Catheter ablation of atrial fibrillation and atrial tachycardia in patients with pulmonary hypertension: a randomized study.

AIMS: Atrial fibrillation (AF), typical atrial flutter (AFL), and other atrial tachycardias (ATs) are common in patients with pulmonary hypertension. Frequently, several supraventricular arrhythmias are successively observed in individual patients. We investigated the hypothesis of whether more extensive radiofrequency catheter ablation of the bi-atrial arrhythmogenic substrate instead of clinical arrhythmia ablation alone results in superior clinical outcomes in patients with pulmonary arterial hypertension (PH) and supraventricular arrhythmias. METHODS AND RESULTS: Patients with combined post- and pre-capillary or isolated pre-capillary PH and supraventricular arrhythmia indicated to catheter ablation were enrolled in three centres and randomized 1:1 into two parallel treatment arms. Patients underwent either clinical arrhythmia ablation only (Limited ablation group) or clinical arrhythmia plus substrate-based ablation (Extended ablation group). The primary endpoint was arrhythmia recurrence >30 s without antiarrhythmic drugs after the 3-month blanking period. A total of 77 patients (mean age 67 ± 10 years; 41 males) were enrolled. The presumable clinical arrhythmia was AF in 38 and AT in 36 patients, including typical AFL in 23 patients. During the median follow-up period of 13 (interquartile range: 12; 19) months, the primary endpoint occurred in 15 patients (42%) vs. 17 patients (45%) in the Extended vs. Limited ablation group (hazard ratio: 0.97, 95% confidence interval: 0.49-2.0). There was no excess of procedural complications and clinical follow-up events including an all-cause death in the Extended ablation group. CONCLUSION: Extensive ablation, compared with a limited approach, was not beneficial in terms of arrhythmia recurrence in patients with AF/AT and PH. CLINICAL TRIALS REGISTRATION: ClinicalTrials.gov; NCT04053361.

Benefits from catheter ablation in patients with pulmonary hypertension: Recent advances.

Supraventricular tachycardia (SVT) is a frequent complication of pulmonary hypertension (PH). The most prevalent SVTs are atrial fibrillation (AF) and typical atrial flutter (AFL), followed by focal and macroreentrant atrial tachycardia (AT) and nodal arrhythmia (AV nodal reentry tachycardia or AV reentry tachycardia). SVT is frequently associated with functional deterioration and right ventricular failure in PH patients. According to some data, reestablishing sinus rhythm is associated with clinical improvement. Catheter ablation of typical AFL, nodal tachyarrhythmias, or other less complex focal ATs have been shown to be feasible, acutely effective, and safe in patients with PH. However, the long-term clinical outcome is modified by the recurrence of index arrhythmia and the onset of a new SVT. Due to right atrial dilatation, technical issues can arise when ablation is carried out. The role of catheter ablation in patients with AF or more complex AT is even less effective. The results mirror the success rate in the general AF population with non-paroxysmal AF. However, the data is limited, and electrophysiological procedures are also more often complicated by specific adverse events in a severely frail population. Despite these limitations, catheter ablation is the treatment of choice in less complex SVT, but the indications for AF ablation must be more individualized.

Pulmonary embolism, part I: Epidemiology, risk factors and risk stratification, pathophysiology, clinical presentation, diagnosis and nonthrombotic pulmonary embolism.

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.

Pulmonary embolism, part II: Management.

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.

Correction: Jansa et al. Hospitalisation Is Prognostic of Survival in Chronic Thromboembolic Pulmonary Hypertension. J. Clin. Med. 2022, 11, 6189.

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Hypertrophic cardiomyopathy due to the mitochondrial DNA mutation m.3303C>T diagnosed in an adult male.

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.

Epidemiology of chronic thromboembolic pulmonary hypertension (CTEPH) in the Czech Republic.

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.

Hospitalisation Is Prognostic of Survival in Chronic Thromboembolic Pulmonary Hypertension.

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.

Long-term prognostic impact of hyponatremia in the ST-elevation myocardial infarction.

AIMS: 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). METHODS AND RESULTS: 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 value < 135 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%, p < 0.05). 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). CONCLUSION: 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.

The impact of riociguat on clinical parameters and quality of life in patients with chronic thromboembolic pulmonary hypertension - results of a retrospective clinical registry.

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.

Balloon Pulmonary Angioplasty in Patients with Chronic Thromboembolic Pulmonary Hypertension: Impact on Clinical and Hemodynamic Parameters, Quality of Life and Risk Profile.

Balloon pulmonary angioplasty (BPA) is a novel treatment option for patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are not eligible for pulmonary endarterectomy (PEA) or suffer from persistent pulmonary hypertension after PEA. The aim of this study was to evaluate the real-life efficacy and safety of BPA in a consecutive group of patients who were diagnosed and treated in the national referral center for CTEPH in the Czech Republic. Here we report data from 160 BPA procedures performed in 64 patients. Efficacy analysis was performed in the subgroup of 25 patients who completed BPA series. Significant improvements were observed in New York Heart Association functional class (4% to 79% in I/II, p < 0.001), 6 min walking test distance (+54.3 m, p < 0.001), risk profile (15.8% to 68.5% with presence of 2/3 low risk criteria, p < 0.001), pulmonary artery mean pressure (-18%, p < 0.001), pulmonary vascular resistance (-32%, p < 0.001), stroke volume (+17%, p = 0.011) and quality of life (+37% in assessment of overall health status by a patient, p < 0.001). We observed 1 fatal periprocedural complication (1.6% of all 64 patients) and 19 BPA-related non-fatal complications (11.9% of all 160 interventions) that predominantly included hemoptysis (10.0% of all sessions). Overall survival at 12 months was 94.6%.

Serum cardiac markers response to biphasic and monophasic electrical cardioversion for supraventricular tachyarrhythmia--a randomised study.

BACKGROUND: Electrical cardioversion in patients with various types of supraventricular tachyarrhythmia (SVT) may induce serum cardiac markers elevation. Only a few studies have evaluated the impact of the type of shock waveform on electrical myocardial injury. The aim of our study was to compare the response of serum cardiac markers to biphasic and monophasic cardioversion for SVT. METHODS: One hundred and forty one patients with various SVTs referred for electrical cardioversion were randomised to monophasic (MP) or biphasic (BP) cardioversion. Serum levels of creatine kinase (CK), MB fraction of CK (CK-MB), myoglobin and troponin I were analysed before cardioversion and 254+/-58 min after the procedure. RESULTS: Average age of the patients was 67.9+/-11.3 years, 71 underwent BP and 70 MP cardioversion. In MP group, cumulative energy (CE)>150J was associated with significant elevation of CK and myoglobin levels after cardioversion (1.52+/-3.81 microkat/l and 187+/-433 microg/l), while CE<150J was not (-0.04+/-0.34 and 4+/-11, p<0.05). In BP group, CE>150J was associated with significant but smaller CK elevation (0.27+/-1.09 microkat/l, p<0.05) and comparable myoglobin elevation (80.7+/-21.4 microg/l, p<0.05). CE>150J was the only independent positive predictor for CK and myoglobin elevation in both groups. No significant changes in CK-MB and Troponin I levels after cardioversion were identified. CONCLUSIONS: According to our study, electrical cardioversion for SVTs is not associated with biochemical signs of myocardial injury. Application of CE>150J can be followed by CK and myoglobin elevation most likely due to skeletal muscle damage. This reaction is more pronounced in MP than in BP cardioversion.

Successfully resuscitated sudden cardiac death in a young homosexual male with HIV myocarditis.

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.