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Application of the chronic thromboembolic pulmonary hypertension (CTEPH) rule out criteria (manual electrocardiogram [ECG] reading and N-terminal pro-brain natriuretic peptide [NTproBNP] test) can rule out CTEPH in pulmonary embolism (PE) patients with persistent dyspnea (InShape II algorithm). Increased pulmonary pressure may also be identified using automated ECG-derived ventricular gradient optimized for right ventricular pressure overload (VG-RVPO). A predefined analysis of the InShape II study was performed. The diagnostic performance of the VG-RVPO for the detection of CTEPH and the incremental diagnostic value of the VG-RVPO as new rule-out criteria in the InShape II algorithm were evaluated. 60 patients were included; 5 (8.3%) were ultimately diagnosed with CTEPH. The mean baseline VG-RVPO (at time of PE diagnosis) was −18.12 mV·ms for CTEPH patients and − 21.57 mV·ms for non-CTEPH patients (mean difference 3.46 mV·ms [95%CI −29.03 to 35.94]). The VG-RVPO (after 3–6 months follow-up) normalized in patients with and without CTEPH, without a clear between-group difference (mean Δ VG-RVPO of −8.68 and − 8.42 mV·ms respectively; mean difference of −0.25 mV·ms, [95%CI −12.94 to 12.44]). The overall predictive accuracy of baseline VG-RVPO, follow-up RVPO and Δ VG-RVPO for CTEPH was moderate to poor (ROC AUC 0.611, 0.514 and 0.539, respectively). Up to 76% of the required echocardiograms could have been avoided with VG-RVPO criteria replacing the InShape II rule-out criteria, however at cost of missing up to 80% of the CTEPH diagnoses. We could not demonstrate (additional) diagnostic value of VG-RVPO as standalone test or as on top of the InShape II algorithm. • The VG-RVPO improved over time after acute PE for CTEPH and non-CTEPH patients. • The VG-RVPO as a standalone test does not accurately discriminate CTEPH from non-CTEPH patients. • Additional diagnostic value of VG-RVPO on top of the InShape II algorithm could not be shown. • Our findings should be regarded as hypothesis generating due to low sample size. [ABSTRACT FROM AUTHOR]

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.ahj.2005.03.058 Byline: Thomas Oosterhof (a)(c), Barbara J.M. Mulder (c), Hubert W. Vliegen (b), Albert de Roos (a) Abstract: 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. Author Affiliation: (a) Department of Radiology, Leiden University Medical Center, Leyden, The Netherlands (b) Department of Cardiology, Leiden University Medical Center, Leyden, The Netherlands (c) Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands Article History: Received 10 February 2005; Accepted 26 March 2005

Abstract Objectives Variations in coronary anatomy are common and may relate to the position of the coronary ostium relative to the aortic sinus, the angle of coronary take-off, or the course of the coronary arterial branches. Several classification systems have been proposed. However, they all lack a simple rationale that is applicable irrespective of the relative position of the great arteries, as well as in bicuspid aortic valves. We present a modification of a relatively simple system introduced in the early 1980s, designated the "Leiden Convention." Methods The first step of the Leiden Convention is that the clinician takes position in the nonfacing sinus of the aorta looking toward the pulmonary orifice. The right-hand facing sinus is sinus 1 , and the left-hand facing sinus is sinus 2. The coronary branches arising from sinus 1 are annotated proceeding in a counterclockwise fashion toward sinus 2. "Usual" (normal) coronary anatomy would be 1R-2LCx. Given their clinical relevance, single sinus coronary arteries are discussed separately. Results This system was originally designed and highly applicable in hearts with an altered great artery relationship, such as in the variable and complicated patterns seen in transposition of the great arteries and double outlet right ventricle. The modified system also can be used in cases with normally related great arteries, cases with single sinus coronary arteries, and cases with bicuspid aortic valves. Conclusions The modified Leiden Convention is not a strict classification but a simple coronary coding system that is broadly applicable. [ABSTRACT FROM AUTHOR]

Renin-angiotensin-aldosterone system (RAAS) inhibition with angiotensin II receptor blockers or angiotensin-converting enzyme inhibitors is beneficial in patients with acquired left ventricular dysfunction. Adult patients with tetralogy of Fallot (TOF) with right ventricular (RV) dysfunction are at high risk for heart failure, arrhythmias, and sudden cardiac death. However, the efficacy of RAAS inhibition has not been established in these patients.Methods: The REDEFINE is an investigator-initiated, multicenter, prospective, randomized, double-blind, placebo-controlled trial to study the effects of the angiotensin II receptor blocker losartan (target dosage of 150 mg once daily) in adult patients with TOF. Patients with RV dysfunction in the absence of severe valvular dysfunction are eligible for inclusion. The primary end point is the change in RV ejection fraction after 18 to 24 months, as measured by cardiovascular magnetic resonance imaging. In addition, laboratory measurements, echocardiography, and cardiopulmonary exercise testing are performed.Conclusion: The REDEFINE trial will study the effects of RAAS inhibition with losartan in TOF patients with RV dysfunction. [ABSTRACT FROM AUTHOR]

Background: The aim was to assess the diagnostic value of the Inverse Dower (INVD)-derived vectorcardiogram (VCG) and the Kors-derived VCG to detect elevated systolic pulmonary artery pressure (SPAP) in suspected pulmonary hypertension (PH).Methods: In 132 patients, morphologic variables were evaluated by comparing the VCG parameters synthesized by INVD and Kors matrix. Comparison of the diagnostic accuracy of detecting SPAP ≥50mmHg between the matrices was performed by ROC curve analysis and logistic regression analysis.Results: Most VCG parameters differed significantly between INVD and Kors. ROC analysis for detection of SPAP ≥50mmHg by VG projected on the X-axis demonstrated no difference (p=0.99) between INVD (AUC=0.80) and Kors (AUC=0.80). Both the INVD- and Kors-derived VCG provided significant diagnostic information on the presence of SPAP ≥50mmHg (INVD, OR 1.05, 95%CI 1.03-1.07; P<0.001; Kors, OR 1.05, 95%CI 1.03-1.08; P<0.001).Conclusion: Although there were significant differences in measures of vector morphology, both INVD- and Kors-derived VCG demonstrated equal clinical performance in case of elevated SPAP. [ABSTRACT FROM AUTHOR]

Abstract: Background and Purpose: Early, preferably noninvasive, detection of pulmonary hypertension improves prognosis. Our study evaluated the diagnostic accuracy of the electrocardiographically derived Butler–Leggett (BL) score and ventricular gradient (VG) to estimate mean pulmonary artery pressure (PAP). Methods: In 63 patients with suspected pulmonary hypertension, BL score and VG were calculated. The VG was projected on a direction optimized for detection of right ventricular pressure overload (VG-RVPO). BL score and VG-RVPO were entered in multiple linear regression analysis and the diagnostic performance to detect PH (invasively measured mean PAP≥25mmHg) was assessed with receiver operating characteristic analysis. Results: Both BL score and VG-RVPO correlated significantly with mean PAP (r=0.45 and r=0.61, respectively; P <0.001). Combining BL score and VG-RVPO increased the correlation to 0.67 (P <0.001). The diagnostic performance of this combination for the detection of PH was good with an area under the curve of 0.79 (P <0.001). Conclusion: Combination of the BL score and VG-RVPO allows for accurate detection of increased PAP. [Copyright &y& Elsevier]

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]

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]

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]

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]

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]

Objectives: The aim of this research was to assess right ventricular diastolic and systolic function before and after pulmonary valve replacement (PVR) in adult patients after repair of tetralogy of Fallot. Background: Pulmonary valve replacement (PVR) in adult patients late after repair of tetralogy of Fallot leads to rapid improvement of right ventricular (RV) systolic function. Methods: A total of 16 patients and 8 healthy subjects were included. Median age at initial repair was 4.9 (0.9 to 13.1) years, and mean age at PVR was 28.7 (19.5 to 45.6) years. Cardiac magnetic resonance imaging was performed before and 8 and 22 months after PVR. Right ventricular volumes and function as well as RV in- and outflow patterns were assessed. Results: The volume of the early filling of the RV (Evol) increased from 49.8 ± 14.7 ml to 53.8 ± 19.3 ml (not significant) and 62.0 ± 18.9 ml, respectively (p < 0.05), whereas the volume of the atrial contraction (Avol) remained unchanged. Consequently, the Evol/Avol ratio increased from 1.4 ± 0.7 before PVR to 1.6 ± 0.7 at 8 months (not significant) and 2.3 ± 1.2 at 22 months (p < 0.01). The Evol/Avol ratio was not significantly different from the healthy subjects at 22 months, indicating late recovery of diastolic function. Systolic function improved rapidly after PVR; the indexed RV end-systolic volume decreased from 93.7 ± 33.0 ml/m2 to 60.9 ± 18.4 ml/m2 (p < 0.01) and 54.8 ± 21.0 ml/m2 (p < 0.01). Conclusions: In adult patients late after total repair of Fallot, PVR leads to late improvement of diastolic function. We speculate that the rapid volume unloading after PVR increases systolic performance, whereas improvement in diastolic function requires long-term remodeling. [Copyright &y& Elsevier]

Investigates the additional value of magnetic resonance angiography to delineate the course of the coronary artery. Role of echomagnetic resonance angiography in coronary atheriography; Patient characteristics, origin, and course of coronary anomalies; Interpretation of magnetic resonance angiograms.

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.

An abstract of the paper "Serial Follow-Up of Biventricular Function, Exercise Capacity and NT-ProBNP Measurements in Repaired Tetralogy of Fallot: Is There a Role for MR Stress Imaging?," by Saskia E. Luijnenburg and colleagues is presented.

Background: Previous research has shown that women with congenital heart disease (CHD) are more susceptible to cardiovascular, obstetric, and offspring events. The causative pathophysiologic mechanisms are incompletely understood. Inadequate uteroplacental circulation is an important denominator in adverse obstetric events and offspring outcome. The relation between cardiac function and uteroplacental perfusion has not been investigated in women with CHD. Moreover, the effects of physiologic changes on pregnancy-related events are unknown. In addition, long-term effects of pregnancy on cardiac function and exercise capacity are scarce. Methods: Zwangerschap bij Aangeboren Hartafwijking (ZAHARA) II, a prospective multicenter cohort study, investigates changes in and relations between cardiovascular parameters and uteroplacental Doppler flow patterns during pregnancy in women with CHD compared to matched healthy controls. The relation between cardiovascular parameters and uteroplacental Doppler flow patterns and the occurrence of cardiac, obstetric, and offspring events will be investigated. At 20 and 32 weeks of gestation, clinical, neurohumoral, and echocardiographic evaluation and fetal growth together with Doppler flow measurements in fetal and maternal circulation are performed. Maternal evaluation is repeated 1 year postpartum. Implications: By identifying the factors responsible for pregnancy-related events in women with CHD, risk stratification can be refined, which may lead to better pre-pregnancy counseling and eventually improve treatment of these women. [Copyright &y& Elsevier]

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]

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]

Background Adult post-coarctectomy patients (CoA) demonstrate increased cardiovascular morbidity and mortality. The carotid intima-media thickness (CIMT), a marker for atherosclerosis, is increased in CoA. The aim was to evaluate the predictive value of CIMT for cardiovascular events. Methods and results Consecutive CoA patients were prospectively studied during 10.1 ± 0.7 years follow-up. At baseline and follow-up echocardiography, MRI imaging and CIMT imaging were performed, while cardiovascular events were registered. CIMT data were compared with controls. The composite endpoint included: myocardial infarction, cerebrovascular events (CVAs), and (sudden) cardiac death. 160 CoA patients were studied (median age 31.7 (18-74 years), 64% male). Events occurred in 11 patients (7%), five (3%) with myocardial infarction, four (2.5%) with an ischemic CVA and two (1%) died suddenly. An increased CIMT (≥ 0.8 mm) (HR = 15.44, P = < 0.001) was predictive for the occurrence of cardiovascular events. Baseline CIMT was increased in CoA compared to controls (0.64 ± 0.12 mm vs 0.57 ± 0.07 mm, P = 0.005). CIMT progression rates were similar (0.0091 ± 0.016 mm/year vs 0.0097 ± 0.018 mm/year, P = 0.84). Signs of atherosclerosis occurred significantly earlier in CoA patients. Conclusion The contemporary cardiovascular event rate in CoA is 11% in 10 years. Atherosclerosis seems to appear earlier in CoA patients as compared to controls. CoA patients with a CIMT exceeding 0.8 mm have a fifteen fold higher cardiovascular risk. CIMT seems to be a useful tool for cardiovascular risk assessment in CoA. [ABSTRACT FROM AUTHOR]

Objective: To study the changes over time in biventricular size and function, and clinical parameters in patients after repair of tetralogy of Fallot (TOF) without subsequent pulmonary valve replacement (PVR). Methods: We prospectively included 78 non-PVR patients (age 20(6–60)years at baseline), who were studied twice with a 5-year interval. Patients underwent magnetic resonance imaging for assessment of biventricular size and function. Exercise testing and electrocardiography were performed to determine peak oxygen uptake (peak VO2) and QRS duration. N-terminal prohormone brain natriuretic peptide (NT-proBNP) was assessed additionally. Results: Pulmonary regurgitation (PR), right ventricular (RV) volumes and QRS duration increased during 5-year follow-up (RV end-diastolic volume (EDV) 130±30ml/m2 to 138±34ml/m2; QRS 132±27msec to 139±27msec); peak VO2 decreased (96±19% to 91±17%). RV ejection fraction, RV effective stroke volume (eff.SV), and NT-proBNP levels remained unchanged. The slope of RVEDV increase was 1.6±3.0ml/m2/year, and depended on RVeff.SV, not on RVEDV, at baseline. Increase in RVEDV correlated with increase in QRS duration over time (r =0.28, p =0.016), and with decrease in RV mass/EDV ratio over time (r =−0.42, p <0.001), not with decrease in peak VO2. In subgroup analysis, patients with larger RVs at baseline showed larger increase in PR during follow-up and larger decrease in NYHA class over time. Conclusions: In TOF patients with moderate RV dilatation, RVEDV increased by 1.6±3.0ml/m2/year, irrespective of RV size at baseline, but depended on RVeff.SV at baseline. Despite limited progression in RV size, unfavourable changes occurred during 5years follow-up, which suggests there is a need for close follow-up. [ABSTRACT FROM AUTHOR]

Abstract: Background: This study aims to investigate indications and complications of permanent cardiac pacing in adults with congenital heart disease (CHD). Methods and results: Two-hundred and seventy-four CHD patients were identified who underwent permanent pacemaker implantation between 1972 and 2009. The indication for pacing was acquired sinus node or AV node conduction disease (63%), sinus node or AV node conduction disease after cardiac surgery (28%), and drug/arrhythmia-related indications (9%). Patients with complex CHD received a pacemaker at younger age (23 versus 31years, p<0.0001) and more often received an epicardial pacing system (51% versus 23%, p<0.0001) compared to those with simple or moderate CHD. Twenty-nine patients (10.6%) had a periprocedural complication during the primary pacemaker implantation (general population: 5.2%). The most common acute complications were lead dysfunction (4.0%), bleeding (2.6%), pocket infection (1.5%) and pneumothorax (1.5%). During a median follow-up of 12years, pacemaker-related complications requiring intervention occurred in 95 patients (34.6%). The most common late pacemaker-related complications included lead failure (24.8%), pacemaker dysfunction/early battery depletion (5.1%), pacemaker migration (4.7%) and erosion (4.7%). Pacemaker implantation at younger age (<18years) was an independent predictor of late pacemaker-related complication (adjusted hazard ratio 1.68, 95% confidence interval 1.07 to 2.63, p=0.023). Conclusions: The risk of periprocedural complications seems higher in the CHD population compared to the general population and more than one-third of CHD patients encountered a pacemaker-related complication during long-term follow-up. This risk increases for those who receive a pacemaker at younger age. [Copyright &y& Elsevier]

Background: Patients with moderate pulmonary valve restenosis late after relief of severe pulmonary stenosis (PS) may show decreased exercise tolerance. To elucidate the mechanism of decreased exercise tolerance, we evaluated cardiac response to physical and pharmacological stress in these patients and compared results with those of patients with native moderate PS. Methods: Twenty asymptomatic patients with moderate PS were divided into 2 groups: Group I (late after relief of severe PS, n=9), and Group II (no previous intervention, n=11). All patients underwent an exercise test, dobutamine stress (DS) MRI, and delayed contrast enhanced MRI. The response to physical and pharmacological stress was compared between both groups. Results: Group I showed impaired exercise capacity compared to Group II (VO2max=72.8%±3.5% vs. 102.5%±16.3%, p<0.001). During DS-MRI, RV-SV increased in Group II, but not in Group I (+13±8ml, −5±8ml, p<0.001). RV end‐diastolic volume decreased significantly in Group I patients (p=0.006) while it did not significantly change in Group II patients. The amount of RV-SV increase (∆ RV-SV) correlated negatively with the period of moderate PS existence and the current PG in Group I (r=−0.82, p=0.007, and r=−0.68, p=0.04, respectively) but not in Group II (r=0.45, p=0.1, and r=0.40, p=0.2, respectively). Furthermore, ∆ RV-SV correlated negatively with the PG before valvuloplasty (r=−0.76, p=0.02). Conclusion: Impaired exercise capacity in patients with moderate pulmonary restenosis after relief of severe PS is probably caused by inability to increase RV-SV. Disturbed RV filling properties, worsening in time, might play a role. [ABSTRACT FROM AUTHOR]

Purpose: Aortic valve dysfunction is common in coarctation patients(CoA). Bicuspid aortic valve (BAV) in CoA is associated with aortic valve stenosis (AS), aortic valve regurgitation (AR), and ascending aortic dilatation. The aim of this study was to evaluate the progression of and predictors for aortic valve dysfunction in CoA. Methods: 96 CoA patients prospectively underwent echocardiography twice between 2001 and 2010. AS was defined as an aortic valve gradient ≥20mmHg, AR as none/minor, or moderate/severe. Aortic dilatation as an ascending aortic diameter ≥37mm. Results: All patients (median age 28.0years, range 17–61years; male 57%) were followed with a median follow-up of 7.0years. Sixty patients (63%) had BAV. At baseline 10 patients had AS (10%, 9 BAV), 6 patients AR (6%, 3 BAV) and 11 patients aortic dilatation (11%, 11 BAV). At follow-up 15 patients had AS (15%, 13 BAV) and 12 patients AR. (13%, 8 BAV). Median AS progression was 1.1mmHg/5years (range — 13–28). Determinants for AS at follow-up were age (ß=0.20, P=0.01), aortic dilatation (ß=4.6, P=0.03), and baseline aortic valve gradient (ß=0.93, P<0.001). BAV was predictive for AR. (ß=0.91, P=0.049). Conclusion: Progression of AS in adult CoA patients is mild in this young population. Older age, aortic dilatation and the baseline aortic valve gradient are determinants for AS at follow-up. BAV is predictive for AR. These findings point towards a common embryological pathway of both valvular and aortic disease in CoA. [ABSTRACT FROM AUTHOR]

Objectives: The aim of this study was to identify which patients with a systemic right ventricle are at risk for clinical events. Background: In patients with congenitally or atrially corrected transposition of the great arteries, worsening of the systemic right ventricle is accompanied by clinical events such as clinical heart failure or the occurrence of arrhythmia. Methods: At baseline, all subjects underwent electrocardiography, echocardiography, cardiopulmonary exercise testing, and cardiovascular magnetic resonance imaging. Clinical events comprised death, vascular events, tricuspid regurgitation requiring surgery, worsening heart failure, and (supra)ventricular arrhythmia. A Cox proportional hazards analysis was used to assess the most valuable determinants of clinical events. Results: A total of 88 patients with a mean age of 33 years were included in the study. Sixty-five percent were men, and 28% had congenitally corrected transposition of the great arteries. During a follow-up period of 4.3 years, 31 patients (35%) experienced 46 clinical events for an annual risk of 12%. Right ventricular end-diastolic volume index measured by means of cardiovascular magnetic resonance imaging or multirow detector computed tomography (hazard ratio: 1.20; p < 0.01) and peak exercise systolic blood pressure (hazard ratio: 0.86; p = 0.02) were the strongest determinants of clinical events. Patients with a right ventricular end-diastolic volume index above 150 ml/m2 and peak exercise systolic blood pressure below 180 mm Hg were most likely to experience clinical events with an annual event rate of 19% versus 0.9% in patients without these risk factors. Conclusions: Patients with a right ventricular end-diastolic volume index above 150 ml/m2 and peak exercise systolic blood pressure below 180 mm Hg had a 20-fold higher annual event rate than patients without these risk factors. Regular cardiovascular magnetic resonance imaging and exercise testing are important in the risk assessment of these patients. [Copyright &y& Elsevier]

Background: Atrial enlargement may reflect ventricular diastolic dysfunction. Although patients with tetralogy of Fallot (TOF) have been studied extensively, little is known about atrial size and function. We assessed bi-atrial size and function in patients after TOF repair, and related them to biventricular systolic and diastolic function, and clinical parameters. Methods: 51 Patients (21±8years) and 30 healthy controls (31±7years) were included and underwent magnetic resonance imaging to assess bi-atrial and biventricular size, systolic and diastolic function. Patients also underwent exercise testing, and N-terminal prohormone brain natriuretic peptide (NT-proBNP) assessment. Results: In patients, right atrial (RA) minimal volume (34±8ml/m2 vs. 28±8ml/m2, p =0.001) and late emptying fraction were increased; RA early emptying fraction was decreased. Patients had longer right ventricular (RV) deceleration time (0.24±0.10 vs. 0.13±0.04, p <0.001), reflecting impaired RV relaxation, and larger RV volumes. Patients with end-diastolic forward flow (EDFF) had larger RA and RV size, abnormal RA emptying, higher NT-proBNP levels, higher VE/VCO2 slope (ventilatory response to carbon dioxide production), and the most abnormal LV diastolic function (impaired compliance). Patients with abnormal RA emptying (reservoir function <30% and pump function >24%) had higher NT-proBNP levels and worse exercise capacity. RA minimal volume was associated with RV end-diastolic volume (r =0.35, p =0.013). Conclusions: In TOF patients with moderate RV dilatation, abnormal bi-atrial function and biventricular diastolic dysfunction are common. Abnormal RA emptying was associated with signs of impaired clinical condition, as was the presence of EDFF. These parameters, together with RA enlargement, could serve as useful markers for clinically relevant RV diastolic dysfunction. [ABSTRACT FROM AUTHOR]

Aims: Aortic coarctation (CoA) occurs as a “simple” isolated disorder, and in a more “complex” form, combined with associated congenital cardiac abnormalities. Long term outcome of all CoA patients may be complicated by dilatation of the thoracic aorta. The aim of this study was to quantify progressive aortic dilatation, and identify determinants for progressive aortic dilatation. Methods and Results: Cardiovascular Magnetic Resonance Imaging (CMR) and echocardiographic data of 93 CoA patients were analyzed retrospectively on the progression, and determinants, of progressive thoracic aortic dilatation. Outcome of simple- versus complex CoA patients were compared. 93 CoA patients (mean age 39±12years, male 59%) were followed with CMR (follow-up 5.3±1.8years). Twenty-eight patients were classified as simple- and 68 as complex CoA. The mean progression rate of thoracic aortic dilatation was highest in the ascending aorta with 2.2±2.0mm/5years (range 0–7.2mm/5years). History of VSD (β=1.77, P=0.004) and an increased left ventricular mass index (β=0.02, P=0.04) were associated with progressive ascending aortic dilatation. Complex CoA patients show an increased progression rate compared to simple CoA patients with 2.4mm/5years versus 1.5mm/5years respectively. (P=0.03). Conclusion: Adult post-coarctectomy patients show an increased mean progression rate of ascending aortic dilatation with 2.2mm/5years. The progression rate of ascending aortic dilatation is increased in complex CoA patients, as compared to simple CoA patients. These findings point towards a more comprehensive genetic subset of patients with an increased risk for progressive ascending aortic dilatation. [ABSTRACT FROM AUTHOR]

Abstract: Background: We hypothesized 1) that long-term ventricular outcome and exercise capacity would be better in patients with isolated pulmonary valve stenosis (PS) treated with balloon pulmonary valvuloplasty (BPV) than in patients operated for tetralogy of Fallot (TOF), and 2) that ventricular outcome and exercise capacity would not be different in PS patients and healthy controls. Methods: We included 21 PS patients after BPV (16.2±5.2years) and 21 patients operated for TOF (16.6±5.6years), matching them for gender, age at treatment, and age at study. Patients underwent cardiovascular magnetic resonance (CMR) imaging, exercise testing, 12-lead ECG and 24-hour Holter monitoring for assessment of right ventricular (RV) size and function, pulmonary regurgitation (PR), exercise capacity and electrocardiographic status. Healthy controls for CMR imaging and exercise testing were matched for gender and age at study. Results: RV volumes and PR percentage were significantly larger in TOF patients than in PS patients; biventricular ejection fraction (EF) was not different. PR was mild in most PS patients. RV end-systolic volume was significantly larger in PS patients than in healthy controls; RVEF was significantly lower. Both patient groups had similar exercise test results. Peak workload and VO2 max. were significantly lower in PS patients than in healthy controls. Conclusions: Longstanding mild PR in PS patients can lead to an enlarged RV, reduced RV function and reduced exercise capacity. Despite more PR and larger RV volumes in TOF patients, exercise capacity and biventricular function are similar in both patient groups. [Copyright &y& Elsevier]

Atrial arrhythmia (AA) is common in adult patients with congenital heart disease (CHD). To enable the prevention of AA or its complications, timely identification of adult patients with CHD at risk of AA is crucial. Long total atrial activation times have been related to AA. Tissue Doppler imaging (TDI) permits noninvasive evaluation of the total atrial conduction time (PA-TDI duration). The present study evaluated the association between the PA-TDI duration and the development of AA in adult patients with CHD. A total of 223 adult patients with CHD were followed up for the occurrence of AA after PA-TDI duration assessment. The PA-TDI duration was defined as the interval from the onset of the P wave on the electrocardiogram to the peak of the A′ wave at the lateral atrial wall on TDI tracings. Among the various clinical and echocardiographic parameters, the association between the PA-TDI duration and AA occurrence was investigated. The median follow-up was 39 months (interquartile range 21 to 57). A PA-TDI duration of ≥126 ms was associated with AA during follow-up (log-rank, p <0.001). On multivariate analysis, a PA-TDI duration >126 ms (hazard ratio 2.25, 95% confidence interval 1.21 to 4.19) and history of AA (hazard ratio 4.89, 95% confidence interval 2.75 to 8.71) were independently associated with the occurrence of AA. In conclusion, PA-TDI duration and a history of AA were independently associated with the occurrence of AA in adult patients with CHD. The PA-TDI duration is a useful tool to identify patients with CHD at risk of AA during follow-up. [ABSTRACT FROM AUTHOR]

Abstract: Background: HMG-coA-reductase-inhibitors (statins) have been proven to reduce atherosclerosis progression as observed by carotid intima-media thickness in patients with known coronary heart disease, independent of lipid lowering. Cardiovascular complications are common in patients after successful coarctation repair. The effect of statins on cardiovascular risk in adults after successful coarctation repair has not yet been established. Methods: We designed a multicentre, prospective, randomised, open label trial to evaluate the effect of the HMGcoA-reductase-inhibitor (Atorvastatin) on atherosclerotic progression in adult post-coarctectomy patients. The primary endpoint in this study is the carotid intima-media thickness as measured by Bmode ultrasonography of the carotid arteries. Conclusion: This large prospective, randomised, open label trial will establish the effect of HMG-coA-reductase inhibitors (Atorvastatin) on cardiovascular risk in adult patients after successful coarctation repair. [Copyright &y& Elsevier]

Chronic hypertension is a major concern in adults who have undergone resection of coarctation of the aorta (CoA) in childhood. In otherwise healthy subjects, exercise-induced hypertension is prognostic for chronic hypertension; however, the prognostic value in patients with CoA remains unknown. The aim of the present study was to evaluate the predictive value of exercise-induced hypertension for chronic hypertension in these patients. In the present prospective follow-up study, 74 patients with CoA (58% men, age 30.9 ± 9.5 years) underwent ambulatory blood pressure (BP) monitoring and exercise testing twice from 2001 to 2009 with a follow-up period of 6.3 ± 0.8 years. Hypertension was defined as a mean systolic BP ≥140 mm Hg and/or mean diastolic BP ≥90 mm Hg or the need for antihypertensive treatment. Exercise-induced hypertension was defined as a mean systolic BP of <140 mm Hg and peak exercise systolic BP of ≥200 mm Hg. At baseline, 27 patients (36%) were hypertensive, 11 (15%) had exercise-induced hypertension, and 36 (49%) were normotensive. At follow-up, all 27 hypertensive patients remained hypertensive. Of the 11 with exercise-induced hypertension, 7 (64%) had developed chronic hypertension, and 4 (36%) continued to have exercise-induced hypertension. Of the 36 normotensive patients, 7 (19%) had developed hypertension, 12 (33%) had developed exercise-induced hypertension, and 17 (47%) remained normotensive. On multivariate analysis, baseline maximum exercise systolic BP was independently associated with the mean systolic BP at follow-up (β = 0.13, p = 0.005). In conclusion, the maximum exercise systolic BP was a predictor for chronic hypertension in patients with CoA. These findings demonstrate the clinical importance of exercise-induced hypertension and warrant additional study into the long-term consequences of exercise-induced hypertension and the potential beneficial role of early antihypertensive treatment in adult patients after CoA repair with exercise-induced hypertension. [Copyright &y& Elsevier]

Abstract: Background: The Fontan circulation is a palliative procedure performed in patients with complex congenital heart disease (CHD), making transpulmonary blood flow dependent on the systemic venous pressure. In a Fontan circulation a low pulmonary vascular resistance (PVR) is crucial, as is epitomized by the observation that a high PVR is a strong predictor of mortality. Long-term follow-up has shown that PVR may rise many years after the Fontan procedure has been performed, possibly due to micro-emboli from a dilated right atrium or from the venous system. Other mechanisms of increased PVR might be aging, obstructed airways caused by lymphatic dysfunction, lack of pulsatile pulmonary flow causing a release of endothelium-derived vasoactive molecules, and prolonged overexpression of vasoconstrictors such as endothelin-1. Mean plasma level of endothelin-1 has been shown to be significantly higher in Fontan patients compared to healthy controls. In patients with pulmonary arterial hypertension (PAH), therapy with bosentan, an endothelin-1 receptor antagonist, has demonstrated to improve exercise capacity and to reduce the elevated PVR. In addition, reduction of PVR is shown early and late after the Fontan procedure on treatment with exogenous NO, another advanced PAH therapy. However, the long term effect of reducing the PVR by bosentan treatment on exercise capacity in Fontan patients is still unknown. Methods: We designed a prospective, multicenter, randomized open label trial to study the effect of bosentan in Fontan patients. The primary endpoint will be the change in maximum exercise capacity (peak V''O2). Conclusion: We hypothesize that treatment with bosentan, an endothelin-1 receptor antagonist, improves maximum exercise capacity and functional capacity in adult Fontan patients. [Copyright &y& Elsevier]

Objectives: The aim of this study was to analyze whether QRS duration, before and after pulmonary valve replacement (PVR), is related to long-term outcome in patients with tetralogy of Fallot (TOF). Background: Key factors that determine outcome after PVR in adult TOF patients are largely unknown. Recognition of such factors assists the identification of patients at increased risk of adverse events. Methods: Adults who previously underwent total correction for TOF (n = 90; age 31.4 ± 10.3 years) and required PVR for pulmonary regurgitation were included. The QRS duration was measured pre-operatively and 6 months after PVR. The post-operative changes in QRS duration were calculated. Adverse events (death, re-PVR, ventricular tachycardia, and symptomatic heart failure) were noted during follow-up. Results: During 5.5 ± 3.5 years of follow-up, 13 adverse events occurred. The 5-year event-free survival rate was 76% for patients with a pre-operative QRS duration >180 ms and 90% in patients with a QRS duration ≤180 ms (p = 0.037). For patients with a post-operative QRS duration >180 ms, 5-year event-free survival was 71%, whereas it was 91% for patients with a post-operative QRS duration ≤180 ms (p = 0.004). After multivariate correction, a post-operative QRS duration >180 ms (hazard ratio: 3.685, 95% confidence interval: 1.104 to 12.304, p < 0.05) and the absence of a reduction in QRS duration post-PVR (hazard ratio: 6.767, 95% confidence interval: 1.704 to 26.878, p < 0.01), was significantly associated with adverse outcome. Conclusions: Severe QRS prolongation, before or after PVR, and the absence of a reduction in QRS duration after PVR, are major determinants of adverse outcome during long-term follow-up of patients with TOF. [ABSTRACT FROM AUTHOR]

Heart rate (HR) at rest is an important marker of prognosis in heart failure, but has not been addressed in pulmonary arterial hypertension (PAH). To determine the prognostic value of HR at rest in patients with PAH, we retrospectively analyzed 140 consecutive patients with idiopathic PAH. Electrocardiogram (ECG)-derived HR at rest was evaluated as a potential predictor of adverse prognosis (death or lung transplantation), in addition to World Health Organization functional class, 6-minute walk distance, and hemodynamics before and approximately 1 year and 2 years after initiation of PAH treatment. During follow-up, 49 patients (35%) died, and 5 patients (4%) underwent lung transplantation. Before treatment initiation and after 1 year and 2 years of treatment, respectively, a higher HR at rest was an independent predictor of adverse prognosis (hazard ratios per 10-beats/min increase 1.76, 95% confidence interval 1.42 to 2.18, 2.31, 95% confidence interval 1.58 to 3.38, 2.1, 95% confidence interval 1.39 to 3.19, respectively, p <0.001 for all). Change in HR between the first and last ECG also independently predicted prognosis (hazard ratio per 1-beat/min increase 1.03, 95% confidence interval 1.01 to 1.06). In conclusion, a higher HR at rest and an important increase in HR at rest during follow-up signify a considerable risk of death in patients with PAH. ECG-derived HR at rest is an important marker of prognosis and should be assessed before and at frequent intervals after initiation of treatment for PAH. [Copyright &y& Elsevier]