Key result
Tailored chemotherapy yields lymphoma remission in ~80% of adults with Fontan circulation.
Why the study?
There is a paucity of data regarding the outcome of lymphoma treatment among adult patients with Fontan circulation despite the growing population and cancer being a leading cause of mortality in congenital heart disease.
Case Report (n=5)
No
Adult patients with Fontan circulation can successfully undergo curative chemotherapy for lymphoma when managed with careful dose modifications, rigorous monitoring of volume status and ventricular function, and multidisciplinary cardio-oncology collaboration.
May support monitored, adjusted chemotherapy in select Fontan patients with lymphoma; hypothesis-generating and should not yet change practice.
Background While cancer is the fourth leading cause of mortality in adults with congenital heart disease, and despite the approximately 30,000 patients with Fontan circulation living in the United States, there is a paucity of data regarding the outcome of lymphoma treatment among single ventricle patients with Fontan circulation. Case presentation We present a case series of 5 patients with Fontan circulation who developed lymphoma and underwent curative chemotherapy, including 2 patients with Hodgkins Lymphoma and 3 patients with large B-cell lymphoma. We describe their congenital anatomy including systemic ventricular morphology, Fontan operation each patient underwent, their clinical presentations, cancer treatments, and relevant diagnostic data. We discuss the pathophysiology relevant to Fontan circulation and illustrate the important considerations regarding treatment of lymphoma. Conclusion Fontan circulation presents complex pathophysiologic manifestations with respect to cardiotoxic cancer therapeutics that require vigilance, close monitoring, and multidisciplinary collaboration. In this case series, we explain our institutional experience regarding treatment of lymphoma among patients with Fontan circulation. Further studies regarding treatment of hematologic malignancy in this unique and ever-growing population are needed.
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Hirschbaum et al. (2026) conducted a case report in Lymphoma in patients with Fontan circulation (n=5). Chemotherapy (e.g., ABVD, DA-R-EPOCH, ICE) was evaluated on Survival and remission status. Among 5 adult patients with Fontan circulation and lymphoma, 4 survived and achieved remission following carefully monitored and often dose-adjusted chemotherapy regimens.
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