Extensive coronary artery thrombosis in a paediatric patient with Kawasaki disease: a case report.

Background: Kawasaki disease (KD) is a paediatric multi-system vasculitis. Mainly, the coronary arteries become affected due to acute inflammation and formation of coronary artery aneurysms (CAAs) may occur. As the size of the CAA increases, so does the risk of clinical complications and serious cardiac outcomes. These patients may experience life-threatening thrombotic coronary artery occlusion and myocardial ischaemia unless antiplatelet and anticoagulation therapy is not initiated in a timely manner.1. Case summary: This case report presents a 12-year-old patient with KD who developed CAAs in two coronary arteries despite initial administration of intravenous immunoglobulins and acetylsalicylic acid, followed by extensive thrombosis of both coronary arteries, although antithrombotic therapy was started after the diagnosis of CAAs. Discussion: Our case is notable because of the severity of the clinical manifestation despite the administration of antiplatelet agents and anticoagulants. It could be speculated that the development of coronary thrombosis in this case might be strongly correlated with the late initiation of oral anticoagulation. The high-quality images of the affected coronary arteries in such a young patient could be of educational value.

Effect of Leisure Sports on Exercise Capacity and Quality of Life in Patients with a Fontan Circulation.

Despite improvement in survival, patients with a Fontan circulation are at risk for numerous complications, and exercise capacity and quality of life are usually reduced compared with healthy controls. However, only a few studies have assessed the impact of the amount of sporting activity on exercise capacity and health-related quality of life. We analyzed cardiopulmonary exercise tests (CPET) in a large cohort of patients with a Fontan circulation. Questionnaires were used to assess health-related quality of life and sporting activity. A total of 79 patients with a median age of 13.0 (6.5 to 34.4) years at CPET were included (female, n = 31). Questionnaires revealed that 80% of patients (n = 63) do leisure sports with 43% (n = 27) exercising more than 2 hours per /week. In a subgroup analysis on pediatric patients (n = 52) we found that nearly all participate in school sports (n = 51) and report good subjective health (n = 48). In the pediatric subgroup, oxygen uptake at the anaerobic threshold and peak oxygen uptake correlated with subjective health (p <0.05) and the amount of leisure sports activity (p <0.01). In the overall cohort, running time and running distance were significantly associated with the hours of sports per week (p <0.01). CPET results did not significantly differ between single right and single left ventricle patients. Furthermore, an open fenestration was not associated with reduced exercise capacity. In conclusion, most patients with a Fontan circulation participate in leisure sports and report good subjective healthiness. In pediatric patients, increased sports activity is associated with better exercise capacity and subjective healthiness.

Surveillance of Fontan Associated Liver Disease in Childhood and Adolescence.

Fontan associated liver disease (FALD) has been recognized as a potentially serious sequela of the Fontan circulation. Prevalence of FALD among different age groups and risk factors for advanced changes were assessed. FALD screening included abdominal ultrasound and laboratory tests. A "liver disease score (LDS)" incorporating items from ultrasound and blood testing was calculated to grade FALD severity (5 items each, maximum score 10 points). 240 patients (male: n = 139, female: n = 101, systemic right ventricle: n = 160) underwent FALD screening 10 (IQR 7-15) years after Fontan surgery. Ultrasound was abnormal in 184 (76.6%) patients (surface nodularity / blunted liver edge: n = 133, 55.4%; heterogeneous parenchyma: n = 93, 38.8%; splenomegaly: n = 68, 28.3%; ascites: n = 23, 9.6%). At least one abnormal laboratory test was detected in 218 (90.8%) patients. Gamma-glutamyl-transpeptidase was elevated in the majority of patients (n = 206, 85.8%). Median LDS was 3 (2-4). Scores ≥5 were observed in 32 (13.3%) patients. Longer follow-up (15 (11-20) vs 9 (6-14) years, P <0.001), higher central venous (13 (11-15) vs 10 (9-12) mmHg, P <0.001) and end-diastolic pressure (8 (5-10) vs 6 (5-7) mmHg, P = 0.001), impaired ventricular function and absence of sinus rhythm were associated with LDS ≥5. Longer follow-up (OR 1.2 (1.1-1.3), P <0.001) and higher central venous pressure (OR 1.6 (1.3-2.1), p < 0.001) were the only independent predictors of advanced FALD. Abdominal ultrasound and laboratory abnormalities suggestive of FALD are common during routine follow-up already in childhood and adolescence irrespective of ventricular morphology. More advanced findings are associated with longer follow-up and higher central venous pressure.

Non-contrast enhanced magnetic resonance imaging for characterization of Fontan associated liver disease.

OBJECTIVES: To evaluate the ability of non-contrast enhanced magnetic resonance imaging (MRI) techniques to characterize Fontan associated liver disease (FALD) in adolescent and adult Fontan patients. METHODS: Fontan patients (n = 29) and healthy controls (n = 13) underwent an MRI protocol with T1, T2 and Apparent Diffusion Coefficient (ADC) mapping. Routine FALD screening included abdominal ultrasound and laboratory testing. RESULTS: Median follow-up after Fontan operation was 15.1 (IQR 12.0-16.8) years. Distinct differences in tissue characteristics were visualized. T1 and T2 relaxation times were prolonged in Fontan patients, particularly of the right lobe (T1: 745 (IQR 715-784) ms vs. 586 (IQR 555-602) ms, p < 0.001; T2: 63 (IQR 59-64) ms vs. 58 (IQR 56-60) ms, p = 0.002). Left lobe ADC was lower in Fontan patients (1.10 (IQR 1.06-1.18) x 10-3 mm2/s vs. 1.23 (IQR 1.19-1.29) x 10-3 mm2/s, p < 0.001). T2 mapping was able to differentiate between controls and Fontan patients with different FALD severity. Right lobe T2 was higher in patients with moderate or severe in comparison to those with no or mild changes and healthy controls (64 (IQR 61-67) ms vs. 60 (IQR 59-63) ms vs. 58 (IQR 56-60) ms, p = 0.001). CONCLUSIONS: Non-contrast enhanced MRI methods are able to visualize regional differences in liver tissue characteristics. T1 and T2 relaxation times were prolonged in Fontan patients suggestive of fibrosis or congestive hepatopathy, while reduced ADC might reflect impaired microperfusion. These methods have promising clinical potential for detection of liver abnormalities in Fontan patients. The usefulness of T2 mapping to grade FALD severity merits further investigation.