How Good Is a Good Fontan? Quality of Life and Exercise Capacity of Fontans Without Arrhythmias

d’Udekem, Yves; Cheung, Michael; Setyapranata, Stella; Iyengar, Ajay J.; Kelly, Patricia J.; Buckland, Naomi; Grigg, Leeanne; Weintraub, Robert G.; Vance, Alasdair; Brizard, Christian P.; Penny, Dan J.

doi:10.1016/j.athoracsur.2009.07.079

Cited by 64 publications

(39 citation statements)

References 31 publications

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“…Peak oxygen consumption increased 2.0 mL·kg Interestingly, levels of the potent pulmonary vasoconstrictor endothelin-1 (ET-1) are frequently elevated before surgical palliation, 13 and raised levels of ET-1 have been shown to correlate with failure of the Fontan circulation. 14,15 These observations suggest that pulmonary vasodilators and especially endothelin receptor antagonists (ERAs) may play a role in the treatment of these patients. Few studies have investigated the safety and efficacy of pulmonary vasodilators after the Fontan operation, and only 2 have tested ERAs.…”

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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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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“…Only a few studies have investigated the quality of life (QOL) of adult survivors with SVP [6,7,8,9]. These studies were limited by the fact that they did not use a control group and did not take functional class or disease severity into account.…”

Section: Introductionmentioning

confidence: 99%

Patient-Reported Outcomes in Adult Survivors with Single-Ventricle Physiology

et al. 2011

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Objectives: Data on patient-reported outcomes (PROs) in patients with single-ventricle physiology (SVP) are scarce. We sought (1) to describe the perceived health status, quality of life, symptoms of anxiety and depression, and sense of coherence in adult survivors with SVP, (2) to compare PROs across functional classes, and (3) to compare PROs between patients and controls. Methods: A case-control study in two adult congenital heart programmes with 62 adult survivors with SVP were matched to 172 healthy controls. A wide range of PROs were measured using validated questionnaires. The treating physician classified patients according to the Ability Index. Results: Patients with SVP have a good functional status. Patients in Ability Index class I consistently reported the best scores, similar to those of healthy controls. Negative associations were found between functional class and outcomes of perceived health and quality of life. For patients in Ability Index class II and III, PROs were poorer. Conclusions: PROs in patients with SVP are generally good.

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“…It has been consistently reported that patients with Fontan circulation have decreased maximal exercise capacity with peak oxygen consumption averaging around 65% of predicted values [94,95]. There is a wide variation in the capacity of these individuals to participate to individual and even group sporting activities, with some being able to function at levels that are surprising for patients bearing only one functional ventricle.…”

Section: Exercise Capacitymentioning

confidence: 99%

Current Therapy for Hypoplastic Left Heart Syndrome and Related Single Ventricle Lesions

2016

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Universally fatal only four decades ago, the progress in the three-stage palliation of hypoplastic left heart syndrome and related single right ventricular lesions has drastically improved the outlook for these patients. While the stage II operation (hemi-Fontan or bidirectional Glenn) and stage III Fontan procedure have evolved into relatively low risk operations, the stage I Norwood procedure remains one of the highest risk and costliest common operations performed in congenital heart surgery. Yet, despite this fact, experienced centers now report hospital survivals in excess of 90% for the Norwood. This traditional three-stage surgical palliation has seen several innovations in the past decade aimed at improving outcomes, particularly for the Norwood procedure. One significant change is a renewed interest in the right ventricle-to-pulmonary artery shunt as the source of pulmonary blood flow, rather than the modified Blalock-Taussig shunt for the Norwood. The multi-institutional Single Ventricle Reconstruction trial randomized 555 patients to one or the other shunt, and these subjects continue to be followed closely as they now approach 10 years post-randomization. In addition to modifications to the Norwood procedure, the “hybrid procedure,” a combined catheter-based and surgical approach, avoids the Norwood procedure in the newborn period entirely. The initial hybrid procedure is then followed by a “comprehensive” stage II, that combines components of both the Norwood and the traditional stage II, and later completion of the Fontan. Proponents of this approach hope to not only improve short-term survival, but potentially longer-term outcomes, such as neurodevelopment, as well. Regardless of the approach, traditional surgical staged palliation or the hybrid procedure, survivals have vastly improved, and large numbers of these patients are surviving not only through their Fontan in early childhood, but into adolescence and young adulthood. As this population grows, it becomes increasingly important to understand the longer-term outcomes of these Fontan patients, not only in terms of survival, but also burden of disease, neurodevelopmental outcomes, psychosocial development and quality of life.

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How Good Is a Good Fontan? Quality of Life and Exercise Capacity of Fontans Without Arrhythmias

Cited by 64 publications

References 31 publications

Bosentan Improves Exercise Capacity in Adolescents and Adults After Fontan Operation

Bosentan Improves Exercise Capacity in Adolescents and Adults After Fontan Operation

Patient-Reported Outcomes in Adult Survivors with Single-Ventricle Physiology

Current Therapy for Hypoplastic Left Heart Syndrome and Related Single Ventricle Lesions

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