Inhaled iloprost for the treatment of patient with Fontan circulation

Kim, Yong Hyun; Chae, Moon Hee; Choi, Do Il

doi:10.3345/kjp.2014.57.10.461

Cited by 6 publications

(5 citation statements)

References 14 publications

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“…Reducing pulmonary vascular resistance improves blood flow across the pulmonary capillaries, which results in a reduced central venous pressure and improved cardiac output. Various pharmacological agents have shown to reduce pulmonary artery pressures such as endothelial receptor antagonists [ 5 ] prostanoids [ 6 ] and phosphodiesterase type 5 inhibitors [ 7 ]. This case report involves the first use of macitentan (an endothelin-receptor antagonist) in a patient with a Fontan circulation.…”

Section: Introductionmentioning

confidence: 99%

The use of Macitentan in Fontan circulation: a case report

Demetriades

Aziz

Condliffe

et al. 2017

BMC Cardiovasc Disord

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BackgroundThe Fontan circulation, a result of a palliative procedure in patients with single systemic ventricles, is defined by chronically elevated pulmonary vascular resistance. When traditional heart failure therapies fail, pharmacological agents that reduce pulmonary artery pressures may be used. These include endothelial-receptor antagonists, prostanoids and phosphodiesterase type 5 inhibitors. We report the first use of macitentan, an endothelin-receptor antagonist, in a patient with a Fontan circulation.Case presentationWe describe the case of a 50 year old female with tricuspid atresia and transposition of the great arteries. Following complex surgery as a child, she subsequently underwent a fenestrated modified atrial pulmonary Fontan operation which was later converted to a total cavopulmonary anastomosis Fontan circulation. Due to failure of various medications to relieve her worsening symptoms, she was commenced on macitentan in April 2016. Few months later, she demonstrated a significant symptomatic improvement and associated increase in her incremental shuttle walking test distance.ConclusionsMacitentan has slower receptor dissociation kinetics compared to other endothelin-receptor antagonists, leading to enhanced pharmacological activity with promising effects in patients with pulmonary arterial hypertension. The patient we report has shown considerable improvement in exercise capacity following introduction of this medication and thus we suggest further randomised trials to establish the role of different endothelin-receptor antagonists in the management of the Fontan circulation.

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Section: Introductionmentioning

confidence: 99%

The use of Macitentan in Fontan circulation: a case report

Demetriades

Aziz

Condliffe

et al. 2017

BMC Cardiovasc Disord

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“…Prostanoid, E-type 1 prostaglandin (PGE1), is commonly used in HLHS neonates prior to Norwood palliation to maintain patency of the ductus arteriosus [ 51 , 52 , 53 ]. HLHS patients are dependent on a patent ductus arteriosus to provide systemic blood flow, due to severe obstruction of the left ventricular outflow tract.…”

Section: Current Hlhs Heart Failure Therapiesmentioning

confidence: 99%

“…PGE1 dilates the ductus arteriosus as a postnatal therapeutic to maintain the right to left shunting of systemic blood flow prior to surgical intervention. Prostanoid use in older SV patients, particularly post-Fontan is rare; however, several small studies have looked at inhaled iloprost in patients with Fontan circulation and found improvement in peak oxygen pulse and maximum VO2, as well as improved exercise capacity and cardiac output [ 52 , 53 ].…”

Section: Current Hlhs Heart Failure Therapiesmentioning

confidence: 99%

Medical Therapies for Heart Failure in Hypoplastic Left Heart Syndrome

Baybayon-Grandgeorge

Pietra

Miyamoto

et al. 2022

JCDD

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Significant surgical and medical advances over the past several decades have resulted in a growing number of infants and children surviving with hypoplastic left heart syndrome (HLHS) and other congenital heart defects associated with a single systemic right ventricle (RV). However, cardiac dysfunction and ultimately heart failure (HF) remain the most common cause of death and indication for transplantation in this population. Moreover, while early recognition and treatment of single ventricle-related complications are essential to improving outcomes, there are no proven therapeutic strategies for single systemic RV HF in the pediatric population. Importantly, prototypical adult HF therapies have been relatively ineffective in mitigating the need for cardiac transplantation in HLHS, likely due to several unique attributes of the failing HLHS myocardium. Here, we discuss the most commonly used medical therapies for the treatment of HF symptoms in HLHS and other single systemic RV patients. Additionally, we provide an overview of potential novel therapies for systemic ventricular failure in the HLHS and related populations based on fundamental science, pre-clinical, clinical, and observational studies in the current literature.

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“…The role of prostacyclines has been investigated in only two trials, which found beneficial effects of inhaled iloprost on symptoms and exercise performance [ 86 , 87 ].…”

Section: Group 1 Ph ( Table 1 )mentioning

confidence: 99%

Focused Update on Pulmonary Hypertension in Children—Selected Topics of Interest for the Adult Cardiologist

2020

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Pulmonary hypertensive vascular disease (PHVD), and pulmonary hypertension (PH), which is a broader term, are severe conditions associated with high morbidity and mortality at all ages. Treatment guidelines in childhood are widely adopted from adult data and experience, though big differences may exist regarding aetiology, concomitant conditions and presentation. Over the past few years, paediatric aspects have been incorporated into the common guidelines, which currently address both children and adults with pulmonary hypertension (PH). There are multiple facets of PH in the context of cardiac conditions in childhood. Apart from Eisenmenger syndrome (ES), the broad spectrum of congenital heart disease (CHD) comprises PH in failing Fontan physiology, as well as segmental PH. In this review we provide current data and novel aspects on the pathophysiological background and individual management concepts of these conditions. Moreover, we focus on paediatric left heart failure with PH and its challenging issues, including end stage treatment options, such as mechanical support and paediatric transplantation. PH in the context of rare congenital disorders, such as Scimitar Syndrome and sickle cell disease is discussed. Based on current data, we provide an overview on multiple underlying mechanisms of PH involved in these conditions, and different management strategies in children and adulthood. In addition, we summarize the paediatric aspects and the pros and cons of the recently updated definitions of PH. This review provides deeper insights into some challenging conditions of paediatric PH in order to improve current knowledge and care for children and young adults.

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Inhaled iloprost for the treatment of patient with Fontan circulation

Cited by 6 publications

References 14 publications

The use of Macitentan in Fontan circulation: a case report

The use of Macitentan in Fontan circulation: a case report

Medical Therapies for Heart Failure in Hypoplastic Left Heart Syndrome

Focused Update on Pulmonary Hypertension in Children—Selected Topics of Interest for the Adult Cardiologist

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