In-stent restenosis estimation following carotid artery stenting: The robust predictive value of atherogenic index of plasma and other non-traditional lipid profiles.

OBJECTIVE: Atherosclerotic carotid artery stenosis is a significant contributor to ischemic strokes, and carotid artery stenting (CAS) has emerged as a pivotal treatment option. However, in-stent restenosis (ISR) remains a concern, impacting the long-term patency of CAS. This study aimed to investigate the predictive value of non-traditional lipid profiles, including the atherogenic index of plasma (AIP), in ISR development. METHODS: This retrospective single-center study involved patients presenting at a tertiary healthcare facility with severe carotid artery disease between 2016 and 2020 who subsequently underwent CAS. A total of 719 patients were included in the study. The study cohort was divided into ISR and non-ISR groups based on restenosis presence, confirmed by angiography following ultrasonographic follow-up assessments. Non-traditional lipid indices, such as AIP, atherogenic index (AI), and lipoprotein combined index (LCI), were evaluated along with traditional risk factors. RESULTS: During a 24-month follow-up, ISR occurred in 4.03% of patients. To determine the predictors of restenosis, three different models were constructed in multivariate analysis for non-traditional lipid indices. Multivariate analysis revealed AIP as a robust independent predictor of ISR (OR: 4.83 (CI 95 % 3.05-6.63, p < .001). Notably, AIP demonstrated superior predictive accuracy compared to AI and LCI, with a higher Area Under the Curve (AUC) of 0.971. CONCLUSION: Non-traditional lipid profiles, especially AIP, were found to be associated with an increased risk of ISR and may serve as predictors of ISR in patients undergoing CAS.

A novel method for the evaluation of right ventricular dysfunction in acute pulmonary embolism: Myocardial work indices.

PURPOSE: The presence of right ventricular dysfunction indicates a higher risk status in patients with pulmonary embolism (PE). The RV strain evaluated by speckle-tracking echocardiography seems to be more reliable method in the evaluation of RV dysfunction as compared to standard echocardiographic measures. In this study, we aimed to determine the value of myocardial-work indices in evaluating serial changes of RV function in acute PE. METHODS: Our study comprised 83 consecutive acute PE patients who admitted to our tertiary cardiovascular hospital. Echocardiography was performed within the first 24-hours of hospitalization, and RV and LV myocardial-work parameters were obtained along with standard echocardiographic parameters. The change in the RV/LVr detected on tomography was selected as the primary outcome measure, and its' predictors were analyzed with classical linear regression and a generalized additive model (GAM). RESULTS: Among the LV-RV strain and myocardial work parameters, the RV global longitudinal strain (GLS) has borderline statistical significance in predicting the RV/LVr change whereas the RV global work efficiency (RV-GWE) strongly predicted RV/LVr change (p: 0.049 and <0.001, respectively). CONCLUSION: In this study, classical linear regression and GAM analyses showed that RV-GWE seems to offer a better prediction of RV/LVr change in patients with acute PE.

Surgical treatment of a difficult case of atrial tachycardia requiring multiple electrophysiologic procedures.

A 34-year-old male with incessant drug-refractory atrial tachycardia (AT) was referred to our clinic for catheter ablation. The procedure began with endocardial activation mapping. The earliest endocardial activation site was in the right atrial appendage (RAA). The procedure continued with mapping of the left atrium through a transseptal approach. The earliest local activation was recorded at the anterior site of the right pulmonary veins. Radiofrequency (RF) ablation of both localizations was performed synchronously but failed to terminate the arrhythmia. The procedure continued with isolation of the RAA using cryoballoon but failed again due to the anomalous structure of the RAA. Then, epicardial RF ablation was attempted but failed. Finally, AT could only be terminated by surgical excision of the RAA.

Temporal association of contamination obsession on the prehospital delay of STEMI during COVID-19 pandemic.

BACKGROUND: One of the modifiable risk factors for ST elevation myocardial infarction is prehospital delay. The purpose of our study was to look at the effect of contamination contamination obsession on prehospital delay compared with other measurements during the Covid-19 pandemic. METHOD: A total of 139 patients with acute STEMI admitted to our heart center from 20 March 2020 to 20 June 2020 were included in this study. If the time interval between the estimated onset of symptoms and admission to the emergency room was >120 min, it was considered as a prehospital delay. The Beck Depression Inventory (BDI), the Beck Anxiety Inventory (BAI), and Padua Inventory-Washington State University Revision (PI-WSUR) test were used to assess Contamination-Obbsessive compulsive disorder (C-OCD). RESULT: The same period STEMI count compared to the previous year decreased 25%. The duration of symptoms onset to hospital admission was longer in the first month compared to second and third months (180 (120-360), 120 (60-180), and 105 (60-180), respectively; P = 0.012). Multivariable logistic regression (model-2) was used to examine the association between 7 candidate predictors (age, gender, diabetes mellitus (DM), hypertension, smoking, pain-onset time, and coronary artery disease (CAD) history), PI-WSUR C-OCD, and admission month with prehospital delay. Among variables, PI-WSUR C-OCD and admission month were independently associated with prehospital delay (OR 5.36 (2.11-13.61) (P = 0.01); 0.26 (0.09-0.87) p < 0.001] respectively]. CONCLUSION: Our study confirmed that contamination obsession was associated with prehospital delay of STEMI patients, however anxiety and depression level was not associated during the pandemic.

Potential renoprotective effect of SGLT2 inhibitors against contrast-induced AKI in diabetic STEMI patients undergoing primary PCI.

BACKGROUND: It has been demonstrated that there is a significant reduction in the incidence of cardiovascular events, mortality rates, and worsening kidney disease in patients using sodium-glucose cotransporter 2 inhibitors (SGLT2i). However, there is limited information about the effect of SGLT2i on the incidence of contrast-induced acute kidney injury (CI-AKI) in patients undergoing primary percutaneous intervention (pPCI). AIMS: Our research was focused on examining how SGLT2i exposure impacts CI-AKI occurrence in patients with ST-segment elevation myocardial infarction (STEMI) and undergoing pPCI. RESULTS: This retrospective, single-center, case-control study included diabetic patients diagnosed with STEMI who underwent pPCI in a tertiary healthcare center between 2021 and 2022. The study population included SGLT2i users (n = 130) and non-SGLT2i users (n = 165). Inverse probability propensity score weighting and doubly robust estimation were performed to decrease bias and to balance covariate distribution for estimating average treatment for those treated. In a doubly robust inverse probability weighted regression model, in which covariates were balanced, CI-AKI risk was also found to be lower in the SGLT2i-user group (OR: 0.86 [0.76-0.98]; 95% CI; P = 0.028). In addition, ejection fraction, admission creatinine, albumin, and volume of contrast media were found to be independent predictors of CI-AKI in patients presenting with STEMI and undergoing pPCI. CONCLUSION: Our study provides evidence supporting the potential protective effect of SGLT2i against CI-AKI in diabetic patients presenting with STEMI and undergoing pPCI.

Complete Percutaneous Retrieval of Migrated VSD Occluder Device from the Pulmonary Artery: Management of a Catastrophic Complication.

Transcatheter closure of muscular ventricular septal defects (VSD) remains a safe and effective method with low complication rates. However, device migration can pose a significant challenge to interventional cardiologists due to potential mortal consequences. A 21-year-old female presented to our clinic with exertional dyspnea and was diagnosed with a muscular VSD. The defect was percutaneously closed using an Amplatzer occluder device. On the first post-procedural day, the patient experienced repeated episodes of coughing and mild hemoptysis. Imaging revealed migration of the VSD occluder device to the right pulmonary artery (PA). Percutaneous retrieval of the device was then decided upon. The right PA was accessed using a hydrophilic guidewire and a pigtail catheter. This catheter was exchanged for an 8-Fr sheathless guide catheter, and a 6-Fr Judkins right catheter was advanced into the right PA through the sheathless guide catheter using the mother-and-child technique. Multiple attempts using a snare were made to retrieve the migrated device. Eventually, the proximal marker point, the hub of the device, was grasped and pulled back from the PA, then externalized through the sheath without the need for surgical cutdown. Our report represents a case of complete percutaneous retrieval of an embolized VSD occluder device from the PA.

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.

Spontaneous Coronary Dissection Induced Cardiac Arrest During Posterior Instrumentation in Prone Position: A Case Report.

Intraoperative cardiac arrest (ICA) is a crucial property of morbidity and mortality for patients undergoing surgical operations. Spontaneous coronary artery dissection (SCAD) is an important cause of ICA and perioperative myocardial infarction, especially in young women. In this case report, we presented the successful management of SCAD-induced ICA in a 46-year-old female patient who underwent posterior spinal instrumentation in the prone position due to lumbar intervertebral disc extrusion.

An Unusual Cause of Syncope: Persistent Hypoglossal Artery, A Case Report.

Persistent hypoglossal artery is a rare primitive anastomose which mainly supplies the posterior cerebral circulation by itself in the case of the absent vertebral arteries. This artery is an embryological remnant and is commonly found incidentally. It originates from the upper cervical vertebra (C1-C2 level) of the internal carotid artery. Here we present a unique case suffering from syncope related to persistent hypoglossal artery which supplies the posterior cerebral blood flow in the absence of the vertebral arteries with the classical findings on magnetic resonance imaging and our clinical approach.

Intravascular Ultrasound-Guided Stent Implantation in the Left Main Coronary Artery Extrinsic Compression by Pulmonary Artery Aneurysm Due to Eisenmenger Syndrome.

Pulmonary arterial hypertension (PAH) is a profoundly destructive condition marked by the gradual narrowing and restructuring of small pulmonary arteries, leading to a rise in pulmonary vascular resistance (PVR), causing right-sided heart failure and, ultimately, mortality. During more advanced stages of this disease, patients may present with rare manifestations of pulmonary artery aneurysm (PAA) which are exertional chest pain, and hoarseness. The left main coronary artery compression (LMCA-Co) has been an increasingly recognized and possibly life-threatening entity in patients with severe PAH. The lack of well-established decision-making strategies for the management of both PAA and LMCA-Co has been remained as an unsolved issue in this setting. In this report, we present a case of percutaneous intervention of LMCA-Co with the guidance of intracoronary imaging in a patient with patent ductus arteriosus-Eisenmenger syndrome. Percutaneous intervention with intravascular guidance appears to be a safe and effective option for relieving symptoms and achieving positive clinical outcomes in patients with LMCA-Co.

Current Insights for Catheter-Directed Therapies in Acute Pulmonary Embolism: Systematic Review and Our Single-Center Experience.

In this review, the current status of the worldwide experience on different catheter-directed treatment systems utilized as alternative reperfusion methods in acute pulmonary-embolism was evaluated, and the risk stratification algorithms in which catheter-directed treatments may be implemented, source of evidence in this setting, adjudication of benefits and risks of available techniques, and innovative multidisciplinary frameworks for referral patterns and care delivery were discussed. Moreover, our perspectives on risk-based catheter-directed treatment utilization strategies in acute pulmonary embolism were summarized.

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.

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.

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.

Modafinil-induced ventricular arrhythmia: A case report.

Modafinil is a central nervous system stimulant that promotes wakefulness and is approved for the treatment of narcolepsy and several other conditions. However, there is a big concern about drug abuse, especially among students to enhance cognitive performance and to reduce the need for sleep. In this case report, we present a 23-year-old female admitted to the cardiology outpatient clinic owing to recurrent palpitations. She stated that she started modafinil 100 mg twice a day one month earlier to increase performance while studying for her exams. Her electrocardiogram (ECG) demonstrated sinus rhythm and a right bundle branch block (RBBB). No structural heart disease or metabolic pathology was detected. A 24-hour ambulatory ECG record showed 11 attacks of non-sustained ventricular tachycardia (NSVT), the longest of which was eight beats. The drug was discontinued and two weeks later, the patient was symptom-free, and her control ECG showed normal sinus rhythm with no RBBB. A control ambulatory ECG was performed, and no ventricular tachycardia was observed. Modafinil, which is considered safer than amphetamine derivatives in terms of cardiovascular side effects, rarely causes serious arrhythmic events, even in healthy subjects. Thus, we suggest evaluating patients for cardiac symptoms after starting on modafinil, and they should be also interrogated regarding the abuse of this drug.

Evaluation of the relationship between diabetic retinopathy and left atrial deformation parameters.

BACKGROUND: Left ventricular systolic dysfunction (LVSD) may develop without coronary artery disease, hypertension (HT), or valvular pathologies in patients with diabetes mellitus (DM), which is defined as diabetic cardiomyopathy (DCM) and its pathophysiology is still unclear. Diabetic retinopathy (DR) is a microvascular complication of DM, and patients with DR have increased risk for the development of heart failure (HF). Two-dimensional speckle tracking echocardiography (2D-STE) evaluates longitudinal deformation in left atrium (LA) myocardium and previous studies utilizing 2D-STE have revealed the detrimental effects of DM on LA functions. Although some studies have shown the association between DR and left ventricle (LV) systolic functions, as far as the researchers of this study investigated, there is no study evaluating the relationship between LA deformation parameters and DR. Hence, we aimed to investigate the relationship between the presence and the degree of DR and LA deformation parameters. RESULTS: LA deformation parameters were analyzed in terms of LA reservoir, conduit, and contractile functions according to the degree of DR. LA reservoir strain value was 14.2 ± 3.6 in normal retina group, 12.2 ± 4.1 in non-proliferative diabetic retinopathy (NPDR) group, and 13 ± 3.7 in proliferative diabetic retinopathy (PDR) group (P = 0.04). LA contractile strain was 15.9 ± 6.8 in normal retina group, 13.1 ± 47.4 in NPDR group, and 9.9 ± 4.7 in PDR group (P < 0.001). LA conduit strain was 30.1 ± 6.6 in normal retina group, 25.3 ± 6.5 in NPDR group, and 22.9 ± 4.9 in PDR group (P < 0.001). Proportional odds regression for association between clinical data, echocardiographic parameters, and LA contractile strain function showed that increasing creatinine (from 0.7 to 1.0; OR 0.71; 95% CI 0.51-0.99; P = 0.04), DR presence (OR 0.24; 95% CI 0.11-0.50; P = 0.001), and increasing left atrial volume index (LAVI) (from 33.5 to 52.6; OR 0.62; 95% CI 0.43-0.89; P = 0.01) were associated with decreasing LA function; however, other variables indicated no association. CONCLUSIONS: Our results showed the relationship between LA deformation parameters and DR, although microvascular involvement is not a certainly defined cardiovascular risk factor. Further prospective studies are needed to determine the clinical importance of DR presence and its degree for deformation parameters.

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.

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.