Current practice of care for adolescent and adult patients after Fontan operation in Poland

Abstract Introduction For the recent decades advances in surgical and medical care have led to rapid growth in the population of adults after Fontan operation. These patients require regular surveillance testing for both cardiovascular status and multiorgan complications and comorbidities. The practice of care for patients after Fontan operation in Poland has not been systematically evaluated. We conducted an observational study in which frequency and selection of surveillance testing at large centers specialized in congenital heart diseases across Poland were investigated on the basis of multicenter survey. Material and methods Eight centers were included into the study - 5 adult congenital heart disease (ACHD) centers and 3 adolescent centers. To objectify the comparison between the centers and facilitate the interpretation of the results we have developed a model that assesses the quality of patient care - the Fontan Surveillance Score (FSS) (Table 1). The higher score is consistent with better care with the maximum of 19 points. Results The overall number of 398 Fontan patients (176 females, 222 males, mean ages: 22.5±7.9 ys) were included into the study. The group consisted of 243 adults (age > 18 ys) and 155 adolescents (age: 15-18 ys). There was difference in the number of Fontan patients cared in each center. The median FFS was 13 points with considerable variability between the centers (interquartile range 7-14 pts). Centers providing continuous care from pediatric period until 18 years of age achieved a higher FFS as compared to ACHD centers (median: 14 pts vs 12 points, p< 0.001). The majority of patients, both in ACHD (82,3%) and pediatric centers (89%) were seen annually and had respectively: physical examination (83.1% and 90.3%, p<0.05), electrocardiogram (83.1% vs 90.3%, p<0.05) and echocardiogram (82.3% and 90.3%, p=0.052) performed at each visit. Nevertheless, unsatisfactory utilization of tests identifying early stages of Fontan – associated complications was observed. The percentage of, respectively, adult and adolescent patients in whom the diagnostic tests were performed were as follows: the cardio-pulmonary exercise tests (45.7% vs 41.3%, p=0.039), cardiac magnetic resonance (7.8% vs 1.3%, p=0.004), liver biochemical (51.9% vs 67.1%, p=0.003) and morphological (6.6% vs 7.7%, p=0.68) assessment and screening for protein-losing enteropathy (31.7% vs 77.4%, p<0.001). Summary The results of the study proved that there is no systemic surveillance approach for Fontan patients in Poland what according to the previous data seems to be a worldwide problem. The practice for care of adolescent Fontan patients differed from the one of adults patients on many levels. The introduction of systemic solutions for the transition period and organization of ACHD centers dedicated to care for Fontan patients is needed.Table 1