RESUMO
Heart failure is the leading cause of morbidity and mortality in patients with Fontan circulation. Sodium-glucose-cotransporter 2 inhibitors (SGLT2i) have become a mainstay of heart failure therapy in adult patients, however, there remains a paucity of literature to describe its use in pediatric heart failure patients, especially those with single ventricle physiology. We describe our early experience using SGLT2i in patients with single ventricle congenital heart disease surgically palliated to the Fontan circulation. We conducted a single-center retrospective chart review of all patients with Fontan circulation who were initiated on an SGLT2i from January 1, 2022 to March 1, 2023. Patient demographics, diagnoses, clinical status, and other therapies were collected from the electronic medical record. During the study period, 14 patients (median age 14.5 years, range 2.0-26.4 years) with Fontan circulation were started on a SGLT2i. Mean weight was 54 kg (range 11.6-80.4 kg). Median follow-up since SGLT2i initiation was 4.1 months (range 13 days-7.7 months). Four patients had a systemic left ventricle and 10 had a systemic right ventricle. Half the patients had Fontan Circulatory Failure with reduced Ejection Fraction (FCFrEF) of the systemic ventricle and the other half had Fontan Circulatory Failure with preserved Ejection Fraction (FCFpEF) of the systemic ventricle. In addition, 3 patients experienced Protein Losing Enteropathy (PLE) and 2 patients had plastic bronchitis, one of whom also was diagnosed with chylothorax. There were no genitourinary infections, hypoglycemia, ketoacidosis, hypotension or other significant adverse effects noted in our patient population. One patient experienced significant diuresis and transient acute kidney injury. Patients with FCFrEF showed a decrease in natriuretic peptide levels. Given the lack of proven therapies, demonstrated benefits of SGLT2i in other populations, and some suggestion of efficacy in Fontan circulation, further study of SGTLT2i in patients with Fontan circulation is warranted.
RESUMO
Anemia is a predictor of morbidity and mortality in both pediatric and adult patients with heart failure. This risk is increased in patients who require ventricular assist device (VAD) placement. The most common mechanism suggested for why these patients develop anemia is chronic inflammation caused by the immune system reacting to the VAD components. The inflammatory response that occurs can suppress erythropoiesis by inhibiting production of erythropoietin. Studies have demonstrated that anemic VAD patients have lower-than-expected erythropoietin levels, which leads to the consideration of erythropoiesis-stimulating agents (ESAs) in this population. Therapy with ESAs can increase hemoglobin and hematocrit levels, thereby decreasing the need for transfusions, subsequently reducing the risk of anti-human leukocyte antigen antibody development. Concerns that ESAs may increase the risk of thrombotic complications in a population already plagued with physiologic disturbances due to the VAD device remain a main barrier in routine use of these medications. The goal of this case series is to discuss a single center's experience with epoetin alfa in pediatric VAD patients at an academic children's hospital. A total of 4 patients were included with no evidence of adverse effects during a total of 120 patient-days of epoetin therapy. One patient was able to discontinue ESA therapy secondary to robust improvement in cell line counts at the time of discharge, while the other 3 patients received heart transplant prior to the discontinuation of ESA therapy. An increase in hematocrit of 1% to 5.5% was seen from epoetin initiation to discontinuation.
