Impact of bosentan on exercise capacity in adults after the Fontan procedure: a randomized controlled trial

Schuuring, Mark J.; Vis, Jeroen C.; Dijk, Arie P.J. van; Melle, Joost P. van; Vliegen, Hubert W.; Pieper, Petronella G.; Sieswerda, Gertjan T.; Bruin‐Bon, Rianne H.A.C.M. de; Mulder, Barbara J.M.; Bouma, Berto J.

doi:10.1093/eurjhf/hft017

Cited by 129 publications

(101 citation statements)

References 34 publications

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“…Treatment with bosentan was not beneficial at 6 months in a population of Fontan patients with elevated N-terminal pro-B-type natriuretic peptide. 162 However, sildenafil in children and adults improved ventilatory efficiency and exercise in Fontan patients. 163 Further study will be necessary before pulmonary arterial vasodilator drugs can be considered part of the armamentarium for treatment of the Fontan patient.…”

Section: Progressive Pulmonary Vascular Diseasementioning

confidence: 99%

Congenital Heart Disease in the Older Adult

Bhatt¹,

Foster²,

Kuehl³

et al. 2015

Circulation

205

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Section: Progressive Pulmonary Vascular Diseasementioning

confidence: 99%

Congenital Heart Disease in the Older Adult

Bhatt¹,

Foster²,

Kuehl³

et al. 2015

Circulation

205

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“…22 A recent open-label study on 42 Fontan patients failed to show effects of 6 months of bosentan treatment. 17 However, the study was hampered by the facts that 24% of patients withdrew before the final visit and that the CPET was performed *Statistically significant treatment effect. †A negative change in these end points is a beneficial treatment response.…”

Section: Discussionmentioning

confidence: 99%

“…Few studies have investigated the safety and efficacy of pulmonary vasodilators after the Fontan operation, and only 2 have tested ERAs. 16,17 Randomized, placebo-controlled studies with ERAs have not been performed.…”

Section: Editorial See P 1999 Clinical Perspective On P 2030mentioning

confidence: 99%

Bosentan Improves Exercise Capacity in Adolescents and Adults After Fontan Operation

et al. 2014

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Background— The Fontan procedure has improved survival in children with functionally univentricular hearts. With time, however, complications such as reduced exercise capacity are seen more frequently. Exercise intolerance is multifactorial, but pulmonary vascular resistance probably plays a crucial role. Elevated pulmonary vascular resistance has been associated with raised levels of endothelin-1, which are common both before and after Fontan operations. Treatment with endothelin-1 receptor antagonists could theoretically improve cardiopulmonary hemodynamics and exercise capacity. The aim of this study was therefore to examine the efficacy and safety of bosentan in Fontan patients. Methods and Results— Seventy-five adolescents and adults were randomized 1:1 to 14 weeks of treatment with bosentan or placebo. Cardiopulmonary exercise test, functional class, blood samples, and quality-of-life questionnaires were evaluated at baseline and at the end of treatment. Sixty-nine patients (92%) completed the study. Peak oxygen consumption increased 2.0 mL·kg −1 ·min −1 (from 28.7 to 30.7 mL·kg −1 ·min −1 ) in the bosentan group compared with 0.6 mL·kg −1 ·min −1 (from 28.4 to 29.0 mL·kg −1 ·min −1 ) in the placebo group ( P =0.02). Cardiopulmonary exercise test time increased by 0.48 minute (from 6.79 to 7.27 minutes) versus 0.08 minute (from 6.94 to 7.02 minutes; P =0.04). Nine bosentan-treated patients improved 1 functional class, whereas none improved in the placebo group ( P =0.0085). Side effects were mild and occurred equally in both groups. No serious adverse effects were seen, and no patients had liver enzyme levels above the 3-fold upper limit. Conclusions— Bosentan improves exercise capacity, exercise time, and functional class in Fontan patients without serious adverse events or hepatotoxicity. Clinical Trial Registration— URL: http://www.clinicaltrials.gov . Unique identifier: NCT01292551.

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“…85 The application of pulmonary vasodilator therapy in these clinical scenarios is an area of active investigation. [86][87][88] Pulmonary vasodilator therapy may also have a role in the management of CHD patients with ventricular-arterial uncoupling despite an absence of pulmonary vascular disease or PH. Examples include patients with severe subpulmonary ventricular dysfunction or tricuspid regurgitation (eg, Ebstein anomaly).…”

Section: Emerging Roles For Pulmonary Vasodilator Therapymentioning

confidence: 99%

Clinical Evaluation and Management of Pulmonary Hypertension in the Adult With Congenital Heart Disease

Opotowsky

2015

Circulation

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200P ulmonary hypertension (PH) refers to elevated pulmonary artery (PA) pressure (PAP), defined as mean PAP≥25 mm Hg. PH is not synonymous with pulmonary arterial hypertension (PAH), which specifies resistive PH characterized by elevated pulmonary vascular resistance (PVR) and normal pulmonary venous pressure. Approximately 5% to 10% of adults with congenital heart disease (CHD) have PH, and this subset of CHD patients is at higher risk of hospitalization and death. [1][2][3] Understanding the epidemiology of PH associated with CHD (PH-CHD) is not straightforward, however, because definitions of PH-CHD and diagnostic techniques vary. Appropriate diagnosis and the management of PH-CHD rest on identifying the cause of elevated PAP: high pulmonary venous pressure, high pulmonary vascular resistance, high pulmonary flow, or some combination of these factors, which are referred to throughout this review as passive, resistive, hyperkinetic, and polygenic PH, respectively (Table 1).Prognosis in PH-CHD depends on the underlying cause, pulmonary vascular pathophysiology, and the interaction between the right heart and pulmonary circulation. Adult CHD is heterogeneous, with myriad permutations of underlying defects, previous interventions, and associated comorbidities. Although comprehensive understanding of clinical context is a prerequisite, identifying the optimal therapy for a patient with PH-CHD depends more heavily on an understanding of pathophysiology than it does on the underlying diagnosis. Therefore, the goal of this review is to frame an understanding of PH-CHD in terms of fundamental cardiopulmonary hemodynamic pathophysiology and to emphasize the clinical assessment and management of situations commonly encountered by adult cardiologists caring for patients with PH-CHD. Classifications of PH and PH-CHDClassification schemes in PH represent clinical perspectives of underlying pathophysiology and tend to parallel fundamental hemodynamic relationships of the pulmonary circulation. PVR

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Impact of bosentan on exercise capacity in adults after the Fontan procedure: a randomized controlled trial

Cited by 129 publications

References 34 publications

Congenital Heart Disease in the Older Adult

Congenital Heart Disease in the Older Adult

Bosentan Improves Exercise Capacity in Adolescents and Adults After Fontan Operation

Clinical Evaluation and Management of Pulmonary Hypertension in the Adult With Congenital Heart Disease

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