Factors associated with long-term mortality after Fontan procedures: a systematic review

Alsaied, Tarek; Bokma, Jouke P.; Engel, Mark E; Kuijpers, Joey M.; Hanke, Samuel P.; Zühlke, Liesl; Zhang, Bin; Veldtman, Gruschen

doi:10.1136/heartjnl-2016-310108

Cited by 122 publications

(81 citation statements)

References 49 publications

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“…Post-operatively six patients were extubated at a median of 3 days (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19), and the other three required tracheostomy at a median of 17 days (14)(15)(16)(17)(18)(19)(20)(21)(22)(23). Post-operatively six patients were extubated at a median of 3 days (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19), and the other three required tracheostomy at a median of 17 days (14)(15)…”

Section: Peri-operativementioning

confidence: 99%

“…1 Fontan failure occurs due to systolic or diastolic dysfunction, atrioventricular valve regurgitation, arrhythmia, and/or lymphatic abnormalities. 1 Fontan failure occurs due to systolic or diastolic dysfunction, atrioventricular valve regurgitation, arrhythmia, and/or lymphatic abnormalities.…”

Section: Introductionmentioning

confidence: 99%

“…Heart failure is the leading cause of death in single ventricle patients palliated with the Fontan procedure. 1 Fontan failure occurs due to systolic or diastolic dysfunction, atrioventricular valve regurgitation, arrhythmia, and/or lymphatic abnormalities. 2,3 Additionally, multi-organ dysfunction is common in this population; particularly, hepatic dysfunction referred to as Fontan-associated liver disease (FALD).…”

Section: Introductionmentioning

confidence: 99%

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Short‐term outcomes of en bloc combined heart and liver transplantation in the failing Fontan

Vaikunth

Concepción

Daugherty

et al. 2019

Clinical Transplantation

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Patients with failing Fontan physiology and liver cirrhosis are being considered for combined heart and liver transplantation. We performed a retrospective review of our experience with en bloc combined heart and liver transplantation in Fontan patients > 10 years old from 2006 to 18 per Institutional Review Board approval. Six females and 3 males (median age 20.7, range 14.2-41.3 years) underwent en bloc combined heart and liver transplantation. Indications for heart transplant included ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia, and/or lymphatic abnormalities. Indication for liver transplant included portal hypertension and cirrhosis. Median Fontan/single ventricular end-diastolic pressure was 18/12 mm Hg, respectively. Median Model for End-Stage Liver Disease excluding International Normalized Ratio score was 10 (7-26), eight patients had a varices, ascites, splenomegaly, thrombocytopenia score of ≥ 2, and all patients had cirrhosis. Median cardiopulmonary bypass and donor ischemic times were 262 (178-307) and 287 (227-396) minutes, respectively. Median intensive care and hospital stay were 19 (5-96) and 29(13-197) days, respectively. Survival was 100%, and rejection was 0% at 30 days and 1 year post-transplant. En bloc combined heart and liver transplantation is an acceptable treatment in the failing Fontan patient with liver cirrhosis.

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Section: Peri-operativementioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Short‐term outcomes of en bloc combined heart and liver transplantation in the failing Fontan

Vaikunth

Concepción

Daugherty

et al. 2019

Clinical Transplantation

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“…Death in these patients is mostly related to sudden death with presumed arrhythmic origin, thromboembolic complications, or heart failure. [1][2][3] Ventricular dysfunction is common in young adults with Fontan circulation and has been identified as a risk factor for mortality. [4][5][6] However, the etiology of ventricular dysfunction over time in Fontan patients is unknown.…”

Section: Introductionmentioning

confidence: 99%

Ventricular mechanics in adolescent and adult patients with a Fontan circulation: Relation to geometry and wall stress

et al. 2018

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Background Patients with single ventricle physiology and Fontan circulation are at increased risk for late complications and reduced survival. The aim of the study was to investigate the correlation between ventricular geometry and systolic regional function in different underlying anatomic conditions in adolescent and adult Fontan‐palliated patients. Method In a retrospective cross‐sectional study, we measured 2D strain, ventricular diameters, ventricular volumes, ejection fraction (EF), global and segmental wall stress, and sphericity index. The same analyses were performed in 99 age‐ and gender‐matched healthy individuals. Results One hundred and one patients were included at a mean age of 21 (range 14–59) years. In comparison with healthy subjects, patients with Fontan circulation displayed larger ventricular volumes (153 ± 78 mL vs 116 ± 38 mL P < 0.05), reduced EF (43% ± 15% vs 55% ± 8% P < 0.05), reduced longitudinal (−13% ± 6% vs −21% ± 4% P < 0.05) and circumferential strain values (−15% ± 7% vs −22% ± 4% P < 0.05). Functionally single ventricles were more spherical (ratio of longitudinal to short‐axis diameters 1.3 ± 0.3 vs 1.7 ± 0.2 P < 0.05). Circumferential strain correlated well with global wall stress and the degree of sphericity (R2 = 0.320), while segmental strain did not correlate with segmental wall stress. The percentage of segments with akinesia was relatively high (16 ± 16% vs 0 ± 0% P < 0.05) indicating reduced segmental contractile function. Conclusion Functionally single ventricles after Fontan palliation have significantly reduced systolic regional and global function with a high intersegmental inhomogeneity. The underlying pathological mechanisms might be multifactorial, including ventricular geometry, sphericity, and regional contractile properties. Future studies are needed to investigate the role of ventricular geometry for clinical performance and outcome.

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“…31 In patients who survive the initial series of corrective surgeries, SCD due to malignant VAs is a common cause of mortality in patients with single-ventricle physiology, following ventricular failure. 32 Owing to a lack of data identifying risk factors for VAs in this population, proposed predictors of SCD are based on expert opinion. The generally accepted risk factors are summarized in Table 1.…”

mentioning

confidence: 99%

The Use of Implantable Cardioverter-defibrillators in the Prevention of Sudden Cardiac Death: A Focus on Congenital Heart Disease and Inherited Arrhythmia Syndromes

Goldstein

Ward

Al‐Khatib

2017

J Innov Cardiac Rhythm Manage

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Factors associated with long-term mortality after Fontan procedures: a systematic review

Abstract: Causes and factors associated with late mortality after the Fontan operation are summarised in this study. The presented information will aid in identifying patients at highest risk for mortality and guide our risk stratification efforts in this patient population.

Cited by 122 publications

References 49 publications

Short‐term outcomes of en bloc combined heart and liver transplantation in the failing Fontan

Short‐term outcomes of en bloc combined heart and liver transplantation in the failing Fontan

Ventricular mechanics in adolescent and adult patients with a Fontan circulation: Relation to geometry and wall stress

The Use of Implantable Cardioverter-defibrillators in the Prevention of Sudden Cardiac Death: A Focus on Congenital Heart Disease and Inherited Arrhythmia Syndromes

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