Significant Improvements in Mortality After the Fontan Operation in Children With Down Syndrome

Sarno, Lauren; Walters, Henry L.; Bondarenko, Igor; Thomas, Ronald; Kobayashi, Daisuke

doi:10.1016/j.athoracsur.2019.07.085

Cited by 11 publications

(11 citation statements)

References 10 publications

(13 reference statements)

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“…A study of 683 adult Fontan patients published in 2018 from the Australian and New Zealand Fontan Registry found that, by age 40, mortality reached 20%, only 53% of patients were free of heart failure symptoms, and only 41% were free of serious adverse events [1]. Similar data has been widely reported [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18]. Approximately half of the instances of mortality and significant morbidity can be attributed to the failure of the unique Fontan circulatory system.…”

Section: Introductionmentioning

confidence: 78%

Parametric Investigation of an Injection-Jet Self-Powered Fontan Circulation

Prather

Das²,

Farias

et al. 2021

Preprint

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Rationale: Approximately 1/2500 babies are born with only one functioning ventricle and the Fontan is the third and, ideally final staged palliative operation for these patients. This altered circulation is prone to failure with survival rates below 50% into adulthood in some series. Chronically elevated inferior vena cava (IVC) pressure is implicated as one cause of the mortality and morbidity in this population.Objective: An injection jet shunt (IJS) drawing blood-flow directly from the aortic arch to significantly lower IVC pressure is proposed.Methods and Results: A computer-generated 3D model of a 2–4 year old patient with a fenestrated Fontan and a cardiac output of 2.3L/min was generated. The detailed 3D pulsatile hemodynamics are resolved in a zero-dimensional lumped parameter network tightly-coupled to a 3D computational fluid dynamics model accounting for non-Newtonian blood rheology and resolving turbulence using large eddy simulation. IVC pressure and systemic oxygen saturation were tracked for various IJS-assisted Fontan configurations, altering design parameters such as shunt and fenestration diameters and locations.A baseline “failing” Fontan with 4mm fenestration was tuned to elevated IVC pressure ($+17.8mmHg$). Enlargement of the fenestration to 8mm resulted in a 3mmHg IVC pressure drop but an unacceptable reduction in systemic oxygen saturation below 80%. Addition of an IJS with a 2mm nozzle and minor volume load to the ventricle improved the IVC pressure drop to 3.2mmHg while increasing systemic oxygen saturation above 80%.Conclusions: The salutary effects of the IJS to effectively lower IVC pressure while retaining acceptable levels of oxygen saturation are successfully demonstrated.

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Section: Introductionmentioning

confidence: 78%

Parametric Investigation of an Injection-Jet Self-Powered Fontan Circulation

Prather

Das²,

Farias

et al. 2021

Preprint

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“…Previous studies describing outcomes of FO in ECD patients have smaller number of patients (between 80 and 151 patients) than our study of 360 pateints, 7–9 are over a longer study period spanning several decades resulting in an era effect (1966–2016), 7–9,13 look at the longitudinal outcomes of ECD patients undergoing single ventricle palliation overall rather than just the FO and without any direct comparison with a non‐ECD cohort during the same study period 5,8,9 . Our study thus has a larger cohort of ECD patients undergoing a FO, over shorter study period and directly compares short‐term outcomes of the FO in the ECD group versus the non‐ECD group.…”

Section: Discussionmentioning

confidence: 95%

“…14 Outcomes of FO in Down syndrome have been mixed with some data suggesting better outcomes and others worse outcomes. 13,15,17 The outcomes in Down syndrome are suboptimal especially when associated with AV valvar insufficiency and pulmonary hypertension. 16,18,19 However, in our cohort, despite a slightly higher incidence of Down syndrome in the ECD cohort, it was not an independent predictor of mortality on multivariable analysis.…”

Section: Discussionmentioning

confidence: 99%

“…7 However, once a patient can undergo a successful FO, the long-term prospects are reasonable in the absence of more than moderate valvular insufficiency and good ventricular function, particularly in the recent times. 8,9 Suboptimal results for ECD are likely due to association with Down syndrome, 6,13 HS, 6,22 AV valvar insufficiency, 8,23 and early propensity to develop pulmonary vascular disease particularly if the pulmonary vascular bed is unprotected. 19,26 Interestingly, despite the higher incidence of pulmonary hypertension in ECD patients, it was not an independent predictor for mortality for a FO.…”

Section: Discussionmentioning

confidence: 99%

“…When Down syndrome patients undergo a FO, the underlying primary cardiac diagnosis is generally ECD in ≥75% of the patients 14 . Outcomes of FO in Down syndrome have been mixed with some data suggesting better outcomes and others worse outcomes 13,15,17 . The outcomes in Down syndrome are suboptimal especially when associated with AV valvar insufficiency and pulmonary hypertension 16,18,19 .…”

Section: Discussionmentioning

confidence: 99%

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National outcomes of the Fontan operation with endocardial cushion defect

Sainathan

Said

Agala

et al. 2022

Journal of Cardiac Surgery

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Background: The traditional outcomes of the Fontan operation (FO) in endocardial cushion defect (ECD) patients have been suboptimal. Previous studies have been limited by the smaller number of ECD patients, longer study period with an era effect, and do not directly compare short-term outcomes of FO in ECD patients with non-ECD patients. Our study aims to address these shortcomings.Methods: A retrospective analysis of the Kids Inpatient Database (2009Database ( , 2012Database ( , and 2016 for the FO was done. The groups were divided into those who underwent FO with ECD as compared to non-ECD diagnosis. The data were abstracted for demographics, clinical characteristics, and operative outcomes. Standard statistical tests were used.Results: Three thousand three hundred eighty patients underwent the FO of which 360 patients (11%) were FO-ECD. ECD patients were more likely to have Down syndrome, Heterotaxy syndrome, transposition/DORV, and TAPVR as compared to non-ECD patients. FO-ECD had a higher discharge-mortality (2.84% vs. 0.45%, p = .04). The length of stay (16 vs. 13 days, p = .05) and total charges incurred ($283, 280 vs. $234, 106, p = .03) for the admission were higher in the FO-ECD as compared to non-ECD patients. In multivariable analysis, ECD diagnosis, cardiac arrest, acute kidney injury, and postoperative hemorrhage were predictors of mortality. Conclusion:Contemporary outcomes for FO are excellent with very low overall operative mortality. However, the outcomes in ECD patients are inferior with higher operative mortality than in non-ECD patients. The occurrence of postoperation complications and a diagnosis of ECD were predictive of a negative outcome.

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