Journal of Rare Cardiovascular Diseases: Moving towards maturity, and implementing the Krakow RCD Classification as a milestone

Abstract

Dear Readers – our Friends and Colleagues, The present, third, issues of your quarterly Journal of Rare Cardiovascular Diseases is an important landmark in the life of the Journal; the life that presents a process from inception to full maturity, with an increase in overall impact. The content of the issue brings to you a happy (we believe) combination of continuity and novelty. I am convinced that you will judge the issues that we bring to your attention today as relevant to your patients with rare cardiovascular disease (RCD), fulfilling one of the principal aims of the Journal. We start with a review of cardiac manifestations in Churg Strauss syndrome, a rare but important form of necrotizing small‑vessel vasculitis. The review is by the internationally‑recognized experts in the fileld, Professors Wojciech Szczeklik and Tomasz Miszalski‑Jamka, who have designed and carried out significant original research in this field and today have a large experience in cardiac imaging and managing patients with the disease. The original contribution that follows is a collaborative work from two Krakow centers that investigated the effect of cardiac involvement in systemic sclerosis and systemic lupus erythematosus on clinical and spiroergometric parameters of exercise tolerance. Those patients require a truly multi‑specialty management that will no doubt benefit from the new knowledge generated by the investigators reporting their work in this issue of Journal. Traditionally, you will also find a mixture of case reports from different corners of the world that, beautifully illustrated, present the diagnostic and management path in RCD patients that are likely to cross your professional path one day. Dr Aleksander Trabka‑Zawidzki and colleagues present the impact of accidentally discovered non‑compaction cardiomyopathy in a patient with the initial diagnosis of acute coronary syndrome. Dr Stephen J. Pettit and colleagues from the Scottish Advanced Heart failure Service discuss the challenges of mechanical support of circulation in a young man with acute fulminant myocarditis, where a timely an appropriate diagnosis leads to a pharmaco‑mechanical battle with the diseases, having done ‘it all’ (by what is achievable today) live saving may not necessarily come as a prize to the management and consultation team. This exemplifies how much is yet to be researched and developed in this area. Dr Grzegorz Kopec and colleagues share some nerve‑wracking challenges they experienced in a pregnant woman with Eisenmenger syndrome (with a happy end of home discharge of both for the child and the mother). Finally, Dr Kaznica‑Wiatr and colleagues discuss the issues of pulmonary arterial hypertension after the correction of cardiac shunts, while Dr Hanna Dziedzic‑Oleksy and her friends from the department and surgical colleagues discuss their management of a young patient with severe spine deformity and Marfan syndrome. Again, these reports reflect the basic feature of RCD field: multi‑specialty collaboration that enables some clinical successes that, until recently, remained unimaginable. The final article in this issue is a report by Dr Jakub Stepniewski on our launching a new EU‑supported project in rare cardiovascular diseases, entitled Development of the European Network in Orphan Cardiovascular Diseases. We are extremely happy to share with you the joy of our successful application and plans (some – quickly implemented) to formally extend the European collaboration RCD network. Please continue your support to your Journal. We look forward to your submissions in the form of original articles, reviews or RCD case reports that will not only highlight your work and experience to your colleagues in the field and will make it formally quotable. We also hope that you will join us this year at the ESC Congress Rare Cardiovascular Diseases Symposium in August/September 2013 in Amsterdam. Last but – by no means – least, please note the mixtures of letters and numbers that complete the “RCD code” closing the titles of Journal publications for the first time in the present issue. The RCD classification, aimed to introduce some systematic clarity and facilitate grouping of experts, is – as you can witness – being implemented!