RESUMO
Myocardial dysfunction after Fontan palliation for univentricular congenital heart disease is a challenging clinical problem. The medical treatment has a limited impact, with cardiac transplant being the ultimate management step. Cell-based therapies are evolving as a new treatment for heart failure. Phase 1 clinical trials using regenerative therapeutic strategies in congenital heart disease are ongoing. We report the first case of autologous bone marrow-derived mononuclear cell administration for ventricular dysfunction, 23 years after Fontan operation in a patient with hypoplastic left heart syndrome. The cells were delivered into the coronary circulation by cardiac catheterization. Ventricular size decreased and several parameters reflecting ventricular function improved, with maximum change noted 3 months after cell delivery. Such regenerative therapeutic options may help in delaying and preventing cardiac transplant.
RESUMO
OBJECTIVE: After Fontan operation, hemodynamically significant veno-venous collateral (VVC) vessels can lead to systemic arterial desaturation. Outcomes after embolization of VVCs have not been determined. We sought to determine the frequency of and outcomes for patients undergoing VVCs embolization after Fontan operation. DESIGN: We retrospectively analyzed clinical and hemodynamic data of patients who underwent cardiac catheterization after the Fontan operation from 1995 to 2014 at Mayo Clinic. Clinical, imaging, and hemodynamic data from patients with VVCs were compared based on intervention (embolization) vs. nonintervention. RESULTS: Four hundred ninety-six patients with prior Fontan operation had catheterization; 109 VVCs were identified in 72 patients (37 males, mean age 26 ± 12 years). Embolization was performed in 31/72 patients (43%). Following embolization, no improvement was demonstrated in oxygen saturation or hemoglobin concentration at ambulatory follow-up (2.5 ± 2 years), and cardiac index declined at follow-up catheterization (5.6 ± 3 years). Overall, 15 patients (21%) died at a mean of 2.8 ± 4 years after embolization. Fifty percent of deaths after embolization occurred in patients with Fontan pressures ≥18 mm Hg. Five-year survival of patients with VVCs undergoing embolization was 74% compared with 92% in those patients who did not undergo embolization (P < .01). In multivariate analysis, significant predictors of death on follow-up were embolization [hazard ratio (HR) = 9.3 (95% confidence interval [CI], 2.8-42), P = .0001], atrio-pulmonary Fontan (HR = 4.2 [95% CI, 1.4-15], P = .01), and heterotaxy (HR = 3.7 [95% CI, 1.0-15], P = .05). CONCLUSION: We observed decreased 5-year survival in patients who had VVC embolization. Embolization of VVC in patients after Fontan should be evaluated very carefully in patients with atrio-pulmonary type Fontan, heterotaxy, and those with Fontan pressure above 18 mm Hg. These patients may benefit from the "natural" fenestration that VVCs provide.