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A Case of IgG4-Related Disease Developing During Long-Term High-Dose Intravenous Epoprostenol Therapy in a Patient With Idiopathic Pulmonary Arterial Hypertension. (PubMed, Pulm Circ) - "Systemic corticosteroids were initiated, and the patient was transitioned from IV epoprostenol to a combination of IV and inhaled treprostinil; IgG4-RD has remained in remission to date, with normalisation of serum IgG4. This case highlights a practical, minimally invasive diagnostic approach for suspected IgG4-RD in severe PAH and demonstrates the feasibility of prostacyclin modification to sustain PAH control while reducing immunosuppression."

Journal • Cardiovascular • Hypertension • Inflammation • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

A treprostinil inhaler (Yutrepia) for pulmonary hypertension. (PubMed, Med Lett Drugs Ther) - No abstract available

Journal • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Increased Incidence of Thrombosis in Patients at Risk of Heparin-Induced Thrombocytopenia Receiving Epoprostenol for Cardiac Surgery. (PubMed, J Cardiothorac Vasc Anesth) - "The rate of thrombosis in our review was considerably higher than the published data on the use of prostacyclin analogues with heparin in patients with HIT, suggesting that this strategy may not be as effective for preventing thrombosis as previously thought. Until larger studies can be conducted, it may be in the best interest of patients with HIT for surgical teams to utilize alternative agents, such as bivalirudin, for anticoagulation in cardiac surgery."

Journal • Cardiovascular • Hematological Disorders • Thrombocytopenia • Thrombosis

Marked improvement in severe pulmonary arterial hypertension following airway infection in a patient with a heterozygous BMP9 nonsense mutation: a case report. (PubMed, Eur Heart J Case Rep) - "Despite receiving combination therapy with macitentan, riociguat, and intravenous epoprostenol, she continued to have severe pulmonary hypertension with suprasystemic pressure [mean pulmonary artery pressure (PAP) = 65 mmHg] for 4 years following treatment initiation. A patient with severe, treatment-resistant pulmonary hypertension exhibiting a dramatic improvement in pulmonary pressure following an AI is exceedingly rare. The present case may provide new insights into the pathophysiology of PAH."

Journal • Cardiovascular • Hypertension • Infectious Disease • Inflammation • Pneumonia • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

SEVERE PORTOPULMONARY HYPERTENSION IN A POST-LIVER TRANSPLANT PATIENT LEADING TO ACUTE RESPIRATORY FAILURE (CHEST 2025) - "He was started on intravenous epoprostenol and gradually transitioned to sildenafil and macitentan...(2023)[1] reported successful management of POPH with combined tadalafil and macitentan... Severe POPH following LT presents significant management challenges. Early recognition and aggressive treatment are crucial to improving patient outcomes. This case highlights the importance of routine hemodynamic monitoring and pulmonary screening in liver transplant recipients to ensure timely intervention for evolving pulmonary vascular complications."

Clinical • Atrial Fibrillation • Cardiovascular • Chronic Kidney Disease • Fibrosis • Gastroenterology • Gastrointestinal Disorder • Hepatology • Immunology • Metabolic Disorders • Metabolic Dysfunction-Associated Steatohepatitis • Nephrology • Portal Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Pulmonary Embolism • Renal Disease • Respiratory Diseases • Transplantation

BRIDGE TO TRANSPLANT: A CASE OF SEVERE IDIOPATHIC PULMONARY ARTERIAL HYPERTENSION MANAGED WITH VENOARTERIAL EXTRACORPOREAL MEMBRANE OXYGENATION AND DOUBLE-LUNG TRANSPLANTATION (CHEST 2025) - "CASE PRESENTATION: We have a 26-year-old female with cor pulmonale secondary to IPAH, listed for lung transplantation while on maximal medical therapy, including Ambrisentan, Adempas, intravenous epoprostenol, and had just started on Soteracept. DLT remains a feasible and life-saving option for patients with severe PAH requiring mechanical support, and it continues to be preferred over HLT under specified conditions. This makes the use of rescue therapies, like ECMO, critical for survival while awaiting transplantation(8)."

Clinical • Cardiovascular • Hematological Disorders • Pulmonary Arterial Hypertension • Respiratory Diseases • Thrombocytopenia • Transplantation

ECMO AS A BRIDGE TO INITIATING THERAPY IN PULMONARY HYPERTENSION: A CASE REPORT (CHEST 2025) - "While there, the patient was started on inhaled epoprostenol and required multiple vasopressors...However, the patient was able to be supported through ECMO while intravenous epoprostenol, sildenafil, and inhaled nitric oxide were titrated with improvement in her pulmonary artery pressures... In conclusion, this case demonstrates the importance of minimizing delays in diagnosis of pulmonary hypertension and also adds to the sparse literature about the utility of ECMO as a bridge to definitive pulmonary hypertension therapy."

Case report • Clinical • Cardiovascular • Gynecology • Hypotension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Urinary Incontinence • Urology

OPTIC PERINEURITIS IN A PATIENT WITH LIMITED SYSTEMIC SCLEROSIS-ASSOCIATED PULMONARY HYPERTENSION (CHEST 2025) - "She was started on intravenous epoprostenol and a phosphodiesterase type-5 inhibitor, but she developed severe headaches and visual changes... OPN with ssSSc can be difficult to diagnose in PAH patients on prostacyclins, as the symptoms overlap with the side effect of PAH treatment. The management of OPN with corticosteroids should be considered with caution as corticosteroid use is rarely associated with SRC."

Clinical • Cardiovascular • Dermatology • Endocrine Disorders • Hypertension • Immunology • Ocular Inflammation • Optic Neuritis • Pulmonary Arterial Hypertension • Pulmonary Disease • Renal Disease • Respiratory Diseases • Rheumatology • Scleroderma • Systemic Sclerosis

DEVELOPMENT OF ERASMUS SYNDROME IN THE SETTING OF CHRONIC SILICOSIS (CHEST 2025) - "Given the constellation of findings including Raynaud's phenomenon, gastritis, and silicosis, he was diagnosed with Erasmus syndrome and started on a regimen of sildenafil, nifedipine and nitroglycerin paste. Due to worsening digital ischemia, intravenous epoprostenol was initiated and he was eventually discharged with sildenafil... Patients with silicosis can develop pulmonary infections, PAH, and autoimmune diseases like Erasmus syndrome. Close monitoring of cutaneous findings concerning for systemic sclerosis can initiate an evaluation for autoimmune disorders which was key to our case. Primary occupational prevention of silica exposure can reduce the risk and incidence of this disease and its complications."

Anemia • Fibrosis • Gastrointestinal Disorder • Human Immunodeficiency Virus • Immunology • Infectious Disease • Inflammatory Arthritis • Lung Cancer • Lupus • Pulmonary Arterial Hypertension • Pulmonary Disease • Rare Diseases • Respiratory Diseases • Rheumatology • Scleroderma • Solid Tumor • Systemic Sclerosis • Tuberculosis

TO TAP OR NOT TO TAP: RETHINKING PERICARDIAL DRAINAGE IN CARDIOGENIC SHOCK (CHEST 2025) - "Given his severe PAH, intravenous epoprostenol was initiated... This case underscores the importance of cautious, individualized management in severe PAH complicated by pericardial effusion. Balancing the risks and benefits of invasive interventions is crucial, as conservative strategies focusing on RV optimization may prevent additional hemodynamic compromise and improve clinical outcomes."

Cardiovascular • Congestive Heart Failure • Heart Failure • Hypotension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

THE UNCLASSIFIED PHENOTYPE: SEVERE PULMONARY HYPERTENSION WITH LOW DLCO AND CAPILLARY REMODELING CHALLENGING DIAGNOSTIC BOUNDARIES: A CASE REPORT (CHEST 2025) - "He was initiated on IV steroids, DuoNeb, and budesonide nebulizations, along with IV diureses...The patient was aggressively diuresed and was initiated on inhaled Flolan therapy. Given the severity of his PH with reduced CI and severely elevated PVR, the patient was then transferred to a tertiary care center for IV Epoprostenol therapy and evaluation for a lung transplant... This case report highlights the diagnostic challenges and the importance of considering capillary remodeling in patients with rapidly progressing severe PH with low DLCO and smoking history and a need for early referral to lung transplant. Advanced imaging, genetic studies, and more detailed clinical profiles can aid in refining diagnostic criteria and potentially lead to new subcategories in PH classification that are more inclusive of these phenotypes."

Case report • Clinical • Cardiovascular • Congestive Heart Failure • Dyslipidemia • Heart Failure • Immunology • Nicotine Addiction • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Tobacco Addiction

NOT QUITE COPD? PULMONARY HYPERTENSION IN A PATIENT WITH TOBACCO USE (CHEST 2025) - "During vasoreactivty testing with intravenous epoprostenol, he showed partial hemodynamic improvement after titration to 10ng/kg/min (mPAP 49, PCWP 11, CI 2.5), however he felt subjectively worse with each dose increase... Given the high mortality associated with PVOD, early recognition of its cardinal signs on imaging and noninvasive testing is crucial . Increased awareness of environmental and occupational exposures may aid in earlier recognition and risk stratification of at-risk individuals."

Clinical • Cardiovascular • Chronic Obstructive Pulmonary Disease • Congestive Heart Failure • Fibrosis • Heart Failure • Hematological Disorders • Hepatology • Immunology • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Management of Perioperative Heparin-Induced Thrombocytopenia (HIT) with Pre-Operative Plasmapheresis and Intra-Operative IV Epoprostenol Infusion in a Patient with Thrombosed Mechanical Tricuspid Valve (TV) (ASA 2025) - "Intravenous heparin (UFH) was initiated and re-do TV replacement scheduled...Considering the risks of using bivalirudin or argatroban during cardiopulmonary bypass (CPB), the decision was made to proceed with pre-operative plasmapheresis and use of UFH during CPB. This was facilitated by IV epoprostenol use prior to and during systemic anticoagulation. No complications related to HIT were reported post-operatively."

Clinical • Hematological Disorders • Thrombocytopenia

Tailored Perioperative Approach for High-Risk iPAH Patient Undergoing Hemorrhoidectomy Pre-Lung Transplant (ASA 2025) - "A 51-year-old with BMPR2-associated idiopathic pulmonary arterial hypertension (iPAH) on intravenous epoprostenol, sildenafil, and ambrisentan, awaiting lung transplantation, presented for pre-transplant hemorrhoidectomy...The patient was pretreated with vasopressin and epinephrine; sedation was achieved with midazolam, propofol, and remifentanyl. ECMO and cardiothoracic surgery backup were available. This case highlights perioperative considerations in pre-lung-transplant iPAH patients presenting for non-cardiac surgery."

Clinical • Anesthesia • Cardiovascular • Gastroenterology • Hematological Disorders • Pulmonary Arterial Hypertension • Respiratory Diseases • Thrombocytopenia • Transplantation

Unmasking an Intracardiac Shunt in a Case of Persistent Unexplained Hypoxia: A Case Report. (PubMed, Reports (MDPI)) - "Right heart catheterization (RHC) revealed precapillary pulmonary hypertension (88/37/54 mmHg), prompting the initiation of intravenous epoprostenol. The patient was eventually discharged with a treatment regimen that included subcutaneous Treprostinil. It is important to recognize that the consequences of PH are extensive, and that a rare yet significant etiology for persistent hypoxemia may be attributed to right-to-left shunting."

Journal • Cardiovascular • Chronic Kidney Disease • Congestive Heart Failure • Heart Failure • Hypertension • Nephrology • Pulmonary Arterial Hypertension • Pulmonary Disease • Renal Disease • Respiratory Diseases

FEASIBILITY OF IV EPOPROSTENOL FOR ACUTE LIMB ISCHEMIA IN ANTIPHOSPHOLIPID SYNDROME: A COMPARATIVE REVIEW OF SAFETY OUTCOMES (EHA 2025) - "We demonstrate that the tolerability of intravenous prostacyclin is comparable to its use in the setting of the treatment of Reynaud's phenomenon. This suggests that with appropriate counselling regarding expected mild side effects, IV epoprostenol may be safely considered for treatment of cases ineligible for or refractory to endovascular surgery and should be reconsidered for study in larger cohorts for evidence of efficacy in APLS."

Clinical • Review • Cardiovascular • Complement-mediated Rare Disorders • Genetic Disorders • Hematological Disorders • Hypotension • Immunology • Pain • Rheumatology • Scleroderma • Systemic Sclerosis

High Prevalence of Thyroid Gland Dysfunction after Pulmonary Arterial Hypertension Treatment According to Real-World Data. (PubMed, Int Heart J) - "The overall prevalence rate of thyroid gland dysfunction was 15.4%, with a significantly higher rate in patients initiated with continuous intravenous epoprostenol therapy than in those receiving only oral agents (2.9% versus 38.9%, P = 0.0007).Patients receiving continuous intravenous epoprostenol therapy had a high prevalence rate of thyroid gland dysfunction. Thus, severe PAH should be treated with an understanding that thyroid gland dysfunction is an important side effect in patients with PAH."

Journal • Real-world evidence • Retrospective data • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Maternal Pulmonary Arterial Hypertension Treated With IV Epoprostenol - A Case Report (ATS 2025) - "Her pre-gestation medications included Ambrisentan, Selexipag, and Sildenafil...The patient was then transitioned from IV Epoprostenol to inhaled Treprostinil via dry powder inhaler...IV Epoprostenol may be used adjunctively in the management of pregnant PAH patients and could improve peri and postpartum outcomes. This case adds to a growing literature base on this subject; the difficulties of performing a blinded, randomized clinical trial in this cohort are acknowledged."

Case report • Clinical • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

Discussion on Role of Imatinib Therapy in Scleroderma with Suspected PVOD (ATS 2025) - "We report a case of a patient with cardiogenic shock and suspected PVOD related to scleroderma, managed with Imatinib.Case Report: A 55-year-old male with CREST syndrome-associated Group 1 PAH, on home subcutaneous Remodulin, presented with dyspnea and leg edema...Due to worsening hypoxemia, he was cannulated for venoarterial extracorporeal membrane oxygenation (VA ECMO), diuresed, and started on Ambrisentan and inhaled Veletri...PVOD remains a challenge in pulmonary hypertension due to diagnostic difficulties, limited treatment options, and poor prognosis. Continued research into its pathogenesis, improved diagnostics, and targeted therapies, supported by advances in genetics and molecular biology, is essential for better management of this condition."

Cardiovascular • Fibrosis • Heart Failure • Hepatology • Immunology • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Scleroderma • Systemic Sclerosis

Hemodynamic Effects of IV Prostacyclins in Patients With PAH-CHD (ATS 2025) - "Continuous parenteral prostanoids such as IV epoprostenol and IV/SC treprostinil are a treatment of choice for patients with advanced PAH. Conclusions Treatment with IV prostacyclins is associated with statistically significant improvement in hemodynamics of patients with PAH-CHD at the first follow-up RHC. Future studies will need to explore whether this change correlates to changes in clinically significant markers of function including 6-minute walk test and functional status, as well as whether or not there are differences in effect amongst various subgroups such as type of CHD or repair status."

Clinical • Cardiovascular • Heart Failure • Hepatology • Pulmonary Arterial Hypertension • Respiratory Diseases

Thrombocytopenia Associated With Epoprostenol and Treprostinil Alone and in Combination With Sotatercept (ATS 2025) - "In the pivotal trials for subcutaneous treprostinil and intravenous epoprostenol, clinically significant changes in hematologic variables were not observed; however, laboratory values were not included in the publications. When PPs were combined with sotatercept, thrombocytopenia incidence increased; however, the specific prostacyclin, and dose were unavailable (Hoeper, 2023).ConclusionEpoprostenol may be more likely to cause thrombocytopenia, and amongst patients receiving epoprostenol and experiencing thrombocytopenia, transitioning to parenteral treprostinil may be considered. However, additional higher quality data on rates of thrombocytopenia associated with PAH medication use, in light of sotatercept, is warranted."

Combination therapy • Cardiovascular • Hematological Disorders • Pulmonary Arterial Hypertension • Respiratory Diseases • Thrombocytopenia

Comparison of Triple Oral and Triple Parenteral Combination Therapy in Pulmonary Arterial Hypertension (ATS 2025) - "Purpose: We compared the effects of Selexipag (S) vs subcutaneous Treprostinil (T) vs intravenous Epoprostenol (E) in PAH patients on both ERA and PDE5-I. S was prescribed to the oldest and least severe PAH patients, while T and E to the youngest and most severe PAH patients and led to a stronger improvement of exercise capacity and haemodynamic profile."

Combination therapy • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

Pulmonary Capillary Hemangiomatosis and Successful Use of Pulmonary Vasodilators During and After an Acute Decompensation of Pulmonary Hypertension (ATS 2025) - "After a discussion at Montefiore, he was started on IV epoprostenol... We present a case of a 58-year-old man with newly diagnosed PCH with severe pulmonary hypertension who successfully tolerated treatment with pulmonary vasodilators. Though patients historically have responded poorly to pulmonary vasodilators with PCH, early diagnosis through biopsy may play a crucial role in discussion regarding treatment options, including trials of IV prostanoids versus lung transplant referral."

Cardiovascular • Chronic Obstructive Pulmonary Disease • Congestive Heart Failure • Heart Failure • Immunology • Infectious Disease • Novel Coronavirus Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • CRP

Pregnancy Unmasking Pulmonary Veno-occlusive Disease Variant Pulmonary Hypertension (ATS 2025) - "Empiric pneumonia treatment with ceftriaxone and azithromycin was initiated...The patient underwent cesarean section, and was initiated on IV epoprostenol, dobutamine, and norepinephrine...Sildenafil was restarted, and she was discharged on day 13 on room air with sildenafil and furosemide, and referred for lung transplant evaluation...This case illustrates how PVOD, while also WHO group I, is often managed differently from PAH, as pre-capillary vasodilation can worsen oxygenation due to downstream pulmonary vein resistance. This case highlights the diagnostic and treatment nuances in managing rare conditions like PVOD, particularly in the high-risk context of pregnancy."

Cardiovascular • Cough • Fatigue • Hepatology • Infectious Disease • Pneumonia • Pulmonary Arterial Hypertension • Pulmonary Disease • Pulmonary Embolism • Respiratory Diseases

Teamwork Makes the Dream Work: Conquering Pulmonary Hypertension in Pregnancy (ATS 2025) - "Intravenous epoprostenol was initiated at 2 ng/kg/min and gradually titrated based on symptoms, lactate level, and venous oxygen saturation, reaching a maximum of 13.5 ng/kg/min in two weeks. Headache and hypotension were dose-limiting side effects, prompting the addition of sildenafil to support therapy...Epoprostenol proved effective for acute management in this case, providing symptomatic relief and hemodynamic stability, thereby enabling a successful delivery. Multidisciplinary collaboration was crucial, combining diverse expertise to address the complex needs of PAH in pregnancy and ensure comprehensive patient care."

Cardiovascular • Human Immunodeficiency Virus • Hypotension • Infectious Disease • Pain • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases