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Background: Measurement of ventricular volumes and function using MRI is an important tool in pediatric congenital heart disease. However, normal values for children are sparce and analysis methods are inconsistent. Purpose: To propose biventricular reference values in children for two MRI postprocessing (contouring) techniques. Study Type: Retrospective. Subjects: A total of 154 children from two institutions (13.9 ± 2.8 years; 101 male) that were referred for a clinical MRI study. Field Strength/Sequence: 1.5 T; balanced steady‐state free precession (bSSFP) sequence. Assessment: Left ventricular (LV) and right ventricular (RV) end‐diastolic and end‐systolic volumes (LVEDV, LVESV, RVEDV, RVESV) and end‐diastolic and end‐systolic myocardial mass (LVEDMM, LVESMM, RVEDMM, RVESMM) were measured from short‐axis images using two contouring techniques: 1) papillary muscles, trabeculations and the moderator band were included in the ventricular blood volume and excluded from the myocardial mass, 2) papillary muscles, trabeculations and the moderator band were excluded from the ventricular volume and included in the ventricular mass. Statistical Tests: Univariable and multivariable linear regression models were used to evaluate relationships between sex, weight, height, body surface area (BSA) and age and volumetric results. Reference graphs and tables were created with the LMS‐method. Contouring techniques were compared by intraclass correlation, regression analysis and Bland–Altman plots. A P value < 0.05 was considered statistically significant. Results: Height and BSA were significantly associated with LVESV (method 1) and with LVEDV and RVEDV (method 2). LVESV (method 2), RVESV (both methods), RVEDV (method 1), and LVEDMM and RVEDMM (both methods), showed significant associations with height and weight. LVSV and RVSV (both methods) were significantly associated with BSA and weight. RVESV (method 1) was significantly associated with age. Gender showed significant associations for all parameters. Data Conclusion: The proposed pediatric reference values can be used in the diagnosis and follow‐up of congenital or acquired heart disease and for research purposes. Evidence Level: 3 Technical Efficacy: Stage 2 [ABSTRACT FROM AUTHOR]

(1) Secundum type atrial septal defect (ASD II) is usually considered a relatively benign cardiac lesion amenable to elective closure at preschool age. Patients with trisomy 21 (T21), however, are known to have a higher susceptibility for pulmonary vascular disease (PVD). Therefore, T21 children may present with clinical symptoms earlier than those without associated anomalies. In addition, early PVD may even preclude closure in selected T21 patients. (2) We performed a retrospective analysis of the German National Register for Congenital Heart Defects including T21 patients with associated isolated ASD II. We report incidence, demographics, therapeutic strategy, outcome, and survival of this cohort. (3) Of 46,628 patients included in the registry, 1549 (3.3%) had T21. Of these, 156 (49.4% female) had an isolated ASD II. Fifty-four patients (34.6%) underwent closure at 6.4 ± 9.9 years of age. Over a cumulative follow-up (FU) of 1148 patient-years, (median 7.4 years), only one patient developed Eisenmenger syndrome and five patients died. Survival of T21 patients without PVD was not statistically different to age- and gender-matched controls from the normal population (p = 0.62), whereas children with uncorrected T21/ASD II (including patients with severe PVD, in whom ASD-closure was considered contraindicated) showed a significantly higher mortality. (4) The outcome of T21-patients with ASD II and without PVD is excellent. However, PVD, either precluding ASD-closure or development of progressive PVD after ASD-closure, is associated with significant mortality in this cohort. Thus T21 patients with ASD II who fulfill general criteria for closure and without PVD should be offered defect closure analogous to patients without T21. [ABSTRACT FROM AUTHOR]

Abstract: Objectives: To examine long-term efficacy of disease targeting therapies (DTT) in patients with Eisenmenger syndrome. Methods: All adult patients with Eisenmenger syndrome treated with DTT at our institution were included. Functional class (FC), oxygen saturation and 6-minute walk test distance (6MWTd) were analysed retrospectively. Results: Between 2002 and 2010, 79 Eisenmenger patients (21 males, 16 with Down syndrome) aged 34±10years (range 17–68years) were included. Median follow-up was 3.3years (range 0.2 to 8.9years). 6MWTd increased early after initiation of DTT, with a plateau after approximately 3years and no obvious trend towards a deterioration on average during longer-term follow-up. Two patients died during follow-up and escalation of treatment was required in 18 patients after a median period of 2.5years. Escalation of therapy was also associated with an increase in 6MWTd. In addition, FC improved on DTT and oxygen saturation, increased, both at rest and peak exercise. This effect was more pronounced in the patients with the lowest baseline oxygen saturation at rest. Conclusions: Long-term DTT is safe and improves objective exercise capacity and subjective symptoms. Response to DTT was typically observed early after initiation of DTT and was, on average, maintained longer-term. However, 1 in 5 patients required escalation of DTT, with time, due to symptomatic deterioration and this was associated with an afresh improvement in 6MWTd. [Copyright &y& Elsevier]