Twenty-Five Year Outcomes of the Lateral Tunnel Fontan Procedure

Wilson, Thomas G.; Shi, William Y.; Iyengar, Ajay J.; Winlaw, David S.; Cordina, Rachael; Wheaton, Gavin; Bullock, Andrew; Gentles, Thomas L.; Weintraub, Robert G.; Justo, Robert; Grigg, Leeanne; Radford, Dorothy J.; d’Udekem, Yves

doi:10.1053/j.semtcvs.2017.06.002

Cited by 23 publications

(19 citation statements)

References 18 publications

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“…Retrospective studies of patients with functional SV with long-term follow-up data reported other risk factors that negatively impact prognosis including arrhythmia, atrioventricular valve regurgitation, and prolonged pleural effusion drainage [6,34]. Two large consortium studies (Single Ventricle Reconstruction Trial from North America [4,5,35] and the Australian and New Zealand Stage III Registry [8]) followed patients with functional SV physiology over time.…”

Section: Discussionmentioning

confidence: 99%

Loss of Ventricular Function After Bidirectional Cavopulmonary Connection: Who Is at Risk?

Vincenti¹,

Qureshi

Niaz

et al. 2020

Pediatr Cardiol

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Decline of single ventricle systolic function after bidirectional cavopulmonary connection (BDCPC) is thought to be a transient phenomenon. We analyzed ventricular function after BDCPC according to ventricular morphology and correlated this evolution to long-term prognosis. A review from Mayo Clinic databases was performed. Visually estimated ejection fraction (EF) was reported from pre-BDCPC to pre-Fontan procedure. The last cardiovascular update was collected to assess longterm prognosis. A freedom from major cardiac event survival curve and a risk factor analysis were performed. 92 patients were included; 52 had left ventricle (LV) morphology and 40 had right ventricle (RV) morphology (28/40 had hypoplastic left heart syndrome (HLHS)). There were no significant differences in groups regarding BDCPC procedure or immediate post-operative outcome. EF showed a significant and relevant decrease from baseline to discharge in the HLHS group: 59 ± 4% to 49 ± 7% or − 9% (p < 0.01) vs. 58 ± 3% to 54 ± 6% or − 4% in the non-HLHS RV group (p = 0.04) and 61 ± 4% to 60 ± 4% or − 1% in the LV group (p = 0.14). Long-term recovery was the least in the HLHS group: EF prior to Fontan 54 ± 2% vs. 56 ± 6% and 60 ± 4%, respectively (p < 0.01). With a median follow-up of 8 years post-BDCPC, six patients had Fontan circulation failure, four died, and three had heart transplantation. EF less than 50% at hospital discharge after BDCPC was strongly correlated to these major cardiac events (HR 3.89; 95% Cl 1.04-14.52). Patients with HLHS are at great risk of ventricular dysfunction after BDCPC. This is not a transient phenomenon and contributes to worse prognosis.

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Section: Discussionmentioning

confidence: 99%

Loss of Ventricular Function After Bidirectional Cavopulmonary Connection: Who Is at Risk?

Vincenti¹,

Qureshi

Niaz

et al. 2020

Pediatr Cardiol

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“…In a subgroup analysis, freedom from thromboembolism in extracardiac Fontan patients was 91% at 12 years. Lateral tunnel Fontan patients had a thromboembolism incidence of 16% [239,240].…”

Section: Incidence and Timing Of Thrombosismentioning

confidence: 99%

Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement

Zentner

Celermajer

Gentles

et al. 2020

Heart, Lung and Circulation

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The Fontan circulation describes the circulatory state resulting from an operation in congenital heart disease where systemic venous return is directed to the lungs without an intervening active pumping chamber. As survival increases, so too does recognition of the potential health challenges. This document aims to allow clinicians, people with a Fontan circulation, and their families to benefit from consensus agreement about management of the person with a Fontan circulation. The document was crafted with input from a multidisciplinary group of health care providers as well as individuals with a Fontan circulation and families. It is hoped that the shared common vision of long-term wellbeing will continue to drive improvements in care and quality of life in this patient population and eventually translate into improved survival.

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“…Plastic bronchitis is a rare complication (1–5%) after Fontan type single‐ventricle palliation, but with significant morbidity and mortality 3 . Adequate treatment should be based on understanding of the pathophysiology of the problem.…”

Section: Discussionmentioning

confidence: 99%

Direct EBUS‐guided transtracheal lymphosclerosis for plastic bronchitis after Fontan

Hubrechts¹,

Dooms²,

Maleux

et al. 2020

Cathet Cardio Intervent

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We report on a new puncture technique with direct transtracheal mediastinal lymphatic access to treat plastic bronchitis after Fontan repair. High resolution contrast‐enhanced spiral CT identified enlarged lymph nodes in the paratracheal region. Inguinal intranodal Gadolinium Dynamic Contrast‐enhanced Magnetic Resonance lymphangiography (DCMRL) confirmed the pathologic centrifugal lymph flow passing through these lymph nodes before leaking into the bronchial tree. The abnormal hypertrophic paratracheal, subcarinal, and hilar lymph nodes were punctured with a 22G needle through an endobronchial ultrasound bronchoscope. Occlusion of the lymph vessels was obtained by injecting a mixture of lipiodol/NBCA N‐butyl cyanoacrylate (Histoacryl) 5/1 under fluoroscopic control. There was a total remission of PB with now 10 months of follow‐up.

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Twenty-Five Year Outcomes of the Lateral Tunnel Fontan Procedure

Cited by 23 publications

References 18 publications

Loss of Ventricular Function After Bidirectional Cavopulmonary Connection: Who Is at Risk?

Loss of Ventricular Function After Bidirectional Cavopulmonary Connection: Who Is at Risk?

Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement

Direct EBUS‐guided transtracheal lymphosclerosis for plastic bronchitis after Fontan

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