Long-acting cilostazol versus isosorbide mononitrate for patients with vasospastic angina: a randomized controlled trial.

BACKGROUND: Cilostazol has a vasodilatory function that may be beneficial for patients with vasospastic angina (VSA). We conducted a randomized, open-label, controlled trial to compare the efficacy and safety of long-acting cilostazol and isosorbide mononitrate (ISMN) for VSA. METHODS: The study included patients with confirmed VSA between September 2019 and May 2021. Participants were randomly assigned to receive long-acting cilostazol (test group, 200 mg once daily) or conventional ISMN therapy (control group, 20 mg twice daily) for 4 weeks. The clinical efficacy and safety were evaluated using weekly questionnaires. RESULTS: Forty patients were enrolled in the study (long-acting cilostazol, n = 20; ISMN, n = 20). Baseline characteristics were balanced between the two groups. Long acting cilostazol showed better angina symptom control within the first week compared to ISMN [reduction of pain intensity score, 6.0 (4.0-8.0) vs. 4.0 (1.0-5.0), P = 0.005; frequency of angina symptom, 0 (0-2.0) vs. 2.0 (0-3.0), P = 0.027, respectively]. The rate of neurological adverse reactions was lower in the cilostazol group than in the ISMN group (headache or dizziness, 40 vs. 85%, P = 0.009; headache, 30 vs. 70%, P = 0.027). CONCLUSION: Long-acting cilostazol provided comparable control of angina and fewer adverse neurologic reactions within 4 weeks compared to ISMN. Long-acting cilostazol provides more intensive control of angina within 1 week, suggesting that it may be an initial choice for the treatment of VSA.

Novel percutaneous technique for creation of porcine model of tricuspid regurgitation via two routes.

OBJECTIVE: To develop an experimental porcine model of tricuspid regurgitation using two induction routes: the inferior vena cava and superior vena cava. METHODS: Tricuspid regurgitation was generated using the loop wire cutting method. The tricuspid regurgitation jet direction was controlled by accessing the valve through the inferior (n = 7) or superior (n = 6) vena cava. The occurrence, direction, and progression of tricuspid regurgitation were assessed postoperatively, and echocardiography was performed at 4 to 6 weeks. Right heart dilatation was assessed using computed tomography after 6 weeks. RESULTS: Moderate to severe or torrential tricuspid regurgitation occurred immediately after the procedure in 12 of 13 animals. The jet was directed toward the septum in five of seven animals in the inferior vena cava group and toward the posterolateral side in four of six animals in the superior vena cava group. The dimensions of the right heart (right atrium, ventricle, and tricuspid valve annulus) were significantly enlarged at the 4- to 6-week follow-up echocardiographic examination and confirmed to be enlarged by computed tomography, independent of the route used. CONCLUSION: The loop wire cutting method successfully induced a disease model of tricuspid regurgitation while controlling the regurgitation jet direction via two procedural routes.

Conversion of the bronchial tree into a conforming electrode to ablate the lung nodule in a porcine model.

BACKGROUND: Radiofrequency ablation (RFA) is one of the treatment options for lung nodules. However, the need for exact delivery of the rigid metal electrode into the center of the target mass often leads to complications or suboptimal results. To overcome these limitations, a concept of conforming electrodes using a flexible material has been tested in this study. METHODS: A bronchoscopy-guided RFA (CAROL) under a temperature-controlled mode was tested in in-vivo and ex-vivo porcine lungs. Gallium-based liquid metal was used for turning the bronchial tree into temporary RF electrodes. A customized bronchoscopy-guided balloon-tipped guiding catheter (CAROL catheter) was used to make the procedure feasible under fluoroscopy imaging guidance. The computer simulation was also performed to gain further insight into the ablation results. Safety was also assessed including the liquid metal remaining in the body. RESULTS: The bronchial electrode injected from the CAROL catheter was able to turn the target site bronchial air pipe into a temporally multi-tined RF electrode. The mean volume of Gallium for each effective CAROL was 0.46 ± 0.47 ml. The ablation results showed highly efficacious and consistent results, especially in the peripheral lung. Most bronchial electrodes were also retrieved by either bronchoscopic suction immediately after the procedure or by natural expectoration thereafter. The liquid metal used in these experiments did not have any significant safety issues. Computer simulation also supports these results. CONCLUSION: The CAROL ablation was very effective and safe in porcine lungs showing encouraging potential to overcome the conventional approaches.

Lung cancer can be treated by inserting a metal device into the lung via the throat and using this to send radio waves into the cancer. However, using a rigid metal device can cause damage to other areas of the lung and can only treat small cancers. Here, we describe an alternative method to treat lung cancers in which liquid metal is used to fill the spaces within the lung closest to the cancer. We demonstrate that this method can be used to treat cancer in a swine model of lung cancer. Given the positive results we obtained, we think this approach should be tested in a clinical trial in human patients with lung cancer, as it might improve cancer treatment.

The Development of a Permanent Implantable Spacer with the Function of Size Adjustability for Customized Treatment of Regurgitant Heart Valve Disease.

The Pivot Mandu is an innovative device featuring a leak-tight adjustable 3D balloon spacer, incorporating inner mesh support, an outer e-PTFE layer, and a compliant balloon in the middle layer with a specialized detachable system. To assess its feasibility, proof of concept was rigorously evaluated through bench testing and survival porcine animal experiments. The results demonstrated successful remote inflation of the balloon system, with the balloon spacer exhibiting sustained patent and functional integrity over an extended observation period of up to 6 months. A noteworthy feature of the newly designed 3D balloon spacer is its capability for easy size adjustment during procedures, enhancing its adaptability and practicality in clinical settings. This three-layered 3D balloon spacer, with its established long-term patency, exhibits highly encouraging outcomes that hold promise in overcoming the current limitations of spacer devices for heart valve diseases. Given the compelling results from preclinical investigations, the translation of the Pivot Mandu into human trials is strongly warranted.

The Clinical Outcomes of Marginal Donor Hearts: A Single Center Experience.

BACKGROUND AND OBJECTIVES: Although the shortage of donor is a common problem worldwide, a significant portion of unutilized hearts are classified as marginal donor (MD) hearts. However, research on the correlation between the MD and the prognosis of heart transplantation (HTx) is lacking. This study was conducted to investigate the clinical impact of MD in HTx. METHODS: Consecutive 73 HTxs during 2014 and 2021 in a tertiary hospital were analyzed. MD was defined as follows; a donor age >55 years, left ventricular ejection fraction <50%, cold ischemic time >240 minutes, or significant cardiac structural problems. Preoperative characteristics and postoperative hemodynamic data, primary graft dysfunction (PGD), and the survival rate were analyzed. Risk stratification by Index for Mortality Prediction after Cardiac Transplantation (IMPACT) score was performed to examine the outcomes according to the recipient state. Each group was sub-divided into 2 risk groups according to the IMPACT score (low <10 vs. high ≥10). RESULTS: A total of 32 (43.8%) patients received an organ from MDs. Extracorporeal membrane oxygenation was more frequent in the non-MD group (34.4% vs. 70.7, p=0.007) There was no significant difference in PGD, 30-day mortality and long-term survival between groups. In the subgroup analysis, early outcomes did not differ between low- and high-risk groups. However, the long-term survival was better in the low-risk group (p=0.01). CONCLUSIONS: The outcomes of MD group were not significantly different from non-MD group. Particularly, in low-risk recipient, the MD group showed excellent early and long-term outcomes. These results suggest the usability of selected MD hearts without increasing adverse events.

Direct Extracorporeal Membrane Oxygenation Bridged Heart Transplantation: The Importance of Multi-Organ Failure.

Background and Objectives: Recently, approximately 40% of all heart transplantation (HTx) in South Korea are performed using the direct extracorporeal membrane oxygenation (ECMO) bridging method. We conducted a study to examine the clinical outcome of direct ECMO-bridged HTx and to investigate the impact of multi-organ failure (MOF). Methods: From June 2014 to September 2022, a total of 96 adult patients who underwent isolated HTx at a single tertiary hospital were included in the study. The patients were sub-grouped into ECMO (n=48) and non-ECMO group (n=48), and the ECMO group was subdivided into awake (n=22) and non-awake (n=26) groups based on mechanical ventilator (MV) dependency. Baseline characteristics, 30-day, and 1-year mortality were analyzed retrospectively. Results: The 1-year survival rate was significantly lower in the ECMO group (72.9% vs. 95.8%, p=0.002). There was a significant difference in the 30-day survival rate between the awake and non-awake ECMO groups (81.8% vs. 65.4%, p=0.032). In the univariate analysis of logistic regression for 1-year mortality, the odds ratio was 8.5 for ECMO bridged HTx compared to the non-ECMO group, 12.3 in patients who required MV (p=0.003), and 23 with additional hemodialysis (p<0.001). Conclusions: Patients who required MV in ECMO bridged HTx showed higher preoperative MOF rates and early mortality than those extubated. When considering ECMO bridged HTx, the severity of MOF should be thoroughly investigated, and careful patient selection is necessary.

Oral anticoagulants and concurrent rifampin administration in tuberculosis patients with non-valvular atrial fibrillation.

BACKGROUND: Evidence and guidelines for Non-vitamin K antagonist oral anticoagulants (NOACs) use when prescribing concurrent rifampin for tuberculosis treatment in patients with non-valvular atrial fibrillation (NVAF) are limited. METHODS: Using the Korean National Health Insurance Service database from January 2009 to December 2018, we performed a population-based retrospective cohort study to assess the net adverse clinical events (NACE), a composite of ischemic stroke or systemic embolism and major bleeding, of NOACs compared with warfarin among NVAF patients taking concurrent rifampin administration for tuberculosis treatment. After a propensity matching score (PSM) analysis, Cox proportional hazards regression was performed in matched cohorts to investigate the clinical outcomes. RESULTS: Of the 735 consecutive patients selected, 465 (63.3%) received warfarin and 270 (36.7%) received NOACs. Among 254 pairs of patients after PSM, the crude incidence rate of NACE was 25.6 in NOAC group and 32.8 per 100 person-years in warfarin group. There was no significant difference between NOAC and warfarin use in NACE (hazard ratio [HR], 0.74; 95% confidence interval [CI], 0.48-1.14; P = 0.172). Major bleeding was the main driver of NACE, and NOAC use was associated with a statistically significantly lower risk of major bleeding than that with warfarin use (HR, 0.63; 95% CI, 0.40-1.00; P = 0.0499). CONCLUSIONS: In our population-based study, there was no statically significant difference in the occurrence of NACE between NOAC and warfarin use. NOAC use may be associated with a lower risk of major bleeding than that with warfarin use.

Safety and efficacy of immediate heparin reversal with protamine after complex percutaneous coronary intervention.

BACKGROUND: Compared to simple percutaneous coronary intervention (PCI), complex PCI is associated with higher bleeding and thrombotic risk. No previous study has evaluated the use of protamine after PCI with contemporary technologies. This study aimed to evaluate the safety and efficacy of manual compression with and without protamine after transfemoral complex PCI. METHODS: We retrospectively analyzed 160 patients (protamine group, n = 92; non-protamine group, n = 68) who underwent complex PCI via the femoral artery. The primary outcome was a composite of in-hospital death, myocardial infarction, stent thrombosis, stroke/systemic embolism, bleeding requiring blood transfusion, and vascular access complications. RESULTS: The primary outcome was significantly lower in the protamine group than in the non-protamine group (4.3% vs. 17.6%; p = 0.006). This was driven mainly by the lower incidences of hematoma in the protamine group (3.3% vs. 13.2%, p = 0.020). Furthermore, the protamine group had a significantly shorter hospital stay than the non-protamine group (4.8 ± 3.7 days vs. 8.4 ± 8.3 days, p = 0.001). While > 90% of the patients had acute coronary syndrome, there were no incidences of myocardial infarction or stent thrombosis in either group. CONCLUSIONS: Among patients who underwent complex PCI via transfemoral access, immediate protamine administration was associated with a significantly lower rate of vascular access complications, especially hematoma, and shorter hospital stay than no protamine administration.

The Cobra Catheter: A Novel Concept and Device for the Thru-Septal Myocardium Approach.

Objectives: In our previous study, we suggested the novel septal traversing technique as effective and safe in catheter-based approach for septal myocardium. However, it is limited by its dependence on the septal perforator vein. This study aimed to evaluate the Cobra catheter as a backup catheter to overcome this limitation in swine. Methods: We designed the guiding Cobra catheter. It consisted of three major parts (the external pull-wire steerable distal tip, the C-shaped shaft, and the steering adjustment handle). We tested the difference in force between the guidewire passing through the muscle and the vessel wall using a push-pull gauge. We performed a septal wire engage procedure in swine using the Cobra catheter. The guidewire engagement of the septal vein and Cobra catheter were compared visually and histopathologically. Results: A total of ten swine were enrolled in this study. The success rate was 100% under fluoroscopy. The experiments confirmed the medical potential of the septal approach even in a location irrelevant to the septal perforator vein anatomy and confirmed that the wire passed well in the target direction in the harvested heart. There was no serious physical damage or pathological abnormalities in the vessel wall and myocardium. Conclusion: These results showed that the novel Cobra catheter with a septal vein-independent trans-septal approach may be a safe and effective alternative for the treatment of structural heart diseases.

A Novel Device for Tricuspid Regurgitation Reduction Featuring 3-Dimensional Leaflet and Atraumatic Anchor: Pivot-TR System.

A new device called the Pivot-TR system was designed to treat tricuspid regurgitation with a novel spacer crossing the valve vertically. Its unique atraumatic anchoring system composed of both the elephant long nose and the inferior vena cava spiral anchor, in addition to the relatively easy implantation mechanism, enabled easy retrieval of the system later on. The system showed promising feasibility and safety results in this swine-based animal experiment, which should encourage human translation study.

Sodium/glucose Co-Transporter 2 Inhibitor, Empagliflozin, Alleviated Transient Expression of SGLT2 after Myocardial Infarction.

BACKGROUND AND OBJECTIVES: Large clinical studies of sodium/glucose cotransporter 2 (SGLT2) inhibitors have shown a significant beneficial effect on heart failure-associated hospitalization and cardiovascular events. As SGLT2 is known to be absent in heart cells, improved cardiovascular outcomes are thought to be accounted for by the indirect effects of the drug. We sought to confirm whether such benefits were mediated through SGLT2 expressed in the heart using myocardial infarction (MI) model. METHODS: Mice pre-treated with empagliflozin (EMPA), an SGLT2 inhibitor, showed a significantly reduced infarct size compared with the vehicle group three days post-MI. Interestingly, we confirmed SGLT2 localized in the infarct zone. The sequential changes of SGLT2 expression after MI were also evaluated. RESULTS: One day after MI, SGLT2 transiently appeared in the ischemic areas in the vehicle group and increased until 72 hours. The appearance of SGLT2 was delayed and less in amount compared with the vehicle group. Additionally, there was a significant difference in metabolites, including glucose and amino acids in the ¹H nuclear magnetic resonance analysis between groups. CONCLUSIONS: Our work demonstrates that SGLT2 is transiently expressed in heart tissue early after MI and EMPA may directly operate on SGLT2 to facilitate metabolic substrates shifts.

Septal Reduction Using Transvenous Intramyocardial Cerclage Radiofrequency Ablation: Preclinical Feasibility.

Debulking of left ventricular septal mass is typically accomplished using surgical myectomy, which is morbid, or using transcoronary alcohol septal ablation, which can result in geographic miss and occasional catastrophic nontarget coronary injury. The authors developed and tested operational parameters in vitro and vivo for a device to accomplish transvenous intraseptal radiofrequency ablation to reduce ventricular septal mass using a technique derived from mitral cerclage, which the authors call cerclage ablation. Cerclage ablation appeared feasible in vitro and safe and effective in vivo. Cerclage ablation is an attractive new approach to debulk the interventricular septum in obstructive hypertrophic cardiomyopathy. These data support clinical investigation.

Incidence and clinical impact of early recurrence of atrial tachyarrhythmia after surgical ablation for atrial fibrillation.

BACKGROUND: Atrial tachyarrhythmias (ATAs) are common within the 3-month blanking period after catheter ablation of atrial fibrillation (AF). However, little evidence is available regarding the current guidelines on the blanking period after surgical AF ablation. We investigate the incidence and significance of early recurrence of atrial tachyarrhythmia (ERAT) and evaluate the optimal blanking period after surgical AF ablation. METHODS: Data from 259 patients who underwent surgical AF ablation from 2009 to 2016 were collected. ERAT was defined as documented ATA episodes lasting for 30 s. A multivariate Cox proportional hazard model was constructed to evaluate the role of ERAT as a predictor of late recurrences (LR) for AF. RESULTS: In total, 127 patients (49.0%) experienced their last episodes of ERAT during the first (n = 65), second (n = 14), or third (n = 48) month of the 3-month blanking period (p < .001). One year freedom from ATAs was 97.8% in patients without ERAT compared with 95.4%, 64.3%, and 8.3% in patients with ERAT in the first, second, and third months after the index procedure, respectively (p < .001). Hazard ratios of LR according to the timing of the last episode of ERAT first, second, and third months after the procedure were 2.84, 16.70, and 119.75, respectively. CONCLUSIONS: The ERAT occurred in 49.0% of patients within the first 3 months after surgical ablation. The occurrence of ERAT within 3 months after surgical AF ablation was a significant independent predictor of LR. Hence, the currently accepted 3-month blanking period may be considered for redefining in patients with AF surgical ablation.

Incidence of Cardiovascular Events and Safety Profile of Prasugrel in Korean Patients With Acute Coronary Syndrome.

BACKGROUND: Dual antiplatelet therapy is commonly used for patients with acute coronary syndrome (ACS). This study aimed to evaluate the safety and efficacy of aspirin and prasugrel at standard dosages in Korean patients using clinical outcome data.Methods and Results:For this prospective multicenter phase IV post-marketing surveillance (PMS) study, ACS patients from 29 July 2012 to 28 July 2016 were recruited. Patients received aspirin at a dose of 75-150 mg daily and a standard dose of prasugrel. Bleeding events were recorded and summarized to evaluate safety. Data on adverse events (AEs) and composite events such as cardiovascular (CV) death, myocardial infarction (MI), and stroke were recorded and summarized to assess efficacy. Of the 3,283 patients recruited, data from 3,110 and 3,044 patients were included in the safety and efficacy analyses, respectively (median treatment duration, 172 days). The most frequently reported AE was ecchymosis (2.8%). The number of patients with major bleeding was 29/3,110 (0.93%). The discontinuation rate for any reason was 12.6%. The number of cases that ended in CV death, MI, stroke, stent thrombosis, or unplanned coronary revascularization was 26/3,044 (0.85%). CONCLUSIONS: The present results are similar to those observed in clinical trials where administration of low-dose aspirin plus prasugrel was associated with a low rate of major bleeding and CV events.

Heart-lung transplantation for refractory diffuse alveolar hemorrhage supported by prolonged extracorporeal membrane oxygenation after cardiogenic shock from ST segment elevation myocardial infarction: a case report.

Diffuse alveolar hemorrhage after percutaneous coronary intervention is a rare but fatal complication. Although timely application of extracorporeal membrane oxygenator and discontinuation of antiplatelet/anticoagulation is the treatment of choice, bleeding is often irreversible. Herein, we introduce a patient with refractory diffuse alveolar hemorrhage after prolonged extracorporeal membrane oxygenator and percutaneous coronary intervention, who was eventually rescued with heart-lung transplantation.

Value of Coronary Angiography in the Cardiac Myxoma.

Preoperative coronary angiography for cardiac myxoma not only excludes coronary artery disease but also detects the artery feeding the cardiac myxoma, which has several clinical implications. In this study, we examined cardiac myxoma cases in two tertiary hospitals using coronary angiography to identify the artery feeding the myxoma. We retrospectively reviewed 42 patients with cardiac myxoma who had undergone surgical removal between July 2008 and December 2015 in two tertiary hospitals, and recorded their baseline characteristics, echocardiographic findings, and coronary angiography. Among those 42 patients, 23 (55%) had coronary angiography before surgery and in no case was significant luminal narrowing observed. In 21 of the coronary angiograms, the artery feeding the cardiac myxoma had a vascular branch (100%), clusters of tortuous vessels with contrast medium pooling (67%), an arteriocavity fistula (33%), and a mobile feeding artery (67%). No significant relationship was found between coronary artery dominance type and the origin of the artery feeding the cardiac myxoma (P = 0.362). Identification of the artery feeding the cardiac myxoma, with a distinctive vascular appearance in coronary angiography, is important for several clinical applications such as helping to diagnose cardiac myxoma and to plan the surgical approach. Clin. Anat. 33:833-838, 2020. © 2019 Wiley Periodicals, Inc.

Radiation reduction during percutaneous coronary intervention: A new protocol with a low frame rate and selective fluoroscopic image storage.

The percutaneous coronary intervention (PCI) procedure is associated with potentially high levels of radiation exposure and therefore increased risk of adverse radiation-induced outcomes, ranging from cataract to malignancy. Frame rate reduction and selective fluoroscopy storage may help reduce radiation exposure. In this study, we evaluated the efficacy of a radiation reduction protocol that uses a lower frame rate and selective storage of fluoroscopic images in terms of its effect on reducing the radiation dose during PCI.The new protocol incorporated a lower frame rate as compared with the conventional protocol, and used selective storage of fluoroscopic images. We reviewed the medical records of patients who underwent PCI under the conventional protocol from January 2013 to December 2013, and compared them with those who underwent PCI with the new protocol from January 2015 to December 2015. The primary endpoint was radiation dose reduction expressed as cumulative air kerma and dose-area product (DAP). The image quality was assessed by 3 independent well-trained cardiologists.One hundred fifty-five patients were enrolled in the conventional protocol group, and 152 were enrolled in the radiation reduction protocol group (total, n = 307). There was no statistical significance in terms of the baseline characteristics, including body mass index. Overall, the radiation reduction protocol group showed a significant reduction in both cumulative air kerma (1634.39 ±â€Š717.95 vs 2074.75 ±â€Š1003.72 mGy, P < .001) and DAP (12344.86 ±â€Š5371.75 vs 15312.19 ±â€Š7136.58 µGy m, P < .001). Image quality was acceptable in both groups.The radiation reduction protocol, which uses a lower frame rate and selective storage of fluoroscopic images, may be an alternative approach to reducing PCI radiation dose.

Mitral Loop Cerclage Annuloplasty for Secondary Mitral Regurgitation: First Human Results.

OBJECTIVES: This is an early feasibility clinical test of mitral loop cerclage annuloplasty to treat secondary mitral valve regurgitation. BACKGROUND: Secondary mitral regurgitation is characterized by cardiomyopathy, mitral annular enlargement, and leaflet traction contributing to malcoaptation. Transcatheter mitral loop cerclage applies circumferential compression to the mitral annulus by creating a loop through the coronary sinus across the interventricular septum, protecting entrapped coronary arteries from compression, and interactive annular reduction under echocardiographic guidance. This is the first human test of mitral loop annuloplasty. METHODS: Five subjects with severe symptomatic secondary mitral regurgitation underwent mitral loop cerclage, with echocardiographic and computed tomography follow-up over 6 months. RESULTS: Mitral loop cerclage was successful in 4 of 5 subjects and aborted in 1 of the 5 because of unsuitable septal coronary vein anatomy. Immediately and over 6 months, measures of both mitral valve regurgitation (effective orifice area and regurgitation fraction) and chamber dimensions (left atrial and left ventricular volumes) were reduced progressively and ejection fractions increased. Two with persistent and permanent atrial fibrillation spontaneously reverted to sinus rhythm during follow-up. One subject experienced a small myocardial infarction from an unrecognized small branch coronary occlusion. Another, experiencing cardiogenic shock at baseline, died of intractable heart failure after 6 weeks. CONCLUSIONS: In this first human test, mitral loop cerclage annuloplasty was successful in 4 of 5 attempts, caused reverse remodeling (reduction in secondary mitral regurgitation and heart chamber volumes), and suggested electrical remodeling (reversion of atrial fibrillation). Further evaluation is warranted.

Effects of long-term iloprost treatment on right ventricular function in patients with Eisenmenger syndrome.

BACKGROUND: Right ventricular (RV) function is an important prognostic factor of pulmonary arterial hypertension (PAH), but there is insufficient data regarding RV function after long-term inhaled iloprost treatment. We evaluated the effect of long-term iloprost treatment on RV function in patients with Eisenmenger syndrome (ES). METHODS: Eleven consecutive patients with ES associated with congenital heart disease underwent echocardiographic measurements at baseline and 48 weeks after iloprost therapy. In addition, we recorded World Health Organization (WHO) functional class, 6-minute walk distance (6MWD), systemic arterial oxygen saturation (SaO2), and laboratory values such as hemoglobin, serum creatinine, and N-terminal pro-B natriuretic peptide. RESULTS: After 48 weeks of iloprost therapy, mean pulmonary arterial pressure (mPAP), pulmonary arterial systolic pressure (PASP), and pulmonary vascular resistance (PVR) were significantly decreased [mPAP, 42.5 (38.5-61.0) to 36.5 (29.1-40.0)mmHg; PASP, 92.6±19.9 to 74.5±23.8mmHg; PVR, 23.4 (19.8-26.0) to 23.4 (19.8-26.0)Wood unit respectively, all p<0.05]. There was also significant improvement in RV myocardial performance index [0.68 (0.61-0.80) to 0.52 (0.51-0.62), p=0.003] and RV longitudinal strain (-15.7±1.6 to -18.1±1.5%, p<0.001). In clinical assessment, WHO functional class (p=0.006), 6MWD (310.6±44.7 to 399.7±80.8m, p<0.001), and SaO2 (90.9±6.0% to 92.5±6.0%, p=0.022) were significantly improved. CONCLUSION: The improvement in echocardiographic parameters of the RV function after 48 weeks of iloprost therapy may provide insight on the efficacy of long-term iloprost treatment for RV functional improvement, which is a prognostic factor in patients with ES.