Congenital Heart Disease
Meta-Analysis of the Effectiveness of Heart Transplantation in Patients With a Failing Fontan

https://doi.org/10.1016/j.amjcard.2017.01.001Get rights and content

The Fontan procedure is increasingly being used to palliate univentricular physiology. It is a complex anatomic and physiologic repair that can fail at any age, often leaving heart transplantation as the only remaining solution. A meta-analysis was performed to achieve the aim of systematically evaluating the existing evidence for survival after heart transplantation in patients who have undergone a Fontan palliation. MEDLINE, Embase, PubMed, and Web of Science were searched for original research studies. The primary outcome was mortality at 1 and 5 years after transplantation. Five hundred eighty-two records were screened, after the removal of duplicates, 12 retrospective observational studies were selected for inclusion in our meta-analysis. This encompassed a total of 351 Fontan patients undergoing heart transplantation. Mean age was 14 years (range 7 to 24 years) and 65% were men. One- and 5-year survival rates after heart transplantation were found to be 80.3% (95% CI 75.9% to 84.2%) and 71.2% (95% CI 66.3% to 75.7%), respectively. No significant association was found between age, gender, and pulmonary pressures and 1-year mortality. In conclusion, in the largest analysis to date, we found that heart transplantation in younger patients after Fontan procedure has an acceptable early and mid-term mortality. It is comparable to published mortality data of heart transplantation for other forms of congenital heart disease. Heart transplantation in the younger failing Fontan population appears to be a reasonable option when all other avenues have been exhausted and appropriate screening has taken place.

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Methods

A comprehensive literature search of PubMed, MEDLINE, Embase, and Web of Science was conducted in March 2015. Our search strategy included keywords Fontan AND “exp. heart transplantation.” In addition to keyword searches, separate title searches were also conducted in MEDLINE, Embase, and Web of Science. No limitations were used for date or publication status. Citations of eligible studies were reviewed for additional references; however, none were identified that were not retrieved already in

Results

Database searches using the previously described strategy yielded 996 results (Figure 1). Five hundred twenty-six studies irrelevant to the topic were excluded, and 44 were excluded for factors noted in Figure 1 with the use of duplicate data being the main reason for exclusion. A total of 12 studies published between 1995 and 2015 were included in the meta-analysis, with 351 patients undergoing heart transplantation (Table 1).

All the 12 studies selected for inclusion in this meta-analysis are

Discussion

There are multiple smaller studies that report on outcomes following heart transplantation in the Fontan population. We considered it important to add to the literature a more global perspective on transplant outcomes in this complex population. Our meta-analysis, inclusive of 351 patients, revealed 1- and 5-year survival rates of 80% and 71%, respectively, in Fontan patients after heart transplantation. This mostly included studies that looked at transplantation performed in a younger

Disclosures

The authors have no conflicts of interest to disclose.

References (30)

Cited by (35)

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    2022, Canadian Journal of Cardiology
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    An earlier report from this registry provided the detail that 53% of HTx procedures were performed during childhood, and 47% during ACHD follow-up.16 The 2 meta-analyses of HTx for FCF that have been conducted both included data from patients across the age spectrum, the first from 351 patients (mean age: 14 years), and the second from 426 patients (mean age: 21 years).17,18 Both identified that substantially more HTx procedures have been performed in male patients, despite the approximately equal gender distribution among Fontan patients.

  • Improving outcomes for transplantation in failing Fontan—what is the next target?

    2021, JTCVS Open
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    In our cohort, mortality plateaued at 6 months, with good medium-term survival of 71% at 1 year and 67% at 5 years. These findings are in line with the results of a recently published meta-analysis that included a total of 351 Fontan patients undergoing OHT and showed 1- and 5-year survival rates of 80.3% (95% CI, 75.9%-84.2%) and 71.2% (95% CI, 66.3%-75.7%), respectively.17 Primary graft failure is a known complication after OHT for failing Fontan circulation.18,19

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