Clinical outcome of patients 20 years after Fontan operation — effect of fenestration on late morbidity

Ono, Masamichi; Boethig, Dietmar; Goerler, Heidi; Lange, Melanie; Westhoff-Bleck, Mechthild; Breymann, Thomas

doi:10.1016/j.ejcts.2006.08.025

Cited by 151 publications

(107 citation statements)

References 25 publications

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“…There was no follow-up information available in 40 patients (4%). Of the 1006 hospital survivors, 203 had an AP (1975AP ( -1995, 271 a lateral tunnel (LT;1988-2006, and 532 an extracardiac conduit (ECC; 1997-2010). The characteristics of the patients are given in Table 1.…”

Section: Methodsmentioning

confidence: 99%

“…[1][2][3] Twenty-year survival estimates have only been delineated for the atriopulmonary connection (AP), and it is likely that some of these reported poor outcomes have misrepresented the longevity of those treated with more recent surgical strategies. 4,5 To clarify these late outcomes, we created the Australia and New Zealand Fontan Registry, which collects health data of all Fontan patients prospectively.…”

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confidence: 99%

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Redefining Expectations of Long-Term Survival After the Fontan Procedure

et al. 2014

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Background— The life expectancy of patients undergoing a Fontan procedure is unknown. Methods and Results— Follow-up of all 1006 survivors of the 1089 patients who underwent a Fontan procedure in Australia and New Zealand was obtained from a binational population-based registry including all pediatric and adult cardiac centers. There were 203 atriopulmonary connections (AP; 1975–1995), 271 lateral tunnels (1988–2006), and 532 extracardiac conduits (1997–2010). The proportion with hypoplastic left heart syndrome increased from 1/173 (1%) before 1990 to 80/500 (16%) after 2000. Survival at 10 years was 89% (84%–93%) for AP and 97% (95% confidence interval [CI], 94%–99%) for lateral tunnels and extracardiac conduits. The longest survival estimate was 76% (95% CI, 67%–82%) at 25 years for AP. AP independently predicted worse survival compared with extracardiac conduits (hazard ratio, 6.2; P <0.001; 95% CI, 2.4–16.0). Freedom from failure (death, transplantation, takedown, conversion to extracardiac conduits, New York Heart Association III/IV, or protein-losing enteropathy/plastic bronchitis) 20 years after Fontan was 70% (95% CI, 63%–76%). Hypoplastic left heart syndrome was the primary predictor of Fontan failure (hazard ratio, 3.8; P <0.001; 95% CI, 2.0–7.1). Ten-year freedom from failure was 79% (95% CI, 61%–89%) for hypoplastic left heart syndrome versus 92% (95% CI, 87%–95%) for other morphologies. Conclusions— The long-term survival of the Australia and New Zealand Fontan population is excellent. Patients with an AP Fontan experience survival of 76% at 25 years. Technical modifications have further improved survival. Patients with hypoplastic left heart syndrome are at higher risk of failure. Large, comprehensive registries such as this will further improve our understanding of late outcomes after the Fontan procedure.

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Section: Methodsmentioning

confidence: 99%

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confidence: 99%

Redefining Expectations of Long-Term Survival After the Fontan Procedure

et al. 2014

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“…545,546 TE remains a major cause of early and late morbidity and mortality. Reported incidences of VTE and stroke ranged from 3% to 16% and 3% to 19%, respectively, in retrospective cohort studies where thrombosis was the primary outcome and from 1% to 7% in retrospective studies assessing multiple outcomes.…”

Section: Fontan Surgerymentioning

confidence: 99%

Antithrombotic Therapy in Neonates and Children

Monagle

Chan

Goldenberg

et al. 2012

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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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Clinical outcome of patients 20 years after Fontan operation — effect of fenestration on late morbidity

Cited by 151 publications

References 25 publications

Redefining Expectations of Long-Term Survival After the Fontan Procedure

Redefining Expectations of Long-Term Survival After the Fontan Procedure

Antithrombotic Therapy in Neonates and Children

Bosentan Improves Exercise Capacity in Adolescents and Adults After Fontan Operation

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