Screening for pulmonary arterial hypertension in patients with systemic sclerosis in the era of new pulmonary arterial hypertension definitions.

OBJECTIVES: This study compares the performance of three composite pulmonary arterial hypertension (PAH) screening tools in a real-life SSc cohort, according to both the previous 2015 ESC/ERS guideline and the recent 2022 ESC/ERS guideline haemodynamic criteria. METHODS: Consecutive SSc patients without a previous diagnosis of pulmonary hypertension (PH) were screened for PAH using the European Society of Cardiology/European Respiratory Society (ESC/ERS), DETECT, and Australian Scleroderma Interest Group (ASIG) algorithms. Right heart catheterisation (RHC) referral performances for PAH were compared according to the 2022 ESC/ERS PAH criteria. RESULTS: Thirty-five of the 81 patients required RHC; 15 (18.5%) according to ESC/ERS, 27 (33.3%) according to DETECT, and 25 (31%) according to ASIG. The final diagnoses were no-PH in 17 patients, WHO group 1 PH (PAH) in 8 patients, WHO group 2 PH in 8 patients, and WHO group 3 PH in 2 patients. When the hemodynamic criteria of the previous ESC/ERS guideline were applied, only one patient was diagnosed with PAH. The sensitivities of the algorithms for the diagnosis of PAH were 62.5% for ESC/ERS, 75% for DETECT, 87.5% for ASIG according to the 2022 ESC/ERS guideline definition, and 100% for all according to the previous ESC/ERS guideline. CONCLUSIONS: With the recent criteria, PAH diagnosis in patients with SSc increased by 1.8-fold. Current algorithms for screening PAH are less sensitive with these revised criteria. Although the ASIG algorithm seems more sensitive, it can still miss the diagnosis. The multimodal/algorithmic approach seems to be the best option for predicting PAH.

Role of Optical Coherence Tomography in Vasculitis-Associated Pulmonary Hypertension and Chronic Thromboembolic Pulmonary Hypertension.

BACKGROUND: Identifying and understanding the microstructural changes within the wall of the pulmonary artery (PA) is crucial for elucidating disease mechanisms and guiding treatment strategies. We assessed the utility of optical coherence tomography (OCT) in identifying such changes within segmental/subsegmental PAs and compared the morphological variations in WHO group 4 pulmonary hypertension associated with Behcet Disease (BD), Takayasu arteritis (TA) and chronic thromboembolic pulmonary hypertension (CTEPH). Idiopathic pulmonary arterial hypertension (IPAH) patients served as controls.Methods and Results: A total of 197 cross-sectional images were analyzed from 20 consecutive patients. BD patients exhibited lower %wall area and mean wall thickness (MWT) compared with CTEPH, TA and, IPAH patients. TA patients showed a notably higher %wall area, which was significant in IPAH and BD patients. Variations in %wall area measurements were observed across distinct cross-sectional segments of the PA within individual patients (22% in CTEPH, 19% in BD, 16% in TA, 23% in IPAH patients). Intravascular webs, bands, and thrombi were observed in BD and CTEPH patients. OCT provided clear delineation of vascular wall calcifications and adventitial vasa vasorum. No procedure-related complications were observed. CONCLUSIONS: PA involvement differs among the various etiologies of PH, with the PA being heterogeneously affected. OCT offers promise in elucidating microstructural vascular wall changes and providing insights into disease mechanisms and treatment effects.

The clinical significance of premature atrial contractions: How frequent should they become predictive of new-onset atrial fibrillation.

BACKGROUND: Although previous studies reported frequent premature atrial contractions(fPACs) increased the risk of adverse cardiovascular outcomes, especially atrial fibrillation(AF), there is a substantial inconsistency between reports concerning the definition of fPAC. In this study, we aimed to investigate the relationship between fPAC and cardiovascular outcomes, especially AF. We further searched for a cutoff value of fPAC for prediction of AF. METHODS: We retrospectively analyzed the ambulatory 24-hr Holter monitoring records and 392 patients included. Frequent PAC was defined as more than 720 PAC/24 hr as used for frequent ventricular premature beats. Patients' baseline characteristics, echocardiographic variables and medical history were recorded. RESULTS: There were 189 patients with fPAC and 203 patients without fPAC. Patients with fPAC had more comorbidities in terms of hypertension, diabetes mellitus, coronary artery disease and congestive heart failure. CHA2DS2-VaSc was higher in patients with fPAC. Mean follow-up duration was 31 months, and the number of patients with new-onset AF during follow-up was significantly higher in fPAC group (22% vs. 5%, p < .001). fPAC was significantly and independently associated with new-onset AF and predicted AF with a cutoff value of 3,459 PAC/24 hr, and the risk of AF was 11-fold higher than those with <3,000 PAC/24 hr. In addition, an increased CHA2DS2-VaSc score was also associated with new-onset atrial fibrillation. CONCLUSION: In our study, we have demonstrated that fPAC is significantly associated with new-onset AF, and this association is the strongest among those patients who have more than 3,000 PAC in 24 hr.

Circulating miR-21 and eNOS in subclinical atherosclerosis in patients with hypertension.

OBJECTIVES: The aim of this study is to evaluate the relationship of miR-21, nitric oxide (NOx) and endothelial nitric oxide synthase (eNOS) with subclinical atherosclerosis in carotid arteries by measuring carotid intima media thickness (CIMT) in patients with hypertension and healthy controls. DESIGN AND METHODS: A total of 28 hypertensive and 28 healthy controls were enrolled. MiR-21 expression was analyzed by quantitative reverse transcription-PCR and NOx, and eNOS levels were measured by ELISA assay. CIMT was evaluated by ultrasonography and CIMT ≥ 0.8 mm was accepted as increased CIMT (iCIMT). RESULTS: C-reactive protein (CRP) level, plasma miR-21 expression level and CIMT were found to be significantly higher in the hypertension group when compared to the control group (p = 0.009, p = 0.002 and p < 0.001, respectively). NOx and eNOS levels were significantly lower in the hypertension group compared to the control group (p < 0.001, both). MiR-21 level was positively correlated with the clinical systolic blood pressure, clinical diastolic blood pressure, CRP and CIMT. MiR-21 was also negatively correlated with NOx and eNOS. Eighteen patients with hypertension had iCIMT. MiR-21 and CRP levels were significantly higher (p < 0.001 and p = 0.001), whereas NOx and eNOS levels were significantly lower in patients with iCIMT (p < 0.001, both). CONCLUSION: The decreased levels of NOx and eNOS found in this study indicate the co-existence of endothelial dysfunction and hypertension once more. In the absence of microalbuminuria, the increased miR-21 expression in patients with iCIMT made us conclude that this miRNA might be involved in the early stages of atherosclerotic process in hypertensive patients.

Unilateral absence of the left pulmonary artery with patent ductus arteriosus and interrupted inferior vena cava.

Unilateral absence of the pulmonary artery and interrupted inferior vena cava are rare congenital disorders. The clinical presentation is variable, and many patients can be asymptomatic for many years and even throughout their lives. We report the case of a 44-year-old female patient with a history of hemoptysis. She was referred to our clinic with a diagnosis of pulmonary artery agenesis. Computed tomography revealed absence of the left main pulmonary artery and long-tract patent ductus arteriosus (PDA). Blood supply to the left lung was provided by major aortopulmonary collateral arteries (MAPCAs). Right heart catheterization through the right femoral vein was problematic, as the catheter could not be negotiated from the inferior vena cava to the right atrium. We revealed the interrupted inferior vena cava, which continued as a dilated azygos vein to the superior vena cava. The magnitude of the shunt flow from the PDA was too small, and the calculated shunt fraction was not significant. Hemoptysis was possibly due to MAPCAs. However, as the patient had no active bleeding when she was referred, the exact source of the hemoptysis could not be identified.

Treatment of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review and meta-analysis.

The evidence for the treatment of connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) mostly depends on subgroup or post hoc analysis of randomized controlled trials (RCTs). Thus, we performed a meta-analysis of RCTs that reported outcomes for CTD-PAH. PubMed and EMBASE were searched for CTD-PAH treatment. The selected outcomes were functional class (FC) change, survival rates, 6-min walk distance (6-MWD), clinical worsening (CW), N-terminal prohormone BNP (NT-proBNP), pulmonary vascular resistance (PVR), mean pulmonary arterial pressure (mPAP), right atrial pressure (RAP), and cardiac index (CI). The meta-analysis was conducted according to the PRISMA guidelines and registered in PROSPERO (CRD42020153560). Twelve RCTs conducted with 1837 patients were included. The diagnoses were systemic sclerosis in 59%, SLE in 20%, and other CTDs in 21%. The pharmacological interventions were epoprostenol, treprostinil, sildenafil, tadalafil, bosentan, macitentan, ambrisentan, riociguat, and selexipag. There was a significant difference between interventions and placebo in FC, 6MWD, CW, PVR, RAP, and CI that favored intervention. Our analysis showed a 39% reduction in the CW risk with PAH treatment. The short-term survival rates and mean serum NT-proBNP changes were similar between the study and control groups. Treatment for CTD-PAH had favorable effects on clinical and hemodynamic outcomes but not on survival and NT-proBNP levels. Different from the previous meta-analyses that focused on 6-MWD, time to clinical worsening, and CW as outcomes, this meta-analysis additionally reports the pooled analysis of change in FC, hemodynamic measurements (RAP, PVR, CI), and NT-proBNP, some of which have prognostic value for PAH.

Ascites does not accompany pleural effusion developing under dasatinib therapy in patients with CML-CP.

Objectives: Pleural effusion (PE) is the most frequent pulmonary complication of dasatinib, a tyrosine kinase inhibitor (TKI). Concurrent pericardial effusions have been reported in about one-third of the cases. In this study, we aimed to investigate ascites generation in chronic-phase chronic myeloid leukemia (CML-CP) patients developing PE under dasatinib. Methods: We conducted a cross-sectional study to evaluate whether pericardial effusion and ascites accompany PE in CML-CP patients treated with dasatinib. For this purpose, consecutive patients with CML-CP who developed PE under dasatinib therapy have been evaluated with chest X-ray, transthoracic echocardiography, and abdominal ultrasonography. Results: There were seven patients, and the median age was 50 years (range, 31-73 years). Most of patients were male (n=5). All patients received imatinib as first-line TKI. Six patients received dasatinib following imatinib failure in second line. The median duration from dasatinib initiation to PE generation was 58 months (range, 8-135 months). Consequently, four patients had grade 1 pericardial effusion, and no patient had ascites. Conclusions: In our small study, dasatinib-related PE was associated with low-grade pericardial effusion but no ascites. There are hypothetical explanations of this phenomenon including the simultaneous activation/inhibition of kinases; however, more research needs to be performed on this topic.

Post-COVID-19 vaccination inflammatory syndrome: A case report.

A previously healthy 24-year-old male patient was referred to our clinic with bilateral lower extremity pain and dark urine, which were developed 2 weeks after receiving the second dose of the BNT162b2 vaccine against severe acute respiratory coronavirus 2. Laboratory tests indicated rhabdomyolysis. Lower extremity magnetic resonance imaging was compatible with myositis. Myositis-related antibodies were negative. Biopsy taken from gastrocnemius muscle revealed muscle necrosis and striking expression of major histocompatibility complex class I antigen. He was successfully treated, and his complaints were resolved. One week later at follow-up, he reported new-onset exertional dyspnoea with palpitations. ST-segment depressions were spotted on electrocardiography. Troponin T was found elevated as 0.595 ng/ml (normal <0.014 ng/ml). Echocardiography showed a hypokinetic left ventricle with an ejection fraction of 40% and pericardial effusion of 2 mm. An appropriate treatment plan was formulated for the diagnosis of myocarditis, eventually, the patient recovered within 10 days. The BNT162b2 messenger ribonucleic acid (mRNA) vaccine was felt to cause the aforementioned condition since no other aetiology could be identified. Although it is known that BNT162b2 may induce myocarditis, myositis concomitant myocarditis appears to be a very rare adverse effect of this vaccine.

Risk Assessment Tool Implementation in Congenital Heart Disease-Associated Pulmonary Arterial Hypertension.

BACKGROUND: Risk assessment is recommended for patients with congenital heart disease-associated pulmonary arterial hypertension. This study aims to compare an abbreviated version of the risk assessment strategy, noninvasive French model, and an abridged version of the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management 2.0 risk score calculator, Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2. METHODS: We enrolled a mixed prevalent and incident cohort of patients with congenital heart disease-associated pulmonary arterial hypertension (n = 126). Noninvasive French model comprising World Health Organization functional class, 6-minute walk distance, and N-terminal pro-hormone of brain natriuretic peptide or brain natriuretic peptide was used. Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2 includes functional class, systolic blood pressure, heart rate, 6-minute walk distance, brain natriuretic peptide/N-terminal pro-hormone of brain natriuretic peptide, and estimated glomerular filtration rate. RESULTS: The mean age was 32.17 ± 16.3 years. The mean follow-up was 99.41 ± 58.2 months. Thirty-two patients died during follow-up period. Most patients were Eisenmenger syndrome (31%) and simple defects (29.4%). Most patients received monotherapy (76.2%). Most patients were World Health Organization functional class I-II (66.6%). Both models effectively identified risk in our cohort (P =.0001). Patients achieving 2 or 3 noninva-sive low-risk criteria or low-risk category by Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2 at follow-up had a significantly reduced risk of death. Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2 approximates noninvasive French model at discriminating among patients based on c-index. Age, high risk by Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2, and the presence of 2 or 3 low-risk criteria by noninvasive French model emerged as an independent predictors of mortality (multivariate hazard ratio: 1.031, 95% CI: 1.005-1.058, P =.02; hazard ratio: 4.258, CI: 1.143-15.860, P =.031; hazard ratio: 0.095, CI: 0.013-0.672, P =.018, respectively). CONCLUSIONS: Both abbreviated risk assessment tools may provide a simplified and robust method of risk assessment for congenital heart disease-associated pulmonary arterial hypertension. Patients not achieving low risk at follow-up may benefit from aggressive use of available therapies.

Pulmonary Hypertension Due to High-Output Heart Failure: Hereditary Hemorrhagic Telangiectasia.

Pulmonary hypertension (PH) is a complex disorder that should be managed with a multidisciplinary approach. Although most of the underlying causes of left heart disease can be easily diagnosed with cardiac imaging, some pathologies might necessitate careful investigation to go beyond the obvious. High-output heart failure (HF) due to arteriovenous malformation (AVMs) is an unnoticeable cause for HF and PH. Patients with hepatic AVMs should always be carefully evaluated with regard to hereditary hemorrhagic telangiectasia (HHT) since they can have multiple signs related to the other systems without any symptoms. In this case report, we discussed a patient who was initially diagnosed as PH associated with HF with preserved ejection fraction but eventually was found to have PH associated with high-output HF due to hereditary hemorrhagic telangiectasia (HHT, or Osler Weber Rendu syndrome) after detailed evaluation.

Six-Minute Walk Test: How to do Guide for Performing a Reliable Test, Interpretation, and Prognostication in Pulmonary Hypertension.

Six-minute walk test (6MWT) is the most widely used exercise capacity measurement worldwide in patients with pulmonary hypertension (PH). Although cardiopulmonary exercise testing (CPET) is the gold standard for the assessment of exercise capacity in cardiovascular diseases; the limited accessibility of the device, the need for experience in interpreting the results, and the difficulties in performing CPET in advanced PH have aroused the interest in the application of easier methods for the measurement of exercise capacity. Since then, accumulated data proved that; 6-minutes walking distance (6MWD) can be used to determine exercise capacity and is highly correlated with maximum oxygen consumption (peak VO2) detected by CPET in patients with heart failure and/or PH. Moreover, 6MWT is very easy and practical to apply in all PH subgroups. This review is focused on the application of a reliable 6MWT and the interpretation of the results in patients with PH.

A 19-Year-Old Pregnant Woman With Pulmonary Hypertension With Progressive Dyspnea.

CASE PRESENTATION: A 19-year-old pregnant woman at week 32 of gestation was referred to our clinic with progressive shortness of breath for the further evaluation and treatment of high-risk pregnancy. Her complaints had been existing since her childhood. Two years prior to her admission, she had been diagnosed with heart failure with preserved ejection fraction due to cardiomyopathy and associated pulmonary hypertension. The patient had no family history of any cardiac disease. She had never smoked or drunk alcohol. Her clinical condition had deteriorated progressively with the pregnancy.

Assessment of atrial electromechanical delay and P wave dispersion in patients with chronic obstructive pulmonary disease.

OBJECTIVE: Chronic obstructive pulmonary disease (COPD) is associated with atrial fibrillation (AF) and reduced forced expiratory volume (FEV1) is an independent predictor for new onset AF. The aims of this study were (1) to analyze the atrial electromechanical delay (AEMD) and P wave dispersion which are two predictors of AF development in patients with COPD and without any cardiovascular disease, and (2) to assess the relationship of those with pulmonary functions as quantified by FEV1 measurements. METHODS: The study included 41 patients with COPD (33 male; mean age: 51 years) and 32 healthy controls. P wave dispersion was calculated as the difference between the maximum and minimum P wave duration in a 12-lead surface electrocardiography (ECG) recording. AEMD, defined as the time interval from the P wave onset on the ECG to the initiation of the late diastolic (Am) wave using a tissue Doppler examination, was measured from the lateral mitral annulus (LAEMD), septal annulus (SAEMD), and tricuspid lateral annulus (TAEMD). RESULTS: P wave dispersion was significantly longer in the COPD group than those in the controls (76±19 ms vs. 45±10 ms; p<0.001). All of the AEMD measurements demonstrated significant prolongation in patients with COPD (LAEMD: 74±9 ms vs. 64±11 ms; SAEMD: 66±10 ms vs. 57±12 ms; and TAEMD: 65±9 ms vs. 46±7 ms; p<0.001 for all). The only correlation with FEV1 was observed in the TAEMD values of the COPD group (rs: -401; p<0.009). CONCLUSION: Both P wave dispersion and AEMD parameters were significantly longer in COPD patients without any established structural or functional cardiac abnormalities, indicating an increased tendency for AF development, beginning from the initial stages of the disease.

The Prevalence of Fabry Disease Among Turkish Patients with Non-Obstructive Hypertrophic Cardiomyopathy: Insights from a Screening Study

Aims: Fabry disease is an X-linked lysosomal storage disorder due to a deficiency of the α-galactosidase A enzyme. Cardiac involvement is present in over 60% of adult cases of Fabry disease. Hypertrophic cardiomyopathy without left ventricular outflow tract obstruction is the most common phenotype. The aim of the study was to screen adult patients with hypertrophic cardiomyopathy without left ventricular outflow tract. Methods: A total of 80 patients between the ages of 18 and 65 years old, were referred to a tertiary center for trans-thoracic echocardiography for various clinical indications. They were investigated for the presence of idiopathic left ventricular hypertrophy without resting or dynamic left ventricular outflow tract obstruction. Plasma α-galactosidase A enzyme activity and α-galactosidase GLA gene mutations were investigated. Results: The mean age was 41.5±12.7 years and 66.25% of patients were males. The mean echocardiographic parameters were as follows: left ventricular ejection fraction 60.7±7.4%, interventricular septum thickness 18.2±4.4 mm, left ventricular posterior wall 13.5±2.1 mm, left ventricular end-diastolic diameter 47.4±6.2 mm, left ventricular end-systolic diameter 27.8±6.5 mm, and left ventricular mass index 171.05±48.5 g/m². Hemizygous mutations associated with Fabry disease were detected in two male patients (2.50% of the screened population): NM_000169.2:c.334C>T(p.Arg112Cys), NM_000169.2:c.902G>A(p.Arg301Gln). Conclusion: Fabry disease should be considered in the differential diagnosis in a highly selected patient population with unexplained left ventricular hypertrophy. The cardiologist may play an important role in the screening and diagnosis of the disease.

Risk assessment and survival of patients with pulmonary hypertension: Multicenter experience in Turkey.

OBJECTIVE: Risk stratification continues to evolve in pulmonary arterial hypertension (PAH). Our aim was to further confirm the risk assessment strategy in our cohort and to determine the most reliable model. METHODS: We enrolled incident patients with idiopathic PAH (IPAH), heritable, drug-induced, congenital heart disease (CHD), connective tissue diseases (CTD) subsets, and chronic thromboembolic pulmonary hypertension (CTEPH) from January 2008 to February 2018. Data from the baseline and subsequent follow-ups within 1 year of diagnosis were included. An abbreviated risk assessment strategy was applied using the following variables: functional class (FC), 6-minute walk distance (6 MWD), N-terminal pro-brain natriuretic peptide (NT-proBNP) or BNP, right atrial (RA) area, pericardial effusion, the mean RA pressure, cardiac index, and mixed venous oxygen saturation. Three different methods were applied to categorize patients. RESULTS: A total of 189 subjects (46+-17 years, 23% male) were included. Sixty-one patients had died. The survival differed significantly between the risk groups both at diagnosis and during the follow-up. Patients with a low-risk profile had a better survival rate. An abbreviated risk assessment tool predicted mortality at early follow-up in the entire group and CHD, CTD subsets, and CTEPH, separately. An overall mortality among risk categories was significantly different according to each categorization method. The most reliable model comprised FC, 6 MWD, NT pro-BNP/BNP, and the RA area at the follow-up. CONCLUSION: The abbreviated risk assessment tool may be valid for the PAH subsets and CTEPH. Echocardiographic variables do matter. A model comprising FC, 6 MWD, NT pro-BNP/BNP, and the RA area at the follow-up could be useful for better prognostication.

Thrombocytopenia associated with antithrombotic therapy in patients with cardiovascular diseases: diagnosis and treatment.

Agents with antiplatelet and anticoagulant activity have been proved to be effective in reducing the incidence of complications following acute coronary syndrome, percutaneous coronary intervention, and cardiopulmonary bypass. However, these agents, including heparin, glycoprotein IIb/IIIa receptor inhibitors, and thienopyridines, are associated with increased risk of bleeding and thrombocytopenia and have been administered together with increasing frequency in a variety of cardiovascular settings. Therefore, clinicians must be familiar with the safety and rational use of these potent antithrombotic agents. Clinical features of thrombocytopenia range from bleeding to thrombosis, even death, and therapy is very different depending on the underlying cause. Additionally, patients may sometimes need urgent intervention or surgery. Thus, it is essential to quickly discriminate the etiology and start appropriate therapy. This review highlights the pathogenesis, clinical and laboratory manifestation, differential diagnosis, and treatment of antithrombotic drug-induced thrombocytopenia in cardiovascular diseases.

Renin-angiotensin-aldosterone system blockers and cardiovascular outcomes: a meta-analysis of randomized clinical trials.

OBJECTIVE: Hypertension is the most prevalent modifiable risk factor for cardiovascular (CV) and cerebrovascular morbidity and mortality. This study aimed to assess the effects of renin-angiotensin-aldosterone system (RAAS) blockade on CV outcomes. METHODS: This study was designed according to the Preferred Reporting Items for Systemic reviews and Meta-Analyses statement. Databases were searched for articles published as of December 2014. Two sets of studies were selected. One set included randomized clinical trials comparing RAAS blocker (angiotensin II receptor blocker [ARB] or angiotensin-converting enzyme inhibitor [ACEI]) with placebo or active treatment. Second set included head-to-head randomized clinical trials comparing an ARB with an ACEI. Studies in both sets had reported any CV outcome parameter or death, i.e., all-cause mortality, CV mortality, emergence of CV events, myocardial infarction, cerebrovascular event, stroke, heart failure, and hospitalization for heart failure. RESULTS: Fifty-four pairwise comparisons of 51 trials with 277,609 patients were included. Statistically significant differences in favor of RAAS blockers vs non-RAAS blockers (risk ratio [RR] ranging from 0.805 to 0.967) were observed in terms of most CV outcomes, including all-cause mortality, CV mortality, CV events, myocardial infarction, heart failure and stroke. ARBs and ACEIs were found to be completely comparable (RR ranging from 0.923 to 1.090, all non-significant). CONCLUSION: RAAS blockers are superior to medications other than RAAS blockers with respect to impact on CV outcomes in patients with hypertension. ARBs and ACEIs are comparable in terms of these outcomes.

Preliminary results from a nationwide adult cardiology perspective for pulmonary hypertension: RegiStry on clInical outcoMe and sUrvival in pulmonaRy hypertension Groups (SIMURG).

OBJECTIVE: The present study was designed to evaluate the characteristics of pulmonary hypertension (PH) and adult cardiology practice patterns for PH in our country. METHODS: We evaluated preliminary survey data of 1501 patients with PH (females, 69%; age, 44.8±5.45) from 20 adult cardiology centers (AdCCs). RESULTS: The average experience of AdCCs in diagnosing and treating patients with PH was 8.5±3.7 years. Pulmonary arterial hypertension (PAH) was the most frequent group (69%) followed by group 4 PH (19%), group 3 PH (8%), and combined pre- and post-capillary PH (4%). PAH associated with congenital heart disease (APAH-CHD) was the most frequent subgroup (47%) of PAH. Most of the patients' functional class (FC) at the time of diagnosis was III. The right heart catheterization (RHC) rate was 11.9±11.6 per month. Most frequently used vasoreactivity agent was intravenous adenosine (60%). All patients under targeted treatments were periodically for FC, six-minute walking test, and echo measures at 3-month intervals. AdCCs repeated RHC in case of clinical worsening (CW). The annual rate of hospitalization was 14.9±19.5. In-hospital use of intravenous iloprost reported from 16 AdCCs in CWs. Bosentan and ambrisentan, as monotreatment or combination treatment (CT), were noted in 845 and 28 patients, respectively, and inhaled iloprost, subcutaneous treprostinil, and intravenous epoprostenol were noted in 283, 30, and four patients, respectively. Bosentan was the first agent used for CT in all AdCCs and iloprost was the second. Routine use of antiaggregant, anticoagulant, and pneumococcal and influenza prophylaxis were restricted in only two AdCCs. CONCLUSION: Our nationwide data illustrate the current status of PH regarding clinical characteristics and practice patterns.

The Trends in Utilizing Nonvitamin K Antagonist Oral Anticoagulants in Patients With Nonvalvular Atrial Fibrillation: A Real-Life Experience.

Dabigatran and rivaroxaban are novel nonvitamin K antagonist oral anticoagulants (NOACs) approved for thromboprophylaxis in atrial fibrillation (AF). In Turkey, like other countries, the efficacy of translation of the clinical trial results and current guideline recommendations into daily clinical practice is yet to be discovered. Using data from medical records of three tertiary care cardiology centers, we identified patients with nonvalvular AF on dabigatran or rivaroxaban treatment. Baseline characteristics and utilization trends were compared between dabigatran and rivaroxaban groups. Secondarily, clinical events including ischemic stroke and/or transient ischemic attack, systemic embolism, and bleeding were evaluated. Among 294 patients with AF included, dabigatran was utilized in 177 (60.2%) and rivaroxaban in 117 (39.8%). Overall, 76% of patients had received long-term warfarin therapy. The use of 110 mg twice a day (55.4%) was the prevailing strategy in dabigatran group, whereas in rivaroxaban group 20 mg every day (67.5%) was the preferred option. Of the patients, 37.3% had severe valvular disease in which mitral regurgitation was the predominant valve abnormality. Scores of CHADS2, CHA2DS2VASc, and HAS-BLED were similar in both the groups. Of the patients, 24% in dabigatran group and 13.7% in rivaroxaban group were prescribed the lower dose inappropriately. The two NOACs did not differ significantly in terms of clinical events. The results of this study indicate that in daily practice, the physicians' behavior in utilizing the NOACs is shaped by the clinical trials and the guideline recommendations. On the other hand, in dose selection, this adherence is not of high quality.

Renin-angiotensin system blockade in the treatment of heart failure and the role of valsartan in this treatment.

Heart failure which occurs due to various causes including primarily coronary artery diseases and hypertension is a syndrome with complex physiopathology and clinic that can impair patients' quality of life or lead to death. However, it is well known that the activation of renin-angiotensin system (RAS) has an important role in the physiopathology of heart failure with reduced ejection fraction. Therefore, suppression of this system for achieving a gain in the treatment of the disease has been among prominent concerns. In this review, the place of RAS suppressive drugs and valsartan, which is an angiotensin receptor blocker, in heart failure will be examined.