Trends in Fontan surgery and risk factors for early adverse outcomes after Fontan surgery: The Australia and New Zealand Fontan Registry experience

Iyengar, Ajay J.; Winlaw, David S.; Galati, John C.; Celermajer, David S.; Wheaton, Gavin; Gentles, Thomas L.; Grigg, Leeanne; Weintraub, Robert G.; Bullock, Andrew; Justo, Robert; d’Udekem, Yves

doi:10.1016/j.jtcvs.2013.09.074

Cited by 91 publications

(79 citation statements)

References 27 publications

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“…The analysis of their early outcomes is published elsewhere. 7 The 1006 patients surviving to hospital discharge with a Fontan procedure constitute the cohort of this study. There was no follow-up information available in 40 patients (4%).…”

Section: Methodsmentioning

confidence: 99%

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%

Redefining Expectations of Long-Term Survival After the Fontan Procedure

et al. 2014

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“…Our hospital mortality of 13% was much higher than that which could be expected, even in a historical series. In the same population, over the same time period the overall hospital mortality was 3.5% with the peak of 8% in the initial period 14 . All series relating experience with the Fontan procedure performed in adults and adolescents have, however, reported hospital mortality ranging between 4 and 13%.…”

Section: Discussionmentioning

confidence: 77%

“…In recent years, the teams who have reported their experience with adults and adolescents undergoing Fontan surgery have demonstrated their hospital mortality to be between 4 and 13%, higher than the 1% usually reported recently for this surgery when performed at a younger age 1,3,[8][9][10][11][12][13][14][15] . But more importantly, in limited follow-up extending to 15 years, outcomes seem to be equivalent to those achieved by younger patients 1-3, 5, 8-10 .…”

Section: Introductionmentioning

confidence: 99%

Unsatisfactory Early and Late Outcomes After Fontan Surgery Delayed to Adolescence and Adulthood

Forsdick

Iyengar

Carins

et al. 2015

Seminars in Thoracic and Cardiovascular Surgery

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“…15 Yet, these improvements also came with the sobering observation that patients were prone to numerous long-term complications. 17 While no doubt multi-factorial, a number of these have been associated with the hemodynamics of the surgically created bypass connection, driving an increasing number of fluid dynamic investigations with the hope of ultimately improving patient outcome.…”

Section: Single-ventricle Heart Defects and Fontan Hemodynamicsmentioning

confidence: 99%

Patient-Specific Surgical Planning, Where Do We Stand? The Example of the Fontan Procedure

Zélicourt

Kurtcuoglu

2015

Ann Biomed Eng

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The Fontan surgery for single ventricle heart defects is a typical example of a clinical intervention in which patient-specific computational modeling can improve patient outcome: with the functional heterogeneity of the presenting patients, which precludes generic solutions, and the clear influence of the surgically-created Fontan connection on hemodynamics, it is acknowledged that individualized computational optimization of the post-operative hemodynamics can be of clinical value. A large body of literature has thus emerged seeking to provide clinically relevant answers and innovative solutions, with an increasing emphasis on patient-specific approaches. In this review we discuss the benefits and challenges of patient-specific simulations for the Fontan surgery, reviewing state of the art solutions and avenues for future development. We first discuss the clinical impact of patient-specific simulations, notably how they have contributed to our understanding of the link between Fontan hemodynamics and patient outcome. This is followed by a survey of methodologies for capturing patient-specific hemodynamics, with an emphasis on the challenges of defining patient-specific boundary conditions and their extension for prediction of post-operative outcome. We conclude with insights into potential future directions, noting that one of the most pressing issues might be the validation of the predictive capabilities of the developed framework.

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Trends in Fontan surgery and risk factors for early adverse outcomes after Fontan surgery: The Australia and New Zealand Fontan Registry experience

Cited by 91 publications

References 27 publications

Redefining Expectations of Long-Term Survival After the Fontan Procedure

Redefining Expectations of Long-Term Survival After the Fontan Procedure

Unsatisfactory Early and Late Outcomes After Fontan Surgery Delayed to Adolescence and Adulthood

Patient-Specific Surgical Planning, Where Do We Stand? The Example of the Fontan Procedure

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