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This analysis investigated the prognostic value of hospitalisation in chronic thromboembolic pulmonary hypertension (CTEPH) using data from the Czech Republic, wherein pulmonary endarterectomy (PEA) was the only targeted treatment option until 2015. Using a landmark method, this analysis quantified the association between a first CTEPH-related hospitalisation event occurring before 3-, 6-, 9-, and 12-month landmark timepoints and subsequent all-cause mortality in adult CTEPH patients diagnosed between 2003 and 2016 in the Czech Republic. Patients were stratified into operable and inoperable, according to PEA eligibility. CTEPH-related hospitalisations were defined as non-elective. Hospitalisations related to CTEPH diagnosis, PEA, balloon pulmonary angioplasty, or clinical trial participation were excluded. Of 436 patients who survived to ≥3 months post diagnosis, 309 were operable, and 127 were inoperable. Sex- and age-adjusted hazard ratios (HRs) showed CTEPH-related hospitalisation was a statistically significant prognostic indicator of mortality at 3, 9, and 12 months in inoperable patients, with an approximately 2-fold increased risk of death in the hospitalisation group (HRs [95% CI] ranging from 1.98 [1.06–3.70] to 2.17 [1.01–4.63]). There was also a trend of worse survival probabilities in the hospitalisation groups for operable patients, with the difference most pronounced at 3 months, with a 76% increased risk of death (adjusted HR [95% CI] 1.76 [1.15–2.68]). This first analysis on the prognostic value of CTEPH-related hospitalisations demonstrates that a first CTEPH-related hospitalisation is prognostic of mortality in CTEPH, particularly for inoperable patients. These patients may benefit from medical and/or interventional therapy.

Aims. The primary objective of the registry was to assess the impact of riociguat on clinical parameters and quality of life in patients with chronic thromboembolic pulmonary hypertension (CTEPH) that was inoperable or persistent/recurrent after pulmonary endarterectomy (PEA). In contrast to randomized pivotal trials, this non-interventional registry evaluated the effectiveness and safety of riociguat in a real-world setting. Methods. Retrospective data were collected from patients' charts as recorded in routine clinical practice from the initiation of riociguat therapy up to approximately 5 months and 1 year after this initiation. Results. In total, 51 patients from a single site were enrolled. After 5 months (mean duration) of riociguat treatment, the following improvements from baseline were observed: change of distance in the 6-minute walking distance (6MWD) (P=0.066); change of score from the quality of life questionnaire (EQ5D-5L) (P=0.020), and overall self-assessment of health status (P=0.001). New York Heart Association (NYHA) class improved in 24.3% of patients. After 11.2 months (mean duration) of riociguat treatment, the following improvements from baseline were observed: change of distance in the 6MWD test (P=0.006), and overall self-assessment of health status (P=0.009). NYHA class improved in 46.4% of patients. Riociguat was well tolerated. In total, 4 patients reported side effects, with hospitalization required in one case and 2 patients who had to discontinue the treatment. Annual survival rate was 89.1%. Conclusion. Riociguat improves functional NYHA class, distance in the 6MWD test and quality of life in a real-world patient population.

This study investigated the epidemiology and survival outcomes of chronic thromboembolic pulmonary hypertension (CTEPH) in the Czech Republic, wherein pulmonary endarterectomy (PEA) surgery was the only targeted treatment option until 2015. This study included all consecutive adults newly diagnosed with CTEPH in the Czech Republic between 2003 and 2016. Incidence/prevalence rates were calculated using general population data extracted from the Institute of Health Information and Statistics of the Czech Republic. Kaplan-Meier estimates of survival from diagnosis until 2018 were calculated. Of a total of 453 patients observed, 236 (52.1%) underwent PEA (median time from diagnosis to PEA: 2.9 months) and 71 (34.1%) had residual pulmonary hypertension (PH) post-PEA. CTEPH incidence rate (95% confidence interval [CI]) between 2006 and 2016 was 4.47 (4.05; 4.91) patients per million (ppm) per year, and the prevalence (95% CI) was 37.43 (33.46; 41.73) ppm in 2016. The rate of CTEPH-related hospitalizations (95% CI) per 100 person-years was 24.4 (22.1; 26.9) for operated patients and 34.2 (30.9; 37.7) for not-operated patients. Median overall survival (95% CI) for all patients from CTEPH diagnosis was 11.2 (9.4; not reached) years. Five-year survival probability (95% CI) was 95.3% (89.9; 97.9) for operated patients without residual PH, 86.3% (75.3; 92.7) for operated patients with residual PH and 61.2% (54.0; 67.6) for not-operated patients. This study reported epidemiological estimates of CTEPH in the Czech Republic consistent with estimates from other national systematic registries; and indicates an unmet medical need in not-operated patients and operated patients with residual PH.

CTEPH is a severe progressive pulmonary vascular disease for which multimodal treatment became recently available. We report the epidemiology and survival of all adult CTEPH patients diagnosed between 2003–2016 in the Czech Republic, where diagnosis and management are centralised in a single expert centre. During the study period, the main treatment option was pulmonary endarterectomy (PEA), the gold standard treatment for CTEPH. Medical treatment was limited to riociguat since 2015, and to participation in clinical trials with other drugs, as off-label use is not allowed. Balloon pulmonary angioplasty (BPA) was introduced in 2016. For incidence/prevalence rates calculation, adult general population data were extracted from the Institute of Health Information and Statistics. Survival from diagnosis was calculated using Kaplan-Meier estimates. Characteristics of patients (N=453) at diagnosis were: median age of 65.2 years, 55% male, 92% NYHA functional class III–IV, and median 6-minute walk test of 336 m. Median time from diagnosis to last follow-up was 6.1 years. Incidence (2006–2016) was 4.47/1,000,000 person-years and prevalence was 37.43 (2016). 236 (52%) patients benefitted from PEA. Of the not operated patients (n=217), 59% (n=129) were technically inoperable (distal disease) and 41% (n=88) were medically inoperable (comorbidities/refusal). 5-year survival probability (95% confidence interval) was: 72.3% (67.8; 76.3) for overall, 82.2% (76.5; 86.5) for operated, 65.3% (56.0; 73.2) for technically inoperable and 55.2% (43.6; 65.4) for medically inoperable. These data indicate an unmet need in inoperable patients, during a time period with limited use of BPA/medical treatment options.

The aim of the study was to analyse in-hospital outcomes and long-term prognostic implications of reduced sodium serum level (S-Na) in the early phase of ST elevation myocardial infarction (STEMI) treated, primarily, with direct percutaneous coronary intervention (dPCI).The study included 218 consecutive patients (144 males, the mean age 64 ± 13 years) with no history of heart failure admitted with acute STEMI. Out of them, 193 (88.5%) patients were treated with dPCI. The mean follow-up period was 39 ± 21 months. Hyponatremia was defined as S-Na value135 mmol/L. A total of 72 (33%) patients reached hyponatremia level; 51(23.4%) of them at admission and 21 (9.6%) later during hospitalization. The hyponatremic patients more frequently presented with reduced left ventricular systolic function, Killip class III or IV and were at increased risk of developing cardiogenic shock compared to patients with normonatremia. Compared to the rest of the population, patients who developed hyponatremia later during hospitalization had higher incidence of acute renal failure; (12 patients/6.1% vs. 5 patients/25.5%, p0.05). The difference in long-term survival between the hyponatremia and normonatremia groups was significant (p = 0.01, log-rank test). The multiple analysis of variance identified decrease of S-Na levels at admission independently associated with total mortality (p = 0.05).Patients who developed hyponatremia in the early phase of STEMI were at higher risk of worse in-hospital clinical outcome. During the long-term follow-up, higher mortality rates were recorded in hyponatremic patients.

Východisko: Rizikova stratifikace a výběr terapeutickeho postupu u pacientů s akutni plicni embolii (PE) jsou ztiženy nedostatkem klinických udajů z velkých prospektivnich randomizovaných studii. Recentně publikovana ceska a evropska doporuceni diagnostiky a lecby akutni PE se lisi v nomenklatuře a v některých aspektech rizikove stratifikace.Cil: Autoři prezentuji hypoteticke srovnani obou doporuceni a dopad jejich odlisnosti na rizikovou stratifikaci a z toho vyplývajici výběr lecby na vlastnim souboru nemocných s akutni a subakutni PE.Metodika: Studie byla navržena jako retrospektivni analýza souboru 209 konsekutivnich nemocných hospitalizovaných na pracovisti autorů v obdobi od ledna 2003 do řijna 2008 pro symptomatickou akutni a subakutni plicni embolii; byli leceni podle v te době dostupných doporuceni. Je zhodnoceno rozděleni do podskupin na zakladě klinicke zavažnosti PE podle ceských doporuceni (masivni, submasivni, mala) a evropských doporuceni (vysoce rizikova, středně rizikova, nizkorizikova).Výsledky: Na zakladě ceských doporuceni byla PE zhodnocena jako masivni u 45 pacientů (21,5 %), jako submasivni u 93 pacientů (44,5 %) a jako mala u 71 pacientů (34 %). Podle evropských doporuceni byla PE hodnocena jako vysoce rizikova u 36 pacientů (17,2 %), jako středně rizikova při hodnoceni přitomnosti dysfunkce/přetiženi prave komory podle echokardiografie a elevace troponinu I u 106 pacientů (51 %), při soucasnem hodnoceni BNP u 112 pacientů (53,6 %). Podle hodnocených kardiomarkerů by do skupiny s nizkým rizikem patřilo 67, resp. 61 nemocných (32 %, resp. 29,2 %). Rozdil v poctu PE klasifikovaných jako masivni vs. vysoce rizikove byl dan skutecnosti, že u deviti pacientů s echokardiografickým průkazem dysfunkce/přetiženi PK byla PE klasifikovana jako masivni jen na zakladě prodělane synkopy při absenci dalsich znamek hemodynamicke nestability; casna mortalita v teto podskupině byla ale nulova. Rozdil v zastoupeni dalsich forem PE podle obou klasifikaci byl dan tim, že u desiti pacientů s malou PE byla plicni embolie klasifikovana jako středně rizikova jen na zakladě zvýsene vstupni hodnoty kardiomarkerů.Zavěr: Nase porovnani v soucasnosti platných dvou doporuceni pro diagnostiku a lecbu PE ukazuje, že rizikova stratifikace a z toho vyplývajici připadný výběr lecby se v obou doporucenich v některých aspektech lisi. Na zakladě nasich udajů se zda, že synkopa u pacientů bez přetrvavajicich projevů hemodynamicke nestability nemusi být znamkou zvýseneho rizika. Budou nutne dalsi analýzy a prospektivni srovnani, aby nedochazelo ani k podceněni, ani přeceněni individualniho rizika nemocných s PE.

Akutni plicni embolie je relativně casta a jeji klinický průběh je variabilni. Autoři clanku podrobně rozebiraji soucasne indikace trombolyticke lecby u tohoto onemocněni a význam rizikove stratifikace, ktera by měla hrat hlavni ulohu v dalsim terapeutickem rozhodovani.

Successfully treated sudden cardiac death due to malignant arrhythmia related to HIV myocarditis in a young male with favorable clinical and virological profile is not described in current literature. HIV myocarditis as a possible cause of malignant ventricular arrhythmia and sudden cardiac death is discussed.

Acute pulmonary embolism (PE) bears a significant burden on health and survival. Rapid and accurate risk stratification and management are of paramount importance to ensure the highest quality of care. This present article summarizes currently available and emerging management strategies for the disease. The authors not only review current evidence regarding early therapy of acute PE, including supportive care, anticoagulation, thrombolysis, surgical and catheter-based treatment, but also the possible role of mechanical circulatory support in PE. The authors also discuss complications related to PE and its management.

Pulmonary embolism is an important clinical entity with considerable mortality despite advances in diagnosis and treatment. In the present article, the authors offer a comprehensive review focused mainly on epidemiology, risk factors, risk stratification, pathophysiological considerations and clinical presentation. Diagnosis based on assessment of clinical likelihood, electrocardiography, chest x-ray, D-dimer levels, markers of myocardial injury and overload, and blood gases is discussed in detail. Special attention is devoted to the clinical use of computed tomography, pulmonary angiography and echocardiography in the setting of pulmonary embolism.

Mitochondrial disorders comprise a heterogeneous group of diseases with multisystem involvement including myocardium. Most cases of mitochondrial cardiomyopathy are associated with myopathy and encephalopathy and are generally present in infancy or childhood. The disease often exhibits a rapid downward course with death frequently occuring within the first year of life. We describe a unique case of hypertrophic cardiomyopathy due to mitochondrial DNA mutation m.3303C >T in the MT-TL1 gene, diagnosed accidentally in a 35-year-old male. The patient initially presented with stroke of assumed cardioembolic origin due to the presence of two interatrial communications associated with mobile aneurysm of the interatrial septum. No other extracardiac manifestations of mitochondrial disorder were observed.