This interview was originally published in Turkish on Nefes, the magazine of the Turkish Pulmonary Hypertension Association PAHSSc, in June 2022. We extend our sincerest thanks to our friends and colleagues at the PAHSSc for graciously permitting us to republish and share it with the wider pulmonary hypertension community.
Professor Maurice Beghetti is Head of the Pediatric Subspecialties Division (HUG), Head of Pediatric Cardiology Unit (HUG) Director of Pediatric Cardiology Unit (HUG), Director of PH Program (HUG) of Children’s University Hospital – Geneva (Switzerland).
The Turkish version of the interview is available on the Nefes magazine, which can be found at this link on the PAHSSc website, at page 12.
Interview
Would you kindly give us some information about yourself. What triggered your interest in children with Pulmonary Hypertension?
My interest in pulmonary hypertension started in the early 90’s when inhaled nitric oxide started to be used. I was involved in post-operative cardiac patients and this was a major change for the treatment of postoperative PH. Then I just continued my interest in this pathology and had the chance to see and participate to all the changes we have seen over the last two decades.
It was rather difficult to get a diagnosis in the past. With regards to children and adolescents, is it more difficult nowadays? If this is the case what kind of problems are being encountered?
Not sure the diagnosis was difficult but treatment was a real problem and as such not many doctors were interested and aware of this disease. Most of the patients died in a very short period of time.
The symptoms are not always typical and the physicians need to keep in mind that pulmonary hypertension is a possible diagnosis in particular with patients presenting with fatigue, shortness of breath and syncope.
For sure the patients need to be diagnosed and treated by experts in an expert center having access to all diagnosis prosedures and treatment possibilities.
As regards Eisenmenger Syndrome, is its first symptom being short of breath accompanied by getting tired quite easily? Are there other noticeable symptoms?
This is a real problem. It is a cardiac malformation that leads to pulmonary hypertension. So the cardiac disease should be diagnosed early and cardiac surgery performed to avoid pulmonary vascular lesions. So here the problem is the diagnosis of the cardiac malformation! And access to early surgery. If the patient is not operated early it may become asymptomatic or seems asymptomatic and then he develops dyspnea and cyanosis but this may be too late for cardiac surgery.
It is advocated that the best treatment could be made through fully equipped multidisciplinary centers frequented by a high number of patients. Do you share this view?
For sure we need experts and centers that have a lot of expertise. This is true to obtain correct cardiac diagnosis and in particular for cardiac catheterization. If a center does not have access to all diagnostic procedure there is a risk of wrong diagnosis and this is a problem for inadequate treatment!!
In the case of Turkey, it is a relatively big country with medical services of different standards. In the case of children the means is rather limited. What do you suggest be done?
To concentrate is an option and organize this then in a sort of collaborative organization with a few real expert centers that can collaborate with the remote centers to be sure that the patients are well followed.
I do personally this with some other centers and it works well. You need a good collaboration and trust. Now with virtual meeting experience this can be adapted and the best could be made for the patients. Still access to real experts is mandatory to obtain better results.
Are there emerging effective treatments for pulmonary hypertension in children and adolescent patients?
Yes, several studies are ongoing with treatments used in adults (Selexipag, Macitentan, Riociguat, Trepostinil) but regarding treatments that are currently being studied for adults as well, things have changed a little bit recently so that we study treatments trialed in adults rapidly in children to give the opportunity to have the treatments available for children. Some studies for the new treatments including Sotarcept are currently considered for children.
Are the same medical treatments offered in identical amounts for adult patients and for children and adolescent patients with pulmonary hypertension as well?
Yes, indeed for most if not all the treatments in adults are used on children and there seems to have the same results. Some studies are still ongoing and awaiting approval in children. But this has changed now, we really have studies in children that give very important data not only on efficacy but on safety of these treatments in young patients. One of the most important aspects is to find the ideal and correct dosage for children.
When young patients become young adults are they referred to other hospitals or is their treatment followed in the same hospital? How many hospitals are there for young patients in Switzerland?
The transition, this is the term used to pass from children to adult clinic, is a very important period. This should be prepared and organized . Either this is done in the same hospital and it is a bit easier (this is the case in my hospital) or the patient needs to go to another hospital. This needs to be organized with the adult and pediatric team, the patient and his family to avoid any problems. Education of the patient is very important so that he is in charge of himself which is an important part of the transition to adult care. In children we do not have so far a centralized care and at least the 4 university hospitals take care of the children with PH.
As a physician do you find it hard to explain the child’s illness to his parents?
Yes and No. With experience you know better how to do it but each patient is different and it is not the same to diagnose the diagnoses at a very young age or in adolescent. In addition you do not have only the patients but all the family. It takes time, empathy education…similarly to medical treatment this requires experience and understanding. But for sure it remains difficult to explain a disease that has no cure.
Do you find it difficult to communicate with a young patient with PH?
See the previous combined answer. Getting together both parents and children you need to approach this as a family problem.
As a result of Covid-19 epidemic were treatments given to children partly disrupted? Were they brought to hospital during the confinement period? Could tele-medicine facilities be used for them?
Personally I manage to have the patient in the hospital but I can also manage this virtually. I, we could really continue to treat patients in quite good conditions in my center even if not ideal. We do this for several patients not living in Switzerland. Covid-19 period has thought us that we can do it even if not ideal. This is really an option.
Your patients are very young and they have a long way to go. What do you feel about lung and heart transplantation surgeries? Is it easy to find donors? As in In Japan are there lung and heart transplantation surgeries from live donors?
This is a real question as transplant survival remains quite low if we consider young and very young patients. Even if we try to postpone lung transplant it is of utmost importance to list the patient in what I would call good conditions to survive surgery…. Therefore adequate medical treatment is necessary to improve and stabilize patients and this requires expertise and access to all treatments including parenteral prostanoids. In addition new approaches such as Pott’s shunt may help for this.
What recommendations would you give doctors who have recently started their profession and who are dealing with PH?
They need a good training in this domain and the Association for Pediatric Cardiology (AEPC) has published a curriculum for this.
Is it easier to improve the exercise capacity of children more easily with respect to that of an adult?
This a good point sometimes exercise capacity measured with 6MWT seems not to be as good for children as for adults but data support the use of maximal exercise capacity using CPET as better for this evaluation. Interestingly children may have a quite good exercise capacity despite advanced disease and compromised hemodynamics.
What kind of physical activities should children with PH be allowed to join? Do you believe children with PH should be exempt from physical education classes?
This seems to be a case by case approach and depends on the situation and hemodynamics children should be kept in good “social” conditions and not isolated because of their disease. Strenuous exercise should be avoided but some exercises adapted to the situation may be possible. It is difficult to give guidelines for this. This may also depend on the etiology of PH.
Do you remember the first young patient with pulmonary hypertension you diagnosed?
Yes, the first Idiopathic patient was in 1991. He came for a desaturation because of inhalation of a peanut but finally desaturation remained desaturated after removing the peanut from the bronchus and the patient was diagnosed with PAH. He was one of the first children that was put on inhaled iloprost and then he went on IV eproprostenol.
It seems that recently heart and lung transplants have mainly way only to lung transplants. What is your view on this? When is it imperative to replace both organs?
Most of the centers perform double lung transplant in PH and this is also supported by recent data in pediatrics. After lung transplant the right ventricle recovers even in presence of severe hemodynamic impairment. This may be different in Eisenmenger Syndrome depending on the cardiac malformations that may require heart transplant. This is the most recent reference on the subject: Hansmann et al JHLT 2022
If there are any other comments you would like to add please feel free to do so?
We have made a lot of progress during the last two decades but a lot of work remains to be done. Key points are early diagnose, expertise, and aggressive treatment and we really hope for new treatments for further improving outcome that still remains not ideal.
Professor Beghetti thank you very much for your time and kind contribution to Nefes.