Ana Maria Daraban, Roxana Enache, L. Predescu, P. Platon, T. Constantinescu, Carina Mihai, I.M. Coman, Carmen Ginghină and Ruxandra Jurcuţ
Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary arterial hypertension (PAH). Because of the similar clinical picture of dyspnea on exertion and signs of right heart failure, PVOD is difficult to distinguish from idiopathic PAH. However, the distinction is mandatory because PVOD has a worse prognosis and, more importantly, the administration of PAH specific therapy (vasodilators) can precipitate severe acute pulmonary oedema. We present a challenging case of PAH in a patient with systemic sclerosis in whom a marked decrease in functional capacity after the initiation of bosentan therapy led to the diagnosis of PVOD. Management of PVOD patients is challenging and referral for lung transplantation should be done at the moment of diagnosis.
Ana Maria Daraban, Adrian Pavel Trifa, Radu Anghel Popp, Diana Botezatu, Marinela Șerban, Valentina Uscatescu, Rodica Talmaci, Daniel Coriu, Carmen Ginghina and Ruxandra Oana Jurcut
Objective: The present case-control study aimed at evaluating the contribution of thrombophilic polymorphisms to acute venous (VTE) as well as arterial thrombotic events (ATE) in a population of young women with few traditional thrombotic factors (CVRF).
Methods: We consecutively enrolled patients under 45 years of age, with less than 3 CVRF, evaluated for VTE or ATE, women and men as a comparator. The control group consisted of healthy young women. A thrombophilia panel and genetic testing for Factor V Leiden (FVL), G20210A Prothrombin and MTHFR polimorphisms were done.
Results: A total of 323 persons were enrolled: 71 women and 121 men with thromboembolic events, and 131 healthy female as controls. Hyperhomocysteinemia was more frequent in ATE (30.4%) than VTE female patients (6.25%), p<0.01. Genetic testing was available in 45 women and 84 men with acute thrombotic events and in all controls. Homozygous FVL was associated with VTE in young women (10.3% vs 0% controls, p<0.01). Prothrombin G20210A polymorphism had the lowest prevalence – 5.4% and only heterozygosity was found. MTHFR C677T heterozygosity showed no significant difference between women patients and controls (62.2 % vs 43.5% respectively, p=0.1). The homozygous status, less frequent (6.6%), was not associated with ATE or VTE. Homozygous MTHFR A1298C was associated with VTE in women (17.2% patients vs 4.5% controls, OR 4.34, p 0.02, CI 1.22-15.3).
Conclusion: In young women with few CVRF, mild hyperhomocysteinemia, homozygosity for FVL and for MTHFR A1298C polymorphisms increase the risk for VTE but not ATE. MTHFR polymorphisms are found with increased frequency in both healthy persons and patients therefore, their significance as an important thrombotic risk modifier remains unclear.
Paula Ionilă, Ruxandra Jurcuţ, Nicoleta Ferariu, Monica Roşca, Monica Chivulescu, Adriana Mursă, Sebastian Militaru, Alin A. Ionescu, Cristina Căldăraru, Ana G. Fruntelată, Silvia F. Goanţă, Simina Crişan, Adina Ionac, Ana-Maria Avram, Attila Frigy, Radu Sascău, Cătălina Arsenescu-Georgescu, Ioan M. Coman, Bogdan A. Popescu, Carmen Ginghină and Eduard Apetrei
Introduction. Hypertrophic cardiomyopathy (HCM) is a disease with increased left ventricular (LV) wall thickness not solely explained by abnormal loading conditions, with great heterogeneity regarding clinical expression and prognosis. The aim of the present study was to collect data on HCM patients from different centres across the country, in order to assess the general characteristics and therapeutic choices in this population.
Methods. Between December 2014 and April 2017, 210 patients from 11 Romanian Cardiology centres were enrolled in the National Registry of HCM. All patients had to fulfil the diagnosis criteria for HCM according to the European Society of Cardiology guidelines. Clinical, electrocardiographic, imaging and therapeutic characteristics were included in a predesigned online file.
Results. Median age at enrolment was 55 ± 15 years with male predominance (60%). 43.6% of the patients had obstructive HCM, 50% non-obstructive HCM, while 6.4% had an apical pattern. Maximal wall thickness was 20.3 ± 4.8 mm (limits 15-37 mm) while LV ejection fraction was 60 ± 8%. Heart failure symptoms dominated the clinical picture, mainly NYHA functional class II (51.4%). Most frequent arrhythmias were atrial fibrillation (28.1%) and non-sustained ventricular tachycardia (19.9%). Mean sudden cardiac death risk score (SCD-RS) was 3.0 ± 2.3%, with 10.4% of the patients with high risk of SCD. However, only 5.7% received an ICD. Patients were mainly treated with beta-blockers (72.9%), diuretics (28.1%) and oral anticoagulants (28.6%). Invasive treatment of LVOT obstruction was performed in a small number of patients: 22 received myomectomy and 13 septal ablation. Cardiac magnetic resonance was reported in only 14 patients (6.6%).
Conclusions. The Romanian registry of HCM illustrates patient characteristics at a national level as well as the gaps in management which need improvement – accessibility to high-end diagnostic tests and invasive methods of treatment.
Fernando Alfonso, Karlen Adamyan, Jean-Yves Artigou, Michael Aschermann, Michael Boehm, Alfonso Buendia, Pao-Hsien Chu, Ariel Cohen, Livio Dei Cas, Mirza Dilic, Anton Doubell, Dario Echeverri, Nuray Enç, Ignacio Ferreira-González, Krzysztof J. Filipiak, Andreas Flammer, Eckart Fleck, Plamen Gatzov, Carmen Ginghina, Lino Goncalves, Habib Haouala, Mahmoud Hassanein, Gerd Heusch, Kurt Huber, Ivan Hulín, Mario Ivanusa, Rungroj Krittayaphong, Chu-Pak Lau, Germanas Marinskis, François Mach, Luiz Felipe Moreira, Tuomo Nieminen, Latifa Oukerraj, Stefan Perings, Luc Pierard, Tatjana Potpara, Walter Reyes-Caorsi, Se-Joong Rim, Olaf Rødevand, Georges Saade, Mikael Sander, Evgeny Shlyakhto, Bilgin Timuralp, Dimitris Tousoulis, Dilek Ural, J.J. Piek, Albert Varga, Thomas F. Lüscher and
The International Committee of Medical Journal Editors (ICMJE) provides recommendations to improve the editorial standards and scientific quality of biomedical journals. These recommendations range from uniform technical requirements to more complex and elusive editorial issues including ethical aspects of the scientific process. Recently, registration of clinical trials, conflicts of interest disclosure, and new criteria for authorship, emphasizing the importance of responsibility and accountability, have been proposed. Last year, a new editorial initiative to foster sharing of clinical trial data was launched. This review discusses this novel initiative with the aim of increasing awareness among readers, investigators, authors and editors belonging to the Editors’ Network of the European Society of Cardiology.