Factors influencing early and late outcome following the Fontan procedure in the current era. The ‘Two Commandments’?☆

Hosein, Riad B.M.; Clarke, Andrew; McGuirk, Simon; Griselli, Massimo; Stümper, Oliver; Giovanni, Joseph V. De; Barron, David J.; Brawn, William J.

doi:10.1016/j.ejcts.2006.11.043

Cited by 249 publications

(202 citation statements)

References 24 publications

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“…18,19 In our study, one third of patients were alive after 45 years of follow-up, with the majority of patients dying at a young age. The late survival of patients operated from 1953 to 1989 and 1990 to 2009 was comparable until 8 years after the operation; thereafter, the late survival of patients operated on from 1990 to 2009 was significantly higher than that for patients operated on from 1953 to 1989.…”

Section: Survivalmentioning

confidence: 98%

Progress in Late Results Among Pediatric Cardiac Surgery Patients

et al. 2015

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Section: Survivalmentioning

confidence: 98%

Progress in Late Results Among Pediatric Cardiac Surgery Patients

et al. 2015

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“…A recent study with a large cohort indicated the importance of "2 recommendations": that is, pulmonary artery resistance and ventricular function. 6 Other possible relevant risk factors may be atrioventricular valve dysfunction and surgery-related issues, such as a longer cardiopulmonary bypass time. 15 Our results do not contradict the previous reports and the early postoperative hemodynamics, especially a lower CVP and no history of an AVVR repair, emerged as important independent predictors of an excellent status for the next 15 postoperative years.…”

Section: Traditional Risk Factorsmentioning

confidence: 99%

Long-Term Serial Aerobic Exercise Capacity and Hemodynamic Properties in Clinically and Hemodynamically Good, "Excellent", Fontan Survivors

Ohuchi

Tanabe

et al. 2012

Circ J

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Circulation Journal Official Journal of the Japanese Circulation Society http://www. j-circ.or.jp our decades have passed since the first Fontan operation was performed in a patient with tricuspid atresia 1 and since then a lot of patients with congenital heart disease who were not suitable for a biventricular repair have enjoyed the benefits of this procedure. The lack of a pulmonary ventricle forced us to rely on an elevated central venous pressure (CVP) and the sucking property of the systemic ventricle to maintain the pulmonary circulation. However, these inadequate compensatory adaptations lead to a diminished preload for the systemic ventricle, resulting in a low cardiac output (CO) and, therefore, chronically elevated CVP and low CO characterize the Fontan circulation. 2 In general, we categorize Fontan patients with a low CVP and high cardiac index (CI) as "good" in terms of the hemodynamics. However, the definition of a low or high CI remains unknown. The other important clinical characteristic of the Fontan patients are a high prevalence of postoperative complications, 3 such as arrhythmias, 4 re-intervention, and non-cardiac lesions that include protein-losing enteropathy. 5-7 Thus, long-term Fontan survivors without those complications can also be categorized as those with a good clinical status. We thought that our ongoing management strategy of seeking a better long-term outcome based on serial comprehensive assessments, including cardiac catheterization and cardiopulmonary exercise testing (CPX), would enable us to clarify clinically and hemodynamically the long-term good Fontan survivors. 8 Accordingly, our purpose for the present study was to identify the long-term clinically (ie, no clinical events) and hemodynamically good Fontan survivors, to characterize the hemodynamics, and finally to determine the clinical predictors of long-term good Fontan survivors.

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“…2 Although the surgical techniques have evolved, 3,4 the procedure remains palliative with current 10-year survival rates reaching 90%. 5,6 The prevalence of Fontan patients has consequently increased significantly over the past decades, 7 and clinicians are now frequently caring for patients who suffer from a number of late comorbidities, including arrhythmias and exercise intolerance. These problems will continue to rise markedly.…”

Section: Introduction Of the Fontan Operation In 1971mentioning

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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Factors influencing early and late outcome following the Fontan procedure in the current era. The ‘Two Commandments’?☆

Cited by 249 publications

References 24 publications

Progress in Late Results Among Pediatric Cardiac Surgery Patients

Progress in Late Results Among Pediatric Cardiac Surgery Patients

Long-Term Serial Aerobic Exercise Capacity and Hemodynamic Properties in Clinically and Hemodynamically Good, "Excellent", Fontan Survivors

Bosentan Improves Exercise Capacity in Adolescents and Adults After Fontan Operation

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