Improved survival after heart transplant for failed Fontan patients with preserved ventricular function

doi:10.1016/j.healun.2016.02.005

The Journal of Heart and Lung Transplantation

Volume 35, Issue 7, July 2016, Pages 877-883

https://doi.org/10.1016/j.healun.2016.02.005 Get rights and content

Background

Patients with a failing Fontan continue to have decreased survival after heart transplant (HT), particularly those with preserved ventricular function (PVF) compared with impaired ventricular function (IVF). In this study we evaluated the effect of institutional changes on post-HT outcomes.

Methods

Data were retrospectively collected for all Fontan patients who underwent HT. Mode of failure was defined by the last echocardiogram before HT, with mild or no dysfunction considered PVF and moderate or severe considered IVF. Outcomes were compared between early era (EE, 1995 to 2008) and current era (CE, 2009 to 2014). Management changes in the CE included volume load reduction with aortopulmonary collateral (APC) embolization, advanced cardiothoracic imaging, higher goal donor/recipient weight ratio and aggressive monitoring for post-HT vasoplegia.

Results

A total of 47 patients were included: 27 in the EE (13 PVF, 14 IVF) and 20 in the CE (12 PVF, 8 IVF). Groups were similar pre-HT, except for more PLE in PVF patients. More patients underwent APC embolization in the CE (80% vs 28%, p < 0.01). There was no difference in donor/recipient weight ratio between eras. There was a trend toward higher primary graft failure for PVF in the EE (77% vs 36%, p = 0.05) but not the CE (42% vs 75%, p = 0.20). Overall, 1-year survival improved in the CE (90%) from the EE (63%) (p = 0.05), mainly due to increased survival for PVF (82 vs 38%, p = 0.04).

Conclusions

Post-HT survival for failing Fontan patients has improved, particularly for PVF. In the CE, our Fontan patients had a 1-year post-HT survival similar to other indications.

Section snippets

Methods

An institutional database was queried for all patients who underwent HT at St. Louis Children’s Hospital from January of 1995 through December of 2014. All patients transplanted with a previous Fontan, but who had not undergone Fontan takedown, were included. Only single-organ transplant patients were included. This study protocol was approved by the internal review board of the Washington University School of Medicine, with need for patient consent waived.

Patient characteristics by era

During the study period, 337 HTs were performed at St. Louis Children’s Hospital: 217 in the EE and 120 in the CE. Of these, 47 Fontan patients underwent heart transplant, including 27 in the EE and 20 in the CE. Among the EE patients, 13 had PVF and 14 had IVF, as compared with the CE with 12 PVF and 8 IVF cases.

Pre-transplant characteristics were similar between eras, including age at transplant, body surface area (BSA), gender, systemic ventricle morphology and presence of Fontan

Discussion

Previous reports have shown that failing Fontan patients have a decreased post-HT survival compared with other indications, particularly for patients with PVF.4, 8, 14 Earlier experience from our institution also suggested a trend toward decreased post-HT survival in the PVF Fontan patient population.⁵ In line with these reports, our EE overall Fontan 1-year survival was a discouraging 63%, with the majority of mortality concentrated in the PVF population. However, the CE survival analysis is

Disclosure statement

C.E.C. reports having received travel reimbursement from Berlin Heart. This study was supported by a grant from the National Institutes of Health (T32 HL007776).

The remaining authors have no conflicts of interest to disclose.

These data were presented at the 35th annual meeting and scientific sessions of the International Society for Heart and Lung Transplantation, April 2015, Nice, France.

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Citation Excerpt :
The importance of careful patient selection was emphasized in a study reporting the lowest pediatric single-centre early mortality rate of 4%.68 An improved 1-year survival rate, from 63% in 1995-2008, (n = 27) to 90% in 2009-2014 (n = 20) was felt to be driven largely by systematic changes in approach and increased institutional experience.32 Factors related to the transplant program also influence both HTx waitlist mortality and early post-transplant outcomes in ACHD patients.72
In the current era, 5%-10% of Fontan patients die or need a transplant in childhood, and approximately 50% will experience the same fate by age 40 years. Heart transplant (HTx) can be successful for selected children and adults with Fontan circulatory failure of any mechanism, with a 1-year post-transplant survival rate approaching 90% in children and 80% in the largest single-centre adult Fontan HTx experience. Protein-losing enteropathy and plastic bronchitis can be expected to resolve post-transplant, and limited data suggest patients with Fontan-associated liver disease who survive HTx can expect improvement in liver health. Early Fontan failure, within 12 months of Fontan completion, is not easily rescued by HTx, and late referrals and failure to refer adult patients remain problematic. Very little is known about the numbers of patients who are not referred, are turned down following assessment for HTx, or die on the waiting list—numbers that are needed to understand the complete picture of HTx in the Fontan population and to identify where best to focus quality-improvement efforts. Recent revisions to listing prioritization in Canada with considerations specific to the Fontan population aim to mitigate the fact that the status-listing criteria are not tailored to the congenital heart population. Transplanting high-risk children prior to Fontan completion, developing adult congenital heart disease transplant centres with expertise that can also offer combined heart-liver transplant when appropriate, and improving single-ventricle mechanical support options and criteria for both adults and children may help mitigate the early post-listing mortality.
De nos jours, les patients qui ont subi l’intervention de Fontan meurent ou doivent subir une transplantation cardiaque pendant leur enfance dans 5 à 10 % des cas ou avant l’âge de 40 ans dans environ 50 % des cas. La transplantation cardiaque (TC) peut être pratiquée avec succès chez certains enfants et adultes qui ont subi l’intervention de Fontan et qui viennent à présenter une insuffisance circulatoire, quelle qu’en soit la cause. Par exemple, dans le cadre de la plus grande expérience monocentrique portant sur la TC postérieure à l’intervention de Fontan, le taux de survie à 1 an des personnes ayant subi une transplantation était de près de 90 % chez les enfants et de 80 % chez les adultes suivis. On peut s’attendre à ce que l’entéropathie avec perte de protéines et la bronchite plastique se résolvent après la transplantation, et les données limitées dont nous disposons donnent à penser que les patients atteints d’une maladie du foie associée à la circulation de Fontan qui survivent à la TC devraient voir leur fonction hépatique s’améliorer. La TC ne permet pas de remédier facilement à l’échec rapide de l’intervention de Fontan, c’est-à-dire moins de 12 mois après sa réalisation, et l’aiguillage tardif ou l’absence d’aiguillage des patients adultes vers les services de transplantation restent problématiques. On sait très peu de choses sur le nombre de patients qui ne sont pas aiguillés vers les services de transplantation, qui ne sont pas retenus comme candidats à la TC au terme d’une évaluation de leur admissibilité ou qui meurent dans l’attente d’une TC. Des données quantitatives sont nécessaires pour se faire une idée plus complète de la question des TC chez les patients ayant subi l’intervention de Fontan et pour mieux cibler nos efforts en matière d’amélioration de la qualité. Les révisions récentes de la priorisation des inscriptions au Canada tiennent compte spécifiquement du cas des patients ayant subi l’intervention de Fontan et visent à pallier le fait que les critères d’inscription en fonction du statut des patients ne sont pas adaptés au contexte des cardiopathies congénitales. Le recours à la transplantation chez les enfants à haut risque en amont de l’intervention de Fontan, la création de centres de transplantation ayant la capacité de prendre en charge les adultes atteints de cardiopathies congénitales et possédant l’expertise nécessaire pour pratiquer une transplantation combinée cœur-foie, au besoin, et l’amélioration des options et des critères d’assistance monoventriculaire mécanique applicables chez les adultes et les enfants peuvent contribuer à limiter la mortalité précoce parmi les patients en attente de transplantation.

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Improved survival after heart transplant for failed Fontan patients with preserved ventricular function

Background

Methods

Results

Conclusions

Section snippets

Methods

Patient characteristics by era

Discussion

Disclosure statement

J Heart Lung Transplant

Ann Thorac Surg

J Am Coll Cardiol

Ann Thorac Surg

J Heart Lung Transplant

J Heart Lung Transplant

Ann Thorac Surg

J Am Coll Cardiol

J Heart Lung Transplant

Transplant Proc

J Heart Lung Transplant

J Heart Lung Transplant

J Heart Lung Transplant

Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery

Circulation

Redefining expectations of long-term survival after the Fontan procedure: twenty-five years of follow-up from the entire population of Australia and New Zealand

Circulation

Trends in the indications and survival in pediatric heart transplants: a 24-year single-center experience in 307 patients

Ann Thorac Surg