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Aims The aim of this study was to evaluate the possible value of dobutamine stress cardiac magnetic resonance imaging (CMR) to predict adverse outcome in Tetralogy of Fallot (TOF) patients. Methods and results In previous prospective multicentre studies, TOF patients underwent low-dose dobutamine stress CMR (7.5 µg/kg/min). Subsequently, during regular-care patient follow-up, patients were assessed for reaching the composite endpoint (cardiac death, arrhythmia-related hospitalization, or cardioversion/ablation, VO2 max ≤65% of predicted). A normal stress response was defined as a decrease in end-systolic volume (ESV) and increase in ejection fraction. The relative parameter change during stress was calculated as relative parameter change = [(parameterstress − parameterrest)/parameterrest] * 100. The predictive value of dobutamine stress CMR for the composite endpoint was determined using time-to-event analyses (Kaplan–Meier) and Cox proportional hazard analysis. We studied 100 patients [67 (67%) male, median age at baseline CMR 17.8 years (interquartile range 13.5–34.0), age at TOF repair 0.9 years (0.6–2.1)]. After a median follow-up of 8.6 years (6.7–14.1), 10 patients reached the composite endpoint. An abnormal stress response (30% vs. 4.4%, P  = 0.021) was more frequently observed in composite endpoint patients. Also in endpoint patients, the relative decrease in right ventricular ESV decreased less during stress compared with the patients without an endpoint (−17 ± 15 vs. −26 ± 13 %, P  = 0.045). Multivariable analyses identified an abnormal stress response (hazard ratio 10.4; 95% confidence interval 2.5–43.7; P  = 0.001) as predictor for the composite endpoint. Conclusion An abnormal ventricular response to dobutamine stress is associated with adverse outcome in patients with repaired TOF. [ABSTRACT FROM AUTHOR]

Introduction: Our objective was to evaluate the incidence of chromosomal aberration (both microscopic and submicroscopic) and the clinical outcome of fetuses with isolated muscular ventricular septal defect (VSD). Material and methods: The study included 40 pregnant women whose fetuses were diagnosed with isolated muscular ventricular septal defect (mVSD). Of these, 30 patients underwent amniocentesis and 10 declined. All samples were tested by chromosomal microarray analysis (CMA). Of the 40 women in the study, 32 gave birth and the clinical outcome of the children was retrieved from the patients' medical records. Results: Of the 30 patients who underwent amniocentesis, one was detected with mosaic Klinefelter syndrome and one was detected with a pathogenic copy number variant unrelated to the VSD. Clinical follow-up was performed on 26 children after birth. The first postnatal echocardiography did not detect a VSD in 13 (50%) of the followed-up children. Spontaneous closure occurred in another eight (30.8%) children during the postnatal follow-up period. In only five children (19.2%) VSD was still detected by echocardiography after the first year of life. Discussion: Isolated muscular VSD diagnosed prenatally does not appear to be a significant risk factor for chromosomal abnormalities and has a favorable clinical outcome. [ABSTRACT FROM AUTHOR]

Aims We aimed to analyse the association between right haemodynamic parameters, right ventricular (RV) dysfunction parameters, and outcomes in patients with preserved ejection fraction (EF). Methods and results Retrospective analysis of right haemodynamic (systolic pulmonary pressure and end-diastolic pulmonary pressure based on tricuspid regurgitation (TR) velocity at pulmonary valve opening time), and RV parameters including size (end-diastolic and end-systolic area), function (RV fractional area change, Tei index, Tricuspid Annular Plane Systolic Excursion, and speckle tracking derived free wall strain), from 557 consecutive patients with preserved EF [EF ≥ 50%; age 64.9 + 20; 52% female; co-morbidity Charlson index 4.7 (2.9, 6.4)]. All cause and cardiac mortality were retrospectively analysed and correlated to echo haemodynamic and co-morbid parameters. TR velocity at pulmonary valve opening time and calculated end-diastolic pulmonary artery pressure were obtainable in 71% of patients. The best haemodynamic univariate predictor of mortality was calculated end-diastolic pulmonary artery pressure [hazard ratio 1.06 (1.04–1.07); P  < 0.0001], superior to TR peak velocity and systolic pulmonary artery pressure. Elevated end-diastolic pulmonary artery pressure was associated with all cause and cardiac mortality even when adjusted for all significant clinical (age, gender, and Charlson index), and echo (stroke volume index, left atrial volume index, systolic pulmonary pressure, E / e ', and Tei index) parameters. Tei index was superior to all other RV functional parameters (P  < 0.05 for all parameters). Conclusion TR velocity at pulmonary valve opening time and calculated end-diastolic pulmonary artery pressure are obtainable in most patients, and add prognostic information on top of clinical and routine haemodynamic and diastolic parameters. [ABSTRACT FROM AUTHOR]

Objective: To systematically review the literature and assess the diagnostic value of biomarkers in detection of late-onset left ventricular (LV) dysfunction in childhood cancer survivors (CCS) treated with anthracyclines.Methods: We systematically searched the literature for studies that evaluated the use of biomarkers for detection of LV dysfunction in CCS treated with anthracyclines more than 1 year since childhood cancer diagnosis. LV dysfunction definitions were accepted as an ejection fraction <50% or <55% and/or a fractional shortening <28%, <29% or <30%. Contingency tables were created to assess diagnostic accuracies of biomarkers for diagnosing LV dysfunction.Results: Of 1362 original studies screened, eight heterogeneous studies evaluating four different biomarkers in mostly asymptomatic CCS were included. In four studies, an abnormal N-terminal pro-B-type natriuretic peptide (NT-proBNP, cut-off range 63-125 ng/L) had low sensitivity (maximally 22%) and a specificity of up to 97% for detection of LV dysfunction. For troponin levels, in five studies one patient had an abnormal troponin value as well as LV dysfunction, while in total 127 patients had LV dysfunction without troponin elevations above cut-off values (lowest 0.01 ng/mL). Two studies that evaluated brain natriuretic peptide and nitric oxide were underpowered to draw conclusions.Conclusions: In individual studies, the diagnostic value of NT-proBNP for detection of LV dysfunction in CCS is limited. Troponins have no role in detecting late-onset LV dysfunction with cut-off values as low as 0.01 ng/mL. Further study on optimal NT-proBNP cut-off values for rule out or rule in of LV dysfunction is warranted. [ABSTRACT FROM AUTHOR]

Pulmonary arterial (PA) flow is abnormal after the Fontan operation and is marked by a lack of pulsatility. We assessed the effects of this abnormal flow on the size and function of the PA's in Fontan patients in long-term serial follow-up. Twenty-three Fontan patients with serial follow-up were included. Median age was 11.1 (9.5-16.0) years at baseline and 15.5 (12.5-22.7) years at follow-up. Median follow-up duration was 4.4 (4.0-5.8) years. Flow and size of the left pulmonary artery were determined using phase-contrast MRI. From this wall shear stress (WSS), distensibility and pulsatility were determined. A group of healthy peers was included for reference. Flow and pulsatility were significantly lower in patients than in controls ( p < 0.001). Mean area was comparable in patients and controls, but distensibility was significantly higher in controls ( p < 0.001). Mean and peak WSS were significantly lower in Fontan patients ( p < 0.001). Between baseline and follow-up, there was a significant increase in normalized flow (15.1 (14.3-19.1) to 18.7 (14.0-22.6) ml/s/m, p = 0.023). Area, pulsatility, distensibility and WSS did not change, but there was a trend toward a lower mean WSS ( p = 0.068). Multivariable regression analysis showed that flow, area and age were important predictors for WSS. WSS in Fontan patients is decreased compared to healthy controls and tends to decrease further with age. Pulsatility and distensibility are significantly lower compared to healthy controls. Pulmonary artery size, however, is not significantly different from healthy controls and long-term growth after Fontan operation is proportionate to body size. [ABSTRACT FROM AUTHOR]