Heart transplantation in Fontan patients across Australia and New Zealand

Shi, William Y.; Yong, Matthew S.; McGiffin, David C.; Jain, Pankaj; Ruygrok, Peter; Marasco, Silvana; Finucane, Kirsten; Keogh, Anne; d’Udekem, Yves; Weintraub, Robert G.; Konstantinov, Igor E.

doi:10.1136/heartjnl-2015-308848

Cited by 41 publications

(39 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…We found a difference in rate of transplantation across centers, ranging from 0%–21%. Significant regional differences in rates of heart transplantation among Fontan patients have recently been reported in Australia and New Zealand (21). Although many factors are likely responsible for this variability, our findings suggest that there remains an unmet need for standardization in criteria used for consideration of heart transplantation in the Fontan population.…”

Section: Discussionmentioning

confidence: 99%

Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure

Atz

Zak

Mahony

et al. 2017

Journal of the American College of Cardiology

206

154

View full text Add to dashboard Cite

Background Multicenter longitudinal objective data for Fontan patients surviving into adulthood are lacking. Objectives Describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time. Methods Exercise testing, echocardiography, B-type natriuretic peptide (BNP), functional health assessment, and medical history abstraction were repeated 9.4 ± 0.4 years after the Fontan Cross-Sectional Study (Fontan 1) compared to previous values. Cox regression analysis explored risk factors for interim death or cardiac transplantation. Results From the original cohort of 546 subjects, 466 were recontacted and 373 (80%) were enrolled at 21.2 ± 3.5 years of age. Among subjects with paired testing, percent predicted maximum VO2 decreased (69 ± 14 vs. 61 ± 16, p <0.001, n = 95), ejection fraction decreased (58 ± 11 vs. 55 ± 10, p <0.001, n=259), and BNP increased (Median (IQR) 13 (7,25) vs. 18 (9,36) pg/mol, p <0.001, n = 340). At latest follow-up lower Pediatric Quality of Life Inventory (PedsQL) physical summary score was associated with poorer exercise performance (R2 adjusted = 0.20, p <0.001, n = 274). Cumulative complications since the Fontan included additional cardiac surgery (32%), catheter intervention (62%), arrhythmia treatment (32%), thrombosis (12%), and protein losing enteropathy (8%). Since Fontan 1, 54 subjects (10%) have received a heart transplant (n = 23) or died without transplantation (n = 31). The interval risk of death/transplantation was associated with poorer ventricular performance and functional health status assessed at Fontan 1, but was not associated with ventricular morphology, subject age or type of Fontan connection. Conclusions Interim transplant-free survival over 12 years in this Fontan cohort was 90% and was independent of ventricular morphology. Exercise performance decreased and was associated with worse functional health status. Future interventions might focus on preserving exercise capacity. (Clinical Trials Registration #: NCT00132782)

show abstract

Section: Discussionmentioning

confidence: 99%

Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure

Atz

Zak

Mahony

et al. 2017

Journal of the American College of Cardiology

206

154

View full text Add to dashboard Cite

show abstract

“…In reality, heart transplantation has taken only a very small part in the treatment armentarium of this population with only 4% to 7% reaching this status within 20 years of the Fontan [49, 68]. The reasons for the infrequent use of this ultimate resource are likely the multiplicity of previous operations increasing the risk of reentry and immunoreactivity, the poor general status of the patients and the complexity of the reconstruction necessary [69, 70].…”

Section: Heart Transplantationmentioning

confidence: 99%

“…The reasons for the infrequent use of this ultimate resource are likely the multiplicity of previous operations increasing the risk of reentry and immunoreactivity, the poor general status of the patients and the complexity of the reconstruction necessary [69, 70]. It has been recognized that those with a Fontan circulation did not have equal access to transplantation because this access is dependent on structuring of heart transplantation programs with expertise in congenital heart diseases [68]. A large multi-centric study has suggested that post-transplantation survival in patients with a previous Fontan surgery was inferior to the survival of patients with idiopathic dilated cardiomyopathy and bi-ventricular congenital heart diseases [70].…”

Section: Heart Transplantationmentioning

confidence: 99%

“…Numerous single center studies have since demonstrated equal late outcomes after transplantation of patients with a Fontan circulation including those with HLHS. It demonstrates that heart transplantation should be offered to patients with a Fontan circulation and that restriction of this practice to expert centers may be advisable [68,71–73]. …”

Section: Heart Transplantationmentioning

confidence: 99%

See 1 more Smart Citation

Current Therapy for Hypoplastic Left Heart Syndrome and Related Single Ventricle Lesions

2016

View full text Add to dashboard Cite

Universally fatal only four decades ago, the progress in the three-stage palliation of hypoplastic left heart syndrome and related single right ventricular lesions has drastically improved the outlook for these patients. While the stage II operation (hemi-Fontan or bidirectional Glenn) and stage III Fontan procedure have evolved into relatively low risk operations, the stage I Norwood procedure remains one of the highest risk and costliest common operations performed in congenital heart surgery. Yet, despite this fact, experienced centers now report hospital survivals in excess of 90% for the Norwood. This traditional three-stage surgical palliation has seen several innovations in the past decade aimed at improving outcomes, particularly for the Norwood procedure. One significant change is a renewed interest in the right ventricle-to-pulmonary artery shunt as the source of pulmonary blood flow, rather than the modified Blalock-Taussig shunt for the Norwood. The multi-institutional Single Ventricle Reconstruction trial randomized 555 patients to one or the other shunt, and these subjects continue to be followed closely as they now approach 10 years post-randomization. In addition to modifications to the Norwood procedure, the “hybrid procedure,” a combined catheter-based and surgical approach, avoids the Norwood procedure in the newborn period entirely. The initial hybrid procedure is then followed by a “comprehensive” stage II, that combines components of both the Norwood and the traditional stage II, and later completion of the Fontan. Proponents of this approach hope to not only improve short-term survival, but potentially longer-term outcomes, such as neurodevelopment, as well. Regardless of the approach, traditional surgical staged palliation or the hybrid procedure, survivals have vastly improved, and large numbers of these patients are surviving not only through their Fontan in early childhood, but into adolescence and young adulthood. As this population grows, it becomes increasingly important to understand the longer-term outcomes of these Fontan patients, not only in terms of survival, but also burden of disease, neurodevelopmental outcomes, psychosocial development and quality of life.

show abstract

“…Unfortunately, re‐routing of the systemic venous return to bypass the heart and empty directly into the pulmonary arteries results in complications from a continuously elevated central venous pressure. The threat of arrhythmias, protein losing enteropathy, liver failure, malnutrition, and heart failure continues to plague this population throughout their lives . Despite the excellent survival, freedom from cardiac events is only 29% at 25 years …”

Section: Introductionmentioning

confidence: 99%

A modified LVAD technique to augment caval and pulmonary arterial blood flow in the “failing Fontan” circulation

et al. 2017

View full text Add to dashboard Cite

show abstract

Heart transplantation in Fontan patients across Australia and New Zealand

Cited by 41 publications

References 15 publications

Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure

Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure

Current Therapy for Hypoplastic Left Heart Syndrome and Related Single Ventricle Lesions

A modified LVAD technique to augment caval and pulmonary arterial blood flow in the “failing Fontan” circulation

Contact Info

Product

Resources

About