Dr. Renzo Marcolongo graduated from Padova University in 1983. He is a specialist in Hematology, Cardiology and Clinical immunology who devoted his professional and academic career to the field of systemic autoimmune, immune-mediated and autoinflammatory diseases and cardioimmunology. Together with Professor Alida Caforio, he is a co-founder of the Cardioimmunology branch of the Cardiology Unit at University Hospital Padova. He has been a member of a European expert panel on Therapeutic Patient Education, and he is a lecturer in this field at many Italian and French institutions. He is the author and co-author of over 90 international publications.

Immune-checkpoint inhibitors (specific antibodies used mostly for treatment in oncology) are of great importance in some cancer patients. However, myocarditis is a very rare complication of their administration. What are your experiences with this clinical entity?

So far, we did not treat any patient with Immune-checkpoint myocarditis. They are probably more rare than expected, at least in Italy.

In Padova Hospital, you have implemented a specific safety protocol for patients with autoimmune myocarditis and immunosuppressive treatment. Could you please share more details about the protocol?

In our experience, a diagnostic workup, namely the Safety Checklist, in biopsy-proven acute myocarditis/dilated cardiomyopathy patients eligible for immunosuppressive therapy should include blood and instrumental tests. In brief, patients were screened for hidden infections (especially tuberculosis, HIV and viral hepatitis B and C) and common malignancies according to age, gender and race (in particular, non-invasive first-line investigations for haematological disorders and breast, cervix, colon and/or prostate cancer were performed when appropriate).

(CAFORIO, Alida Linda Patrizia et al. Long‐term efficacy and safety of tailored immunosuppressive therapy in immune‐mediated biopsy‐proven myocarditis: A propensity‐weighted study. Online. European Journal of Heart Failure. ISSN 1388-9842. https://doi.org/10.1002/ejhf.3220. CAFORIO, Alida L. P. (ed.). Myocarditis. Online. Cham: Springer International Publishing, 2020. ISBN 978-3-030-35275-2. https://doi.org/10.1007/978-3-030-35276-9.)

What are the latest trends in immunological therapy of myocarditis, including its specific forms like giant-cell and eosinophilic myocarditis?

We successfully experienced the anti-CD20 monoclonal antibody Rituximab (used for therapy of different autoimmune diseases, targeted against B-lymphocytes producing antibodies; see this article) in the treatment of both giant-cell and eosinophilic myocarditis. In recent years, we also successfully treated several patients with eosinophilic myocarditis with anti-IL5 monoclonal antibody mepolizumab (eosinophils are involved, for example, in allergic reactions; under publication).

What would you like to communicate to the readers of myocarditis.eu?

To be confident. Myocarditis, when promptly and properly diagnosed by endomyocardial biopsy, is a treatable disease. In our experience, immune-mediated/virus-negative forms usually show an excellent response to immunosuppressive/immunomodulatory treatment, with a good prognosis.

Interview with dr. Giordani may be found here.

Příspěvek Dr. Renzo Marcolongo pochází z Myokarditida

Dear Dr. Giordani, could you please share some information about your career?

I am currently working as a Research Fellow at the Padova University Hospital, which is located in Northern Italy and serves as a third-level referral center for almost all kinds of cardiovascular diseases in our region, ranging from congenital heart disease to end-stage heart failure with need to heart transplant referral. I completed both my University studies and Cardiology training in this hospital and I am currently having the opportunity to keep working in this center in the Cardioimmunology field. During my Cardiology training I especially focused on multi-modality cardiac imaging and clinical management of myocarditis and immune-mediated pericarditis patients, with the possibility of working together with an expert Immunologist.

You have published many articles about myocarditis, including its fulminant form. Could you please tell more details about this specific form of myocarditis?

Myocarditis is a heterogeneous disease, as it can present in many forms. Among those, fulminant presentation is the most dangerous. Despite a standardized definition of fulminant myocarditis does not exist, it is usually intended as a form of myocarditis presentation in which hemodynamic support in terms of inotropic drugs or mechanical circulatory devices (i.e., ECMO, Impella) is needed. Moreover, incessant ventricular arrhythmias (i.e. arrhythmic storm) can coexist, also in presence of preserved left ventricular systolic function. An important concept is that fulminant myocarditis, rather than being a distinct form of myocarditis, is instead a peculiar clinical presentation of the disease, carrying a worse prognosis, even long after the resolution of the acute phase of the disease.

How has the treatment of fulminant myocarditis changed in recent years?

Myocarditis treatment in general should be always tailored both on the disease’s characteristics (i.e. histological type, infectious or immune-mediated etiology) and the patients’ features (i.e. hemodynamic stability, comorbidities). The first description of a fulminant myocarditis case was performed at the beginning of the 1990s; since then, the disease management evolved from basic hemodynamic support to etiological treatment, essentially driven by the results of endomyocardial biopsy. When myocarditis presents in a fulminant form, it is mandatory to achieve a sample of myocardial tissue as soon as possible in order to confirm the diagnosis of myocarditis (since myocarditis can mimic many other cardiovascular diseases), to establish the type of inflammatory infiltrate (for example, eosinophilic myocarditis deserves a different treatment than giant cell myocarditis) and to detect any infectious agent in the myocardium (which is a contraindication to immunosuppression and orients towards the use of antimicrobial drugs).

What is the future of fulminant myocarditis management based on your opinion?

The future of myocarditis treatment relies in precision medicine and personalized protocols. Further research is needed to identify individualized prognostic markers, especially the predictors of response to immunosuppressive therapy and the predictors of myocarditis relapse. It is only recently that fulminant myocarditis has been identified as a marker of worse prognosis both in the short and long term, while in the past it was incorrectly believed that this myocarditis form had a paradoxically low rate of mortality after the resolution of the acute phase of the disease. This reflect the amount of work that still has to be done in order to ensure that all suspected myocarditis patients with severe hemodynamic presentation are offered the best diagnostic and therapeutic methods.

What would you like to communicate to the readers of myocarditis.eu?

Fulminant myocarditis is only one of the many different aspects that inflammatory cardiomyopathy can present. Cardioimmunology is a new frontier of medicine with lots of promising advances in the near future. I hope that this will lead to improvement in the patients’ conditions.

Příspěvek Dr. Andrea Silvio Giordani pochází z Myokarditida

Dr Roblová, please, could you share with us some information about your professional life?

After graduation from the medical school (1st Medical School of Charles University in Prague), I have had become employed in my home-town in České Budějovice. Now, I have been working in the 2nd Department of Medicine of General University Hospital in Prague for 2,5 years where I am focused primarily on the issue of cardiomyopathies and echocardiography.

How often in your clinical practice do you meet with the cases of myocarditis and inflammatory cardiomyopathy?

In our centre for cardiomyopathies and on the ward, we treat approximately 5 patients per month.

According to the data of UZIS (Institute of Medical Informatics and Statistics), the number of cases of myocarditis has increased by about 60 % when compared with the time 10 years ago. Would you offer us an explanation for this increase?

It is true. Surely, improved diagnostic tools take a part in this, primarily cardiac magnetic resonance that is a golden standard in non-invasive diagnostics of myocarditis. Most likely, even a better awareness and knowledge about this disease among medical professionals play their roles too.

Is care about patients with myocarditis and inflammatory heart diseases generally somehow specific?

Generally, restriction of the physical activity for 6 months or until the reduction of the inflammation or the improvement of the heart function is recommended. In the case of heart failure, standard pharmacotherapy of the heart failure is administered (ACE inhibitors, beta-blockers, diuretics etc.). Regarding specific therapy, the situation is not clear completely. There is an agreement in the question of giant-cell and eosinophilic myocarditis and sarcoidosis, where immunosuppressive therapy is indicated. (note of the redactor: for a more detailed description of some terms click on blue-coloured words).

What are according to your professional opinion the biggest difficulties in the research of diagnostics and therapeutics methods of myocarditis and inflammatory cardiomyopathy?

The main problem according to my opinion is the heterogeneity of the myocarditis aetiology. Moreover, it a histological diagnosis and for the definitive diagnosis, the endomyocardial biopsy may be needed, what is not a rule and this examination is performed only in specialized centres. Furthermore, in milder forms of myocarditis without the disorders in the heart function or life-threatening arrhythmias, the endomyocardial biopsy is not indicated.

What is your opinion about the website www.myokarditida.cz? Would you recommend it? Alternatively, for whom do you think it could be beneficial?

The website truly pleasantly surprised me. They are well arranged, with up-to-date information. I think that the website is very helpful to public and physicians, and thus I may recommend them glowingly.

Author of the opening picture: Znajdzlekarza

Příspěvek Lenka Roblová M.D. pochází z Myokarditida

1. Could you share something about your career?

I earned my B.A. and M.D. title at the University of Pennsylvania. I completed an internship and a residency at Stanford University, followed by a fellowship in the Division of Cardiology at the University of California, San Diego. I further completed a fellowship in vascular medicine at Mayo Clinic School of Graduate Medical Education. Since 2015, I have chaired the Department of Cardiovascular Medicine at Mayo Clinic in Florida, USA.

I would like to add that Professor Cooper has dedicated his professional life primarily to the issue of inflammatory heart and vascular diseases, including myocarditis and cardiac sarcoidosis. He is a researcher of many scientific projects and publishes in leading world-class medical papers. He is a member of American College of Cardiology and European Society of Cardiology. He lectures on the topic of inflammatory heart and vascular diseases worldwide. He is also the founder and vice president of Myocarditis Foundation (see questions below).

2. What is Myocarditis Foundation?

Myocarditis Foundation is a non-profit organization, formed in 2005 in response to the dire need for more information about myocarditis; myocarditis is an inflammatory disease that attacks the heart muscle leading to cardiac dysfunction and heart failure. The Foundation was founded and is led by volunteers, world-renown physicians, researchers, families who have lost loved ones and patients. It develops and maintains an array of awareness and educational resources for physicians, researchers, patients, and the public. It also provides the only fellowship-mentor research program funding of myocarditis, whose goals are the development of accurate, rapid diagnostic methods and safe, effective therapies that minimize or eliminate myocarditis progression to heart dysfunction, failure, and sudden death. The goal of the Foundation is also to support families and patients with this disease.

3. Could you share some information about the activities of the foundation?

Besides the above-mentioned activities, Myocarditis Foundation organizes a number of events with the goal to increase the knowledge about the disease not only in public but also among physicians. Other goals of the Foundation are to gain financial resources for research projects and to support patients and their families. It is for example Myocarditis Foundation Announces Gala Fundraiser, which took place for the last time this June, and it is also Annual Myocarditis Foundation Family Support/Research Meeting.

4. What message would you like to send to patients with myocarditis and their relatives not only in the Czech Republic?

I would give a message of hope, that with a unified approach to the management of myocarditis we will make progress towards prevention and cure. Collaboration between medical centers is needed to make the major advances needed to help patients and their families.

Autor of the opening picture: Injurymap

Příspěvek Professor Leslie T. Cooper, Jr. pochází z Myokarditida

Příspěvek Veronika Stará M.D. pochází z Myokarditida

Could you please share with us some information about your professional life?

In 1999, I have successfully graduated from the 2nd Medical School of Charles University in Prague. After studies, I have spent a year as a PhD student on the Department of Medical Informatics and afterwards, I have spent 1,5 years on the substitute “civilian” military service. Then, in 2002, I have commenced in the Department of Radiology of the 2nd Medical School of Charles University and Motol Hospital. Except the 2-year intermezzo, when I have worked in a hospital in southern Scotland (Dufries, 2012-2014), I am a doctor on the Department of Radiology in Motol Hospital up to now.

What is currently the significance of cardiac magnetic resonance in the diagnosis of myocarditis?

Magnetic resonance is not absolutely necessary for the diagnosis of myocarditis, however, its importance is increasing gradually. Firstly, it can help to distinguish myocarditis from other diseases of the heart muscle. Secondly, it can display, if there is just an inflammation in the myocardium or if it happened, that irreversibly damaged heart muscle cells have been replaced by a scar. Furthermore, magnetic resonance is a reliable method for the evaluation of the heart ventricles function which is often decreased in myocarditis.

What benefits it brings?

The biggest advantage of the magnetic resonance is that it is non-invasive and without radiation load. Thanks to the fact that it determines the localization of the biggest damage to the heart muscle, it can help during invasive sampling of the heart muscle tissue (endomyocardial biopsy) if this examination is necessary. Further, the extent of the damaged displayed on magnetic resonance helps to determine prognosis, so what will be the progress of the disease. When the magnetic resonance examination is repeated, this progress may be evaluated.

A news in the magnetic resonance is so-called T2-mapping. Could you please tell us more about that?

T2 and also T1 mapping are not completely new methods, however their significance and utilization in heart imaging increases. Mapping is based on basic parameters of magnetic resonance imaging and it enables qualitative evaluation with the determination of T1 and T2 relaxing times in the certain tissue. These parameters are different in the healthy and sick myocardium. In recent years, a number of scientific studies using this mapping for the evaluation of myocarditis increases. T2 mapping is useful primarily for the situation, where oedema of the myocardium is diffuse (equally in the heart muscle) and it is difficult to evaluate it clearly visually.

Is there also some other news in the diagnosis of myocarditis in the question of imaging methods?

Newly used and researched method is T1 mapping, which, as it seems, enables more accurate determination of fibrous changes including general ones, which are difficult to be determined by traditional methods like late enhancement.

What is your opinion about the website www.myokarditida.cz? Would you recommend it? Alternatively, for whom do you think it could be beneficial?

The version for public dealing with the issue of different diseases are quite common abroad and so there are accessible in foreign languages (mostly in English). Website www.myokarditide.cz are also in the Czech language, and therefore they could be and an important source of information for Czech patients and their relatives. Considering the extent and depth of information, there could be interesting not just for non-professionals, but also for medical staff which do not come often in contact with myocarditis.

Příspěvek Theodor Adla M.D. pochází z Myokarditida