Diagnosis and Treatment Patterns of Chronic Thromboembolic Pulmonary Hypertension in Russia, Kazakhstan, Turkey, Lebanon, and Saudi Arabia: A Registry Study.

BACKGROUND: Patients with chronic thromboembolic pulmonary hypertension (CTEPH) in countries with limited resources have, to date, been poorly represented in registries. OBJECTIVE: This work assesses the epidemiology, diagnosis, hemodynamic and functional parameters, and treatment of CTEPH in Russia, Kazakhstan, Turkey, Lebanon, and Saudi Arabia. METHODS: A prospective, cohort, phase IV, observational registry with 3-year follow-up (n = 212) in patients aged ≥ 18 years diagnosed with CTEPH was created. Clinical, hemodynamic, and functional parameters were obtained at an initial visit, follow-up visits, and a final visit at the end of 3 years' observation or end of follow-up. Data were recorded on electronic case report forms. Parameters evaluated included 6-minute walking distance (6MWD), use of pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA), pulmonary hypertension (PH)-targeted therapy, and survival. All statistical analyses were exploratory and descriptive, and were performed in the overall population. RESULTS: The most common symptoms were typical of those expected for CTEPH. Almost 90% of patients underwent right heart catheterization at diagnosis or initial study visit. In total, 66 patients (31%) underwent PEA before the initial visit; 95 patients (45%) were considered operable, 115 (54%) were inoperable, and two (1%) had no operability data. Only 26 patients (12%) had been assessed for BPA at their initial visit. PH-targeted therapy was documented at diagnosis for 77 patients (36%), most commonly a phosphodiesterase type 5 inhibitor (23%). Use of PH-targeted therapy increased to 142 patients (67%) at the initial visit, remaining similar after 3 years. Use of riociguat increased from 6% of patients at diagnosis to 38% at 3 years. Between baseline and end of observation, results for patients with paired data showed an increase in 6MWD. Survival at the end of observation was 88%. CONCLUSIONS: These data highlight the current diagnosis and management of CTEPH in the participating countries. They show that early CTEPH diagnosis remains challenging, and use of off-label PH-targeted therapy is common. CLINICALTRIALS: gov: NCT02637050; registered December 2015.

Long-Term Natural Course of Patients with Pulmonary Artery Pressures in the Range of 21-24 mmHg: Insights from a Single-Center Study.

OBJECTIVE: Slightly elevated mean pulmonary artery pressure (mPAP) was previously termed as ''borderline pulmonary hypertension (PH)''. We examined the long-term prognosis of patients with mPAP values between 21 and 24 mmHg, who were referred with the suspicion of pulmonary hypertension. METHODS: Our retrospective study included patients with moderate-to-high echocardiographic risk who underwent right heart catheterization (RHC) between 2008 and 2021 and were followed for at least 1 year. Patients with mPAP <21 mmHg and mPAP 21-24 mmHg were compared. Demographic and clinical characteristics and prognoses of the groups were compared. All-cause mortality over a mean follow-up of 5 years (min 1-max 13 years) was evaluated. RESULTS: A total of 140 patients (mean age 53.1 ± 14.8 years, female 74.5%) with mPAP values <25 mmHg measured of the 395 diagnostic RHCs. Mean follow-up was 4.92 ± 3.13 years. NT-pro-BNP and 6-min walking distance were better in patients with mPAP <21 mmHg. Echocardiographic findings suggestive of PH were more common in mPAP 21-24 mmHg group (P < 0.05). Both the pulmonary artery wedge pressure and cardiac index values were significantly deteriorated in individuals with mPAP 21-24 mmHg (P = 0.001). All-cause mortality tended to be higher in the borderline PH group but did not reach to statistical significance. CONCLUSION: Our single-center observational study revealed that the individuals with an mPAP of 21-24 mmHg tended to have a worser prognosis than those with mPAP of <21 mmHg for up to 13-year follow-up.

Could Heart Rate Variability Serve as a Prognostic Factor in Patients with Pulmonary Hypertension? A Single-center Pilot Study.

OBJECTIVE: Heart rate variability (HRV), which is defined as cyclic changes in sinus rate with time, is used as a measure of cardiac autonomic tone. Our aim was to determine the impact of HRV on short-term prognosis in pulmonary hypertension (PH). METHODS: We enrolled 64 PH patients and 69 healthy subjects (control group). Patients were evaluated by Holter-ECG, echocardiography, and laboratory tests. 24-h Holter-ECG monitoring was used for HRV. The development of adverse events (right heart failure, hospitalization, syncope, and death) during the 6-month follow-up was evaluated in PH group. RESULTS: PH group (39 ± 16 years, 37.5% males) comprised of 16 patients with idiopathic pulmonary arterial hypertension (PAH) (25%), 36 patients with PAH associated with congenital heart disease (56.3%), 3 PAH associated with connective tissue disease (4.7%), 1 with portopulmonary (1.6%), and 8 chronic thromboembolic PH (12.5%). The time-dependent (standard deviation of all NN intervals for a selected time period [SDNN], standard deviation of the 5-min mean R-R intervals tabulated over an entire day [SDANN], SDNN Index, and Triangular Index) and frequency-dependent HRV indices (low frequency, high-frequency power, and total power,) were significantly reduced in those with PH. Functional class was negatively associated with SDNN, SDANN, SDNN Index, and Triangular Index. Adverse events developed in 25% of the patients during the 6-month follow-up period (200 ± 92 days) (7 patients had right-heart failure, 5 syncope, 12 patients were hospitalized, and 9 had died). All the time and frequency-dependent indices significantly associated with adverse events. Mortality correlated with SDNN (rS = -0.354, P = 0.005), SDANN (rS = -0.368, P = 0.004), SDNN Index (rS = -0.257, P = 0.045), Triangular Index (rS = -0.310, P = 0.014), and VLF (rS = -0.265, P = 0.039). CONCLUSION: HRV is significantly depressed in patients with PH and is associated with the clinical status. HRV indices might predict clinical deterioration, adverse events, and mortality for 6 months. Non-invasive assessment of HRV through Holter-ECG may be a valuable and practical tool in risk stratification of patients with PH for short-term outcomes.

Could Impedance Cardiography be a Non-Invasive Alternative Method of Measuring Cardiac Output in Patients with Pulmonary Hypertension?

BACKGROUND: Pulmonary hypertension guidelines recommend invasive right heart catheterization for diagnosis and clinical follow-up. Our aim was to compare non-invasive impedance cardiography with invasive techniques for cardiac index measurements and mortality prediction in patients with pulmonary hypertension. METHODS: Between 2008 and 2018, 284 right heart catheterizations were performed for the diagnosis of pulmonary hypertension in 215 patients with mean pulmonary artery pressure >25 mm Hg, and at least 2 methods used for cardiac output measurement were included in the study retrospectively. Patients were evaluated with Pearson's correlation in 3 groups: estimated Fick (eFick) method and thermodilution (group 1), eFick method and impedance cardiography (group 2), and thermodilution and impedance cardiography (group 3). We also compared the predictive power of cardiac index measured by different methods for 1-year overall mortality and hospitalizations. RESULTS: There were strong and moderate positive correlations in groups 1 and 3, respectively (r = 0.634, P <.001, r = 0.534, P =.001), and the weakest correlation was in group 2 (r = 0.390, P =.001). The mean difference (bias) between eFick method versus impedance cardiography, impedance cardiography vs. thermodilution, and eFick method vs. thermodilution was 0.6 mL/min, 0.47 mL/min, and -0.2 mL/min respectively, but limits of agreement were wide. In both groups, cardiac index <2.5 L/min/m2 as measured by thermodilution significantly predicted 1-year mortality. Also, impedance cardiography was better than eFick method in predicting mortality (P =.02). CONCLUSIONS: Our single-center real-life data showed that for cardiac output and cardiac index measurements, impedance cardiography provides a moderate correlation with thermodilution and is fair with eFick method methods. Moreover, thermodilution appeared superior to both eFick method and impedance cardiography, while impedance cardiography was even better than eFick method in predicting 1-year adverse events, including total mortality and hospitalization, in patients with pulmonary hypertension.

Takotsubo syndrome early after treatment due to non cardiotoxic chemotherapy agents.

Takotsubo syndrome (TTS), acute stress-induced cardiomyopathy, is known to have a dramatic clinical presentation mimicking acute myocardial infarction. Recently developed chemotherapeutic drugs have resulted in improvements in morbidity and mortality in many forms of cancer. However, some chemotherapeutic drugs are cardiotoxic and may cause heart failure. Gemcitabine and vinorelbine are commonly used drugs for various solid organ neoplasms. While neither of these chemotherapeutic drugs has been directly associated with cardiotoxicity, there are a few case reports in the literature related to gemcitabine treatment- induced cardiomyopathy. This case report describes a case of TTS developing within hours of gemcitabine and vinorelbine chemotherapy.

Serum Endocan Levels Predict Angiographic No-Reflow Phenomenon in Patients With ST-Segment Elevation Myocardial Infarction Undergoing Primary Coronary Intervention.

No-reflow phenomenon (NRP) is an important problem in patients with ST-segment elevation myocardial infarction (STEMI) undergoing primary percutaneous coronary intervention (pPCI). Endocan is synthesized and secreted by activated vascular endothelium, and it has been shown to be related to endothelial dysfunction and inflammation. We aimed to evaluate the relationship between endocan levels and NRP. Consecutive patients (n = 137) with STEMI who had undergone coronary angiography and pPCI were enrolled into the study. The clinical characteristics of the patients were obtained and endocan levels were measured. Endocan levels were significantly higher in the NRP (+) group compared with the NRP (-) group (P < .001). In multivariate analysis, endocan (P < .001, OR = 2.39, 95% CI = 1.37-4.15) was found to be an independent predictor of NRP. An endocan value of >2.7 ng/mL has 89.6% sensitivity and 74.2% specificity for the prediction of the NRP (area under the curve: 0.832, P < .001). The present study demonstrated that the endocan level is an independent predictor of the NRP in patients with STEMI who underwent pPCI. Endocan levels may be helpful in detecting patients with a higher risk of insufficient myocardial perfusion and worse clinical outcome.

Hepatic and splenic sonographic and sonoelastographic findings in pulmonary arterial hypertension.

PURPOSE: The aim of this study was to evaluate the associations of sonographic and sonoelastographic parameters with clinical cardiac parameters, as well as to assess their value in predicting survival in patients with pulmonary arterial hypertension (PAH). METHODS: Thirty-six patients with PAH and normal liver function were prospectively enrolled in this prospective study along with 26 healthy controls, all of whom underwent ultrasound and point shear wave elastography examinations. Additionally, the portal vein pulsatility index (PVPI), inferior vena cava collapsibility index, and clinical cardiac variables were obtained in PAH patients. The values of hepatic (LVs) and splenic shear wave velocity (SVs) were compared between PAH patients and controls. The relationships between all sonographic and clinical parameters in the PAH patients were analyzed. Furthermore, their prognostic value in predicting survival was investigated. RESULTS: LVs values in PAH patients (median, 1.62 m/s) were significantly higher than in controls (median, 0.99 m/s), while no significant difference was observed in SVs values. Patients with higher grades of tricuspid regurgitation (TR) had significantly different values of PVPI (P=0.010) and sonoelastographic parameters (P<0.001 for LVs and P=0.004 for SVs) compared to those with less severe TR. Tricuspid annular plane systolic excursion values were the only investigated parameter found to be associated with survival (hazard ratio, 0.814; 95% confidence interval, 0.694 to 0.954; P=0.011). CONCLUSION: Our results demonstrated a direct association between cardiac congestion (i.e., the severity of TR) and liver stiffness, which should be kept in mind during the assessment of fibrosis in patients with PAH.

Assessment of right ventricular function in patients with pulmonary arterial hypertension-congenital heart disease and repaired and unrepaired defects: Correlation among speckle tracking, conventional echocardiography, and clinical parameters.

OBJECTIVE: The purpose of this study is to compare the analysis of right ventricular (RV) free wall strain via 2D speckle tracking echocardiography with conventional echocardiography and clinical parameters in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) receiving specific treatment. This study also aims to describe the differences between patients with repaired and unrepaired defects. METHODS: This prospective study included 44 adult patients with PAH-CHD who were receiving PAH-specific treatment in a single center. This study excluded patients with complex congenital heart disease. The authors studied the conventional echocardiographic parameters, such as RV fractional area change (FAC), tricuspid annular plane systolic excursion (TAPSE), right atrial (RA) area, Tricuspid S', and hemodynamic parameters, such as functional class, 6-minute walking distance (6MWD), and N-terminal pro-brain natriuretic peptide (NT-proBNP) levels. RESULTS: The mean age of participants was 33.8±11.6 years, and 65.9% of participants were female. The mean RV free wall strain was -14.8±4.7%. Majority of the patients belonged to WHO functional class 2 (61.4%) with a mean NT-proBNP level of 619.2±778.4 and mean 6MWD of 400.2±86.9 meters. During the follow-up of 30.8±9.0 months, 6 patients (13.6%) developed clinical right heart failure, whereas 9 (20.5%) of them died. There was a positive and significant correlation between RV free wall strain and WHO functional class (r=0.320, p=0.03), whereas there was a negative correlation between RV free wall strain and FAC (r=-0.392, p=0.01), TAPSE (r=-0.577, p=0.0001), and Tricuspid S' (r=-0.489, p=0.001). There was no significant correlation of RV free wall strain with either RA area or 6MWD. Patients with repaired congenital heart defects had worse RV functional parameters and RV free wall strain than patients with unrepaired defects. CONCLUSION: The assessment of RV free wall strain via 2D speckle tracking echocardiography is a feasible method and correlates well with conventional echocardiography and clinical parameters in patients with PAH-CHD receiving specific treatment. (Anatol J Cardiol 2020; 23: 277-87).

Effects of cardiopulmonary rehabilitation on pulmonary arterial hypertension: A prospective, randomized study.

OBJECTIVES: This study aims to investigate the effects of cardiopulmonary rehabilitation (CPR) on cardiopulmonary function, quality of life, depression, and hemodynamic parameters in patients with pulmonary arterial hypertension (PAH) and to compare the efficacy of hospital- and home-based exercise programs. PATIENTS AND METHODS: Between December 2014 to May 2016, a total of 30 patients with PAH were included in the study. The patients were randomly assigned to either a hospital-based (n=15) or home-based exercise program (n=15). The hospital group was assigned to a cardiac rehabilitation (three days/week, 1 hour/week 50 to 70% maximal oxygen uptake [PvO2]) program for eight weeks. The home-based exercise group received home exercises alone. Before and after eight weeks of rehabilitation, all patients were evaluated for their functional status (PvO2), pulmonary function including forced expiratory volume in one sec (FEV1), mL, forced vital capacity (FVC), mL and FEV1/FVC%, quality of life using Short Form-36 (SF-36), depression severity using Beck Depression Inventory, and hemodynamic parameters including left ventricular ejection fraction, right ventricular ejection fraction, left ventricular end-systolic diameter, left ventricular end-diastolic diameter, and systolic pulmonary arterial pressure. RESULTS: Of the patients, 12 completed the study in each group. There was no statistically significant difference in the functional status, quality of life, depression severity, and hemodynamic parameters after the rehabilitation compared baseline between the groups. CONCLUSION: Based on our study results, short-term CPR seems not to be beneficial in patients with PAH. We recommend long-term rehabilitation programs to achieve more benefits from aerobic exercise training in this patient population.

Is there a gender gap in secondary prevention of coronary artery disease in Turkey?

OBJECTIVE: It has been reported that women receive fewer preventive recommendations regarding pharmacological treatment, lifestyle modifications, and cardiac rehabilitation compared with men who have a similar risk profile. This study was an investigation of the impact of gender on cardiovascular risk profile and secondary prevention measures for coronary artery disease (CAD) in the Turkish population. METHODS: Statistical analyses were based on the European Action on Secondary and Primary Prevention through Intervention to Reduce Events (EUROASPIRE)-IV cross-sectional survey data obtained from 17 centers in Turkey. Male and female patients, aged 18 to 80 years, who were hospitalized for a first or recurrent coronary event (coronary artery bypass graft, percutaneous coronary intervention, acute myocardial infarction, or acute myocardial ischemia) were eligible. RESULTS: A total of 88 (19.7%) females and 358 males (80.3%) were included. At the time of the index event, the females were significantly older (p=0.003) and had received less formal education (p<0.001). Non-smoking status (p<0.001) and higher levels of depression and anxiety (both p<0.001) were more common in the female patients. At the time of the interview, conducted between 6 and 36 months after the index event, central obesity (p<0.001) and obesity (p=0.004) were significantly more common in females. LDL-C, HDL-C or HbA1c levels did not differ significantly between genders. The fasting blood glucose level was significantly higher (p=0.003) and hypertension was more common in females (p=0.001). There was no significant difference in an increase in physical activity or weight loss after the index event between genders, and there was no significant difference between genders regarding continuity of antiplatelet, statin, beta blocker or ACEi/ARB II receptor blocker usage (p>0.05). CONCLUSION: Achievement of ideal body weight, fasting blood glucose and blood pressure targets was lower in women despite similar reported medication use. This highlights the importance of the implementation of lifestyle measures and adherence to medications in women.

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.

[EUROASPIRE-IV: European Society of Cardiology study of lifestyle, risk factors, and treatment approaches in patients with coronary artery disease: Data from Turkey]. / EUROASPIRE-IV: Avrupa Kardiyoloji Dernegi'nin koroner arter hastalarinda yasam tarzi, risk faktörleri ve tedavi yaklasimi üzerine çalismasi: Türkiye verileri.

OBJECTIVE: Data from EUROASPIRE-IV Turkey report investigating risk factors and adherence to guidelines in patients hospitalized for coronary artery disease are presented and results are compared with those of EUROASPIRE-III Turkey and EUROASPIRE-IV Europe. METHODS: Study was performed in 24 European countries, including Turkey (17 centers). Patients (18-80 years old) hospitalized for coronary (index) event during preceding 3 years were identified from hospital records and interviewed ≥6 months later. Patient information regarding index event was acquired from hospital records. Anamnesis was obtained during the interview, and physical examination and laboratory analyses were performed. RESULTS: Median age at the index coronary event was 58.8 years, and it was significantly decreased compared with last EUROASPIRE-III study (60.5 years), which was conducted at the same centers 6 years earlier (p=0.017). Of all patients, 19.3% were under 50 years of age and mean age was lower than that of EUROASPIRE-IV Europe (62.5 years). Comparing EUROASPIRE-IV Turkey with EUROASPIRE-III Turkey, rate of smokers increased to 25.5% from 23.1% (p=0.499), obesity increased to 40.7% from 35.5% (p=0.211), total cholesterol level increased to 49.6% from 48.3% (p=0.767), and diabetes rate increased to 39.7% from 33.6% (p=0.139), however none of the differences reached a level of statistical significance. Only 11.7% of the smokers quit after coronary event. Rates for these factors were lower in EUROASPIRE-IV Europe (16% for smoking, 37.6% for obesity, and 26.8% for diabetes). CONCLUSION: EUROASPIRE-IV Turkey data revealed that secondary prevention was unsatisfactory and had progressed unfavorably compared with last EUROASPIRE study, some risk factors were more uncontrolled than overall European average, and coronary artery events at young age remain an important problem.

Right ventricular free-wall longitudinal speckle tracking strain in patients with pulmonary arterial hypertension under specific treatment.

BACKGROUND: Right ventricular (RV) dysfunction is a major determinant of outcomes in patients with pulmonary arterial hypertension (PAH), although the optimal measure of RV function is poorly defined. We evaluated the utility of RV free-wall speckle tracking strain as an assessment tool for RV function in patients with PAH who are already under specific treatment compared with conventional echocardiographic parameters and investigated the relationship of RV free-wall strain with clinical hemodynamic parameters of RV performance. METHODS: Right ventricular free-wall strain was evaluated in 92 patients (Group-1 and Group-4 pulmonary hypertension) who were on PAH-specific treatment for at least 3 months. Right atrial (RA) area, RV FAC, TAPSE, tricuspid S, functional class, 6-minute walking distance, and NT-proBNP were studied. The mean duration of follow-up was 222±133 days. RESULTS: All patients were under PAH-specific treatment, and mean RV free-wall strain was -13.16±6.3%. RV free-wall strain correlated well with functional class (r=.312, P=.01), NT-proBNP (r=.423, P=.0001), RA area (r=.427, P=.0001), FAC (r=-.637, P=.0001), TAPSE (r=-.524, P=.0001), tricuspid S (r=-.450, P=.0001), 6-minute walking distance (r=-.333, P=.002). RV free-wall strain significantly correlated with all follow-up adverse events, death, and clinical right heart failure (RHF) (P=.04, P=.03, P=.02, respectively). According to the receiver operator characteristic analysis, the cutoff value for RV free-wall strain for the development of clinical RHF was -12.5% (sensitivity: 71%, specificity: 67%) and for all cardiovascular adverse events (death included) was -12.5% (sensitivity: 54%, specificity: 64%). CONCLUSION: Assessment of RV free-wall strain is a feasible, easy-to-perform method and may be used as a predictor of RHF, clinical deterioration, and mortality in patients already under PAH-specific treatment.

Sequencing of mutations in the serine/threonine kinase domain of the bone morphogenetic protein receptor type 2 gene causing pulmonary arterial hypertension.

OBJECTIVE: Germline mutations in the bone morphogenetic protein receptor type-2 (BMPR2) gene are considered to be a major risk factor for pulmonary arterial hypertension (PAH). BMPR2 mutations have been reported in 10%-20% of idiopathic PAH and in 80% of familial PAH cases. The aim of this study was to evaluate the frequency of mutations in the serine/threonine kinase domain of the BMPR2 gene in a group of patients from a single PAH referral center in Turkey. METHODS: This cross-sectional study used a DNA-sequencing method to investigate BMPR2 mutations in the serine-threonine-kinase domain in 43 patients diagnosed with PAH [8 with idiopathic PAH and 35 with congenital heart disease (CHD)] from a single PAH referral center. Patients were included if they had a hemodynamically measured mean pulmonary arterial pressure of >25 mm Hg with a mean pulmonary capillary wedge pressure of ≤15 mm Hg. Patients with severe left heart disease and/or pulmonary disease that could cause pulmonary hypertension were excluded. Associations between categoric variables were determined using the chi-square test. Differences between idiopathic and CHD-associated PAH groups were compared with the unpaired Student's t-test for continuous variables. RESULTS: We detected a missense mutation, [p.C347Y (c.1040G>A)], in one patient with idiopathic PAH in exon 8 of the BMPR2 gene. The mutation was detected in a 27-year-old female with a remarkable family history for PAH. She had a favorable response to endothelin receptor antagonists. No mutations were detected in the exons 5-11 of the BMPR2 gene in the PAH-CHD group. CONCLUSION: A missense mutation was detected in only one of the eight patients with idiopathic PAH. The BMPR2 missense mutation rate of 12.5% in this cohort of Turkish patients with idiopathic PAH was similar to that seen in European registries. The index patient was a young female with a family history remarkable for PAH; she had a good long-term response to PAH-specific treatment, probably due to the early initiation of the treatment. Genetic screening of families affected by PAH might have great value in identifying the disease at an early stage.

Pulmonary arterial hypertension and pregnancy.

This is the case report of a pregnant woman who refused pregnancy termination when diagnosed with pulmonary arterial hypertension (PAH) functional class 2-3 at the 24th week of gestation and of her newborn. A pregnant woman with PAH functional class 2-3 was treated with inhaled prostacyclin analog (iloprost), oral sildenafil, oxygen, and low molecular weight heparin. She delivered at 32nd week by Cesarean section. The infant required oxygen up to 36th week postconceptional age and had a short steroid treatment. The mother needed close cardiovascular monitorization, intensive oxygen and pulmonary vasodilator therapy for 2 months and was discharged with oxygen and oral iloprost treatment. A multidisciplinary approach together with pulmonary vasodilator therapy may be succesful in such a high-risk pregnant woman.

Antihypertensive drugs and inflammation in acute ischemic stroke as a predictor factor of future cardiovascular mortality.

The beneficial effects of antihypertensive drugs in secondary stroke prevention could not be based on their effects on lowering hypertension only.In this prospective study, the status of blood pressure, treatment regimens, new atherothrombotic event, blood sampling for hs-CRP and fibrinogen were asked at initial visit, 6th, and 12th months in 226 patients with atherothrombotic stroke.Eighty-seven percent of patients had an antihypertensive regimen, but hypertension control was achieved in 34.1% of patients.Neither use of six different antihypertensive drug regimens nor the change in blood pressure levels showed any difference on new atherothrombotic events, outcomes or survival rates.On the other hand, the higher levels of hs-CRP at baseline were found to be associated with higher mortality rates (p=0.020).Our findings emphasize the predictive role of inflammation in future cardiovascular mortality in patients with acute ischemic stroke, indicating that inflammatory mediators underlying the atherothrombotic process play a more important role than it is assumed.

[The psychometric properties of the Turkish version of Myocardial Infarction Dimensional Assessment Scale (MIDAS)]. / Miyokart Enfarktüsü Boyutsal Degerlendirme Ölçegi (MIDAS) Türkçe sürümünün psikometrik özellikleri.

OBJECTIVE: The purpose of this study was to describe the psychometric properties of the Myocardial Infarction Dimensional Assessment Scale (MIDAS). METHODS: This is a methodological cultural adaptation study. The MIDAS consists of 35-items covering seven domains: physical activity, insecurity, emotional reaction, dependency, diet, concerns over medication, and side effects which are rated on a five-point Likert scale from 1: never to 5:always. The highest score of MIDAS is 100.Quality of life (QOL) decreases as the score of scale increases. Overall 185 myocardial infarction (MI) patients were enrolled in this study. Cronbach alpha was used for the reliability analysis. The criterion validity, structural validity, and sensitivity analysis approach was used for validity analysis. New York Heart Association (NYHA) and the Canadian Cardiovascular Society Functional Classifications (CCSFC) for testing the criterion validity; SF-36 for construct validity testing of the Turkish version of the MIDAS were used. RESULTS: The range of Cronbach alpha values is 0.79-0.90 for seven domains of the scale. No problematic items were observed for the entire scale. Medication related domains of the MIDAS showed considerable floor effects (35.7%-22.7%). Confirmatory Factor analysis indicators [Comparative Fit Index (CFI) =0.95 and Root Mean Square Error of Approximation (RMSEA) =0.075] supported the construct validity of MIDAS. Convergent validity of the MIDAS was confirmed with correlation of SF-36 scale where appropriate. Criterion validity results was also satisfactory by comparing different stages of the NYHA and the CCSFC (p<0.05). CONCLUSION: Overall results revealed that Turkish version of the MIDAS is a reliable and valid instrument.

Tachycardia-induced cardiomyopathy in patients with idiopathic ventricular arrhythmias: the incidence, clinical and electrophysiologic characteristics, and the predictors.

INTRODUCTION: Idiopathic ventricular arrhythmias in the form of monomorphic premature ventricular contractions (PVC) and/or ventricular tachycardia (VT) can cause tachycardia-induced cardiomyopathy (TICMP). The aim of this study was to determine the incidence, clinical and electrophysiologic characteristics, and the predictors of TICMP in patients with idiopathic ventricular arrhythmias. METHODS: Study population consisted of 249 consecutive patients (148 F/101 M, 45 ± 20 y/o) with frequent PVCs and/or VT. All patients underwent transthoracic echocardiography and 24-hour Holter monitoring. TICMP was defined as left ventricular ejection fraction (LVEF) of ≤50% in the absence of any detectable underlying heart disease and improvement of LVEF ≥15% following effective treatment of index ventricular arrhythmia. RESULTS: Seventeen (6.8%) patients had TICMP. Patients with TICMP compared to patients with preserved LVEF were more likely to be male (65% vs 39%, P = 0.043) and asymptomatic (29% vs 9%, P = 0.018), and were more likely to have higher PVC burden (29.4 ± 9.2 vs 8.1 ± 7.4, P < 0.001), persistence of PVCs throughout the day (65% vs 22%, P = 0.001), and repetitive monomorphic VT (24% vs 0.9%, P < 0.001). PVC burden of 16% by ROC curve analysis best separated the patients with TICMP compared to patients with preserved LVEF (sensitivity 100%, specificity 87%, area under curve 0.96). CONCLUSIONS: TICMP was relatively common (∼1 in every 15 patients) in our study population. The predictors of TICMP were male gender, absence of symptoms, PVC burden of ≥16%, persistence of PVCs throughout the day, and the presence of repetitive monomorphic VT.