Gabriel Hernandez, MD FACC recently published the article “Heart transplantation and in‐hospital outcomes in adult congenital heart disease patients with Fontan: A decade nationwide analysis from 2004 to 2014” in the Journal of Cardiac Surgery. Read the full article here. Dr. Hernandez tells us about the paper, its findings and potential impact on adult congenital heart disease patients.
Which group of patients did you study?
We sought to evaluate the early outcomes of heart transplants in adult Fontan patients. With the improvement in therapies for infants and children with congenital heart disease, a growing number of these patients reach adulthood. Even though early surgical interventions are lifesaving, long-term complications are frequent. An example of this is the Fontan surgery, which is a palliative correction of single ventricle morphology, a congenital defect non-compatible with life. Adults with Fontan palliation are still afflicted by a heavy burden of disease and have a limited adulthood survival. Early intervention with a definitive therapy, such as heart transplantation, is not widely offered to these patients.
What did your study find?
We found that only 0.5% (n=93) of transplant surgeries performed in the US during a decade of analysis were done in adults with Fontan (worldwide Fontan population exceeds 70,000). Despite these patients being “highly selected” (younger, and with less co‐morbid conditions such as diabetes and chronic lung disease), they had exceptionally high mortality during their index transplant hospitalization (26.3% vs 5.3% OR 18.10, 95% CI 5.06‐65.0 P < 0.001). This finding is likely related to a delayed referral, evidenced by the high rate of overt liver disease and resultant coagulopathy that we found. Interestingly, there were no in‐hospital deaths in the subgroup of patients receiving simultaneous liver transplants during the same hospitalization.
What gaps in knowledge do these findings fill? What gaps still persist?
It is key to understand that adults with Fontan have a limited life expectancy and heart transplant may be the only available therapy to prolong their life; mechanical circulatory support devices (i.e destination LVAD) are not currently on their prime time for them. These patients need to be referred early to centers with an established adult congenital heart disease (ACHD) and heart transplantation program such as UMMC. We believe that earlier transplantation in capable centers may lower this early mortality.
Were these findings surprising to you?
The reason we decided to undertake this study was due to the relatively high number of failing Fontan referred for transplant. It was surprising however to see this significant difference in early mortality. We know from prior studies that the 1-year conditional survival (those who survived the first year) is better in adults with congenital heart disease compared to other forms of cardiomyopathies. If we can decrease the excess in early mortality, with early transplantation then this can be a game changer.
What additional research is needed?
There are substantial opportunities for further research to better define Fontan failure, validate prognostic tools and more clearly define indications for advanced heart failure therapies referral. Moving forward, more intentional training and multi‐disciplinary collaboration and research between ACHD and heart failure providers is necessary to expedite advanced heart failure therapies for these patients.