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Background: Patients with single ventricle (SV) circulations are at risk for ventricular dysfunction. This study investigates whether there is evidence of increased myocardial fibrosis and myocardial dysfunction in children after the Fontan operation., Methods: Consecutive children after the Fontan operation who underwent cardiac magnetic resonance (CMR) T1 relaxometry with a modified look-locker inversion recovery approach were included in this retrospective study. Native T1 times (T1) and extracellular volume fractions (ECV) in the free wall of the dominant ventricle (left, SLV; right, SRV) were compared with controls and correlated with hemodynamic and clinical parameters., Results: Twenty-one SV patients (9.7±4.6years; 13 SLV; 8 SRV) and 24 healthy control children (13.9±2.6years, p=0.002) were included. T1 and ECV were higher in SRV patients than in controls (1036±46ms vs 974±27ms, p<0.001; 28±4% vs 22±3%, p=0.002) and SLV patients (978±39ms, p=0.002; 23±5%, p=0.012) while there was no difference between SLV patients and controls. Age at bidirectional cavopulmonary connection was correlated with T1 (R=0.55, p=0.015), while systolic blood pressure (R=-0.68, p<0.001) and body weight (R=-0.54, p=0.012) inversely correlated with ECV. T1 negatively correlated with radial and circumferential strain by CMR feature tracking., Conclusions: Fontan patients with a SRV show increased CMR markers of diffuse myocardial fibrosis, which are associated with decreased myocardial contractility. Whether their increased fibrosis burden conveys a greater risk for long-term complications in this population remains to be investigated., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Background: Patients undergoing palliative surgeries for single-ventricle conditions are affected by multiple comorbidities or non-cardiac conditions. The prevalence, costs and the cost implications of these conditions have not been assessed., Methods: Administrative costing records from four hospitals in Australia and New Zealand were linked with the Fontan registry database to analyze the inpatient resource use for co-morbid or non-cardiac conditions. Inpatient costing records from the birth year through to Fontan completion were available for 156 patients. The most frequent primary diagnoses were hypoplastic left heart syndrome (33%), double inlet left ventricle (13%), and tricuspid atresia (12%)., Results: During the staged surgical treatment period, children had a mean of 10±6 inpatient admissions and spent 85±64days in hospital. Among these admissions, 3±5 were for non-cardiac conditions, totaling 21±41 inpatient days. Whilst cardiac surgeries were the major reason for resource use (77% of the total cost), other cardiac care that is not surgical contributed 5% and non-cardiac admissions 18% of the total cost. The three most prevalent non-cardiac diagnostic admission categories were 'Respiratory system', 'Digestive system', and 'Ear, nose, mouth and throat', affecting 28%, 21% and 34% of the patients respectively. Multivariate regression estimated that admissions for each of these categories resulted in an increased cost of $34,563 (P=0.08), $52,438 (P=0.05) and $10,525 (P=0.53) per patient respectively for the staged surgical treatment period., Conclusions: Non-cardiac admissions for single-ventricle patients are common and have substantial resource implications. Further research assessing the causes of admission and extent to which admissions are preventable is warranted., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Background: The incidence of children born with functional univentricular heart (UVH) and their prognosis presumably changed substantially in recent years. This is due to introduction of fetal echocardiography and potential termination of pregnancy (TOP) when UVH is diagnosed (UVH TOP), and to improvements in treatment. We aimed to explore changes in incidence, to estimate changes in survival, and to describe predictors of mortality in UVH patients., Methods: Using a population-based design we identified all UVH cases in Denmark from 1977 to 2009., Results: 703 UVH live births and 106 UVH TOP were identified. A dramatic decrease in birth incidence of UVH patients and a corresponding increase in UVH TOP was observed in recent years. Mean incidence rate of UVH (live births and UVH TOP) was 0.39 per 1000 births. In adjusted analysis survival improved significantly from birth era 1977-1989 to 1990-1999 (HR 2.65, 95% confidence interval (CI), 2.06-3.42) but not significantly from 1990-1999 to 2000-2009 (HR 0.77, 95% CI, 0.57-1.05). In the birth era 2000-2009, the lowest five-year survival was seen with hypoplastic left heart syndrome (HLHS) (18.8%), whereas the best survival was seen with tricuspid atresia (79.8%). Adjusted risk of death was 7.3 times higher in the HLHS group compared to the tricuspid atresia group (95% CI, 3.94-13.47)., Conclusions: This study demonstrates a dramatic decrease in birth incidence of UVH patients most probably due to a corresponding increase in UVH TOP. Despite survival improved after introduction of Fontan surgery, survival has not improved significantly during the last 20years., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

Background: Doppler myocardial echocardiography (DME) may be an excellent additional means of assessing determinants of systolic and diastolic ventricular function in patients with tricuspid atresia (TA) after total cavopulmonary connection (TCPC)., Methods and Results: Thirty-three patients with TA and normal systolic shortening/ejection fraction (M-mode) after TCPC were studied by DME at a median age of 7.6 years (range 1.5-17 years). These results were compared to 16 age matched normal controls. Median time under a cavopulmonary shunt was 5.2 years (range 0.6-13.3 years). Isovolumic acceleration and isovolumic velocity did not differ significantly. All other systolic (S-wave acceleration-velocity and S-wave duration) and diastolic DME indices (E-deceleration-velocity, A-velocity and E/A ratio) were significantly lower in TCPC patients in comparison to normals (p<0.0001). Furthermore, isovolumic relaxation time (IRT) was significantly prolonged in the patient group (p<0.0001). Even though there was a significant correlation between the time of volume unloading and left ventricular end-diastolic diameter, exclusion of all patients with short period of volume unloading and relatively large systemic ventricles did not alter the results., Conclusions: Patients with TA after TCPC and normal shortening and ejection fraction have normal isovolumic acceleration. Load dependent DME indices of systolic and diastolic function, however, were significantly reduced. These findings suggest that the described DME indices mirror the "normal" hemodynamics after TCPC in patients with TA. It needs to be assessed, whether this is an index of adverse prognosis in long-term surveillance of these patients.

Background: There is evidence that "inappropriate hypertrophy" of the single left ventricle, which occurs as a result of acute preload reduction, leads to adverse consequences on ventricular function. However, a systematic study of the capability of tissue Doppler imaging (TDI) to assess systolic and diastolic ventricular functions after the Fontan procedure is still missing., Methods: Twenty-four postoperative patients aged 12-33 years were prospectively evaluated with two-dimensional echocardiography equipped with TDI capabilities. Nineteen age-matched normal subjects were selected as controls. Good-quality echoes for the measurement of ejection fractions were available in 21 patients. Ten patients (group 1) had systolic dysfunction (ejection fraction < 50%), and 11 patients (group 2) had normal systolic function. Peak systolic and diastolic wall velocities were acquired from the two-chamber view in the myocardia and mitral annulus., Results: Compared with controls, the Fontan patients had a significantly reduced peak systolic velocity at wall and annulus sites. A linear correlation existed between ejection fraction and systolic myocardial velocity from the annular sites. Group 1 patients had lower wall velocities and lower annulus velocities both in systole and diastole. Group 2 patients had preserved systolic velocities but decreased regional and annular early diastolic velocities, suggesting impaired filling. Multiple correlation analysis showed a relation between peak early diastolic mitral velocity and ventricular ejection fraction, mean mitral annular motion at systole, mass/volume ratio, and the number of years post Fontan revision., Conclusions: Myocardial velocities recorded after the Fontan operation give insight into systolic and diastolic ventricular functions. The peak systolic mitral annular velocity correlated well with the ventricular ejection fraction. The peak early diastolic velocity and the ratio between the early and late diastolic mitral annular velocity are reduced and reflect diastolic dysfunction even in the presence of normal systolic ejection fraction.