Role of digital health in the personalized management of heart failure due to Fontan failure: a case report

Abstract Background Heart failure (HF) represents a major cause of morbidity and mortality in patients with Fontan circulation. Chronic venous congestion, impaired preload, and limited cardiac output reserve make clinical assessment and intermittent invasive evaluations insufficient to capture early hemodynamic deterioration. Pharmacological treatment lack of robust evidence in this subgroup of patients. In this challenging setting, implantable invasive hemodynamic monitoring systems may provide continuous, real-life information capable of guiding personalized HF management. Case summary A 26-year-old Fontan male developed protein losing enteropathy and recurrent hospitalizations for decompensated heart failure. Therefore, the patient was candidate to heart transplantation. Over the time, episodes of acute HF increased with an escalating requirement of dosage of diuretics. At 22 years of age, another episode of acute decompensated HF prompted a catheter-based intervention with Fontan conduit re-dilatation and re-fenestration with an Atrial Flow Regulator (AFR) implant, and a pulmonary artery pressure monitoring system (CardioMEMS™) was deployed to telemonitoring pulmonary artery pressures and adjust the diuretic therapy accordingly. Up to date, 4 years from the intervention, the use of this remote monitoring system were feasible and effective in tailoring medical therapy to prevent further hospitalizations for acute HF. The patient is in clinical follow-up with regular controls. Discussion Systems allowing continuous or repeated assessment of hemodynamic status in real-life conditions may support personalized treatment adjustments, improve symptom control, and potentially reduce HF-related hospitalizations in patients with failing Fontan circulation.