Leading child cardiologist Jan Marek on the challenges and rewards of healing young hearts

Jan Marek

One of the laureates of the 2023 Gratias Agit awards, handed out by the Czech Ministry of Foreign Affairs to people and organizations for promoting the good name of the Czech Republic abroad, is Jan Marek, a leading expert in the field of pediatric and prenatal cardiology. Although he has spent the last 18 years working at Great Ormond Street Hospital for Children in London he has maintained close ties with his home country. I caught up with him on his last visit to Prague to talk about the challenges and rewards of his profession.

Jan Marek and Jan Lipavský | Photo: Czech Foreign Ministry

You are a leading pediatric cardiologist. What made you chose your given specialization in medicine?

Biographical book interview with Professor Marek entitled Srdce napůl  (The Heart in Half) | Photo: Vyšehrad

“My background is medical. Both my parents and grandparents are doctors and I never thought of doing anything different. During medical school I started volunteering at the Pediatric Cardiac Centre in Prague’s Motol Hospital and I was impressed by pediatric cardiology as a profession partly because of the amazing stories of the so-called “blue babies”. The history of this goes back to 1940 when doctors first started saving the lives of “blue babies”. I loved the fact that you admit a baby that is critically ill – that is blue – you give them treatment to fix their heart problem and in two weeks’ time the baby goes home with mum completely fit and healthy. I was just obsessed with this specialty! So I started volunteering and doing research as a medical student and after graduating from medial school I started specializing as a pediatrician and after the first training I focused on pediatric cardiology. I was very lucky that I was appointed by the founder of pediatric cardiology in Czechoslovakia, Professor Šamánek, who used to be my mentor and I think that today I am proud to say that without him I would never be working at Great Ormond Street Children’s Hospital in London – because he was such a fantastic mentor.”

How do you come to be working at Great Ormond Street Hospital?

Great Ormond Street Hospital | Photo: GOSH

“Actually, that was not my intention. I was approached by specialists from Great Ormond Street Hospital who were looking for someone who could introduce and implement new diagnostic procedures and I said OK, after 20 years of working in Prague it sounds like a challenge. Great Ormond Street Hospital is definitely one of the best pediatric hospitals in the world, so I said I would come for a year and I have been there for 18 years now. And I am very happy and very proud to be working in this amazing hospital.”

What do you find most difficult about your work? You work with child patients…

“That is the big difference in pediatric medicine, compared to adults, because we do not have just the one patient but also the family who need a lot of support. Communication is absolutely crucial. When we discuss the treatment of these babies, we need to bring the parents on board. So we create a mini-team and the priority of this mini-team is to save the child’s life, provide good treatment and a good quality of life in the long-term. We need to be frank. We need to explain in detail what the expectations are and what are the child’s chances of surviving without surgery. I always try to make sure that the parents really understand the problem and there is no room for any mystifications or lies. You need to be really frank. And once you achieve that we start working as a team and that is how we can progress together to save the child.”

Saving a child’s life would create a strong emotional bond, would it not? How do you deal with situations where you fail or can’t get a heart in time and stay sane and strong for the next challenge?

Illustrative photo: Ondřej Vaňura,  Czech Radio

“That is the most difficult question you can ask. The basic thing is to understand the disease and to be frank and quite clear about the child’s prospects. Once we reach the ceiling in treatment – and that may happen – we are lucky to have a very strong supporting team, including the palliative team – and they are the experts who can communicate with parents much better than myself. We have the environment and the means to offer ways of managing the situation so that the child does not suffer at the end of their life. There are many options. The child can have a separate room where they can be with their family – that is always very important –and then we discuss how we are going to wean off the treatment. There is an option that the child may pass away in the arms of the family. If the parents wish to move the child to a hospice we can offer that as well.”

I was speaking about you. Where do you get the strength to come to terms with these situations – when you have no heart for a child patient or the body rejects it, when you know you have lost.

“You always learn from every individual case. You need to be confident that there is nothing more you could have done, nothing more to offer in terms of treatment. If you are not confident, you can still communicate with other institutions. It is fair to ask for a second opinion. At Great Ormond Street Hospital we often communicate with the Boston Children’s Hospital or Philadelphia Hospital or our neighboring centres. I need to remain professional, but of course, I always have doubts and bad nights and it spins in a circle and you keep thinking about it. But once you are confident that you did everything possible and the diagnosis is confirmed, when you know why the surgery did not go well you learn from it. When I know that I did the best I could do, I can come to terms with it.”

How have the possibilities of saving children with heart disease changed over the years of your practice? What can you do now that was not possible when you started?

“Things have improved massively. I have worked in this field for nearly 38 years and this specialty has developed hugely. First, we can diagnose most heart conditions even before the babies are born. That was my job in Prague –I helped to implement the fetal cardiac screening program in Czechoslovakia in 1986. And now we are even able to treat the babies before they are delivered. So the typical disease that we often see is tachycardia or very fast heart rate and we know that we can very successfully treat these babies through the mother – that we can administer the medicine and the treatment is very successful. This is a life-saving procedure and very often structurally these hearts are normal so we save a baby’s life before it is born and the child remains well and fit till adulthood –so that is truly rewarding.

Illustrative photo: Myriams-Fotos,  Pixabay,  CC0 1.0 DEED

“We can even try to use small catheters so that we can open up the valves before the babies are born. This is a bit more tricky, because there are risks associated with these procedures including maternal risks and if something goes really wrong we have a 200 percent mortality. So this is still experimental and is a big ethical issue as well. I am very supportive of the view to develop large centres – we do not need many centres, probably just one in the UK and one in Europe – to concentrate these pregnancies because we need to develop the skills to do it and do it safely. It is not routine. This is something new that has emerged since the new millennium.

“And then, there has been massive progress in transplantations, massive progress in the use of prosthetic materials, stents, valves which we can introduce through a catheter –these are the “keyhole” procedures. So that is a completely new field – so called catheter interventions that we can implant in the heart through a small incision in the groin using the catheter and that is a massive improvement.”

What is the life expectancy of a child today with a transplanted heart – and even if it is highly successful, would that child need a new heart in 10 or 20 years’ time?

“Typically, we would transplant the heart from a brain dead person – when the heart is still beating. Nowadays we can use a heart that has stopped beating.  We can reanimate the heart and use it for a transplant. In this way we extended the pool of donors and we have a much higher success rate of transplantations.”

“In transplants we always struggle with the lack of donors for small babies. We can implant a heart that is three times larger than the heart of the recipient, so that is good, and it grows with the child. Our youngest transplant patient was three months old, I believe. And with the new medicine we now use for immune depression, the 5-year survival rate is now at 90 percent. So that is fantastic, but of course there are associated problems – coronary disease, diabetes…so it is not a cure, but we can offer a life for ten, twenty, thirty years. A re-transplant is possible, but again it depends on the availability of a donor. There has been a massive improvement since we started doing DCD transplants. You may not have heard of them. Typically, we would transplant the heart from a brain dead person – when the heart is still beating. Nowadays we can use a heart that has stopped beating. We can reanimate the heart and we have very special perfusion pumps so we can implement the nutrients and the oxygen during transportation and we can use such a heart for a transplant. That is a big achievement. In this way we extended the pool of donors and we have a much higher success rate of the transplantations.”

How has artificial intelligence affected your work? How much can it take over and where will a human doctor always be irreplaceable?

“I have this vision that the mum will go to a shop, or order an ultra-sound machine on Amazon, do the examination by herself at home and send the data to the lab in the hospital for the machine to analyze and then the doctor will just look at the result.”

“This is also a part of my research project, not just artificial intelligence but multi-modality imaging, 3D printing, virtual reality. We have developed a massive program that includes a training program for students where we can use simulators based on virtual reality and of course part of it is artificial intelligence. This is not rocket science. The artificial intelligence is about big data. We need to collect big data. So it is more about collaboration, because our field is rather small and we need to collaborate with many institutions to collect the data. But once you collect good quality data then you can really start using the deep learning machine and eventually the machine will tell us the diagnosis –probably already from the fetal stage. I have this vision that the mum will go to a shop, or order an ultra-sound machine on Amazon, do the examination by herself at home and send the data to the lab in the hospital for the machine to analyze and then the doctor will just look at the result.”

I was just going to ask about that – what would you like to see in your area of expertise develop during your lifetime?

Illustrative photo: fernandozhiminaicela,  Pixabay,  Pixabay License

“From the technical perspective plenty, I would welcome such development. But I definitely would not want to lose the connection with the patient, the human touch. So one part is the technical aspect and I am sure that computers will sort out a lot of problems, on the other hand, I hope that this will give us more time to spend with the patient, to explain, to talk and to really have a good relationship with the patient and the family. That would be my priority. And then, to ask the machines what we should do. OK, the machine says this and I agree, because I supervise the program and then finally – probably –let the machine do the operation.”

There are concerns that Covid may have a bigger impact on some of our organs than was previously thought. Have you seen an increase in heart disease that you suspect may be linked to Covid or that Covid made worse?

“During the acute pandemic we did see cardiac problems that are similar to what we call myocarditis or inflammatory disease of the muscle and also inflammation of the coronary arteries. As you know the coronary arteries are extremely important because they supply the heart with oxygenated blood. We saw patients who developed the aneurysm formation or symptoms which are typical for another inflammatory disease which is called Kawasaki disease. So during the acute phase of the pandemic we witnessed these two problems. Interestingly, all patients in our institutions, except two, recovered completely. So it was a big issue during the pandemic, but it is not an issue any more at this stage.”

So it was not irreversible?

“It was reversible. Even in patients who were critically ill. I remember two or three that we had to put on extra-corporal-circulation because the cardiac function was so depressed, but they recovered within three or four days. So we learned a lot. We still have to wait for more data regarding the long-term outlook for these patients, but so far we havn’t seen any major impairment of the cardiac function in those affected.”

You cooperate closely with Czech specialists and help train young doctors – has Brexit made that more difficult?

Illustrative photo: StockSnap,  Pixabay,  Pixabay License

“Yes, and that applies not only to doctors from the Czech Republic, but from continental Europe in general. It is a shame really and I feel very sorry for them because that has always been the dream of young doctors –to one day come to Great Ormond Street Hospital to get training for six months or one year. I remember this was my dream as well, even before the Iron Curtain fell. Great Ormond Street is almost like a Mecca of pediatric cardiology and now it is extremely difficult to get students here because they have to demonstrate that their pre-graduate training matches perfectly with the training with the UK, they have to pass a language test again and it is also quite costly to get the GMC registration for European colleagues. I think it costs fifteen hundred pounds and for a young doctor that is a lot of money. It is getting better now, a little bit, but I am sure that many young doctors lost the opportunity to come to Great Ormond Street Hospital. It is a shame for us as well, because I love to teach and it is part of my professional career. Fingers crossed that this will resolve itself somehow or that the government will notice that they need to change the rules to get more young doctors to the UK because all of Europe is short of doctors.”

And would you say that cardiology is at a high level in this country?

“If you get a heart attack the best place in the world to be is the Czech Republic. Because they guarantee that you will receive the stent into the coronary arteries within two hours. That is an amazing program.”

“In the Czech Republic? Yes, cardiology is the flagship of medicine here. The adult cardiology is amazing. You probably don’t know this, but if you get a heart attack the best place in the world to be is the Czech Republic. Because they guarantee that you will receive the stent into the coronary arteries within two hours. That is an amazing program. The colleagues who developed it were awarded by the World Health Organization. So cardiology is very well developed in the Czech Republic, including pediatric cardiology and cardiac surgery.”

Many people have a heart problem they are not aware of. How important is prevention in your field of medicine?

“Extremely important. Many cardiologists use the media to get that message across, to raise awareness of the risks, particularly high blood pressure and cholesterol. So you want to keep active, eat a balanced diet, a lot of vegetables, fruit, fish, rather than the pork, cabbage dumplings that are popular in the Czech Republic, I would not advise that. So, a low-fat diet, sports and an active life is what I would recommend.”

We have seen far too often you footballers dying on the playing field of heart arrest. I understand you were prepared to start a project to test footballers’ hearts but nothing came of it. What happened?

“Well, one of my areas of interest was the congenital abnormality of the coronary arteries. Some children can be born with this and they are potentially at risk of the coronary arteries getting compressed during vigorous exercise resulting in sudden cardiac death.

“We see more and more of these diseases as part of the routine screening on ultrasound. So it can be a completely coincidental finding and we know that some of these abnormalities are potentially life-threatening. This problem first emerged when American military recruits who died suddenly underwent an autopsy that revealed that the cause of death was an abnormality of the coronary artery.

Petr Čech | Photo: Filip Jandourek,  Czech Radio

“Now we are in a difficult position because we can incidentally pick up this abnormality but we do not really know whether this particular subject will be at risk of dying during vigorous exercise and it is particularly difficult if you have athletes who are aiming for a professional career. I have a lot of patients in London and Prague and I remember one swimmer who was in training for the Olympics. I picked up this abnormality on her scan and in the end she quit because she was scared that she might die during the competition.

“As regards the project you asked about – I started that due to my connection with Chelsea goalie Petr Cech and he was very keen to support a pilot project in which I and my colleagues would use ultra-sound machines to obtain good pictures of players’ coronary arteries – academy players aged between 12 to 16. So it was to have been a test regarding how well we could image the coronary arteries (the boys have a lot of muscle which could complicate that) and see whether we would find any abnormalities. That was the primary idea. But then the question rose – what would happen if we did and that was the end of the study.”

Because they didn’t want to know?

“Of course. It is a very competitive sport and academy players are all aiming for professional careers in which they hope to earn a lot of money. So the medical director of the Chelsea team actually cancelled the project.”

We should say that you were awarded the Czech Foreign Ministry’s Gratias Agit award for promoting the Czech Republic abroad. How do you feel about that and what did you get the award for specifically?

“Well, first I want to say that I am proud of receiving this award. And I think it is largely for my interest in not being completely disconnected from the Czech Republic. I did not want to leave the country and close the door and it was never my intention to leave the country so as to earn more money. I was lucky to be offered the amazing opportunity to work in one of the best hospitals in the world and I always kept in mind something that comes from my early days working with my mentor who told me “You need to work with young people and you must do everything to make them better than you are.” I always kept that advise in mind and that is why I started a project supporting Czech doctors, I am also supporting a Czech charity for families with children with heart conditions. So I think it is probably all this that got me nominated. That I am a cosmopolitan person, happy to share my experience and help others.”

Jan Marek a Jan Lipavský | Photo: Michal Krumphanzl,  ČTK

Professor Jan Marek is a physician specialising in pediatric and prenatal cardiology. He is currently a Lead Consultant of Echocardiography and Prenatal Cardiology at Great Ormond Street Hospital for Children (GOSH) in London. He is also a professor at the Faculty of Medicine of Charles University in Prague, and a professor at the Institute of Cardiovascular Sciences at University College London (UCL).

Professor Marek has a private practice in Prague and he continues to consult complicated medical cases with colleagues at the Children’s Cardiac Centre of Motol Hospital.

He sponsors Srdíčkáři, an association of parents of children with heart problems, and he works closely with the Be Charity Foundation to help raise funds to support children with heart disease.

In 2023, the Vyšehrad publishers published a biographical book interview with Professor Marek entitled Srdce napůl (The Heart in Half).