Use of the Prostacyclin-Receptor Agonist Selexipag in Pulmonary Arterial Hypertension Associated with Eisenmenger Syndrome

2021 ◽  
Author(s):  
Sotiria C. Apostolopoulou ◽  
Panagiotis Karyofyllis ◽  
Eftychia A. Demerouti
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Receptor Agonist ◽  
Eisenmenger Syndrome ◽  
Prostacyclin Receptor ◽  
Pulmonary Arterial

scholarly journals Selexipag: An Oral and Selective IP Prostacyclin Receptor Agonist for the Treatment of Pulmonary Arterial Hypertension

2015 ◽  
Vol 58 (18) ◽  
pp. 7128-7137 ◽  
Author(s):  
Tetsuo Asaki ◽  
Keiichi Kuwano ◽  
Keith Morrison ◽  
John Gatfield ◽  
Taisuke Hamamoto ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Receptor Agonist ◽  
Prostacyclin Receptor ◽  
Pulmonary Arterial

scholarly journals Prostacyclin receptor agonist selexipag in a patient with high-risk idiopathic pulmonary arterial hypertension: a case report

2021 ◽  
Vol 20 (5) ◽  
pp. 3010
Author(s):  
E. A. Rezukhina ◽  
O. V. Rodnenkov ◽  
T. V. Martynyuk
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
High Risk ◽  
Arterial Hypertension ◽  
Receptor Agonist ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
World Health ◽  
Class Iii ◽  
National Medical ◽  
Prostacyclin Receptor ◽  
Pulmonary Arterial

We present a 48-year-old patient with World Health Organization class III idiopathic pulmonary arterial hypertension (IPAH), taking specific therapy with macitentan 10 mg a day, who was readmitted to the National Medical Research Center of Cardiology due to increase in exercise dyspnea and decrease in effort tolerance. According to a comprehensive examination, single factors of high risk and unfavorable prognosis were identified. Due to systemic hypotension when using inhaled iloprost, selexipag was added to therapy. According to control hospitalization, 8-month selexipag therapy improved the patient's condition, as well as high risk factors were not revealed. Selexipag is a selective oral prostacyclin receptor agonist recommended for longterm IPAH therapy in adult patients.

scholarly journals Selexipag: an oral, selective prostacyclin receptor agonist for the treatment of pulmonary arterial hypertension

2012 ◽  
Vol 40 (4) ◽  
pp. 874-880 ◽  
Author(s):  
Gérald Simonneau ◽  
Adam Torbicki ◽  
Marius M. Hoeper ◽  
Marion Delcroix ◽  
Kristóf Karlócai ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Receptor Agonist ◽  
Prostacyclin Receptor ◽  
Pulmonary Arterial

scholarly journals Heart and lung transplantation in pulmonary arterial hypertension related to congenital heart disease: an unusual indication

2020 ◽  
Vol 4 (S1) ◽  
Author(s):  
Rosaria Barracano ◽  
Heba Nashat ◽  
Andrew Constantine ◽  
Konstantinos Dimopoulos
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Transplantation ◽  
Atrial Septal Defect ◽  
Septal Defect ◽  
Pulmonary Vascular Disease ◽  
Eisenmenger Syndrome ◽  
Pulmonary Arterial ◽  
Recurrent Haemoptysis

Abstract Background Eisenmenger syndrome is a multisystem disorder, characterised by a significant cardiac defect, severe pulmonary hypertension and long-standing cyanosis. Despite the availability of pulmonary hypertension therapies and improved supportive care in specialist centres, Eisenmenger patients are still faced with significant morbidity and mortality. Case presentation We describe the case of a 44-year-old woman with Eisenmenger syndrome secondary to a large secundum atrial septal defect. Her pulmonary vascular disease was treated with pulmonary vasodilators, but she experienced a progressive decline in exercise tolerance, increasing atrial arrhythmias, resulting in referral for transplantation. Her condition was complicated by significant recurrent haemoptysis in the context of extremely dilated pulmonary arteries and in-situ thrombosis, which prompted successful heart and lung transplantation. She made a slow recovery but remains well 3 years post-transplant. Conclusions Patients with Eisenmenger syndrome secondary to a pre-tricuspid lesion, such as an atrial septal defect have a natural history that differs to patients with post-tricuspid shunts; the disease tends to present later in life but is more aggressive, prompting early and aggressive medical intervention with pulmonary arterial hypertension therapies. This case illustrates that severe recurrent haemoptysis can be an indication for expediting transplantation in Eisenmenger syndrome patients.

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