Anesthetic management for percutaneous reverse Potts shunt creation in children with refractory idiopathic pulmonary arterial hypertension: A case series

2021 ◽  
Author(s):  
Arnaud Valent ◽  
Imene Nefzi ◽  
Vanessa Lopez ◽  
Cristian Mirabile ◽  
Gilles Orliaguet
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Anesthetic Management ◽  
Case Series ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Pulmonary Arterial

scholarly journals Contemporary use of Selexipag in pulmonary arterial hypertension associated with congenital heart disease: a case series

2020 ◽  
Author(s):  
Sarah Blissett ◽  
David Blusztein ◽  
Vaikom S Mahadevan
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Side Effects ◽  
Arterial Hypertension ◽  
Exercise Capacity ◽  
Congenital Heart ◽  
Pulmonary Veins ◽  
Case Series ◽  
Pulmonary Arterial

Abstract Background There are significant risks of parenteral prostacyclin use in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), which may limit their use. Selexipag is an oral, selective prostacyclin analogue that has been shown to reduce disease progression and improve exercise capacity in patients with PAH-CHD. Administering Selexipag in patients with PAH-CHD could potentially overcome some of the risks of parenteral therapy while improving clinical outcomes. Case summary We report five cases highlighting the clinical uses of Selexipag in patients with PAH-CHD. In the first two cases, Selexipag was initiated as part of a Treat-to-close strategy. In the third case, initiation of Selexipag improved symptoms and objective exercise capacity in a patient with Eisenmenger syndrome. In the fourth and fifth cases, rapid cross-titration protocols were used to transition from parenteral prostacyclins to Selexipag. In the fourth case, Selexipag was initiated in the context of significant side effects limiting parenteral prostacyclin use. In the fifth case, Selexipag was used to down-titrate from parenteral prostacyclins following closure of a sinus venosus atrial septal defect and redirection of anomalous pulmonary veins. Discussion Selexipag is a promising oral therapy for patients with at various stages of the spectrum of PAH-CHD to improve symptoms, exercise capacity and, in some cases, haemodynamics. Our cases also highlight practical aspects of Selexipag use including targeting the individualized maximally tolerated dose for each patient, managing side effects and managing dose interruptions.

scholarly journals EXPRESS: The transition from Parenteral Prostacyclins to Selexipag: Safety and Feasibility in Select Patients

2021 ◽  
pp. 204589402110366
Author(s):  
Nael Aldweib ◽  
Nate Verlinden ◽  
Hayah Kassis-george ◽  
Amresh Raina
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Case Series ◽  
Outpatient Setting ◽  
Limited Data ◽  
Standardized Protocol ◽  
Pulmonary Arterial

There is limited data regarding the feasibility of transitioning from intravenous prostacyclins to selexipag in pulmonary arterial hypertension (PAH) patients. We present a case series of successful transitions from intravenous prostacyclins to selexipag in the majority of carefully selected five stable PAH patients using a standardized protocol in the outpatient setting.

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