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Background Family screening for hypertrophic cardiomyopathy (HCM) is based on genetic testing and clinical evaluation (maximal left ventricular wall thickness (MWT) ≥15 mm, or ≥13 mm in first-degree relatives of HCM patients). The aim of this study was to assess the effect of gender and body size on diagnosis of HCM and prediction of clinical outcome. Methods This study includes 199 genotype-positive subjects (age 44 ± 15 years, 50% men) referred for cardiac screening. Gender-specific reference values for MWT indexed by body surface area (BSA), height and weight were derived from 147 healthy controls. Predictive accuracy of each method for HCM-related events was assessed by comparing areas under the receiver operating characteristic curves (AUC). Results Men had a higher absolute, but similar BSA- and weight-indexed MWT compared with women (14.0 ± 3.9 mm vs 11.5 ± 3.8 mm, p 2 vs 6.6 ± 2.4 mm/m2; 0.17 ± 0.06 mm/kg vs 0.17 ± 0.06 mm/kg, both p > 0.05). Applying BSA- and weight-indexed cut-off values decreased HCM diagnoses in the study group (48% vs 42%; 48% vs 39%, both p 2: 58% vs 45%; ≤70 kg: 37% vs 46%; ≥85 kg: 53% vs 25%, all p p Conclusions In genotype-positive subjects referred for family screening, differences in MWT across gender are mitigated after indexation by BSA or weight. Indexation decreases the prevalence of HCM, particularly in larger men, and improves the predictive accuracy for HCM-related events.

Speckle tracking analysis (STE) of the left ventricle offers a new method to assess left ventricular (LV) diastolic function. LV diastolic strain measurements offer a non-invasive, global and direct measure of LV diastolic function. However, there is little data on normal values and the influence of anthropomorphic factors which is crucial in clinical practice for new techniques. The aims of this study were to formulate reference values for LV diastolic strain rate, elucidate effects of age and sex on LV diastolic strain analysis and compare STE measurements with conventional LV diastolic measurements. One-hundred-forty-seven healthy subjects aged 20–72 years (≥ 28 subjects per age decade) were prospectively included (Mean age 44 ± 13.7 years, 50% female) and examined with electrocardiography and 2D-echocardiography, including speckle tracking. Left ventricular peak early diastolic strain rate (Sre) was measured in the apical windows, using STE. Men had significantly lower LV Sre values than women (1.02 ± 0.22 vs. 1.18 ± 0.23, p value

ObjectiveGalectin-3 is an emerging biomarker for risk stratification in patients with heart failure. This study aims to investigate the release of galectin-3 and its association with cardiovascular events in patients with adult congenital heart disease (ACHD).MethodsIn this prospective cohort study, 602 consecutive patients with ACHD who routinely visited the outpatient clinic were enrolled between 2011 and 2013. Galectin-3 was measured in thaw serum by batch analysis. The association between galectin-3 and a primary endpoint of all-cause mortality, heart failure, hospitalisation, arrhythmia, thromboembolic events and cardiac interventions was investigated using multivariable Cox models. Reference values and reproducibility were established by duplicate galectin-3 measurements in 143 healthy controls.ResultsGalectin-3 was measured in 591 (98%) patients (median age 33 (25–41) years, 58% male, 90% New York Heart Association (NYHA) class I). Median galectin-3 was 12.7 (range 4.2–45.7) ng/mL and was elevated in 7% of patients. Galectin-3 positively correlated with age, cardiac medication use, NYHA class, loss of sinus rhythm, cardiac dysfunction and N-terminal pro-B-type natriuretic peptide (NT-proBNP). During a median follow-up of 4.4 (IQR 3.9–4.8) years, the primary endpoint occurred in 195 patients (33%). Galectin-3 was significantly associated with the primary endpoint in the univariable analysis (HR per twofold higher value 2.05; 95% CI 1.44 to 2.93, pConclusionsGalectin-3 is significantly associated with functional capacity, cardiac function and adverse cardiovascular events in patients with ACHD. Nevertheless, the additive value of galectin-3 to a more conventional risk marker such as NT-proBNP seems to be limited.

Purpose Previous studies suggest that anterior mitral valve leaflet (AMVL) elongation is a primary phenotypic feature in hypertrophic cardiomyopathy (HCM). Our aim was to assess AMVL length in individuals with HCM gene mutations and in healthy controls and to identify predictors of the development of HCM during follow-up. Methods A total of 133 HCM mutation carriers and 135 controls underwent cardiac examination including electro- and echocardiography. AMVL length was measured in the parasternal long axis and apical three chamber view during diastole. Univariate and multivariable cox proportional hazard regression analyses were performed to identify predictors of HCM. Results There were no significant differences between HCM mutation carriers and controls regarding age and sex. In the parasternal long axis view, AMVL length was similar in mutation carriers and controls (24 ± 4 vs 24 ± 4 mm, p = 0.8). In the apical three chamber view, AMVL were shorter in mutation carriers (29 ± 4 vs 30 ± 4 mm, p = 0.02). When averaged for both views, AMVL length was similar in mutation carriers and controls (27 ± 3 vs 27 ± 3 mm, p = 0.2). During 5.8 ± 3.0 years follow-up, 13 (14%) HCM mutation carriers developed HCM. Pathological Q wave (hazard ratio 9.89, p = 0.004), E/e′ ratio (hazard ratio 2.52, p = 0.001), and maximal wall thickness (hazard ratio 2.15, p = 0.001) were independent predictors of HCM. AMVL length was not predictive of the development of HCM. Conclusions AMVL length is similar in HCM mutation carriers and controls. AMVL length is not predictive of the development of HCM, in contrast to pathological Q wave, E/e′ ratio, and maximal wall thickness.

Purpose: Left atrial (LA) dilatation is predictive for complications in a multitude of cardiac diseases; therefore, adequate assessment is essential. Technological advances have made it possible to quantify LA function with Speckle Tracking Echocardiography (STE); however, there are currently no recommendations for normal values with regard to LA function. We aimed to assess LA myocardial and volumetric function in a healthy cohort and investigate correlations with baseline characteristics. Methods: This prospective cohort study included 147 (aged 20–72) healthy individuals and assessed LA volumetric function using maximum, minimum and pre-a-wave volumes and myocardial function using reservoir function using peak strain in LA relaxation (LA-strain), conduit function using peak strain rate in early LA contraction (LA-SRe) and pump function using peak strain rate in late LA contraction (LA-SRa). Results: Mean LA-strain was 39.7 ± 6.2%, LA-SRe −2.78 ± 0.62 s−1 and LA-SRa −2.56 ± 0.62 s−1. Subjects were divided into 5 age decades (each 50% female). LA-strain and LA-SRe were lower in the oldest groups, whereas LA-SRa was higher. LA-SRa was higher in males(−2.69 ± 0.68 s−1 vs −2.42 ± 0.52 s−1). Age-specific values are provided. Age proved to be an independent predictor for LA-SRa after correction for blood pressure and heart rate. LA expansion index and passive emptying fraction decreased with age, while active emptying fraction increased with age. LA maximum volume did not increase with age. Conclusion: This study provides normal values for the three phasic functions of the LA, assessed with STE and volumetric function. Our results suggest the need for age-specific reference ranges, and normal values for this cohort have been calculated.

Aims The aim of this prospective study was to evaluate the feasibility and establish normal values of functional right ventricle (RV) parameters as assessed in our four, long-axis view RV model using iRotate echocardiography. Furthermore, we evaluated the potential use of this model in patients with abnormally loaded RVs. Method and results One hundred and fifty-five healthy subjects aged 20-72 years (≥28 subjects per decile) were prospectively recruited. We used non-dedicated RV speckle-tracking software to test the feasibility and to establish normal range values of peak systolic global longitudinal RV strain (RV-GLS) from the RV free-walls (septum was excluded). Also normal range values for: dimensions, tricuspid annular plane systolic excursion (TAPSE) and tricuspid annular peak systolic velocity (TDI-S') were established. The feasibility of RV-GLS was 88, 84, and 62%, respectively, in the lateral, inferior, and anterior free wall. Mean RV-GLS normal values were -24.5 ± 4.9% for lateral wall and -25.4 ± 5.0% for anterior wall. Mean RV-GLS in the inferior wall was -23.2 ± 4.4% in the aortic (Ao) view and -20.7 ± 5.0% in the coronal (CV) view. The feasibility of mean RV-GLS was 100% in the anterior, lateral, and inferior walls in abnormally dilated RVs. Conclusion The feasibility of all RV parameters assessed in the four-view iRotate model is good to excellent. Normal values for RV dimension and function of the anterior, inferior, and lateral RV walls have been established. Further studies and dedicated RV speckle-tracking software are warranted to discover the full potential of this new technique.

Background Adults with a systemic right ventricle ( sRV ) have a high risk of cardiac complications. This study aimed to identify prognostic markers in adults with sRV based on clinical evaluation, echocardiography, and blood biomarkers. Methods and Results In this prospective cohort study, consecutive clinically stable adults with sRV caused by Mustard‐ or congenitally corrected transposition of the great arteries were included (2011–2013). Eighty‐six patients were included (age 37±9 years, 65% male, 83% New York Heart Association functional class I, 76% Mustard transposition of the great arteries, 24% congenitally corrected transposition of the great arteries). Venous blood sampling was performed including N‐terminal pro B‐type natriuretic peptide, high‐sensitive‐troponin‐T, high‐sensitivity C‐reactive protein, growth differentiation factor‐15, galectin‐3, red cell distribution width, estimated glomerular filtration rate, and hemoglobin. Besides conventional echocardiographic measurements, longitudinal, circumferential, and radial strain were assessed using strain analysis. During a median follow‐up of 5.9 (interquartile range 5.3–6.3) years, 19 (22%) patients died or had heart failure (primary end point) and 29 (34%) patients died or had arrhythmia (secondary end point). Univariable Cox regression analysis was performed using dichotomous or standardized continuous variables. New York Heart Association functional class >I, systolic blood pressure, and most blood biomarkers were associated with the primary and secondary end point (galectin‐3 not for primary, N‐terminal pro B‐type natriuretic peptide and high‐sensitivity C‐reactive protein not for secondary end point). Growth differentiation factor‐15 showed the strongest association with both end points (hazard ratios; 2.44 [95% CI 1.67–3.57, P CI 1.46–2.73, P CI 1.34–2.85], P CI 1.21–2.38, P =0.002], respectively). Concerning strain analysis, only sRV septal strain was associated with the secondary end point (hazard ratio 0.58 [95% CI 0.39–0.86], P =0.006). Conclusions Clinical, conventional echocardiographic, and blood measurements are important markers for risk stratification in adults with a sRV . The value of novel echocardiographic strain analysis seems limited.

textabstractAims: Reliable evaluation of the severity and consequences of pulmonary regurgitation (PR) in patients with repaired tetralogy of Fallot (TOF) is crucial to timely identify the need for pulmonary valve intervention. We aimed to identify the accuracy of echocardiographic parameters to differentiate between moderate and severe PR, using phase contrast cardiac magnetic resonance imaging (CMR) as gold standard. Methods and results: In this cross-sectional study, 45 TOF patients with both echocardiographic and CMR measurements of PR were enrolled. All quantitative and semiquantitative echocardiographic measurements such as pressure half time (PHT), Color flow jet width (CFJW), ratio CFJW/right ventricle outflow tract (RVOT) diameter, PR index and the presence of early termination of the PR jet, end-diastolic antegrade flow and diastolic backflow in main pulmonary artery (MPA), and PA branches correlated significantly with PR fraction on CMR. Qualitative assessment with color flow on echocardiography overestimated PR Multivariate linear regression analysis identified the ratio of CFJW/RVOT diameter and PHT as independent predictors of PR fraction. Accuracy of echo parameters was tested to differentiate between mild-to-moderate and severe PR Combining different echocardiographic parameters increased sensitivity and specificity. The addition of diastolic flow reversal in the PA branches to PHT below 167 milliseconds increased the NPV from 87% to 89% and PPV from 62% to 76%. Conclusions: Comparison with CMR confirms that echocardiographic parameters are reliable in predicting PR severity. Combined measurement of diastolic flow reversal in the pulmonary artery branches and PHT is reliable in the detection of severe PR in the follow-up of TOF patients.

Background Adult patients with repaired tetralogy of Fallot (ToF) are at risk for complications such as heart failure and sudden cardiac death, and identifying high-risk patients is important. Reduced left ventricular (LV) and right ventricular (RV) function has been identified as a predictor of outcomes. However, LV ejection fraction is often preserved, and RV function is difficult to assess. With the introduction of strain analysis, an easy and more sensitive parameter became available. The aim of this study was to investigate the association between strain variables and cardiovascular events in patients with ToF. Methods Stable adult patients with repaired ToF were consecutively included in a prospective observational study between 2011 and 2013 (N = 151; median age, 33.2 years [interquartile range, 25.5–42.0 years]; 61.6% men). For the left ventricle, global longitudinal strain and apical and basal rotation were measured, and longitudinal strain was measured for the right ventricle. The primary endpoint was a composite of death or heart failure. The secondary endpoint was a composite of death, heart failure, arrhythmia, reintervention, or hospitalization for cardiac reasons. Results During a median follow-up period of 71.5 months (interquartile range, 64.0–75.3 months), the primary and secondary endpoints occurred in 14 (9%) and 62 (41%) patients, respectively. After adjusting for LV ejection fraction and LV global longitudinal strain, RV longitudinal strain remained independently associated with the primary endpoint in a ridge regression analysis. LV apical rotation remained independently associated with the secondary end point (adjusted hazard ratio, 0.72; 95% CI, 0.52–0.98; P = .035) after adjusting for age, New York Heart Association functional class, QRS duration, LV ejection fraction, RV longitudinal strain, and LV global longitudinal strain. Conclusions Myocardial deformation variables of both the left and right ventricles were associated with cardiovascular events in patients with ToF. LV and RV longitudinal strain and LV rotation should become part of the routine assessment of patients with ToF.

Aims Evaluation of left ventricular (LV) myocardial deformation by speckle tracking echocardiography (STE) is useful for clinical and research purposes. However, strain measurements depend on the used software. Normative data for QLAB 10 (Philips) are scarce. Additionally, little is known about the influence of anthropometric factors. We aimed to establish normal adult STE-derived strain and strain rate values and to evaluate associations with anthropometrics. Methods One hundred fifty-five healthy subjects aged 20 to 72 years (≥28 subjects per decile) were prospectively gathered and examined with electrocardiography and two-dimensional echocardiography. With STE, we assessed peak systolic LV global longitudinal strain (GLS), segmental longitudinal strain, and strain rate from the three standard apical views. Results We included 147 healthy subjects (age 44.6±13.7 years, 50% female, GLS −20.8±2.0%). Men had significantly lower GLS than women (−20.1±1.8% vs −21.5±2.0%, P

Despite successful aortic coarctation (CoA) repair, systemic hypertension often recurs which may influence left ventricular (LV) function. We aimed to detect early LV dysfunction using LV global longitudinal strain (GLS) in adults with repaired CoA, and to identify associations with patient and echocardiographic characteristics. In this cross-sectional study, patients with repaired CoA and healthy controls were recruited prospectively. All subjects underwent echocardiography, ECG and blood sampling within 1 day. With speckle-tracking echocardiography, we assessed LV GLS on the apical four-, three- and two-chamber views. We included 150 subjects: 75 patients (57 % male, age 33.4 ± 12.8 years, age at repair 2.5 [IQR: 0.1–11.1] years) and 75 healthy controls of similar sex and age. LV GLS was lower in patients than in controls (−17.1 ± 2.3 vs. −20.2 ± 1.6 %, P

Background: Few prospective data are available regarding long-term outcomes after surgical closure of a ventricular septal defect (VSD).Objectives: The objective of this study was to invest...

Background Hypertrophic cardiomyopathy (HC) is characterized by left ventricular (LV) hypertrophy and associated with papillary muscle (PM) abnormalities. The aim of this study was to evaluate the utility of three-dimensional echocardiography (3DE) for the geometric assessment of LV hypertrophy and PM morphology. Methods The study included 24 patients with an established diagnosis of HC and 31 healthy controls. 3DE was performed using an iE33 or EPIQ 7C ultrasound system with an X5-1 transducer. QLAB software was used for the 3D analysis of LV wall thickness (LVWT) and PM morphology and hypertrophy; the number and cross-sectional area (CSA) of anterolateral and posteromedial PMs; and the presence of bifid or accessory PMs. Results Patients with HC had a larger LVWT compared to controls in all segments (p

Objective: To provide prospective information on long-term outcome after surgical correction of valvular pulmonary stenosis (PS). Methods: Fifty-three consecutive patients operated for PS during childhood between 1968 and 1980 in one centre are followed longitudinally for 37±3.4 years, including extensive in-hospital examination every 10 years. Results: Survival information was available in 100% of the original 53 patients. Cumulative survival was 94% at 20 years and 91% at 40 years. Excluding perioperative mortality (

Aim For accurate interpretation of echocardiographic measurements normative data are required, which are provided by guidelines. For this article, the hypothesis was that these cannot be extrapolated to the Dutch population, since in Dutch clinical practice often higher values are found, which may not be pathological but physiological. Therefore this study aimed to 1) obtain and propose normative values for cardiac chamber quantification in a healthy Dutch population and 2) determine influences of baseline characteristics on these measurements. Methods Prospectively recruited healthy subjects, aged 20–72 years (at least 28 subjects per age decade, equally distributed for gender) underwent physical examination and 2D and 3D echocardiography. Both ventricles and atria were assessed and volumes were calculated. Results 147 subjects were included (age 44 ± 14 years, 50% female). Overall, feasibility was good for both linear and volumetric measurements. Linear and volumetric parameters were consistently higher than current guidelines recommend, while functional parameters were in line with the guidelines. This was more so in the older population. 3D volumes were higher than 2D volumes. Gender dependency was seen in all body surface area (BSA) corrected volumes and with increasing age, ejection fractions decreased. Conclusion This study provides 2D and 3D echocardiographic reference ranges for both ventricles and atria derived from a healthy Dutch population. BSA indexed volumes are gender-dependent, age did not influence ventricular volumes and a rise in blood pressure was independently associated with increased right ventricular volumes. The higher volumes found may be indicative for the Dutch population being the tallest in the world.

Aims Left ventricular (LV) dysfunction is a major determinant of late adverse clinical outcome in adult patients with tetralogy of Fallot (ToF). Therefore, early detection is important. Speckle-tracking echocardiography (STE) has emerged as a quantitative technique to assess LV function. The aim of this study was to evaluate LV rotation and twist with STE in adult ToF patients and their association with right ventricular (RV) and LV dimensions and function, exercise capacity, and NT-proBNP level. Methods Eighty-two ToF patients and 56 healthy controls matched for age and gender underwent echocardiography, electrocardiography, cardiac magnetic resonance imaging (CMR), bicycle ergometry, and NT-proBNP measurement. For STE, short-axis parasternal views were obtained at the LV base and apex. We analysed LV apical and basal rotation curves and calculated LV twist. Results Of the 82 ToF patients (55% male, age 33 ± 10 years, 98% NYHA I), 58 (71%) had normal twist, but lower than the controls [12.5 (IQR: 6.6) vs. 16.9 (IQR: 8.2) degrees, P = 0.002] mainly due to decreased apical rotation. Twenty-one (26%) patients had abnormal apical rotation which was associated with larger LV dimensions and decreased systolic biventricular function. Multivariable regression analyses showed positive relations of LV twist with biventricular systolic function measured with echocardiography as well as CMR. Conclusion The majority of adults with corrected ToF show a reduced LV twist. Strikingly, one-quarter of these patients have an abnormal apical rotation which is associated with decreased systolic LV and RV function. These findings suggest that abnormal apical rotation is a new objective diagnostic criterion for detection of ventricular dysfunction.

To describe the very long-term outcome after surgical closure of an atrial septal defect (ASD). Longitudinal cohort study of 135 consecutive patients who underwent surgical ASD repair at age

Aims The aim of this study was to evaluate the feasibility of transthoracic two-dimensional (2D) iRotate, a new echo modality, to assess the whole right ventricle (RV) from a single transducer position based on anatomic landmarks. Methods and Results The anatomic landmarks were first defined based on three-dimensional echocardiographic datasets using multiplane reconstruction analyses. Thereafter, we included 120 healthy subjects (51% male, age range 21–67 years). Using 2D iRotate, four views of the RV could be acquired based on these landmarks. The anterior, lateral, inferior wall (divided into three segments: basal–mid–apical), and right ventricular outflow tract (RVOT) anterior wall of the RV were determined. The feasibility of visualization of RV segments and tricuspid annular plane systolic excursion (TAPSE) and tissue Doppler imaging (TDI) measurements were assessed. To evaluate this model for diseased RVs, a small pilot study of 20 patients was performed. In 98% of healthy subjects and 100% of patients, iRotate mode was feasible to assess the RV from one single transducer position. In total, 86% and 95%, respectively, of the RV segments could be visualized. The visualization of the RVOT anterior wall was worse 23% and 75%, respectively. TAPSE and TDI measurements on all four views were feasible 93% and 92%, respectively, of the healthy subjects and in 100% of the patients. Conclusion With 2D iRotate, a comprehensive evaluation of the entire normal and diseased RV is feasible from a fixed transducer position based on anatomic landmarks. This is less time-consuming than the multiview approach and enhances accuracy of RV evaluation. Imaging of the RVOT segment remains challenging.

It is unknown whether right-ventricular (RV) volume overload caused by an atrial septal defect (ASD) still has its effect on RV deformation long after repair. We evaluated RV and left-ventricular (LV) deformation beyond 30 years after surgical ASD repair in childhood, and studied relationships with conventional diagnostic parameters. Methods and results In this prospective study, we included 102 subjects: 51 patients with repaired ASD (39% male, age 43.3+ 4.9 years, age at repair 7.9+ 3.6 years) and 51 healthy controls of similar age and sex. All subjects underwent echocardiography and elec- trocardiography. Additionally, ASD patients underwent cardiac magnetic resonance imaging (CMR), bicycle ergometry, and NT-proBNP measurement. With speckle-tracking echocardiography, we analysed peak systolic longitudinal strain of the RV lateral wall, LV lateral wall, and septum. RV lateral wall global longitudinal strain (GLS) was lower in patients (220.4+ 2.7%) than controls (226.8+ 4.2%, P , 0.001), due mainly to decreased apical strain (219.3+ 6.2 vs. 228.8+ 8.3%, P , 0.001). RV lateral wall GLS correlated with CMR-derived RV and LV end-diastolic volumes (r ¼ 0.49, P ¼ 0.014; r ¼ 0.53, P ¼ 0.005), and with RV and LV end-systolic volumes (r ¼ 0.43, P ¼ 0.034; r ¼ 0.46, P ¼ 0.019). LV GLS was similar between patients and controls (P ¼ 0.144). No significant correlations were found with NT-proBNP or exercise capacity. Conclusion Although ASD repair was already performed in childhood, RV longitudinal strain, especially of the apical segment, is decreased in patients 35 years after surgery. This suggests that RV function has still been affected in the long run, probably due to the early RV volume overload, but possibly also as sequel of surgery.

Background— Prospective data on long-term survival and clinical outcome beyond 30 years after surgical correction of tetralogy of Fallot are nonexistent. Methods and Results— This longitudinal cohort study consists of the 144 patients with tetralogy of Fallot who underwent surgical repair at Conclusions— Although many patients needed a reoperation or developed arrhythmias, late mortality was low, and the clinical condition and subjective health status of most patients remained good. Previous shunt, low temperature during surgery, and early postoperative arrhythmias were found to predict late mortality.

Aims Late systolic dysfunction of the systemic right ventricle (RV) in patients with transposition of the great arteries (TGA) is of major concern. Right ventricular global longitudinal strain (GLS) might be able to identify early dysfunction. Methods and results Adults with TGA after Mustard operation (TGA-Mustard) or congenitally corrected-TGA (ccTGA) underwent echocardiography, electrocardiography, and NT-proBNP measurement. Using speckle-tracking echocardiography, we analysed longitudinal strain and strain rate, and compared findings in both patients groups, to healthy controls and with clinical parameters. We included 42 patients (mean age 37 +/- 7 years, 69% male) with a systemic RV [32 TGA-Mustard (34 +/- 4 years after corrective surgery) and 10 ccTGA], and 32 healthy controls (mean age 36 +/- 11 years). Global longitudinal strain of the systemic RV was lower in patients than GLS of the systemic LV in controls (-14.2 +/- 3.5 vs. -20.0 +/- 3.0%, P < 0.001). Average LS of the RV lateral wall was lower in patients with TGA-Mustard (-15.5 +/- 3.4%) than ccTGA (-18.3 +/- 3.6%, P = 0.047). Right ventricular GLS tended to be lower in patients in NYHA class II than I, and correlated with NT-proBNP (r = 0.49, P = 0.001), RV fractional area change (r = -0.39, P = 0.019), RV apex-base-diameter (r = 0.37, P = 0.021), and QRS duration (r = 0.41, P = 0.014). Conclusion Global longitudinal strain of the systemic RV in patients is lower than GLS of the systemic LVin healthy controls, especially in the apical segment, and tended to be lower in TGA-Mustard than ccTGA patients. Since RV GLS correlates with RV function, myocardial deformation is useful as a more quantitative tool to measure systemic RV function. Decreased GLS was associated with elevated NT-proBNP and tended to correlate with worsening NYHA class, which strengthens the potential prognostic value of GLS in patients with a systemic RV.

Few prospective data are available regarding long-term outcomes after surgical closure of a ventricular septal defect (VSD).The objective of this study was to investigate clinical outcomes30 years after surgical VSD closure.Patients who underwent surgical VSD closure during childhood between 1968 and 1980 were reexamined every 10 years. In 2012, we invited eligible patients to undergo another examination, which included electrocardiography, Holter monitoring, echocardiography, bicycle ergometry, measurement of N-terminal pro-B-type natriuretic peptide, and subjective health assessment.Cumulative survival was 86% at 40 years. Causes of mortality were arrhythmia, heart failure, endocarditis, during valvular surgery, pulmonary hypertension, noncardiac causes, and unknown causes. Cumulative event-free survival after surgery was 72% at 40 years. Symptomatic arrhythmias occurred in 13% of patients and surgical or catheter-based reinterventions in 12%. Prevalence of impaired right ventricular systolic function increased from 1% in 2001 to 17% in 2012 (p=0.001). Left ventricular systolic function was impaired but stable in 21% of patients. Aortic regurgitation occurred more often in the last 20 years (p=0.039), and mean exercise capacity decreased (p=0.003). N-terminal pro-B-type natriuretic peptide (median: 11.6 pmol/l [interquartile range: 7.0 to 19.8 pmol/l]) was elevated (14 pmol/l) in 38% of patients. A concomitant cardiac lesion, for example, patent ductus arteriosus, and aortic cross-clamp time were determinants of late events (hazard ratio: 2.84 [95% confidence interval: 1.23 to 6.53] and hazard ratio: 1.47 per 10 min [95% confidence interval: 1.22 to 1.99], respectively). Patients rated their subjective health status significantly better than a reference population.Survival up to 40 years after successful surgical VSD closure is slightly lower than in the general Dutch population. Morbidity is not negligible, especially in patients with a concomitant cardiac lesion.

Background: Amino-terminal B-type natriuretic peptide (NT-proBNP) may detect early cardiac dysfunction in adults with tetralogy of Fallot (ToF) late after corrective surgery. We aimed to determine the value of NT-proBNP in adults with ToF and establish its relationship with echocardiography and exercise capacity. Methods and results: NT-proBNP measurement, electrocardiography and detailed 2D-echocardiography were performed on the same day in 177 consecutive adults with ToF (mean age 34.6 +/- 11.8 years, 58% male, 89% NYHA I, 29.3 +/- 8.5 years after surgical correction). Thirty-eight percent of the patients also underwent a cardiopulmonary-exercise test. Median NT-proBNP was 16 [IQR 6.7-33.6] pmol/L, and was elevated in 55%. NT-proBNP correlated with right ventricular (RV) dilatation (r = 0.271, p < 0.001) and RV systolic dysfunction (r = -0.195, p = 0.022), but more strongly with LV systolic dysfunction (r = -0.367, p < 0.001), which was present in 69 patients (39%). Moderate or severe pulmonary regurgitation was not associated with higher NT-proBNP. Tricuspid and pulmonary regurgitation peak velocities correlated with NT-proBNP (r = 0.305, p < 0.001 and r = 0.186, p = 0.045, respectively). LV twist was measured with speckle-tracking echocardiography in 71 patients. An abnormal LV twist (20 patients, 28%) was associated with elevated NT-proBNP (p = 0.030). No relationship between NT-proBNP and exercise capacity was found. Conclusions: NT-proBNP levels are elevated in more than 50% of adults with corrected ToF, while they are in stable clinical condition. Higher NT-proBNP is most strongly associated with elevated pulmonary pressures, and with LV dysfunction rather than RV dysfunction. NT-proBNP has the potential to become routine examination in patients with ToF to monitor ventricular function and may be used for timely detection of clinical deterioration. (C) 2014 Elsevier Ireland Ltd. All rights reserved.

We thank Dr. Marmoush and colleagues for their response to our paper [(1)][1] describing the survival and clinical outcome in patients up to 40 years after surgical closure of a ventricular septal defect (VSD). Dr. Marmoush and colleagues justly remarked the high incidence of VSD in infants with

Aims Many patients with repaired Tetralogy of Fallot (ToF) have right ventricular (RV) volume overload due to pulmonary regurgitation (PR). We studied the effect of volume overload on global and regional RV and left ventricular (LV) deformation, and their relationships with conventional diagnostic parameters. Methods and results In this cross-sectional study, 94 prospectively recruited ToF patients (61% male, age 32.8 +/- 9.5 years, age at repair 1.9 [0.8-5.7] years, 39% pulmonary homograft) and 85 healthy controls of similar age and sex underwent echocardiography and electrocardiography. In a subset of patients, cardiac magnetic resonance imaging, bicycle ergometry, and NT-proBNP measurement were performed within the same day. With speckle-tracking echocardiography, we analysed peak systolic global longitudinal strain (GLS), segmental longitudinal strain and strain rate of the RV free wall, LV lateral wall, and septum. Patients had a lower RV free wall strain than controls (-18.1 +/- 4.5 vs. -26.5 +/- 4.5%, P < 0.001), especially at the apical segment (-15.9 +/- 7.4 vs. -28.2 +/- 7.7%, P, 0.001), and lower RV strain rate. LV GLS was also lower (-17.4 +/- 2.5 vs. -19.6 +/- 1.9%, P < 0.001), mainly due to the interventricular septum. Patients with PR >25% had higher LV GLS and RV free wall strain than patients with PR

Purpose: To describe mortality and morbidity in the total cohort of patients who underwent a Mustard operation for transposition of the great arteries (TGA) between 1971-1980 in our center and were followed prospectively every 10 years. Methods: Survival status was obtained from the Dutch National Population Registry. Patients who participated in one or both of the previous studies were invited for thorough in-hospital clinical investigation. Patients who could or would not participate were asked to fulfill a written questionnaire. Events were defined as death or heart transplantation, re-intervention, arrhythmia, heart failure, pacemaker or ICD implantation and endocarditis. Results: Information on survival status was obtained in 86 patients (95%). Mean follow-up was 35±2 years, with a range of 32-39 years. Cumulative survival was 80% after 20 years, 77% after 30 years and 68% after 37 years. Event free survival was 36%, 25% and 19% after 20, 30 and 37 years respectively. In the last 10 years 2 patients died due to ventricular fibrillation and 1 patient underwent heart transplantation, three patients (6%) underwent re-interventions for baffle stenosis, 5 (10%) had their first episode of supraventricular tachycardia, three (6%) needed a pacemaker (1 for sick sinus syndrome and 2 for AV-blok) and 3 (6%) received an ICD (1 for secondary and 2 for primary prevention), 5 patients (10%) were treated for de novo heart failure. ![Figure][1] Survival after Mustard operation for TGA Conclusion: Long-term outcome of Mustard patients is clearly hampered, with 68% survival and only 19% event-free survival after 37 years. [1]: pending:yes

Purpose: Left ventricular (LV) dysfunction is a major determinant of late adverse clinical outcome in adult patients with Tetralogy of Fallot (ToF) and therefore early detection is crucial. Today, speckle tracking echocardiography (STE) has emerged as a quantitative technique for assessing LV function. The aim of this study was to evaluate LV rotation and twist with STE in adult patients with ToF and its association with right ventricular (RV) and LV dimensions and function. Methods: Eighty-seven ToF patients (54% male, age 33±10 years, 97% in NYHA I) and 56 healthy controls were investigated with STE. Additionally, 51 patients underwent cardiac magnetic resonance imaging (CMR). Using STE, we analysed the LV rotation curves at apex and base and calculated LV twist. Peak apical rotation (AR) > 4 degrees was defined as normal. Results: In 63 (72%) ToF patients and in all healthy controls a normal AR was found (9.9±4.2 vs 10.7±3.8 degrees, p=0.3). However, in these patients the basal rotation (BR) was impaired compared to controls (-4.8±3.7 vs -7.2±3.4 degrees, p

The answer is a non-ST-elevation myocardial infarction (NSTEMI) of the posterior wall. For interpretation of the electrical forces in the dorsal direction, the additional leads V7 to V9 can be placed dorsally. On the ECG of a posterior myocardial infarction lead V2 is a mirror image of the paravertebral lead V9 (Fig. 2). The ECG of this patient showed an isolated STsegment elevation in V2 and therefore a ST-segment depression and no Q wave in V9. This indicated an NSTEMI of the posterior wall. Coronary angiography confirmed a critical stenosis in the right posterolateral branch of the right coronary artery, a normal left circumflex artery and a normal left anterior descending artery (Fig. 3). Subsequently, a percutaneous coronary intervention of the RCA was performed.