Usability of myocardial work parameters to demonstrate subclinical myocardial involvement in normotensive individuals with exaggerated hypertensive response in treadmill exercise testing.

Early determination of changes in myocardial functions is essential for the protection of cardiovascular diseases. This study aimed to evaluate myocardial work parameters in healthy individuals who developed an exaggerated hypertensive response during the treadmill exercise test procedure. The study included a total of 64 patients for whom an exercise electrocardiography test was planned for functional capacity evaluation. The study population was divided according to the presence of exaggerated hypertensive response to exercise (EBPRE) (SBP/DBP ≥210/105 mmHg in males ≥190/105 mmHg in females) and normal blood pressure response to exercise (NBPRE). Patients' echocardiographic evaluations were made at rest, and myocardial work parameters were calculated. There was no statistical difference between the groups (NBPRE vs. EBPRE, respectively) in terms of left ventricular 2,3 and 4 chamber strains and global longitudinal strain (GLS) values (-20.6 ± -2.3, -19.7 ± -1.9, p:.13; -21.3 ± -2.7, -21 ± -2.4, p:.68; -21.2 ± -2.2, -21.2 ± -2.3, p:.93; and -20.8 ± -1.5, -20.4 ± -1.5, p:.23, respectively). Global constrictive work (GCW), global waste work (GWW), and global work efficiency (GWE) were not statistically different between the two groups (2374 ± 210, 2465 ± 204, p:.10; 142 ± 64, 127 ± 42, p:.31; 94.3 ± 2.5, 95.1 ± 1.5, p:.18, respectively). In contrast, global work index (GWI) parameters were different between the two groups (2036 ± 149, 2147 ± 150, p < .001). The GWI was independently associated with EBPRE (odds ratio with 95% 3.32 (1.02-11.24), p = .03). The partial effect plots were used for GWI to predict EBPRE, according to the results, an increase in GWI predicts probability of exaggerated hypertensive response. In conclusion, Myocardial work analyses might be used to identify early signs of myocardial involvement in normotensive patients with EBPRE.

Is it Time to Reappraise for Black-Box Warning on AngioJet Rheolytic Thrombectomy in Patients with Pulmonary Embolism: A Systematic Review and Meta-analysis.

BACKGROUND: AngioJet rheolytic thrombectomy (ART) system has been widely used as a catheter-directed treatment (CDT) method in acute pulmonary embolism (PE), however, there has been a controversy regarding the safety of its use. In this systematic review and meta-analysis, we evaluated the efficacy and safety outcomes of ART in patients with PE. METHODS: Our meta-analysis have been based on search in the MEDLINE, EMBASE, and Cochrane Library for studies published up to August 2022. The primary outcomes were overall pooled rates of major bleeding (MB) and minor bleeding (mB), worsening renal function (WRF), bradycardia/conduction disturbance (BCD), and PE-related and all-cause mortality in patients who underwent ART. RESULTS: Among the 233 studies documented at initial search, 24 studies were eligible for meta-analysis, and a total of 427 PE patients who underwent ART were evaluated. Overall pooled rates of MB and mB were 9.6% (95% CI 5.9%-15.2%) and 9.2% (95% CI 6.1%-13.6%), transient BCD and WRF were 18.2% (95% CI 12.4%-26%) and 15% (95% CI 10%-21.8%), and PE-related death and all-cause death were 12.7% (95% CI 9.1%-17.3%) and 15% (95% CI 11%-20%), respectively. However, significant heterogeneity and some evidence of funnel plot asymmetry and publication bias were noted for MB, BCD and WRF, but not for PE-related death and all-cause death. CONCLUSION: Overall pooled rates of bleeding events, BCD and WRF episodes, PE-related death and all-cause death may be considered as encouraging results for efficacy and safety issues of ART utilization in specific scenarios of acute PE, and a reappraisal for black-box warning on ART seems to be necessary.

Switching from Deferasirox Dispersible Tablets to Deferasirox Film-Coated Tablets: Is There an Effect on Ferritin Levels in Children and Adolescents with Transfusion-Dependent Anemia?

OBJECTIVE: To investigate the effect of switching from deferasirox dispersible tablet (DT) to deferasirox film-coated tablet (FCT) on serum ferritin (SF) levels in transfusion-dependent patients. MATERIALS AND METHODS: Patients who received regular erythrocyte transfusion and whose treatment was switched from DT to FCT were included in the study. FCT start date was taken as the index date. Patients were followed over 2 equal and long periods, both before and after index date. RESULTS: Thirty-two patients were included, and the comparison periods ranged from 4 to 12 months. The SF values increased from a median of 1723 ng/mL (range 717-5369 ng/mL) to 1.853 ng/mL (range 924-5478 ng/mL) after switching from DT to FCT (P = .036). While there was a significant increase in median SF after switching in Turkish patients (1467 ng/mL to 1778 ng/ mL, P = .010) and patients ≥12 years (1598-1848 ng/mL, P = .009), there was an insignificant (P = .859) decrease in SF in immigrant children. Considering only the post-switch period, there was a non-significant increase in median SF in the entire cohort, while SF decreased significantly in immigrant children (P = .026). No serious side effects were observed in any patient that would cause discontinuation of treatment. CONCLUSION: Overall, higher SF value was observed with FCT compared to DT in short term. There were different results between patient groups. Our results suggest that FCT is more effective than DT in patients with high basal ferritin and who are actually incompatible with treatment and should be preferred first in these patients.

Early Systolic Lengthening Is Associated with SYNTAX Score in Patients with Non-ST-Elevation Acute Coronary Syndrome.

BACKGROUND: Early systolic lengthening is a echocardiographic strain parameter previously used to determine the lesion severity in patients with stable coronary artery disease. In the present study, we aimed to evaluate the relationship between early systolic lengthening and anatomic SYNTAX score in troponin (-) and (+) groups among patients with non-ST-elevation acute coronary syndrome (ACS). METHODS: A total of 95 patients diagnosed with non-ST-elevation ACS were included in the prospective, non-randomized, single-center study. The patients were categorized into 2 groups as troponin (+) and troponin (-). The patients were evaluated in terms of echocardiographic, clinical, and angiographic parameters. RESULTS: The baseline characteristics, including age (58 ± 13 vs. 60 ± 10 respectively, P =.340), a history of hypertension (67.1% vs. 64%, respectively, P =.479), diabetes (28.6% vs. 32%, respectively, P =.467), global longitudinal strain (-14.37 ± 5.11 vs. -16.42 ± 3.93, respectively, P =.095), left ventricular ejection fraction (58.71 ± 8.73 vs. 57.20 ± 8.70, respectively, P =.263), and E/e' (8.44 ± 2.13 vs. 8.33 ± 1.99, respectively, P =.785), were similar between troponin (+) and troponin (-) groups. Left ventricle end-systolic diameter (3.2 ± 0.78; 3.50 ± 0.74 vs. 3.2 ± 0.78, respectively, P =.031), left ventricle end-systolic volume (55.57 ± 32.17 vs. 38.28 ± 13.63, respectively, P =.013), left ventricle end-diastolic volume (115.31 ± 49.54 vs. 91.23 ± 20.57, respectively, P =.042), the rate of early systolic lengthening (65.7% vs. 28%, respectively, P =.001), the duration of early systolic lengthening (24.02 ± 31 ms vs. 15.56 ± 30.19 ms, respectively, P =.009), and the SYNTAX score (16 ± 11 vs. 10 ± 10, respectively, P =.023) were higher in the troponin (+) group. Furthermore, a significant correlation was found between early systolic lengthening and SYNTAX score (r = 0.43, P <.001). CONCLUSION: The rate and duration of early systolic lengthening were higher in patients in the troponin (+) group. Early systolic lengthening is related to SYNTAX score in patients with non-ST-elevation ACS.

Remembering the Occam's Razor: Could Simple Electrocardiographic Findings Provide Relevant Predictions for Current Hemodynamic Criteria of Pulmonary Hypertension?

BACKGROUND: We evaluated the predictive value of electrocardiographic (ECG) findings for pulmonary hemodynamics assessed by right heart catheterization (RHC). METHODS: Our study population comprised 562 retrospectively evaluated patients who underwent RHC between 2006 and 2022. Correlations between ECG measures and pulmonary arterial systolic and mean pressures (PASP and PAMP) and pulmonary vascular resistance (PVR) were investigated. Moreover, receiver operating characteristic (ROC) curve analysis assessed the predictive value of ECG for pulmonary hypertension (PH) and precapillary PH. RESULTS: The P-wave amplitude (Pwa) and R/S ratio (r) in V1 and V2, Ra in augmented voltage right (aVR), right or indeterminate axis, but not P wave duration (Pwd) or right bundle branch block (RBBB) significantly correlated with PASP, PAMP, and PVR (P <.001 for all). The partial R2 analysis revealed that amplitude of R wave (Ra) in aVR, R/Sr in V1 and V2, QRS axis, and Pwa added to the base model provided significant contributions to variance for PASP, PAMP, and PVR, respectively. The Pwa > 0.16 mV, Ra in aVR > 0.05 mV, QRS axis > 100° and R/Sr in V1 > 0.9 showed the highest area under curve (AUC) values for PAMP > 20 mm Hg. Using the same cutoff value, Ra in aVR, Pwa, QRS axis, and R/Sr in V1 showed highest predictions for PVR > 2 Wood Units (WU). CONCLUSION: In this study, Pwa, Ra in aVR, right or indeterminate axis deviations, and R/Sr in V1 and V2 showed statistically significant correlations with pulmonary hemodynamics, and Ra in aVR, R/Sr in V2 and V1, QRS axis, and Pwa contributed to variance for PASP, PAMP, and PVR, respectively. Moreover, Pwa, Ra in aVR, QRS axis, and R/Sr in V1 seem to provide relevant predictions for PH and precapillary PH.

Hoarseness as a Predictor for Pulmonary Arterial Aneurysm and Extrinsic Left Main Coronary Artery Compression in Patients with Severe Pulmonary Hypertension.

OBJECTIVE: Pulmonary artery (PA) enlargement is a common finding in patients with severe pulmonary hypertension (PH) and may be associated with extrinsic compression of the left main coronary artery (LMCA-Co) and/or compression of the left recurrent laryngeal nerve resulting in hoarseness named as Ortner syndrome (OS). In this study, we evaluated the diagnostic impact of OS in predicting the PA aneurysm and significant LMCA-Co in patients with PH. METHODS: Our study population comprised retrospectively evaluated 865 with PH confirmed with the right heart catheterization between 2006 and 2022. Patients underwent coronary angiography due to several indications, including the presence of a PA aneurysm on echocardiography, angina symptoms, or the incidental discovery of LMCA-Co on multidetector computed tomography. The LMCA-Co is defined as diameter stenosis ³ 50% in reference distal LMCA segment on two consecutive angiographic planes. RESULTS: The LMCA-Co and hoarseness were documented in 3.8% and 4.3% of patients with PH, respectively. Increasing PA diameter was significantly associated with worse clinical, hemodynamic, laboratory, and echocardiographic parameters. The receiver operating curves revealed that the PA diameter >41 mm was cutoff for hoarseness (AUC: 0.834; sensitivity 69%, specificity 84%, and negative predictive value 98%), and PA diameter >35 mm was cutoff for LMCA-Co >50% (AUC: 0.794; sensitivity 89%, specificity 58 %, and negative predictive value 99%). An odds ratio of hoarseness for LMCA-Co was 83.3 (95% confidence interval; 36.5-190, P < 0.001) with 3.2% sensitivity, 98.7% specificity, and 59% positive and 98% negative predictive values. CONCLUSION: In this study, a close relationship was found between the presence of hoarseness and the probability of extrinsic LMCA-Co by enlarged PA in patients with severe PH. Therefore, the risk of LMCA-Co should be taken into account in patients with PH suffering from hoarseness.

A Novel Composed Index to Evaluate the Right Ventricle Free-Wall Adaptation Against Ventricular Wall Stress in Acute Pulmonary Embolism.

BACKGROUND: Pulmonary embolism severity index and simplified pulmonary embolism severity index have been utilized in initial risk evaluation in patients with acute pulmonary embolism. However, these models do not include any imaging measure of right ventricle function. In this study, we proposed a novel index and aimed to evaluate the clinical impact. METHODS: Our study population comprised retrospectively evaluated 502 patients with acute pulmonary embolism managed with different treatment modalities. Echocardiographic and computed tomographic pulmonary angiography evaluations were performed at admission to the emergency room within maximally 30 minutes. The formula of our index was as follows: (right ventricle diameter × systolic pulmonary arterial pressure-echo)/(right ventricle free-wall diameter × tricuspid annular plane systolic excursion). RESULTS: This index value showed significant correlations to clinical and hemodynamic severity measures. Only pulmonary embolism severity index, but not our index value, independently predicted in-hospital mortality. However, an index value higher than 17.8 predicted the long-term mortality with a sensitivity of 70% and specificity of 40% (areas under the curve = 0.652, 95% CI, 0.557-0.747, P =.001). According to the adjusted variable plot, the risk of long-term mortality increased until an index level of 30 but remained unchanged thereafter. The cumulative hazard curve also showed a higher mortality with high-index value versus low-index value. CONCLUSIONS: Our index composed from measures of computed tomographic pulmonary angiography and transthoracic echocardiography may provide important insights regarding the adaptation status of right ventricle against pressure/wall stress in acute pulmonary embolism, and a higher value seems to be associated with severity of the clinical and hemodynamic status and long-term mortality but not with in-hospital mortality. However, the pulmonary embolism severity index remained as the only independent predictor for in-hospital mortality.

Reappraisal of the Transthoracic Echocardiographic Algorithm in Predicting Pulmonary Hypertension Redefined by Updated Pulmonary Artery Mean Pressure Threshold.

BACKGROUND: Although an adopted echocardiography algorithm based on tricuspid regurgitation jet peak velocity and suggestive findings for pulmonary hypertension has been utilized in the non-invasive prediction of pulmonary hypertension probability, the reliability of this approach for the updated hemodynamic definition of pulmonary hypertension remains to be determined. In this study, for the first time, we aimed to evaluate the tricuspid regurgitation jet peak velocity and suggestive findings in predicting the probability of pulmonary hypertension as defined by mean pulmonary arterial pressure > 20 mm Hg and > 25 mm Hg, respectively. METHODS: Our study group was comprised of the retrospectively evaluated 1300 patients (age 53.1 ± 18.8 years, female 62.1%) who underwent right heart catheterization with different indications between 2006 and 2018. All echocardiographic and right heart catheterization assessments were performed in accordance with the European Society of Cardiology/European Respiratory Society 2015 Pulmonary Hypertension Guidelines. RESULTS: Although tricuspid regurgitation jet peak velocity showed a significant relation with mean pulmonary arterial pressure in both definitions, suggestive findings offered a significant contribution only in predicting mean pulmonary arterial pressure ≥ 25 mm Hg but not for mean pulmonary arterial pressure > 20 mm Hg. In predicting the mean pulmonary arterial pressure > 20 mm Hg, tricuspid regurgitation jet peak velocity and suggestive findings showed an odds ratio of 2.57 (1.59-4.14, P <.001) and 1.25 (0.86-1.82, P =.16), respectively. In predicting the mean pulmonary arterial pressure ≥ 25 mm Hg, tricuspid regurgitation jet peak velocity, and suggestive findings showed an odds ratio of 2.33 (1.80-3.04, P <.001) and 1.54 (1.15-2.08, P <.001), respectively. The tricuspid regurgitation jet peak velocity > 2.8 m/s and tricuspid regurgitation jet peak velocity > 3.4 m/s were associated with 70% and 84% probability of mean pulmonary arterial pressure > 20 mm Hg and 60% and 76% probability of mean pulmonary arterial pressure ≥ 25 mm Hg, respectively. CONCLUSIONS: In contrast to those in predicting the mean pulmonary arterial pressure ≥ 25 mm Hg, suggestive findings did not provide a significant contribution to the probability of mean pulmonary arterial pressure > 20 mm Hg predicted by tricuspid regurgitation jet peak velocity solely. The impact of the novel mean pulmonary arterial pressure threshold on the echocardiographic prediction of pulmonary hypertension remains to be clarified by future studies.

A New Index for the Prediction of In-Hospital Mortality in Patients with Acute Pulmonary Embolism: The Modified Shock Index.

BACKGROUND: Pulmonary embolism severity index, its simplified version, and shock index have been used for risk stratification in acute pulmonary embolism. In this study, we proposed a modification in severity index and evaluated the correlates and prognostic value of modification in severity index in this setting. METHODS: The study group comprised retrospectively evaluated 181 patients with acute pulmonary embolism. Systematic workup including pulmonary embolism severity index, its simplified version, shock index, biomarkers, and echocardiographic and multidetector computed tomography assessments was performed in all patients. Moreover, we calculated modification in severity index by multiplying original shock index (heart rate/systolic blood pressure ratio) and a third component, 1/pulse oxymetric saturation (pSat O2%) ratio. The primary endpoint was defined as all-cause mortality and hemodynamic collapse during the hospital stay. RESULTS: On the basis of initial risk stratification, ultrasound-assisted thrombolysis, systemic tissue-type plasminogen activator, and unfractionated heparin therapies were utilized in 83 (45.9%), 37 (20.4%), and 61 (33.7%) patients, respectively. The primary end-point occurred in 13 (7.2%) patients. Receiver-operating curve analysis revealed that modification in severity index had the highest area under the curve of 0.739 (0.588-0.890, P =.002) compared with shock index, pulmonary embolism severity index, or its simplified version. The modification in severity index > 0.989 predicted primary endpoint with 73% sensitivity and 54% specificity. CONCLUSIONS: The modification in severity index seems to be a simple, quick, and compre-hensive risk assessment tool for bedside evaluation at initial stratification, in monitoring the clinical benefit from therapies, and decision-making for escalation to other reperfusion strategies in patients with acute pulmonary embolism. However, the prognostic value of modification in severity index needs to be validated with further studies.

A comparison of hypersensitivity reactions between intravenous and intramuscular applications of native E. coli asparaginase in children with acute lymphoblastic leukemia.

INTRODUCTION: Asparaginase is an indispensable drug in treating childhood acute lymphoblastic leukemia (ALL). Hypersensitivity reactions (HSR) are the most common side effects and interfere with the antineoplastic activity of the drug. This study aims to compare the intramuscular (IM) and intravenous (IV) administration routes of Native Escherichia coli Lasparaginase (L-ASNase) in terms of hypersensitive reactions. METHODS: L-ASNase was randomly administered IV or IM to newly diagnosed ALL patients and HSR was monitored in all patients for 1 h following the end of the IV infusion and for 2 h following the end of the IM administration of L-ASNase. Based on a retrospective review of clinical charts, reactions were identified. In order to determine the severity of each allergic reaction, we used the Common Terminology Criteria for Adverse Events (CTCAE) version 4.03 for allergic reactions. RESULTS: A total of 1032 doses of L-ASNase were administered to 85 patients (42 males and 43 females) during the study period. Among 85 patients, 30 reactions were recorded, which means that 35% of the patients reacted. According to the CTCAE, twenty-nine out of 30 reactions (97%) were grade 2, while one (3%) was grade 4. In terms of individual doses, there was a non-significant trend toward increased incidence of reactions with IV administration (3.8% versus 0.9%, p = 0.064). The rate of reactions was higher in patients who received all IV doses (n: 60) as compared to those who received all IM doses (n: 25) (31.7% vs. 3.5%; chi-square= 8.415, p value=0.04). Based on the risk groups and HSR incidence, it was found that high risk group (HRG) patients were significantly more likely to develop HSR compared to the standart risk group (SRG) and intermediate risk group (MRG) patients (chi-square p = 0.003, CI: 95%; odds ratio: 3.12 and 5.91, respectively). CONCLUSIONS: In conclusion, IM administration of L-ASNase causes significantly less HSR to L-ASNase than the IV route. Patients with HRGALL have a higher risk of HSR. Since L-ASNase is still used in many developing countries and there are problems in the supply of Erwinia chrysanthemi ASNase (Erwinia), LASNase can be administered IM to reduce the frequency of HSR.

Comparison of bone marrow biopsy and fluorodeoxyglucose-positron emission tomography for the assessment of bone marrow infiltration in children with Hodgkin's lymphoma.

PURPOSE: To compare bone marrow biopsy (BMB) with [18F]-fluoro-2-deoxy-d-glucose positron emission tomography/computed tomography (PET/CT) imaging in the demonstration of bone marrow involvement in children with Hodgkin's Lymphoma (HL) and to investigate the effectiveness of PET/CT imaging and thus the necessity for BMB at staging. METHODS: Pediatric patients with HL, who underwent both bilateral iliac BMB and PET/CT imaging at disease staging were retrospectively analyzed. In determining bone marrow involvement (BMinv), BMB and/or first/follow-up PET/CT imaging were eligible for review. RESULTS: Fifty-six patients were included. BMinv was detected by PET/CT imaging in 6/56 (10.7%), whereas the proportion was 3/56 (5.3%) in BMB specimens. Bone marrow biopsies and PET/CT images were concordant in 53/56 (94.6%) patients with BMB specimens missing three cases of BMinv detected by PET/CT. When diagnostic accuracy was calculated, sensitivity, specificity, positive predictive value and negative predictive values for PET/CT were 100%, 100%, 100%, 100%, respectively, and the same values for BMB were 50%, 100%, 100%, 94.3%, respectively. CONCLUSIONS: The results of PET/CT and BMB for staging of pediatric HL patients were compatible, and PET/CT imaging was found to provide high diagnostic performance in determining BMinv. In keeping with earlier research, the current study showed that BMB may not be necessary in every patient at staging, and should be reserved for cases where PET/CT is inconclusive.

An Eight-year, Single-center Experience on Ultrasound Assisted Thrombolysis with Moderate-dose, Slow-infusion Regimen in Pulmonary Embolism.

BACKGROUND: There is limited data on moderate-dose with slow-infusion thrombolytic regimen by ultrasound-asssisted-thrombolysis (USAT) in patients with acute pulmonary embolism (PE). AIMS: In this study, our eight-year experience on USAT with moderate-dose, slow-infusion tissue-type plasminogen activator (t-PA) regimen in patients with PE at intermediate-high- and high-risk was presented, and short-, and long-term effectiveness and safety outcomes were evaluated. METHODS: Our study is based on the retrospective evaluation of 225 patients with PE having multiple comorbidities who underwent USAT. RESULTS: High- and intermediate-high-risk were noted in 14.7% and in 85.3% of patients, respectively. Mean t-PA dosage was 35.4±13.3 mg, and the infusion duration was 26.6±7.7 h. Measures of pulmonary artery (PA) obstruction and right ventricle (RV) dysfunction were improved within days (p<0.0001 for all). During the hospital stay, major and minor bleeding and mortality rates were 6.2%, 12.4%, and 6.2%, respectively. Bleeding and unresolved PE accounted for 50% and 42.8% of in-hospital mortality, respectively. Age, rate, and duration of t-PA were not associated with in-hospital major bleeding and mortality. Oxygen saturation exceeded 90% in 91.2% of patients at discharge. During follow-up of median 962 (610-1894) days, high-risk status related to 30-day mortality, whereas age >65 years was associated with long-term mortality. CONCLUSION: Our real-life experience with USAT with moderate-dose, slow-infusion t-PA regimen in patients with PE at high-and intermediate-high risk demonstrated clinically relevant improvements in PA obstructive burden and RV dysfunction. Age, rate or infusion duration of t-PA was not related to major bleeding or mortality risk, whereas unresolved obstruction remained as a lethal issue.

Predictors of pulmonary hypertension after atrial septal defect closure: Impact of atrial fibrillation.

Background: In this study, we aimed to evaluate the course of pulmonary artery systolic pressure, to identify factors associated with pulmonary arterial hypertension, and to determine the impact of atrial septal defect closure on clinical outcomes in long-term follow-up. Methods: Between March 2008 and August 2020, a total of 547 adult patients (193 males, 354 females; median age: 37 years; range, 27.5 to 47 years) with secundum atrial septal defect were retrospectively analyzed. Of these patients, 304 underwent percutaneous defect closure and 243 underwent isolated surgical repairs. Pulmonary arterial hypertension was defined as a noninvasively estimated pulmonary artery systolic pressure of ≥40 mmHg at the final follow-up after atrial septal defect closure. Factors associated with pulmonary arterial hypertension were analyzed. Results: Sixty-nine (12.6%) patients presented with pulmonary arterial hypertension at the final follow-up. A total of 35 (6.4%) patients had persistent atrial fibrillation before atrial septal defect closure, and 22 of these 35 patients had pulmonary arterial hypertension during long-term follow-up. Older age at the time of atrial septal defect closure (HR: 4.76; 95% CI: 2.68-8.44; p<0.001), the presence of persistent atrial fibrillation (HR: 2.18; 95% CI: 1.21-3.91; p=0.009), and greater right ventricular basal diameter (HR: 4.78; 95% CI: 2.57-8.84; p<0.001) were found to be associated with late pulmonary arterial hypertension. Conclusion: The presence of persistent atrial fibrillation may be used to predict patients at higher risk for pulmonary arterial hypertension after atrial septal defect closure.

Clinical, imaging and hemodynamic correlates and prognostic impact of syncope in acute pulmonary embolism: A single-center study.

Background: We aimed to determine the clinical, echocardiographic and hemodynamic correlates of syncope as a presenting symptom in pulmonary embolism and its impact on in-hospital and long-term outcomes. Methods: Between July 2012 and October 2019, a total of 641 patients with PE (277 males, 364 females; median age: 65 years; range, 51 to 74 years) in whom the diagnostic work-up and risk-based management were performed according to the current pulmonary embolism guidelines were retrospectively analyzed. Clinical, laboratory and imaging data of the patients were obtained from hospital database system. Results: Syncope was noted in 193 (30.2%) of patients on admission, and was associated with a significantly higher-risk status manifested by elevated troponin and D-dimer levels, a higher Pulmonary Embolism Severity Index scores, deterioration of right-to-left ventricular diameter ratio, right ventricular longitudinal contraction measures, the higher Qanadli score, and higher rates of thrombolytic therapies (p<0.001) and rheolytic-thrombectomy (p=0.037) therapies. In-hospital mortality (p=0.007) and minor bleeding (p<0.001) were significantly higher in syncope subgroup. Multivariate logistic regression analysis showed that higher Pulmonary Embolism Severity Index scores and right-to-left ventricular diameter ratio were independently associated with syncope, while aging and increased heart rate predicted in-hospital mortality. Malignancy and right-to-left ventricular diameter ratio at discharge, but not syncope, were independent predictors of cumulative mortality during follow-up. Conclusion: Syncope as the presenting symptom is associated with a higher risk due to more severe obstructive pressure load and right ventricular dysfunction requiring more proactive strategies in patients with pulmonary embolism. However, with appropriate risk-based therapies, neither in-hospital mortality nor long-term mortality can be predicted by syncope.

The Consistent Effectiveness and Safety of Macitentan Therapies Across Idiopathic and Congenital Heart Disease-Associated PulmonaryArterial Hypertension: A Single-Center Experience.

BACKGROUND: In this single-center study, we evaluated efficacy and safety issues and predictors of survival in patients with idiopathic and congenital heart disease-associated pulmonary arterial hypertension who were under macitentan therapies. METHOD: Our study retrospectively evaluated 221 patients with pulmonary arterial hypertension enrolled in our single-center study, and mono, dual, and triple macitentan therapies were noted in 30, 115, and 76 patients, respectively. The longitudinal changes in clinical, neurohumoral, and echocardiographic measures of pulmonary arterial hypertension were evaluated. The Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management, Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management 2.0, and Registry to Evaluate Early and Long- Term Pulmonary Arterial Hypertension Disease Management Lite 2 scores at baseline, Swedish PAH Registry, Comparative Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension registry, and French Pulmonary Hypertension Network registry risk status both at baseline and first control were assessed. RESULT: The median follow-up period was 1068 [415-2245] days. Macitentan was associated with significant improvements in functional class, 6-minute walk distance, N-terminal pro-brain natriuretic peptide (NT-proBNP), and echocardiographic measures without any deterioration of hemoglobin or hepatic enzymes. The low-risk scores with each model at baseline and/or first control are related to significantly better survival. Age, gender, and log-NT-proBNP in time-fixed and idiopathic pulmonary arterial hypertension, and log-NT-proBNP in time-dependent Cox proportional hazard regression analyses were independent predictors of mortality. CONCLUSION: Mono- or sequential combination macitentan therapies were associated with sustained benefits in functional class, 6-minute walk distance, NT-proBNP, and echocardiographic measures in patients with idiopathic pulmonary arterial hypertension and congenital heart disease-associated pulmonary arterial hypertension, and low-risk scores at baseline and/or first controls can be translated to better survival.

Eosinophil-to-Monocyte Ratio as a Candidate for a Novel Prognostic Marker in Acute Pulmonary Embolism: Is it a Consumptive Mechanism?

BACKGROUND: The role of eosinophils in thrombotic processes is well known, and the prognostic value of eosinophil to monocyte ratio had been determined in patients with ST elevated myocardial infarction and acute ischemic stroke in recent studies. We aimed to evaluate the impact of the eosinophil-to-monocyte ratio on short- and long-term allcause mortality in patients with pulmonary embolism, which is another clinical condition closely related to the thrombotic pathway. METHODS: In this study, a total of 212 retrospectively evaluated patients with intermediate-high risk and high-risk pulmonary embolism who underwent catheter-directed therapies with ultrasound-assisted thrombolysis or rheolytic thrombectomy (Angiojet©) and intravenous thrombolytic treatment were included. RESULTS: The median Pulmonary Embolism Severity Index score was 105 (86-128; interquartile range: 25-75, min-max: 35-250). The intermediate-high status and high-risk status were noted in 83.5% and 16.5% of the patients, respectively. All of the reperfusion strategies resulted in significant improvements in the measures of pulmonary arterial pressure and right ventricular strain. Death was recorded in 42 (18.6%) patients during the follow-up period (median 1029 days, interquartile range: 651-1358). Multiple Cox regression analysis revealed that a higher pulmonary embolism severity index score (from 85 to 128; hazard ratio=3.00; 95% CI: 2.11-4.29; P < .001) and a lower eosinophil-to-monocyte ratio (from 0.02 to 0.24; hazard ratio=0.56; 95% CI: 0.34-0.98; P = .032) were 2 independent predictors for long-term all-cause mortality. The eosinophil-to-monocyte ratio at the admission of less than 0.03 was documented to be associated with higher mortality (P < .001). CONCLUSION: Our results revealed that a lower eosinophil-to-monocyte ratio and a higher pulmonary embolism severity index score independently predict the long-term mortality in patients with intermediate-high- and high-risk pulmonary embolism.

Maternal and Fetal Outcomes in Pregnant Women with Pulmonary Arterial Hypertension: A Single-Center Experience and Review of Current Literature.

BACKGROUND: Although pregnancy in women with pulmonary arterial hypertension has been considered a high-risk condition, current data regarding pregnancy with pulmonary arterial hypertension are scarce. In this study, we aimed to evaluate our single-center data on maternal and fetal outcomes in pregnant women with PAH and review currently available risk-based management strategies. METHODS: Our single-center study group comprised 35 women who became pregnant after the diagnosis of pulmonary arterial hypertension or in whom pulmonary arterial hypertension was diagnosed within early post-partum period. Clinical, laboratory, echocardiographic, and hemodynamic characteristics of pregnant and non-pregnant productive women with pulmonary arterial hypertension were compared, and similar comparison was also repeated for survivors and non-survivors in pregnant patient group. RESULTS: Pregnancy was noted in 15% of the 228 females with pulmonary arterial hypertension who were of hormonally productive ages, generally well-tolerated until delivery. Elective abortion and pre-term delivery were documented in 1 (2.8%) and 12 (35.3%) pregnant women, respectively. Switching to sildenafil was the standard medication during pregnancy. Cesarian section was the preferred method of delivery in all pregnant women with pulmonary arterial hypertension and was performed without any complication. Clinic deteoriation within the first week of delivery was observed in 5 (41.6%) patients. Maternal mortality was noted in 13 (37.1%) patients and was documented to cumulate within the first month of delivery. However, any sign predicting post-partum clinical deterioration was not found. No fetal mortality was observed. CONCLUSION: Despite the development of advanced therapies, pregnancy in pulmonary arterial hypertension still carries a high mortality risk and requires multi-disciplinary expert center care with more proactive management strategies.

Central Nervous System Fungal Infections in Children With Leukemia and Undergoing Hematopoietic Stem Cell Transplantation: A Retrospective Multicenter Study.

BACKGROUND: Central nervous system fungal infections (CNSFI) are seen in patients with hematologic malignancies and have high morbidity and mortality. Because of their rarity, there is limited data on CNSFI in children with no established treatment protocols or guidelines. MATERIALS AND METHODS: In this multicenter retrospective study, 51 pediatric patients with leukemia, 6 of whom had undergone bone marrow transplantation, with proven or probable CNSFI were evaluated. Fungal infections were defined as proven or probable based on European Organisation for Research and Treatment of Cancer criteria. Proven CNSFI was diagnosed by appropriate central nervous system (CNS) imaging or tissue sample findings in combination with positive microbiological results of cerebrospinal fluid. A positive culture, microscopic evidence of hyphae, a positive result of the galactomannan assays are defined as positive microbiological evidence. Probable CNSFI was defined as appropriate CNS imaging findings together with proven or probable invasive fungal infections at another focus without CNS when there is no other explanatory condition. Data was collected by using the questionnaire form (Supplemental Digital Content 1, http://links.lww.com/JPHO/A541 ). RESULTS: Seventeen patients had proven, 34 patients had probable CNSFI. Headaches and seizures were the most common clinical findings. The median time between the onset of fever and diagnosis was 5 days. The most common fungal agent identified was Aspergillus . Sixteen patients received single-agent, 35 received combination antifungal therapy. Surgery was performed in 23 patients. Twenty-two patients (43%) died, 29 of the CNSFI episodes recovered with a 20% neurological sequelae. CONCLUSION: CNSFIs should be considered in the differential diagnosis in patients with leukemia and refractory/recurrent fever, headache, neurologicalocular symptoms, and a radiologic-serological evaluation should be performed immediately. Early diagnosis and prompt management, both medical and surgical, are essential for improving clinical outcomes.

Relationship between right atrial pressure and the Model for End-Stage Liver Disease (MELD) score in patients with advanced heart failure.

Background: This study aims to investigate the association between right atrial pressure and the Model for End-Stage Liver Disease score and to evaluate the ability of this scoring system to accurately predict the value of right atrial pressure. Methods: Between April 2016 and November 2018, a total of 137 patients (118 males, 19 females; median age: 49 years; range, 40 to 54 years) with advanced heart failure who were candidates for left ventricular assist device implantation or heart transplantation were retrospectively analyzed. We developed a formula calculated by using the biochemical and cardiac catheterization parameters of the patients. Results: There was a strong correlation between the right atrial pressure and the scores (r=0.510, p<0.001). The estimated right atrial pressure was calculated as "2 + (0.92 x Model for End-Stage Liver Disease Score)" (unstandardized coefficient 0.920, t value 7.674, p<0.001). The Model for End-Stage Liver Disease score was found to be an independent predictor of high right atrial pressure (odds ratio=1.491, 95% confidence interval: 1.244-1.786, p<0.001). The calculated area under the curve was 0.789 (95% confidence interval: 0.710-0.867, p<0.001) and the cut-off value of the Model for End-Stage Liver Disease score in the prediction of high right atrial pressure was 10.5 with 75% sensitivity and 73% specificity. Conclusion: We define a method to calculate right atrial pressure obtained by using the Model for End-Stage Liver Disease score without the need for cardiac catheterization during the hospitalization and follow-up period of patients with advanced heart failure.

Prognostic Impact of Nutritional Indices in Candidates for Heart Transplantation.

OBJECTIVE: No study has thus far evaluated the association of controlling nutritional status (CO NUT) score and prognostic nutritional index (PNI) with prognosis in candidates listed for heart transplantation (HT). Therefore, in this study, we aimed to investigate the impact of these nutritional indices on prognosis in these candidates. METHODS: In this retrospective study, a total of 195 candidates for HT were included. Over a median follow-up period of 503.5 days, the patients were grouped as survivors (n=121) and non-survivors (n = 74). Malnutrition was defined as CONUT score ≥2 (CONUT-defined malnu trition) and PNI ≤38 (PNI-defined malnutrition). RESULTS: The CONUT-defined malnutrition was observed in 19.8% and 39.2% of the survivors and non-survivors (P = .003), and the PNI-defined malnutrition was observed in 7.4% and 16.2% of the survivors and non-survivors (P = .032). The univariate analysis revealed that the CONUT score from 0 to 2 (hazard ratio [HR]: 1.41, 95% confidence interval [CI]: 1.11-1.79, P =.004) and PNI from 45.5 to 54.5 (HR: 0.78, 95% CI: 0.64-0.95, P = .001), the CONUT-defined malnutrition (HR: 2.48, 95% CI: 1.55-3.97, P < .001) and the PNI-defined malnutrition (HR: 1.97, 95% CI: 1.01-3.86, P = .04) were associated with mortality. In the multivariate adjusted models, the CO NUT-defined malnutrition was an independent predictor of mortality, whereas the PNI-defined malnutrition was not a predictor of mortality (HR: 1.92, 95% CI: 1.12-3.27, P = .001 and HR: 1.64, 95% CI: 0.80-3.40, P = .18). The log-rank test revealed that the CONUT-defined malnutri tion and the PNI-defined malnutrition were associated with decrease in survival rate. CONCLUSION: Although both the CONUT score and the PNI score were associated with prognosis in candidates for HT, the CONUT score was superior to the PNI score in predicting mortality.