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Aims To determine whether atrial-based pacing prevents atrial arrhythmias in adults with congenital heart disease (CHD) compared with ventricular pacing. Methods and results All adult CHD patients from four participating centres with a permanent pacemaker were identified. Patients with permanent atrial arrhythmias at pacemaker implantation and patients who received a pacemaker for treatment of drug-refractory atrial arrhythmias were excluded. The final study population consisted of 211 patients (52% male, 36% complex CHD) who received a first pacemaker for sick sinus dysfunction (n = 82) or atrioventricular block (n = 129) at a median age of 24 years [interquartile range (IQR), 12–34]. A history of atrial arrhythmias at implantation was present in 49 patients (23%). Atrial-based pacing was the initial pacing mode in 139 patients (66%) while the others (34%) received ventricular pacing. During a median follow-up of 13 years (IQR, 7–21), 90 patients (43%) developed an atrial arrhythmia. Multivariate analysis demonstrated no significant effect of atrial-based pacing on subsequent atrial arrhythmias [hazard ratio (HR), 1.53; 95% confidence interval (CI), 0.91–2.56; P = 0.1]. Independent predictors of atrial arrhythmia were history of atrial arrhythmias (HR, 5.55; 95% CI, 3.47–8.89; P< 0.0001), older age (≥18 years) at pacemaker implantation (HR, 2.29; 95% CI, 1.29–4.04; P = 0.005), and complex CHD (HR, 1.57; 95% CI, 1.01–2.45; P = 0.04). Conclusion In contrast to the general population, atrial-based pacing was not associated with a lower incidence of atrial arrhythmia in adults with CHD. [ABSTRACT FROM PUBLISHER]

Background: The arterial switch operation is the current treatment for transposition of the great arteries. Long-term outcome mainly depends on the patency of the transferred coronary arteries. This study assessed the presence of abnormal coronary findings and neoaortic root dilation late after arterial switch operation. Methods: In 30 adult patients after arterial switch operation (22 men, 22 ± 3 years), computed tomography angiography was performed to assess the coronary anatomy and abnormal coronary findings, defined as significant stenosis, interarterial coronary course, and acute angled coronary origins. Neoaortic root dimensions and coronary takeoff height were also assessed. Results: The most common coronary anatomy pattern was found in 24 of 30 patients. Variant anatomy patterns were seen in 6 patients (5 with aberrant circumflex artery, 1 with a single ostium). The prevalence of abnormal coronary findings was higher in patients with variant coronary pattern as compared with patients with common coronary pattern (100% and 29%, respectively; p = 0.003). In particular, an acute angle of the coronary origin was frequently observed. In patients with an acute angle, larger dimensions of the aortic annulus (p = 0.016) and the sinus of Valsalva (p = 0.002) were observed. Moreover, a higher takeoff of the right (p = 0.030) and left (p = 0.002) coronary ostium was noted in patients with acute angles. Conclusions: Abnormal coronary findings were frequently observed in adult patients after arterial switch operation, especially in patients with a variant coronary anatomy pattern. Neoaortic root dilation and a higher coronary takeoff may explain part of the pathophysiology. Long-term follow-up is needed to determine the clinical significance of these findings. [Copyright &y& Elsevier]

Background: Current surgical strategies that aim at preventing pulmonary regurgitation in patients with corrected tetralogy of Fallot (cToF) may result in a certain grade of residual pulmonary stenosis (PS). The clinical implications of a postoperative residual PS in cToF patients remain unclear. Pulmonary valve replacement (PVR) is frequently needed during follow-up of cToF patients. The aim of the current study was to determine the role of residual PS in the need for PVR during follow-up in cToF patients. Methods: cToF patients were included if clinical follow-up after primary surgical correction had taken place for a minimum of 5 years. Patient characteristics, surgical factors, and postoperative factors were reviewed, with a special focus on the transpulmonic systolic gradient. Cox proportional hazards regression analysis was performed to identify predictors of PVR. Results: Of 171 cToF patients, 71 (41.5%) underwent PVR after 24.2 years (interquartile range, 16.8–31.6 years). Year of birth, older age at corrective operation, and patch use significantly predicted PVR during follow-up. By contrast, a mild residual PS in cToF patients (peak systolic gradient, 15–30 mm Hg) independently reduced the risk of PVR, as compared with patients without PS (hazard ratio, 0.47; p = 0.02) and with moderate-to-severe PS (hazard ratio, 0.35; p = 0.01). Conclusions: In addition to the known risks factors for PVR, a postoperative mild residual PS reduces the risk of PVR during follow-up of cToF patients. This finding provides clinical evidence for a conservative PS relief during correction of ToF. [ABSTRACT FROM AUTHOR]

Abstract: Purpose: The aim of this study was to investigate the use of the electrocardiogram-derived ventricular gradient, projected on the x-axis (VGx), for detection of pulmonary hypertension (PH) and for prediction of all-cause mortality in PH patients. Methods: In patients referred for PH screening (n = 216), the VGx was calculated semiautomatically from the electrocardiogram and was defined as abnormal when less than 24 mV·ms. The VGx of PH patients was compared with the VGx of patients without PH. The association between a reduced VGx and mortality was investigated in PH patients. Results: Patients with PH (n = 117) had a significantly reduced VGx: 14 ± 27 vs 45 ± 23 mV·ms, P < .001. Furthermore, a severely reduced VGx (<0 mV·ms) was associated with increased mortality in PH patients: hazard ratio, 1.025 (95% confidence interval, 1.006-1.045; P = .012) per mV·ms VGx decrease. Conclusion: Reduced VGx is associated with the presence of PH and, more importantly, within PH patients, a severely reduced VGx predicts mortality. [Copyright &y& Elsevier]

Background: In women with corrected tetralogy of Fallot (ToF), pregnancy is associated with maternal cardiac, obstetric, and offspring complications. Our aim is to investigate the magnitude and determinants of pregnancy outcome in women with corrected ToF. Methods: In this retrospective international multicenter study using 2 congenital heart disease registries, 204 women with corrected ToF were identified. Within this group, 74 women had 157 pregnancies, including 30 miscarriages and 4 terminations of pregnancy. Detailed information on each completed pregnancy (n = 123) was obtained using medical records and supplementary interviews. Results: Cardiovascular events occurred during 10 (8.1%) pregnancies, mainly (supra)ventricular arrhythmias. Obstetric and offspring events occurred in 73 (58.9%) and 42 (33.9%) pregnancies, respectively, including offspring mortality in 8 (6.4%). The most important predictor was use of cardiac medication before pregnancy (odds ratio for cardiac events 11.7, 95% CI 2.2-62.7; odds ratio for offspring events 8.4, 95% CI 1.4-48.6). In pregnancies with cardiovascular events, significantly more small-for-gestational-age children were born (P value < .01). Conclusions: Cardiovascular, obstetric, and offspring events occur frequently during pregnancies in women with ToF. Maternal use of cardiovascular medication is associated with pregnancy outcome, and maternal cardiovascular events during pregnancy are highly associated with offspring events. [Copyright &y& Elsevier]

The article discusses the conducted study to evaluate the quality of life (QoL) of patients with a history of acute pulmonary embolism (PE). QoL refers on the effect of a disease, as well as its treatment on the physical, psychologic and social function and well being of the patients. In the study, univariate analysis and linear regression analysis were used to analyze and identify the independent determinants of QoL.

Background: Previous studies demonstrated that ventricular response to stress cardiovascular magnetic resonance (CMR) is frequently abnormal in patients with a systemic right ventricle (RV). However, the clinical implications of these findings remained unknown. We sought to evaluate whether abnormal response to stress CMR predicts adverse outcome in patients with a systemic RV. Methods: Thirty-nine adult patients (54% male; mean age 26, range 18-65 years) with a systemic RV underwent stress CMR to determine the response of RV volumes and ejection fraction (EF). During follow-up, cardiac events, defined as hospitalization for heart failure, cardiac surgery, aborted cardiac arrest, or death, were recorded. The prognostic value of an abnormal response to stress, defined as lack of a decrease in RV end-systolic volume (ESV) or lack of an increase in RV EF, was assessed. Results: We frequently observed an abnormal response to stress, as RV ESV did not decrease in 17 patients (44%), and RV EF did not increase in 15 patients (38%). After a mean follow-up period of 8.1 years, 8 (21%) patients had reached the composite end point. The inability to decrease RV ESV during stress was predictive for cardiac events with a hazard ratio of 2.3 (95% CI 1.19-88.72, P = .034), as was the inability to increase RV EF with a hazard ratio of 2.3 (95% CI 1.31-81.59, P = .027). Conclusions: Stress CMR potentially has important prognostic value in patients with a systemic RV. Patients with a systemic RV who show abnormal cardiac response to stress have a substantially higher risk of adverse outcome. [ABSTRACT FROM AUTHOR]

Background: Angiotensin II receptor blockers have been proven to be beneficial in left ventricular failure. In patients with a morphologic right ventricle supporting the systemic circulation, its efficacy has not yet been established. Methods: We designed a multicenter, prospective, randomized, double-blind, placebo-controlled trial studying the effect of valsartan in patients with a systemic right ventricle due to a congenitally or surgically corrected transposition of the great arteries. The primary end point is the change in right ventricular ejection fraction as measured by cardiovascular magnetic resonance or multidetector row cardiac computed tomography in case of pacemaker patients. Conclusion: This large prospective, double-blind, randomized, placebo-controlled trial will establish the role of angiotensin II receptor blockers (valsartan) in the treatment of patients with a systemic right ventricle. [ABSTRACT FROM AUTHOR]

Aims: Data regarding pregnancy outcome in women with congenital heart disease (CHD) are limited. [ABSTRACT FROM PUBLISHER]

Aims: Mortality in adults with congenital heart disease is known to be increased, yet its extent and the major mortality risks are unclear. [ABSTRACT FROM PUBLISHER]

Abstract: The population of adult survivors with tetralogy of Fallot (TOF) is growing over the last decades due to improvements in perinatal management, intensive care and surgical techniques. Pulmonary regurgitation (PR) plays a crucial role in the long-term outcome of these patients. Although PR may be tolerated well for many years, eventually it may lead to right ventricular (RV) dilatation, RV dysfunction, exercise intolerance, arrhythmia and sudden cardiac death. Cardiovascular magnetic resonance (CMR) imaging is an important tool in the follow-up of patients after TOF repair, because biventricular volumes and function and PR volume and fraction can be measured with great accuracy and reproducibility. CMR imaging studies have identified risk factors for late adverse outcomes in patients after TOF repair. These include increased RV end-diastolic volume (EDV), reduced left ventricular ejection fraction and abnormal RV outflow tract function. Other applications of CMR include stress imaging, assessment of diastolic function and late gadolinium enhancement, which have provided additional insight in the function of the RV. Timing of pulmonary valve replacement (PVR) is controversial and should balance between the preservation of RV function and the need for subsequent PVR surgery, since the life-span of a homograft is limited. Based on CMR imaging studies, PVR will be considered if the RVEDV reaches a threshold of between 150 and 200ml/m2 in the presence of severe PR. However, timing of PVR should be based on multiple factors, other than RV size and PR fraction alone. [Copyright &y& Elsevier]

Cardiovascular magnetic resonance (CMR) imaging provides highly accurate measurements of biventricular volumes and mass and is frequently used in the follow-up of patients with acquired and congenital heart disease (CHD). Data on reproducibility are limited in patients with CHD, while measurements should be reproducible, since CMR imaging has a main contribution to decision making and timing of (re)interventions. The aim of this study was to assess intra-observer and interobserver variability of biventricular function, volumes and mass in a heterogeneous group of patients with CHD using CMR imaging. Thirty-five patients with CHD (7–62 years) were included in this study. A short axis set was acquired using a steady-state free precession pulse sequence. Intra-observer and interobserver variability was assessed for left ventricular (LV) and right ventricular (RV) volumes, function and mass by calculating the coefficient of variability. Intra-observer variability was between 2.9 and 6.8% and interobserver variability was between 3.9 and 10.2%. Overall, variations were smallest for biventricular end-diastolic volume and highest for biventricular end-systolic volume. Intra-observer and interobserver variability of biventricular parameters assessed by CMR imaging is good for a heterogeneous group of patients with CHD. CMR imaging is an accurate and reproducible method and should allow adequate assessment of changes in ventricular size and global ventricular function. [ABSTRACT FROM AUTHOR]

The aim of this study was to evaluate the feasibility of right ventricular (RV) longitudinal peak systolic strain (LPSS) assessment for the follow-up of adult patients with corrected tetralogy of Fallot (TOF). Adult patients ( n = 18) with corrected TOF underwent echocardiography and CMR twice with a time interval of 4.2 ± 1.7 years. RV performance was derived from CMR, and included RV volumes and ejection fraction (EF). LPSS was calculated globally (GLPSS) and in the RV free wall (LPSS FW), with echocardiographic speckle-tracking strain-analysis. Baseline (G)LPSS values were compared between patients and healthy controls; the relation between (G)LPSS and CMR parameters was evaluated and the changes in (G)LPSS and CMR parameters during follow-up were compared. GLPSS and LPSS FW were significantly reduced in patients as compared to controls (−14.9 ± 0.7% vs. −21.6 ± 0.9% and −15.5 ± 0.9% vs. −22.7 ± 1.5%, P < 0.01). Moderate agreement between LPSS and CMR parameters was observed. RV EF remained unchanged during follow-up, whereas GLPSS and LPSS FW demonstrated a significant reduction. RVEF showed a 1% increase, whereas GLPSS decreased by 14%, and LPSS FW by 27%. RV LPSS is reduced in TOF patients as compared to controls; during follow-up RV EF remained unchanged whereas LPSS decreased suggesting that RV LPSS may be a sensitive marker to detect early deterioration in RV performance. [ABSTRACT FROM AUTHOR]

The article assesses whether the electrocardiogram (ECG) is useful for monitoring treatment response based on changes in pulmonary vascular resistance (PVR) in patients with pulmomary arterial hypertension (PAH). An ECG was recorded in 81 PAH patients at the time of diagnostic right heart catheterization and a year after treatment. Results conclude that routine ECG evaluation can be a vital contribution to assess the treatment response in PAH patients.

Abstract: Background and purpose: Normal limits of the spatial QRS-T angle and spatial ventricular gradient (SVG) are only available from Frank vectorcardiograms (VCGs) of male subjects. We determined normal limits for these variables derived from standard 12-lead electrocardiograms (ECGs) of 660 male and female students aged 18 to 29 years. Methods: A computer algorithm was used that constructed approximated VCG leads by inverse Dower matrix transformation of the 12-lead ECG and subsequently calculated the spatial QRS-T angle, SVG magnitude, and orientation. Results: In female subjects, the QRS-T angle was more acute (females, 66° ± 23°; normal, 20°-116°; males, 80° ± 24°; normal, 30°-130°; P < .001), and the SVG magnitude was smaller (females, 81 ± 23 mV·ms; normal, 39-143 mV·ms; males, 110 ± 29 mV·ms; normal, 59-187 mV·ms; P < .001) than in male subjects. The male SVG magnitude in our study was larger than that computed in Frank VCGs (79 ± 28 mV·ms; P < .001). Conclusions: The spatial QRS-T angle and SVG depend strongly on sex. Furthermore, normal limits of SVG derived from Frank VCGs differ markedly from those derived from VCGs synthesized from the standard ECG. As nowadays, VCGs are usually synthesized from the 12-lead ECG; normal limits derived from the standard ECG should preferably be used. [Copyright &y& Elsevier]

The study aimed to assess whether the 12-lead ECG-derived ventricular gradient, a vectorial representation of ventricular action potential duration heterogeneity directed toward the area of shortest action potential duration, can improve ECG diagnosis of chronic right ventricular (RV) pressure load. ECGs from 72 pulmonary arterial hypertension patients recorded <30 days before onset of therapy were compared with ECGs from matched healthy control subjects (n = 144). Conventional ECG criteria for increased RV pressure load were compared with the ventricular gradient. In 38 patients a cardiac magnetic resonance (CMR) study had been performed within 24 h of the ECG. By multivariable analysis, combined use of conventional ECG parameters (rsr' or rsR' in VI, RJS > 1 with R > 0.5 mV in VI, and QRS axis >90°) had a sensitivity of 89% and a specificity of 93% for presence of chronic RV pressure load. However, the ventricular gradient not only had a higher diagnostic accuracy for chronic RV pressure load by receiver operating characteristic analysis [areas under the curve (AUC) = 0.993, SE 0.004 as. AUC = 0.945, SE 0.021, P < 0.05], but also discriminated between mild-to-moderate and severe RV pressure load. CMR identified an inverse relation between the ventricular gradient and RV mass, and a trend toward a similar relation with RV volume. In conclusion, chronically increased RV pressure load is electrocardiographically reflected by an altered ventricular gradient associated with RV remodeling-related changes in ventricular action potential duration heterogeneity. The use of the ventricular gradient allows ECO detection of even mildly increased RV pressure load. [ABSTRACT FROM AUTHOR]

The study aim was to assess three-dimensional electrocardiogram (ECG) changes during development of pulmonary arterial hypertension (PAH). PAH was induced in male Wistar rats (n = 23) using monocrotaline (MCT; 40 mg/kg sc). Untreated healthy rats served as controls (n = 5). ECGs were recorded with an orthogonal three-lead system on days 0, 14, and 25 and analyzed with dedicated computer software. In addition, left ventricular (LV)-to-right ventricular (RV) fractional shortening ratio was determined using echocardiography. Invasively measured RV systolic pressure was 49 (SD 10) mmHg on day 14 and 64 (SD 10) mmHg on day 25 vs. 25 (SD 2) mmHg in controls (both P < 0.001). Baseline ECGs of controls and MCT rats were similar, and ECGs of controls did not change over time. In MCT rats, ECG changes were already present on day 14 but more explicit on day 25: increased RV electromotive forces decreased mean QRS-vector magnitude and changed QRS-axis orientation. Important changes in action potential duration distribution and repolarization sequence were reflected by a decreased spatial ventricular gradient magnitude and increased QRS-T spatial angle. On day 25, LV-to-RV fractional shortening ratio was increased, and RV hypertrophy was found, but not on day 14. In conclusion, developing PAH is characterized by early ECG changes preceding RV hypertrophy, whereas severe PAH is marked by profound ECG changes associated with anatomical and functional changes in the RV. Three-dimensional ECG analysis appears to be very sensitive to early changes in RV afterload. [ABSTRACT FROM AUTHOR]

Background: Predicting changes in right ventricular (RV) size and function after pulmonary valve replacement (PVR) is important for timely reintervention in adult tetralogy of Fallot patients. Methods: We analyzed the influence of pulmonary regurgitation severity and RV size and function before PVR on the outcome of RV size and function after PVR in 27 adult Fallot patients who had cardiac magnetic resonance imaging before and after PVR. RV dimensions were indexed for body surface area. Results: Pulmonary regurgitation (48% ± 11% of RV stroke volume) was not related to RV dimensions and function before PVR. Moreover, severity of pulmonary regurgitation did not influence changes in RV dimensions after PVR. The indexed RV end-systolic volume before PVR (mean, 98 mL/m2; range, 52 to 235 mL/m2) best predicted the indexed RV end-systolic volume after PVR (mean, 59 mL/m2; range, 24 to 132 mL/m2, r = 0.78, p < 0.001) and the indexed RV end-diastolic volume after PVR (mean, 107 mL/m2; range, 70 to 170 mL/m2, r = 0.73, p < 0.001). Baseline RV ejection fraction corrected for valvular insufficiencies and shunting (21% ± 7%) best predicted the RV ejection fraction after PVR (43% ± 10%, r = 0.77, p < 0.001). Conclusions: Timing of PVR should be based on indexed RV end-systolic volume and corrected RV ejection fraction rather than on severity of pulmonary regurgitation. [Copyright &y& Elsevier]

Abstract: Aims: To compare multidetector row computed tomography (MDCT) global and regional left ventricular (LV) function assessment with echocardiography and cardiovascular magnetic resonance (CMR). Methods and results: In 25 patients, who were referred for noninvasive angiography with 16-detector row CT, LV function assessment was also performed. A subsequent echocardiogram was performed, and in a subgroup of patients, CMR examination was completed to evaluate LV function. For global function assessment, the LV ejection fraction (LVEF) was calculated. Regional LV function was scored using a 17-segment model and a 4-point scoring system. MDCT agreed well with echocardiography for the assessment of LVEF (r =0.96; bias 0.54%; p <0.0001) and regional LV function (κ =0.78). Eight patients had no contra-indications and gave informed consent for CMR examination. A fair correlation between MDCT and CMR was demonstrated in the assessment of LVEF (r =0.86; bias −1.5%; p <0.01). Regional LV function agreement between MDCT and CMR was good (κ =0.86). Conclusion: MDCT agreed well with both echocardiography and CMR in the assessment of global and regional LV function. Global and regional LV function may accurately be evaluated by 16-detector row CT, and can be added to a routine CT image analysis protocol without need for additional contrast or imaging time. [Copyright &y& Elsevier]

Cardiovascular magnetic resonance (CMR) is becoming an important tool in the clinical management of patients with congenital heart disease. Because of the diverse problems patients may face after initial correction for tetralogy of Fallot and the large amount of CMR techniques that can be applied, creating a patient-orientated imaging protocol is a difficult issue. Although it is still not certain what the impact of some parameters, provided by CMR, should be on clinical decision making, new techniques are being developed and applied. In this report, we review the current clinical issues in patients with tetralogy of Fallot and review the current implication and limitations of CMR in this patient category. [Copyright &y& Elsevier]

Cardiovascular magnetic resonance (CMR) with flow velocity mapping has emerged as a noninvasive method to measure flow in saphenous vein coronary artery bypass grafts. The aim of the current study was to retrospectively test two previously described analysis methods on a large CMR data set and to compare their diagnostic accuracy in detecting diseased vein grafts. In 125 vein grafts of 68 patients, volume flow parameters (volume flow, systolic and diastolic peak flow, diastolic-to-systolic flow ratio at rest and during adenosine stress, and flow reserve) were derived from the velocity maps. Method 1 implemented basal flow [ABSTRACT FROM AUTHOR]

Background: Multidetector-row computed tomography (MDCT) is a versatile modality to evaluate stenoses in native coronary arteries and bypass grafts. Acquired MDCT data can additionally be used to assess left ventricular ejection fraction (LVEF). The purpose was to use MDCT for the assessment of bypass graft and coronary artery disease combined with evaluation of LVEF. Methods: Twenty-five patients underwent 16-detector-row CT examination and coronary angiography. Bypass grafts and nongrafted coronary artery segments at MDCT were evaluated on eligibility, patency, and > or = 50% stenosis. The MDCT data set was used to calculate LVEF and was divided into patients with no/subendocardial/transmural myocardial infarctions (MIs). Results: Ninety vessels were evaluated: 14 arterial grafts/53 vein grafts/23 nongrafted vessels. Of 225 segments, 17 were ineligible for evaluation because of metal clips. With MDCT, patency in segments of arterial grafts/vein grafts/nongrafted vessels could be evaluated with high accuracy in 100%/100%/97% of segments. In arterial grafts, stenoses > or = 50% did not occur at angiography, which was for all eligible segments correctly diagnosed at MDCT. Stenosis > or = 50% could be correctly detected by MDCT with a sensitivity/specificity of 100%/94% for vein grafts and 100%/89% for nongrafted vessels. Negative predictive value was 100% for vein grafts and nongrafted vessels. In patients with transmural MI, MDCT revealed a significant lower LVEF as compared with patients without or with subendocardial MI (P < .05). Conclusion: Comprehensive assessment of bypass grafts, nongrafted vessels, and LVEF is feasible with MDCT. Owing to the high negative predictive value this noninvasive approach may be used as gatekeeper before coronary angiography. [ABSTRACT FROM AUTHOR]

Background: Coronary angiography is considered the gold standard in evaluating vein graft disease; however, angiography does not allow assessment of hemodynamic consequences of lesions. In this study hemodynamic consequences of significant stenoses in vein grafts were evaluated by Doppler velocity assessment, and results were compared with single photon emission computed tomography (SPECT) perfusion imaging.Methods and Results: Angiography was performed in 58 patients after coronary artery bypass grafting because of recurrent chest pain. During the procedure, Doppler velocity measurements were acquired before and after administration of adenosine. Of 58 patients (with 78 vein grafts), 20 patients (with 24 vein grafts) underwent SPECT perfusion imaging. Grafts were divided into those with nonsignificant percent diameter stenosis (< 50%) (n = 49) and those with significant percent diameter stenosis (> or =50%) (n = 29). When a cutoff value for coronary flow velocity reserve (CFVR) of 1.8 was applied, modest agreement (69%, kappa = 0.25, P < .05) between CFVR and angiography was shown. Agreement between SPECT and angiography was also modest (63%, kappa = 0.28, P = not significant). SPECT and CFVR provided comparable information in 20 of 24 grafts with available SPECT, illustrating good agreement (83%, kappa = 0.61, P = .001).Conclusions: Significant stenoses in vein grafts require further exploration to assess their hemodynamic significance. The Doppler velocity results agreed better with SPECT perfusion imaging than with percent diameter stenosis in the evaluation of vein graft function. [ABSTRACT FROM AUTHOR]

Over the past decades, life expectancy in patients with congenital heart disease has increased dramatically. However, serious complications may develop late after total repair in infancy. These complications are usually the result of longstanding pulmonary regurgitation, which leads to dilatation of the right ventricle and an increased risk for severe arrhythmias. Therefore lifelong follow-up in these patients is required. Cardiac magnetic resonance imaging is the current imaging tool of choice because it offers superior imaging quality and enables accurate quantification of functional parameters such as flow volumes and systolic and diastolic performance. [ABSTRACT FROM AUTHOR]

Background: In patients with an intra-atrial correction for transposition of the great arteries (TGA) an abnormal response to stress testing is common. However, hemodynamic responses may vary substantially when different stress tests are used. We compared the hemodynamic response to dobutamine stress with the response to physical exercise in patients and controls. Methods: Thirty-nine patients and 25 age/sex-matched control subjects underwent either dobutamine stress (15 µg/kg/min) or submaximal physical exercise cardiovascular magnetic resonance. End-systolic and end-diastolic right ventricular volumes (ESV; EDV) were determined. Five representative patients underwent both stress tests. For these patients, wall thickening reserve was calculated as systolic wall thickening during stress minus systolic wall thickening at rest. Results: In controls, dobutamine stress and physical exercise showed similar responses: stroke volume, cardiac output, and ejection fraction increased significantly, whereas ESV decreased significantly and EDV was unchanged. In patients, stroke volume did not increase with either dobutamine or exercise (- 8.6% vs. 2.9%). Ejection fraction increased significantly with dobutamine (16%, p [ABSTRACT FROM AUTHOR]

Myocardial DNA concentration and ventricular weight were measured in the hearts of normal and spontaneously hypertensive rats varying in age from 1 day to 1 year and the ventricular DNA content calculated. A linear relation between ventricular DNA content and ventricular weight was observed in both rat strains. This linear relation was deduced theoretically, assuming that the number of ventricular non-myocytes per heart was proportional to ventricular weight and that the number of ventricular myocytes per heart was constant after the first postnatal week. The fit with experimental data produced values of the number of ventricular myocytes (42.5 × 106) and non-myocytes (199 × 106 per gram of ventricular tissue) in normal rat heart. Using published data on ventricular volume occupied by myocytes (80%) and myocardial specific density (1.06 g·ml−1), the mean(SD) volume of individual myocytes could be calculated (17758(2632) multiplied by ventricular weight, expressed in μm3·g). The values obtained compared well with experimental observations reported in the present study (18504(131) μm3·g) and in other studies. Hearts from spontaneously hypertensive rats had lower numbers of myocytes (37.4 × 106) and non-myocytes (180 × 106 per gram) per heart than normal hearts with identical ventricular weights. Hearts of normal 2 month old rats that were made hypertrophic by various means were identical to non-hypertrophic hearts of normal older rats with identical ventricular weights in relation to the number of ventricular myocytes and non-myocytes per heart. It is concluded that ventricular hypertrophy induced in young adult rats might be regarded as normal growth occurring at an accelerated rate. [ABSTRACT FROM PUBLISHER]

Background: In patients with repaired tetralogy of Fallot (rTOF), multiple reoperations or percutaneous interventions after pulmonary valve replacement (PVR) may be necessary due to limited homograft durability. However, data to guide individualised prediction of homograft durability remain scarce. The aim of this study was to provide risk models for RV to pulmonary artery homograft durability.Methods: This retrospective multicentre study included consecutive patients with rTOF who had undergone PVR at an age of >12 years. Homograft dysfunction was defined as at least moderate pulmonary regurgitation (PR) or pulmonary stenosis (PS) (pressure gradient ≥36 mm Hg) as assessed by echocardiography. Reintervention was defined as percutaneous intervention or redo-PVR.Results: A total of 153 patients with rTOF were included (62% male, mean age at PVR 31±11 years, pulmonary homograft 96%, follow-up 9.6 years (IQR 5.9, 13.3)). Average freedom from homograft dysfunction and reintervention after 10 years was 74% and 89%, respectively. In multivariable Cox proportional hazards analysis, postoperative PS ≥20 mm Hg (HR 6.52, 95% CI 3.09 to 13.7), postoperative PR ≥ grade 1 (HR 3.13, 95% CI 1.45 to 6.74) and age at PVR <18 years (HR 3.52, 95% CI 1.64 to 7.53) were independently predictive for homograft dysfunction. In patients without any risk factor, 10-year freedom from homograft dysfunction and reintervention was excellent (91% and 96%, respectively) in contrast to patients with ≥2 risk factors (25% and 73%, respectively).Conclusions: Individualised prediction of homograft durability in patients with rTOF can be guided by early postoperative echocardiography. In adult patients without early postoperative PS or PR, homograft dysfunction and reintervention are unlikely to occur within 10 years, and follow-up may be less stringent. [ABSTRACT FROM AUTHOR]

An abstract of the conference paper "Bi-atrial function and its relation with biventricular function and clinical parameters in patients operated for tetralogy of fallot," by Saskia E. Luijnenburg and colleagues is presented.

Abstract: Background: This study evaluated the diameters and distensibility of the aortic root as well as the degree of aortic regurgitation (AR) and its effect on left ventricular (LV) function in patients 8.2 ± 3.1 years after they underwent the Ross procedure, with a comparison of these parameters between patients and matched healthy subjects. Methods: Eighteen Ross procedure patients (16 male patients, age [mean ± SD] 19.2 ± 3.8 years) and 18 matched healthy subjects (16 male patients, age [mean ± SD] 19.7 ± 4.2 years) underwent magnetic resonance imaging. Measurements for diameters (at 4 levels) and the distensibility of the aortic root were performed using a steady-state free precession sequence. Aortic flow was assessed with a velocity-encoded phase-contrast sequence. Left ventricular systolic function was assessed with a gradient-echo sequence in the short-axis plane. Comparison of parameters was performed using the Mann-Whitney U test. Correlations between diameters, distensibility, AR fraction, and LV systolic function were expressed with Spearman rank correlation coefficients. Linear regression analysis was used to identify predictors of LV systolic dysfunction. Results: Aortic root diameters were increased in Ross procedure patients as compared with healthy subjects (mean difference 6.3-11.6 mm, P ≤ .02 at all 4 levels). Distensibility of the aortic root was lower in patients (1.9 ± 1.1 vs 7.8 ± 3.3 mm Hg−1, P < .01). An AR fraction >5% was present in 14 of the 18 patients (mean AR fraction 8% ± 5% vs 1% ± 1%, P < .01). Left ventricular ejection fraction was lower in patients (50% ± 6% vs 57% ± 6%, P < .01). Dilatation, decreased distensibility, and AR fraction were correlated with impaired LV systolic function (P < .05 for all). The AR fraction predicted impaired LV systolic function (P < .01). Conclusions: Magnetic resonance imaging shows dilatation and decreased distensibility of the aortic root, AR, and consequent impaired LV systolic function in patients after the Ross procedure. [Copyright &y& Elsevier]

The aim of this study was to analyze the effect of pressure and/or volume overload on right ventricular (RV) function and brain natriuretic peptide (BNP) levels in patients with surgically corrected congenital heart disease. Forty-two consecutive patients aged 17 to 57 years (median 30) with congenital heart disease (32 with tetralogy of Fallot and 10 with pulmonary stenosis) were examined. The RV systolic pressure was estimated using Doppler echocardiography. Cardiovascular magnetic resonance imaging was used to obtain the RV volumes, ejection fraction (EF) and corrected EF (cEF). Plasma BNP levels were determined by immunoradiometric assay. Patients were categorized as having volume overload when pulmonary regurgitation was ≥10% and pressure overload when the RV systolic pressure was >40 mm Hg. Patients with RV volume overload had a lower RVEF compared with patients with pressure overload (p = 0.02) and lower left ventricular EF (p <0.001). BNP was higher in patients with volume overload than in patients with pressure overload (p = 0.002). BNP correlated with pulmonary regurgitation, RVEF, RV cEF, and left ventricular EF. In linear regression analysis, RV cEF was an independent predictor for BNP, after adjustment for age. Without the parameter of RV cEF in the regression model, pulmonary regurgitation and RVEF were independently associated with BNP level, after adjustment for age. In conclusion, patients with RV volume overload had higher BNP levels and lower RV function than patients with RV pressure overload. BNP levels were independently associated with the degree of RV volume overload and RV function. [Copyright &y& Elsevier]

Objectives: This study was designed to perform a head-to-head comparison between single-photon emission computed tomography (SPECT) and cardiovascular magnetic resonance (CMR) to evaluate hemodynamic significance of angiographic findings in bypass grafts. Background: The hemodynamic significance of a bypass graft stenosis may not always accurately be determined from the coronary angiogram. A variety of diagnostic tests (invasive or noninvasive) can further characterize the hemodynamic consequence of a lesion. Methods: Fifty-seven arterial and vein grafts in 25 patients were evaluated by angiography, SPECT perfusion imaging, and coronary flow velocity reserve determination by CMR. Based on angiography and SPECT, four different groups could be identified: 1) no significant stenosis (<50%), normal perfusion; 2) significant stenosis (>/=50%), abnormal perfusion; 3) significant stenosis, normal perfusion (no hemodynamic significance); and 4) no significant stenosis, abnormal perfusion (suggesting microvascular disease). Results: A complete evaluation was obtained in 46 grafts. Single-photon emission computed tomography and CMR provided similar information in 37 of 46 grafts (80%), illustrating good agreement (kappa = 0.61, p < 0.001). Eight grafts perfused a territory with scar tissue. When agreement between SPECT and CMR was restricted to grafts without scar tissue, it improved to 84% (kappa = 0.68). Integration of angiography with SPECT categorized 14 lesions in group 1, 23 in group 2, 6 in group 3, and 3 in group 4. Single-photon emission computed tomography and CMR agreement per group was 86%, 78%, 100%, and 33%, respectively. Conclusions: Head-to-head comparison showed good agreement between SPECT and CMR for functional evaluation of bypass grafts. Cardiovascular magnetic resonance may offer an alternative method to SPECT for functional characterization of angiographic lesions. [ABSTRACT FROM AUTHOR]

The Post-Pulmonary Embolism Syndrome (PPES) comprises heterogeneous entities, including chronic thromboembolic disease with/without pulmonary hypertension (CTEPH/CTEPD), and deconditioning. To assess underlying physiological determinants of PPES, and efficacy and safety of rehabilitation training in these patients. 56 consecutive PE patients with persistent dyspnea and/or functional limitations despite ≥3 months of anticoagulation underwent standardized diagnostic work-up including exercise testing as part of routine practice. All diagnostic (imaging and cardiopulmonary function) tests were interpreted by a core group of experienced clinicians. A subgroup of patients without CTEPH or other treatable conditions was referred for a 12-week personalized rehabilitation program, studying changes in physical condition and patient-reported outcome measures. Persistent vascular occlusions were observed in 21/56 patients (38%) and CTEPH was confirmed in ten (18%). Regarding those without CTEPH, impaired cardiopulmonary responses were evident in 18/39 patients with available CPET data (46%), unrelated to chronic thrombi. Rehabilitation was completed by 27 patients after excluding 29 (patients with CTEPH or treatable comorbidities, refusal, ineligibility, or training elsewhere). Training intensity, PE-specific quality of life (PEmb-QoL) and fatigue (CIS) improved with a median difference of 20 W (p = 0.001), 3.9 points (p < 0.001) and 16 points (p = 0.003), respectively. Functional status (Post-VTE Functional Status Scale) improved ≥1 grade in 18 (67%) patients, and declined in one (3.7%). Our findings suggest that abnormal cardiopulmonary responses to exercise are common in patients with PPES and are not limited to those with chronic thrombi. Offering pulmonary rehabilitation to patients not treated otherwise seems safe and promising. • We studied physiological determinants in patients with Post-Pulmonary Embolism Syndrome (PPES). • Insufficient cardiopulmonary responses to exercise were common in these patients. • These findings were not limited to patients with persistent vascular occlusions. • Offering a 12-week pulmonary rehabilitation program seems safe and promising. • 'Post-VTE Functional Status' improved after completing rehabilitation training. [ABSTRACT FROM AUTHOR]

An anomalous coronary artery is reported in 2% to 23% of patients with tetralogy of Fallot (TOF). Knowledge of coronary anatomy prior to corrective surgery is vital to avoid damage to vessels crossing the right ventricular outflow tract (RVOT). A meta-analysis on the prevalence of anomalous coronary arteries in TOF is lacking to date. Here, an overview of coronary anomalies in TOF is provided and implications for patient management are discussed. PubMed, Embase and Web of Science were searched. Analysis was done using Revman 5.3 (Cochrane Community, London). The primary analysis focused on the origin and proximal course of the right and left coronary arteries. In addition, the prevalence of large conus arteries and coronary arteriovenous fistulas (CAVF) was calculated. Twenty-eight studies, encompassing 6956 patients, were included; 6% of TOF patients have an anomalous coronary artery. Hereof, 72% cross the RVOT; the majority of the remaining 28% courses behind the aorta. Six percent of patients have a large conus artery and 4% a CAVF. Other coronary anomalies include a left or right coronary artery from the pulmonary trunk or left or right pulmonary artery, coronary tree hypoplasia and anastomoses between coronary and bronchial arteries. The prevalence of coronary anomalies in TOF is 4–6%. In patients with an anomalous coronary artery, 72% cross the RVOT. The combined risk of encountering an anomalous coronary artery or a large conus artery crossing the RVOT is 10.3%. Coronary anatomy should be defined before surgery and the surgical approach adapted accordingly. Unlabelled Image • The prevalence of coronary anomalies in TOF is 4–6%. • In patients with an ACA, 72% of the anomalous arteries cross the RVOT. • The risk of encountering an ACA or large conus artery crossing the RVOT is 10.3%. • Before surgery, define the coronary anatomy to avoid damage of anomalous vessel. • Surgical approach should be adapted to the course of an anomalous coronary artery. [ABSTRACT FROM AUTHOR]

Objective: Maternal heart disease (HD) complicates 1-4 % of pregnancies and is associated with adverse maternal and fetal outcomes. Although vaginal birth is generally recommended in the guidelines, cesarean section (CS) rates in women with HD are often high. Aim of the present study was to evaluate mode of birth and pregnancy outcomes in women with HD in a tertiary care hospital in the Netherlands.Study Design: The study population consisted of 128 consecutive pregnancies in 99 women with HD, managed by a pregnancy heart team between 2012-2017 and ending in births after 24 weeks' gestation. Pregnancy risk was assessed per modified World Health Organization class. Mode of birth (planned and performed) and maternal and fetal complications (cardiovascular events, postpartum hemorrhage, prematurity, small for gestational age and death) were assessed for each pregnancy.Results: Pregnancy risk was classified as modified World Health Organization class I in 23 %, class II in 50 %, class III in 21 % and class IV in 6% of pregnancies. Planned mode of birth was vaginal in 114 pregnancies (89 %) and CS in 14 (11 %; nine for obstetric and five for cardiac indication). An unplanned CS was performed in 18 pregnancies (16 %; 16 for obstetric and two for cardiac indications). Overall mode of birth was vaginal in 75 % and CS in 25 %. Twelve cardiovascular events occurred in eight pregnancies (6 %), postpartum hemorrhage in nine (7 %) and small for gestational age in 14 (11 %). No maternal or fetal deaths occurred.Conclusions: Findings of this study indicate that - given that pregnancies are managed and mode of birth is meticulously planned by a multidisciplinary pregnancy heart team - vaginal birth is a suitable option for women with HD. [ABSTRACT FROM AUTHOR]

Abstract Patients presenting with chest pain suggestive of coronary artery disease (CAD) who at coronary arteriography appear to be free of obstructive disease have presented a diagnostic and therapeutic challenge since the 1970's. Studies in female patient populations have suggested that this is predominantly a women's syndrome usually caused by microvascular endothelial dependent and independent dysfunction. A critical review of the literature focusing on studies including both women and men revealed that apart from a higher incidence of this syndrome in women there are no clinical relevant differences between both sexes. In women a lower coronary flow reserve has been reported but this appears to be mainly due to a higher basal flow. Important questions with regard to the clinical implications of microvascular dysfunction have yet to be resolved in studies involving women as well as men in which a distinction is made between patients with normal coronary arteries and those with nonobstructive disease. Highlights • Sex specificity of microvascular angina has not been proven. • Women more often than men have chest pain without coronary obstructions. • Hypertension is a common cause of microvascular disease and in part reversible. • Irrespective of sex normal coronary arteries indicates a favorable prognosis. [ABSTRACT FROM AUTHOR]

Abstract Objectives In the VAL-SERVE (Valsartan in Systemic Right Ventricle) trial, three-year valsartan treatment improved systemic ventricular function only in symptomatic patients with congenitally or with an atrial switch corrected transposition of the great arteries. The aim of the current study was to investigate the longer-term clinical outcomes after valsartan treatment. Methods From 2006 to 2009, 88 adults were randomly allocated 1:1 to either valsartan or placebo for three consecutive years. Endpoints were defined as overall survival and freedom from clinical events (arrhythmia, heart failure, tricuspid valve surgery, death). Results Cardiac drug use and median follow-up after trial close-out (8.3 years) was similar between the randomization groups. Six patients (valsartan n = 3, placebo n = 3) died in 364 and 365 person-years (P = 0.999). No difference in the composite or separate clinical endpoints was found between the randomization groups, with corresponding long-term event-free survival rates of 50% and 34%. Nevertheless, in symptomatic patients valsartan significantly reduced the risk for events compared to placebo (HR 0.37, 95% CI 0.17–0.92). Analysis for repeated events and on-treatment analysis with any renin-angiotensin-aldosterone-system-inhibitor did not alter these results. Conclusions Valsartan treatment in systemic RV patients did not result in improved survival at longer-term follow-up, but was associated with decreased risk of events in symptomatic patients. Highlights • Systemic right ventricular failure is a major problem in transposition of the great arteries. • Limited data on heart failure treatment in this patient group are inconclusive. • The study shows a reduction in morbidity in symptomatic patients with angiotensin II receptor blockers. • In line with the previous trial results, valsartan has a favorable treatment effect in symptomatic patients. [ABSTRACT FROM AUTHOR]