my classes, I get together with a lot of people. In addition, I would also have to use public transportation. So, I’ve been torn between enjoying a nice and relaxed life at home on the one hand and the feeling of complete uncertainty on the other hand. What is going to be the next step? What should and what shouldn’t I do?

Heart-Explorer Magazine: I can see that there are many pressing questions, Professor Diller. Before we delve into them – we’ve noticed you’re wearing a face mask hanging from your neck…?

Gerhard-Paul Diller: Yes, of course, we all need one here. And it goes without saying that we only wear it once. This one is going to end up in the trash right away.

Heart-Explorer Magazine: How do you, as a doctor, feel in this completely new situation? What has your day-to-day routine at the University Hospital of Münster, the UKM, been like in the past seven weeks? What changes did you face?

Gerhard-Paul Diller: Well, quite a few things. First of all, we made all the necessary arrangements for a potential inrush of patients, of course. That is, all routine measures have been reduced, as well as the activities in the outpatient department. Accordingly, we’ve had quite a limited schedule and have been focusing solely on the urgent cases and emergencies. Now, we have to decide how to handle the easing in a reasonable manner and see when we can resume our regular business at least to some degree. But, sure, during the past weeks, everything was about these precautions and measures to enable us to deal with that anticipated wave of severely ill Covid patients and to offer them adequate care.

Heart-Explorer Magazine: For the time being, this wave has not happened. What about now? Is it still to be anticipated, especially in light of the easing?

Gerhard-Paul Diller:  The first big wave has not happened so far, at least here in Germany - I think it’s fair to add that. The situation is much worse in other countries such as northern Italy, France, Spain, but also in some parts of the USA and in Great Britain. At the moment, it’s hard to predict how probable a second wave is. Likewise, only time will tell what the current easing of the lockdown measures will imply. Anything is to be expected. Of course, we hope that we will continue to be spared form the worst.

Heart-Explorer Magazine: Yes, absolutely. Which brings me to my next question. The new virus has not been sufficiently investigated yet. Scientists are doing their utmost to answer all sorts of questions. Which findings surprised you the most so far? And how do current findings match with your clinical routine at the UKM?

Gerhard-Paul Diller: There are indeed a multitude of studies and initial publications are already available too. However, many of these publications cannot be evaluated properly yet, since they haven’t undergone the required peer-review, but were published as preliminary results. We were surprised by the fact that patients reacted to an infection in different ways, their reaction ranging from a total absence of symptoms, especially in children, up to multiple organ failure and neurological damage, particularly in older individuals with underlying diseases. It is our urgent hope, of course, that a substance will be found with which a Covid-19 infection can be treated effectively. Above all, we hope that vaccination research will make quick progress so that we will be able to immunize patients against this disease as soon as possible. Until then, we can more or less just react to an infection with this disease. That is, we’ll be taking appropriate measures to support the patients in handling the infection. This includes treating patients who develop dyspnea with oxygen and even up to artificial respiration if required and providing them with intensive care. That’s the current state of things for us.

Heart-Explorer Magazine: Which disease courses do you encounter in your day-to-day practice with adults with congenital heart disease?

Gerhard-Paul Diller: The disease courses vary a lot. The situation is mostly as we know it from most publications. Many patients have a mild course that is largely comparable to the flu or a cold. Add to this the assumed larger, as-yet unknown, proportion of infected patients that we don’t see since they don’t have any symptoms. What we see here are patients developing symptoms such as breathlessness who have to be admitted and who then recover. It is only in rare cases, fortunately, that the infection involves the lungs being severely affected. These patients require artificial respiration and, of course, also intensive care for a longer period of time.

Eva Niggemeyer: People keep saying that, in the general population, the chance of developing only mild symptoms or no symptoms at all is relatively high. As an at-risk patient, I’ve been asked quite frequently what I’ve been asking myself too, of course: Do I have the same chance of remaining symptom-free? In other words: do I only have to fear worse things to happen in the case of an infection? Or do we at-risk patients face a higher risk of catching the disease in general? Is anything known in this regard yet?

Gerhard-Paul Diller: This is quite a speculative matter at the moment. There are initial data from China that suggest that patients with a cardiovascular risk profile do not only have a more severe disease course, but also a higher risk of getting infected with Covid-19. Accordingly, we have to expect that patients with a severe heart defect, in principal, have a higher risk than individuals without a heart defect.

Heart-Explorer Magazine: Ms. Niggemeyer, what’s on your mind now that you’ve just heard all this? We, of course, hope that this won’t happen in the first place, but, if this were the case, what would be your greatest concern if you were infected by the virus?

Eva Niggemeyer: My greatest fear is intubation. I’ve read a few things by people who claim that it does more harm than good. Apparently, there are different opinions on this. And you can’t always tell which information to trust nowadays. I’m currently considering to decline such an intubation in general. And then, I guess, you have to wait and see. An oxygen mask might be ok. But I guess, an intubation is my greatest fear. I wonder what’s your experience regarding this issue?

Gerhard-Paul Diller: As I said, we don’t really have that much practical experience with severe Covid-19 infections so far. Thank goodness, the big wave has not happened. But, of course, generally speaking, Covid-19 patients rejecting intubation and, accordingly, ventilation, are limiting their therapy. That has to be kept in mind. It also depends on the patient’s individual life situation, their quality of life and their prognosis. It has to be discussed on a case-by-case basis, if that is still possible at all. And this is where it really becomes difficult. That worries me. Such discussions can hardly be had as detailed as required in an acute situation. We haven’t had to face any such cases so far, though. But I can indeed imagine that such a case may happen yet.

Heart-Explorer Magazine: Ms. Niggemeyer mentioned this earlier: Quite recently, there have been various reports stating that the therapy results after intubation and long-term artificial respiration in Covid-19 patients have not been satisfying. What has been the experience in clinical practice so far, what have your colleagues from other hospitals been reporting?

Gerhard-Paul Diller: That differs, of course. In fact, it strongly depends on the risk profile, the age and comorbidities. It is true that younger patients with few comorbidities on average have a mild course. They don’t require to be ventilated that long and are weaned off more quickly afterwards. They recover significantly better than old patients with many comorbidities. But I don’t think that this comes as much of a surprise in this context. That had been expected.

Heart-Explorer Magazine: You can tell quite easily that this is a difficult question to be considered by doctors when a patient rejects such a measure. Which options do patients have to make an autonomous decision here and to thus make it easier also for the physicians in charge of their treatment?

Gerhard-Paul Diller: There is the classical option of the advance health care directive, that is, the option of putting the decision in writing. However, Covid-19 is an urgent situation. And that is completely different than finding that the state of a chronically ill patient is deteriorating successively with a declining number of treatment options being available. Usually, there is enough time for patients in such a situation to discuss their wishes and also what they do not want with the treating physician and family members. We must not discount this. Although the therapy results in the case of artificial respiration over a longer period of time are not all equally good, and even though the treatment can have severe consequences in some cases, with respect to Covid-19, there is in fact hope that the disease can be cured and the body can overcome the virus. And, in such cases, the most important rule is to do anything possible to enable a well-grounded cure.

Heart-Explorer Magazine: Do you think that, in this light, it makes sense for an at-risk patient to discuss a potential infection with a specialized ACHD cardiologist in advance?

Gerhard-Paul Diller: Yes, absolutely. Particularly if you feel that you need more information. For instance: What kind of impact will my disease have on the rest of my life, my quality of life and my life expectancy? In this context, you can also discuss matters such as a potential Covid-19 infection and what it might imply for you in particular.

Heart-Explorer Magazine: There are roughly 300,000 adults with congenital heart disease in Germany, with an average age that is significantly below 60 years. Is it known yet how many of them belong to the high-risk groups, for instance, because they have severe congenital heart disease that also affects the lung? And does this question regarding an affected lung make sense at all, since there have been reports of the virus causing myocarditis, impairing blood coagulation and leading to multiple organ failure?

Gerhard-Paul Diller: This is about patient risk stratification, which involves many factors. One of them is the anatomy of the heart defect, if you’d like to put it that way. Further factors that you have already mentioned have to be added to this. They include an affected lung, for instance in the form of pulmonary hypertension, and heart failure, maybe also kidney disease, liver disease and any additional immune deficiencies. In order to be able to answer this question in a differentiated way, we need to have an overall image that is made up of these individual factors and their interaction with each other. And, so far, the data situation is too limited. There are two registers that collect data on this, one in the USA and one in Europe. Also the German Society of Paediatric Cardiology collects data on the disease courses of children with Covid-19. There have been initial findings, but it is too early to discuss them.

Heart-Explorer Magazine: What is guiding you then, at the moment?

Gerhard-Paul Diller: We have seen various studies, from China at first, and then from Italy and, with the pandemic progressively spreading, also from the USA now. According to these, the virus usually affected older individuals that were aged significantly above 60 years and who had a so-called acquired disease such as hypertension, heart failure, heart attack or diabetes. In contrast, we currently only have a small amount of initial data on adults with congenital heart disease from northern Italy. There, ACHD cardiologists conducted a phone survey that included approximately 600 patients. In this patient group, no single fatal course was recorded that could be ascribed to Covid-19. The fact that this was the case particularly in this severely affected region gives us reason to hope. However, at the same time, we have to be very cautious.

Heart-Explorer Magazine: Why?

Gerhard-Paul Diller: We have to assume that patients haven’t caught the virus because of the shutdown; and that this might be the reason for the absence of severe courses. There are no antibody tests yet to check this. So much is based on hypothetical thinking at the moment. It can be said, however, that patients with severe congenital heart disease who have pulmonary hypertension or cyanosis are in the high risk group. These cases require particular caution. And besides these patients, there is a considerable number of patients with congenital heart disease that have had very successful surgery, who have a normal exercise capacity and who are physically very active. We can assume their risk is not that high. But, of course, they all should protect themselves as best they can from getting infected.

Heart-Explorer Magazine: Certainly, most of the patients know of the recommended protective and sanitary measures by now. That is, practicing social distancing, washing hands and hand sanitation, cough etiquette. This should have been memorized more or less. Now, we are facing an easing in many areas. Ms. Niggemeyer mentioned that earlier: How can ACHD patients protect themselves at all now? What about returning to the workplace, using public transportation or going to the hairdresser or shopping, in light of social distancing and nose and mouth covers?

Gerhard-Paul Diller: It goes without saying that observing the general recommendations continues to be an important thing to do. Specific recommendations regarding work, isolation or attending school have to be made on an individual basis, however. We are currently collaborating with the European Society of Cardiology to create a position paper which will hopefully put such recommendations on a sound basis. At the moment, in Münster, we advise patients with a very high risk, people who have, for instance, a univentricular heart or severe pulmonary hypertension, to work from home, where possible, and to be out in public little as possible. But all these are cases that also require an individual assessment. This decision can’t be a one-way-street, since it also implies serious social and economic consequences.

Heart-Explorer Magazine: Are there work areas that are particularly tricky in your view?

Gerhard-Paul Diller: We are especially skeptical with respect to patients who, for instance, work as educators with children, since children aren’t able to observe the social distancing and sanitizing measures in a consistent manner. In such cases, you just have to expect a higher infection risk than with people who work in an office with other adults. However, such recommendations have to be individually customized for each patient. Since they are, as I said, associated with serious consequences also with respect to the quality of life.

Heart-Explorer Magazine: I think this shows us all quite vividly how hard it is also for physicians to assess the situation and consider measures appropriately. The issue of quality of life plays a central role here. Ms. Niggemeyer, how does the need to brace yourself for the situation impact your quality of life?

Eva Niggemeyer: What I find to be hardest, in fact, is the total lack of social contacts, also in terms of a simple hug or anything like that. I’m experiencing a great lack here. I’m a very social person and I’ve been realizing that I miss the social aspect of my life very much. And, of course, I miss being able to just roam freely outdoors. I guess that I don’t differ that much from healthy people in this respect. However, in the worst case, I am the one who is most likely to lose a lot. On the one hand, I often feel like some kind of an overcautious exaggerator while, on the other hand, I think to myself, oh well… - you get careless quite easily, you see? So, I, too, am thinking that it all won’t be that bad, and also media reports don’t sound that dramatic anymore. People are happy. Why shouldn’t I be, too?

Heart-Explorer Magazine: I think we can all relate to that. Professor Diller, can, in some circumstances, such limitations bear an additional health risk, since they do have an enormous impact on the quality of life?

Gerhard-Paul Diller: This aspect should indeed receive crucial attention. It involves a psychological and also a medical element. For instance: At the moment, many patients have been hesitating to see a doctor. I am concerned that we might be missing a potential deterioration in people’s health that we could have prevented with appropriate measures. Besides the issue of isolation in and of itself, there are related factors such as weight gain and lack of exercise. All these factors have a negative impact on the quality of life. At the Competence Network for Congenital Heart Defects, we just conducted a survey to investigate these issues. Many patients took part. The results are currently being analyzed. A great deal of uncertainty and also a lack of information are already becoming apparent though. In this context, we have to also point the finger at ourselves. Maybe we should have started communicating more quickly. But the problem is that we don’t have any reliable data, you see, to meet this justified need of information. We are also facing a pandemic with a new virus for the first time. This is an unusual situation for all of us.

Heart-Explorer Magazine: In this unusual situation, and also with respect to the easing, does the public debate, and do consulting and decision making groups focus too one-sidedly on the elderly patients with pre-existing conditions, while neglecting people, that is, also younger people, with more rare congenital conditions?

Gerhard-Paul Diller: Yes. From my point of view, you can put it this way. And it is true. Of course, this is also due to the data that were communicated in the beginning and that revealed a particular risk for people of a considerably high age with pre-existing conditions. In this context, chronically ill adult patients and also children with congenital heart disease, who are very concerned at the moment, are easily forgotten, even though they experience this situation to be much more difficult than older fellow citizens do, who are retired. Our daily encounters with patients show us that they are at the beginning or in the middle of their professional life and, accordingly, they have to move about in the public sphere much more. They can’t just easily isolate themselves at home.

Heart-Explorer Magazine: Ms. Niggemeyer, what do you wish for most at the moment, particularly from those around you?

Eva Niggemeyer: Regarding the people close to me, I couldn’t wish for more, since they all have been handling the situation greatly. I’ve been receiving so much support. And I find that to be really enriching. Regarding my wider environment, I wish people would bear in mind that all these regulations aren’t just about them, but about protecting others as well. I don’t think it’s asking too much to cover both your nose and mouth with the face mask instead of only your mouth. I’m doing it too after all. It makes breathing harder for me, but it still works. Let’s just say that I wish there were still more of that wave of solidarity that could be sensed at the beginning. I’d like it to stay that way.

Heart-Explorer Magazine: Are there any more pressing questions that you would like to ask Professor Diller?

Eva Niggemeyer: Well, I’d say that I’m content. That is, as far as this is possible in the current situation. It just isn’t easy. I think the greatest worry of us patients is that, at the end of the day, we have to make decisions on our own since no one can tell us exactly what is the right or the wrong thing to do. If these decisions have a direct bearing on your own life, then this is a great burden of course. I think that all you can do here is listen to your inner voice. No one can do this for you.

Heart-Explorer Magazine: I think that’s a good way of putting it. Ms. Niggemeyer, Professor Diller, thank you so much for the interview. Stay healthy and positive!

The interview took place on May 08, 2020.