RESUMO
BACKGROUND: While the short- and median-term survival has improved considerably in patients with Fontan circulation, cardiac function and exercise capacity are still reduced and may deteriorate over time. Cardiac magnetic resonance (CMR) is the gold standard for the assessment of ventricular volume and function. Speckle-tracking echocardiography (STE) is a myocardial deformation technique to assess ventricular function, with promising results. The aim of our study is to validate STE and conventional echocardiography parameters and to compare them with CMR. Furthermore, we aimed to design a diagnostic algorithm applying some parameters in series for early detection of myocardial dysfunction. MATERIALS AND METHODS: We performed a cross-sectional single-center study in 64 patients with Fontan circulation. Longitudinal and circumferential strain, strain rates, and conventional echocardiographic measurements were registered. Ventricular volumes and ejection fraction (EF) were obtained by CMR. RESULTS: Seven patients presented ventricular dysfunction (EF <45% by CMR), without showing a significant correlation between STE parameters or conventional measures by echocardiography and CMR. After the application of the diagnostic algorithm with the optimal cutoff points (global longitudinal strain - 24.5%, global circumferential strain - 20%, and annular plane systolic excursion - 16.5 mm), we got a sensitivity rate and a negative predictive value of 100%. In 19 patients (40.1%), the absence of ventricular dysfunction was demonstrated without no false-negative cases. CONCLUSIONS: STE should be considered a complementary diagnostic tool in Fontan patients. These suggested parameters applied in series are a useful tool for identifying early ventricular dysfunction and for diagnostic tests improvement with a fewer CMRs in the follow-up of these patients.
RESUMO
INTRODUCTION AND OBJECTIVES: Different percutaneous interventional procedures are needed to reach and maintain adequate anatomical and physiological conditions for the Fontan circulation. We aim to describe the experience gained at a children's hospital in such interventions, and to analyze the clinical outcomes. METHODS: Retrospective study of all patients with Fontan circulation completed between 1995 and 2013. We analyzed the clinical characteristics and the different types of percutaneous interventions performed, considering three different periods of time: before Glenn surgery, between Glenn and Fontan surgeries, and after Fontan was completed. Survival and time to indication of percutaneous interventions in each period were analyzed, as well as the clinical situation at last follow-up. RESULTS: Of the 91 patients analyzed, 46 (50.5%) required percutaneous interventions. The most frequent procedures were pulmonary artery angioplasty and angioplasty of the Fontan conduit. Estimated survival at 10, 20 and 30 years of age was 96.2%, 94.7% and 89.4%, respectively. There were no significant differences in survival of patients undergoing percutaneous interventions or not. Overall survival and time to indication of percutaneous interventions were significantly lower in the group of patients with right morphology systemic ventricle. Patients with fenestrated Fontan required interventions more frequently. At the end of follow-up, 66 patients (72.5%) were asymptomatic, without significant differences between patients who underwent or did not undergo percutaneous interventions. CONCLUSIONS: Interventional catheterization procedures are often necessary to reach and maintain the fragile Fontan circulation, mainly in patients with right morphology systemic ventricles and fenestrated Fontan conduits.
