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Background Heart failure (HF) is the leading cause of mortality and associated with significant morbidity in adults with congenital heart disease. We sought to assess the association between HF and patient‐report outcomes in adults with congenital heart disease. Methods and Results As part of the APPROACH‐IS (Assessment of Patterns of Patient‐Reported Outcomes in Adults with Congenital Heart disease—International Study), we collected data on HF status and patient‐reported outcomes in 3959 patients from 15 countries across 5 continents. Patient‐report outcomes were: perceived health status (12‐item Short Form Health Survey), quality of life (Linear Analogue Scale and Satisfaction with Life Scale), sense of coherence‐13, psychological distress (Hospital Anxiety and Depression Scale), and illness perception (Brief Illness Perception Questionnaire). In this sample, 137 (3.5%) had HF at the time of investigation, 298 (7.5%) had a history of HF, and 3524 (89.0%) had no current or past episode of HF. Patients with current or past HF were older and had a higher prevalence of complex congenital heart disease, arrhythmias, implantable cardioverter‐defibrillators, other clinical comorbidities, and mood disorders than those who never had HF. Patients with HF had worse physical functioning, mental functioning, quality of life, satisfaction with life, sense of coherence, depressive symptoms, and illness perception scores. Magnitudes of differences were large for physical functioning and illness perception and moderate for mental functioning, quality of life, and depressive symptoms. Conclusions HF in adults with congenital heart disease is associated with poorer patient‐reported outcomes, with large effect sizes for physical functioning and illness perception. Registration URL: https://clinicaltrials.gov ; Unique identifier: NCT02150603.

Aims Cardiovascular risk factors are used for risk stratification in primary prevention. We sought to determine if simple cardiac risk scores are associated with magnetic resonance imaging (MRI)-detected subclinical cerebrovascular disease including carotid wall volume (CWV), carotid intraplaque haemorrhage (IPH), and silent brain infarction (SBI). Methods and results A total of 7594 adults with no history of cardiovascular disease (CVD) underwent risk factor assessment and a non-contrast enhanced MRI of the carotid arteries and brain using a standardized protocol in a population-based cohort recruited between 2014 and 2018. The non-lab-based INTERHEART risk score (IHRS) was calculated in all participants; the Framingham Risk Score was calculated in a subset who provided blood samples (n = 3889). The association between these risk scores and MRI measures of CWV, carotid IPH, and SBI was determined. The mean age of the cohort was 58 (8.9) years, 55% were women. Each 5-point increase (∼1 SD) in the IHRS was associated with a 9 mm3 increase in CWV, adjusted for sex (P < 0.0001), a 23% increase in IPH [95% confidence interval (CI) 9–38%], and a 32% (95% CI 20–45%) increase in SBI. These associations were consistent for lacunar and non-lacunar brain infarction. The Framingham Risk Score was also significantly associated with CWV, IPH, and SBI. CWV was additive and independent to the risk scores in its association with IPH and SBI. Conclusion Simple cardiovascular risk scores are significantly associated with the presence of MRI-detected subclinical cerebrovascular disease, including CWV, IPH, and SBI in an adult population without known clinical CVD.

Background There is a paucity of data to guide decisions regarding thromboprophylaxis for atrial arrhythmias in congenital heart disease. Methods A retrospective multicenter cohort study enrolled patients with documented sustained atrial arrhythmias and congenital heart disease from 12 North American centers to quantify thromboembolic and bleeding rates associated with antiplatelet and anticoagulation therapy, and explore associated factors. A blinded committee adjudicated all qualifying arrhythmias and outcomes. Results A total of 482 patients, 45.2% female, age 32.0±18.0years, were followed for 11.3±9.4years since the qualifying arrhythmia. Antiplatelet therapy was administered to 37.8%, anticoagulation to 54.4%, and neither to 7.9%. Congenital heart disease complexity was simple, moderate, and severe in 18.5%, 34.4%, and 47.1%, respectively. Freedom from thromboembolic events was 84.7±2.7% at 15years, with no difference between anticoagulation versus antiplatelet therapy (P=0.97). Congenital heart disease complexity was independently associated with thromboembolic events, with rates of 0.00%, 0.93%, and 1.95%/year in those with simple, moderate, and severe forms (P 2 and CHA 2 DS 2 -VASc scores were not predictive of thromboembolic risk. Annualized bleeding rates with antiplatelet and anticoagulation therapy were 0.66% and 1.82% (P=0.039). In multivariable analyses, anticoagulation [hazard ratio (HR) 4.76, 95% CI (1.05–21.58), P=0.043] and HAS-BLED score [HR 3.15, 95% CI (1.02, 9.78), P=0.047] were independently associated with major bleeds. Conclusion Current management of atrial arrhythmias in congenital heart disease is associated with a modest rate of thromboembolic events, which is predicted by disease complexity but not CHADS 2 /CHA 2 DS 2 -VASc scores. HAS-BLED score is applicable to the congenital population in predicting major bleeds.

Background The tissue-blood partition coefficient (PC) of gadolinium, derived from T1 measurements, reflects myocardial connective tissue fraction and tissue injury, increasing in proportion with edema or fibrosis. We determined the myocardial PC of gadolinium in patients with acute myocarditis, chronic myocardial infarction (MI), and healthy volunteers. We hypothesized that the characteristics of the injured myocardium in patients with MI and myocarditis may differ and that the PC will be higher in chronically injured myocardium (MI) compared with acutely injured myocardium (myocarditis). Methods We performed late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) imaging and T1 mapping before and after administration of gadolinium (0.1 mmol/kg Gd-BOPTA) at 3 Tesla in 10 healthy volunteers (47.1 ± 12.4 years), 18 patients with chronic MI (62.5 ± 8.1 years), and 16 patients with acute myocarditis (42.5 ± 13.9 years). Results In patients with chronic MI and focal scar by LGE, the whole left ventricular myocardial PC (0.45 ± 0.05) was higher compared with patients with MI without focal scar (0.39 ± 0.03, P = 0.02) but not significantly different from whole myocardial PC in volunteers (0.40 ± 0.05) or patients with myocarditis (0.41 ± 0.05). The PC in myocarditis scars was lower than in chronic MI scars (0.60 ± 0.12 vs 0.77 ± 0.16, P = 0.016). The relationships of PC and scar burden, expressed as % LGE, were similar and significant for the 2 groups (P = 0.042). Conclusion The tissue-blood partition coefficient of Gd-BOPTA is elevated in areas of acute and chronic myocardial injury and may serve as a marker for disease activity and density of scars, which was found to be higher in chronic MI than in acute myocarditis.

Purpose A combined breathing maneuver of hyperventilation, followed by a long voluntary breathhold leads to coronary vasodilation. We investigated the impact of breathing maneuvers on MR first-pass cardiac perfusion imaging and its potential clinical utility. Materials and Methods We studied 24 healthy volunteers (37 ± 12 years; 62.5% men) on a clinical 3 Tesla MRI system and performed first-pass perfusion MR at rest, during a short breathhold (S-HVBH) following 60 s of hyperventilation, and at the end of a long breathhold (L-HVBH) following the hyperventilation, performed in random order. A blinded reader analyzed signal intensity upslope, upslope index, and time between 20 and 80% of maximal signal. Results All volunteers tolerated the breathing maneuvers well and completed the study protocol. The upslope of the signal-intensity-over-time curve was increased during S-HVBH (1.86 ± 0.70 units/s, P < 0.05) and at the end of L-LVBH (1.77 ± 0.82 units/s), when compared with baseline results (1.34 ± 0.58 units/s). Corrected for the arterial input, the upslope was higher at the end of the L-HVBH (0.095 ± 0.019 units/s versus 0.077 ± 0.016 units/s at rest, P < 0.01) as was the myocardial perfusion reserve index (1.25 ± 0.22 versus 1.09 ± 0.17; P < 0.001). In a multiple regression model, only gender, rate-pressure product, and breathhold time were independently and significantly related to the upslope (R = 0.771; P < 0.001). Conclusion In conclusion, a voluntary long breathhold after hyperventilation leads to an increase of the myocardial perfusion reserve index. This may impact findings from current practice of first-pass perfusion imaging. The clinical utility of breathing maneuvers as a vasodilatory stimulus for first-pass perfusion imaging may warrant further research. J. MAGN. RESON. IMAGING 2016;44:947–955.

Background: Safety and feasibility data on transvenous lead extraction (TLE) in the challenging population of adults with congenital heart disease (A-CHD) are limited. Herein, we report the results of TLE in A-CHD during a 20-year period. Methods and Results: All consecutive TLE procedures in A-CHD were included in a monocentric prospective registry from 1996. A total of 121 leads were extracted in 49 A-CHD (median age, 38 years; 51% men) during 71 TLE procedures. Twenty-four (49%) patients had transposition of the great arteries. Main indications for extraction were infection in 34 (48%) and lead failure in 22 (31%). A laser sheath was required for 56 (46%) leads and a femoral approach for 10 (8%). Complete TLE was achieved for 111 leads (92%). In multivariable analysis, lead duration (odds ratio, 1.02; 95% confidence interval, 1.00–1.04; P P P =0.03) and valvular vegetation (odds ratio, 7.29; 95% confidence interval, 1.32–40.51; P =0.02). After a median of 54 (19–134) months of follow-up after the first TLE, 3 deaths occurred independently from lead management. Conclusions: Despite complex anatomic issues, TLE can be achieved successfully in most A-CHD using advanced extraction techniques. Subpulmonary atrioventricular valve regurgitation is a prevalent complication, particularly in patients with transposition of the great arteries.

Background There is a paucity of data regarding late‐onset pulmonary hypertension ( PH ) in patients with transposition of the great arteries and atrial switch surgery. Methods and Results A retrospective cohort study was conducted on 140 adults with transposition of the great arteries and atrial switch surgery, age 37.3±7.8, 37.1% female, in order to assess the prevalence and characteristics of late‐onset PH and explore associated factors. Patients were followed for a median of 32.3 years after atrial switch surgery and 10.0 years after their first referral visit. PH was detected in 18 of 33 (54.5%) patients who had invasive hemodynamic studies. Average age at diagnosis of PH was 33.9±8.1 years. PH was postcapillary in all, with a mean pulmonary artery pressure of 36±12 mm Hg and mean pulmonary capillary wedge pressure of 28±8 mm Hg. PH was diagnosed in 13 of 17 (76.5%) patients who had cardiac catheterization for heart failure or decreased exercise tolerance. In multivariable analyses, systemic hypertension (odds ratio 9.4, 95% confidence interval 2.2‐39.4, P =0.002) and heart failure or New York Heart Association class III or IV symptoms (odds ratio 49.8, 95% confidence interval 8.6‐289.0, P PH . Patients with PH were more likely to develop cardiovascular comorbidities including atrial ( P =0.001) and ventricular ( P =0.008) arrhythmias, require hospitalizations for heart failure ( P P PH (hazard ratio 9.4, 95% confidence interval 2.1‐43.0], P Conclusions Late‐onset postcapillary PH is highly prevalent in adults with transposition of the great arteries and atrial switch surgery and is associated with an adverse prognosis.

The evaluation of the right ventricle (RV) is a challenge; as a result six transthoracic echocardiography (TTE) parameters have been suggested. While gated blood-pool single photon electron computed tomography (GBPS) is a promising technique, there is currently no completely automated and validated processing software available clinically. Consequently, cardiac magnetic resonance (CMR) imaging remains the gold standard for RV assessment. We aimed to compare RV evaluation by GBPS and TTE to CMR.Fifty-eight patients underwent CMR, GBPS and TTE for RV assessment, including volumes, RVEF and TTE's indices of RV function (fractional area change (FAC), RV myocardial performance index by pulsed wave Doppler (MPI-PWD) and tissue Doppler (MPI-TDI) and tricuspid annular plane systolic excursion (TAPSE) by M-Mode and tissue Doppler (TAPSE-TDI)). GBPS was performed using both a commercial (QBS) and the Montreal Heart Institute (MHI) proprietary software.Nuclear medicine derived volumes quantification showed very good correlations with CMR, for RV end-diastolic (r=0.84 and 0.77, all p0.001) and end-systolic (r=0.82 and 0.67, all p0.001) volumes by MHI and QBS software respectively. RVEF showed a significant correlation with CMR in patients with RVEF ≤ 45% (r=0.54, p=0.029 and r=0.55, p=0.028, by MHI and QBS respectively). Among TTE parameters, only FAC and MPI-TDI were significantly correlated with CMR-RVEF, mainly for RVEF ≤ 45% (r=0.63, p=0.011 and r=0.58, p=0.046).GBPS, both with MHI and QBS software, exhibited significant correlations with CMR for evaluation of the RV (volumes and decreased RVEF estimation). Among TTE's parameters, only FAC and MPI-TDI showed significant correlation with CMR with RVEF ≤ 45%.

Tetralogy of Fallot is the most common form of cyanotic congenital heart disease and accounts for 10% of all congenital heart defects. Corrective surgery has dramatically improved long-term prognosis, with nearly 90% of patients now surviving well into adulthood. Aortic root dilation is a well-recognized feature of unrepaired tetralogy of Fallot, and is increasingly reported in patients, years after corrective surgery. While the prevalence and severity of aortic root dilation and its associated complications remain to be defined, a clearer portrait is emerging from the growing body of literature. The objective of this contemporary review is to address the scope of the problem regarding aortic dilation in tetralogy of Fallot, explore potential pathophysiological mechanisms, summarize current knowledge regarding adverse events and discuss potential therapeutic options.

Background Shone complex consists of a constellation of left-sided, usually obstructive, cardiac lesions, including supravalvar mitral ring, parachute mitral valve, subaortic stenosis, and aortic coarctation. Incomplete Shone complex consists of a mitral valve anomaly associated with lesions involving the subaortic region, aortic valve, or thoracic aorta. There is a paucity of data regarding long-term outcomes in adults with Shone complex. Methods We reviewed records of adults with complete or incomplete Shone complex followed at the Montreal Heart Institute between 1982 and 2014. Results Among 4189 adults with congenital heart disease, 28 (0.67%) patients (mean age, 35 ± 11 years; 50% women) had complete or incomplete Shone complex and were followed for a median of 8 years. Only 39% were previously diagnosed as having Shone complex. The most common defects were congenital mitral stenosis (93%), aortic coarctation (75%), and bicuspid aortic valve (71%). Heart transplantation was required in 2 patients (7.1%) at age 22 and 28 years, respectively. Overall, 48% had cardiovascular hospitalizations during adulthood, predominantly for arrhythmias or heart failure. Freedom from cardiovascular intervention was 55%, 18%, and 8% at 10, 20, and 30 years of age, respectively. Although aortic coarctation was the most common indication for initial intervention (61%), adult interventions occurred predominantly for aortic valve/left ventricular outflow tract (60%) and mitral valve (33%) lesions. Conclusions Shone complex is an under-recognized entity associated with relatively low mortality in adulthood but substantial morbidity related to arrhythmias, heart failure, and interventions. Increased awareness of this condition and associated complications may allow for more tailored follow-up.

Background Sudden death of presumed arrhythmic etiology is the leading cause of mortality in adults with tetralogy of Fallot. Objectives To assess the impact of exercise on ventricular arrhythmias in adults with tetralogy of Fallot. Methods Adults with repaired tetralogy of Fallot were randomized in a 2:1 ratio to an open-label trial of standard care versus 12weeks of supervised combined aerobic/resistance training with continuous Holter monitoring. Proportion of premature ventricular complexes (PVC) and runs of non-sustained ventricular tachycardia (NSVT) were assessed by mixed and Poisson regression models with generalized estimating equations for repeated measures. Results A total of 152 Holters were performed in 17 patients, median age 35 (interquartile range [IQR] 28, 42) years, 65% male, 13 of whom were randomized to exercise training. Baseline characteristics were similar between groups. Exercise training resulted in significant increases in peak oxygen uptake (11±19%, p=0.028), metabolic equivalents (11±18%, p=0.027), and exercise duration (8±10%, p=0.009) compared to no changes in controls. Frequent (≥30 per hour) PVCs were present in 46% of patients, couplets in 62%, and 3 to 7 beat runs of NSVT in 31%. The median proportion of PVCs was 1.93‰ (IQR 0.41, 5.89) at baseline and 1.45‰ (IQR 0.08, 2.76) during the initial exercise session (p=0.722), and remained stable over time (s coefficient=−0.031, p=0.408). Runs of NSVT decreased significantly over time (s coefficient=−0.032, p=0.018). Conclusion In adults with repaired tetralogy of Fallot, exercise training is safe, improves exercise capacity, and appears to confer a beneficial effect on ventricular arrhythmias.

Prediction of adverse maternal and neonatal events in women with heart disease is not well established. We aimed to assess cardiac, obstetrical and neonatal complications in pregnant women with heart disease referred to our tertiary care center and validate a previously proposed risk index.We included 227 women with cardiac disease followed for 312 pregnancies at our tertiary center from 1992 to 2007. Cardiac risk was assessed using the previously proposed Cardiac Disease in Pregnancy (CARPREG) score and its association with maternal and neonatal outcomes was determined.Maternal cardiac lesions were predominantly congenital (81.4%). CARPREG risk was low (score=0) in 66.3% and intermediate (score=1) in 33.7% pregnancies. Maternal cardiac events complicated 7.4% pregnancies, with pulmonary edema occurring most frequently (3.8%). An intermediate score was associated with a higher rate of cardiac events (19.0% vs. 1.4%, odds ratio [OR] 15.6, 95% confidence interval (95%CI) 4.5-54.4, p0.0001). Adverse events occurred in 27.5% neonates. Preterm deliveries occurred in 16.7% pregnancies, more commonly in patients with intermediate scores (OR 2.4, 95%CI 1.2-4.6, p=0.01). The sensitivity and negative predictive values of a low score were respectively 87% and 99% for total cardiac events and both 100% for primary cardiac events including pulmonary edema and sustained arrhythmia.The CARPREG risk index has a high sensitivity and negative predictive value with regards to cardiac complications in pregnant women with heart disease. It may, therefore, be routinely used to improve the assessment of cardiac risk before and during pregnancy.

As a result of major achievements in pediatric cardiac care, a growing number of patients with congenital heart disease (CHD) are flourishing well into adulthood. This heterogeneous and aging population of patients, many of whom represent the first generation of middle-age survivors, faces unique issues and challenges. As a field, adult CHD has evolved markedly during the past decade on several fronts, including imaging, arrhythmia management, percutaneous interventions, surgical techniques, research, and multidisciplinary care that extends beyond the cardiac realm. This review highlights recent advances across the wide spectrum of key issues encountered by adults with CHD.

We evaluated the accuracy of planar radionuclide angiography and different count-based and space-based electrocardiogram (ECG)-gated blood-pool single-photon emission computed tomography (GBPS) algorithms for assessment of left ventricular end-diastolic volume (LVEDV), end-systolic volume (LVESV), and ejection fraction (LVEF) compared with the gold standard of cardiac magnetic resonance imaging (cMRI). The goal is to assess the accuracy of a recently developed GBPS algorithm.Subjects had planar, GBPS, and cMRI sequentially. Datasets were processed by QBS software (Cedar-Sinai) and by MHI software (Montreal Heart Institute). Space-based approaches were used to compute LVEDV, LVESV, and LVEF. Count-based techniques were also used to assess LVEF. All results were compared to cMRI. Fifty-five patients (85% male; mean age 63 +/- 9 years) completed the study. LVEFs and their correlations to cMRI values were 43 +/- 12% (r = .82), 39 +/- 14% (r = .82), and 39 +/- 13% for MHI(space), QBS(space), and cMRI methodologies, respectively. LVEF by count-based methods also demonstrated good correlation to LVEF provided by cMRI (42 +/- 13%, r = .88 for MHI(count) and 46 +/- 15%, r = .84 for QBS(count)). Strong correlations were obtained for LVEDV (r = .96 for MHI and r = .92 for QBS) and for LVESV (.97 for MHI and r = .94 for QBS).All Gated blood-pool SPECT algorithms had significant variation in estimating LVEF. Nevertheless our software provides good estimates of LV volumes and LVEF. Such software may, therefore, be applied to assess LV morphology and function.

Background— Pulmonary arterial hypertension (PAH) may develop in patients with atrial septal defects (ASD); however, little is known about associated risk factors and its evolution after transcatheter ASD closure. Methods and Results— We conducted a cohort study on 215 adults with attempted transcatheter ASD closure from 1999 to 2006. Patients were classified according to baseline systolic pulmonary artery pressures as having no (I, P P =0.0052), female sex (OR, 3.9; P =0.0313), and at least moderate tricuspid regurgitation (OR, 3.6; P =0.0043). At 15 (interquartile range, 8 to 43) months post–ASD closure, patients with higher baseline pressures were more likely to experience a ≥5-mm Hg decrease (33.7%, 73.9%, 79.2%, and 100.0% in groups I to IV, P P P P =0.0418) and no more than mild tricuspid regurgitation (OR, 0.14; P =0.0420). Conclusion— In adults with ASDs, severity of PAH is modulated by age, sex, defect size, and degree of tricuspid regurgitation. Patients with moderate or severe PAH may benefit from substantial reductions in pulmonary artery pressures after transcatheter ASD closure, although the PAH values remain elevated in a sizeable proportion.

Tetralogy of Fallot is the most common form of congenital heart disease in implantable cardioverter-defibrillator (ICD) recipients. Indeed, sudden death of presumed arrhythmic etiology is the most frequent mode of demise in patients with surgically repaired tetralogy of Fallot, often in early-to-mid adulthood. Nevertheless, the overall annual incidence of sudden death is actually low. Therein lies the major challenge of risk stratification. This review highlights some of the complexities involved in risk stratifying patients with tetralogy of Fallot, underscoring the importance of avoiding oversimplification by means of a rigid algorithm that dictates therapy. To complement sound clinical judgment, a quantitative probabilistic approach is presented, which considers the body of literature from primary prevention ICD trials, risk factors identified by observational studies, the value of programmed ventricular stimulation and insights gleaned from studying ICD recipients with tetralogy of Fallot.

A combined breathing maneuver of hyperventilation, followed by a long voluntary breathhold leads to coronary vasodilation. We investigated the impact of breathing maneuvers on MR first-pass cardiac perfusion imaging and its potential clinical utility.We studied 24 healthy volunteers (37 ± 12 years; 62.5% men) on a clinical 3 Tesla MRI system and performed first-pass perfusion MR at rest, during a short breathhold (S-HVBH) following 60 s of hyperventilation, and at the end of a long breathhold (L-HVBH) following the hyperventilation, performed in random order. A blinded reader analyzed signal intensity upslope, upslope index, and time between 20 and 80% of maximal signal.All volunteers tolerated the breathing maneuvers well and completed the study protocol. The upslope of the signal-intensity-over-time curve was increased during S-HVBH (1.86 ± 0.70 units/s, P0.05) and at the end of L-LVBH (1.77 ± 0.82 units/s), when compared with baseline results (1.34 ± 0.58 units/s). Corrected for the arterial input, the upslope was higher at the end of the L-HVBH (0.095 ± 0.019 units/s versus 0.077 ± 0.016 units/s at rest, P0.01) as was the myocardial perfusion reserve index (1.25 ± 0.22 versus 1.09 ± 0.17; P0.001). In a multiple regression model, only gender, rate-pressure product, and breathhold time were independently and significantly related to the upslope (R = 0.771; P0.001).In conclusion, a voluntary long breathhold after hyperventilation leads to an increase of the myocardial perfusion reserve index. This may impact findings from current practice of first-pass perfusion imaging. The clinical utility of breathing maneuvers as a vasodilatory stimulus for first-pass perfusion imaging may warrant further research. J. MAGN. RESON. IMAGING 2016;44:947-955.

Exercise capacity in adults with various forms of congenital heart disease is substantially lower than that of the general population. Although the underlying congenital heart defect, and its sequelae, certainly contribute to observed exercise limitations, there is evidence suggesting that deconditioning and a sedentary lifestyle are important implicated factors. The prevalence of acquired cardiovascular comorbidities is on the increase in the aging population with congenital heart disease, such that obesity and a sedentary lifestyle confer increased risk. Health fears and misconceptions are common barriers to regular physical activity in adults with congenital heart disease, despite evidence linking lower functional capacity to poor outcomes, and data supporting the safety and efficacy of exercise in bestowing numerous physical and psychosocial rewards. With few exceptions, adults with congenital heart disease should be counselled to exercise regularly. In this contemporary review, we provide a practical approach to assessing adults with congenital heart disease before exercise training. We examine available evidence supporting the safety and benefits of exercise training. Risks associated with exercise training in adults with congenital heart disease are discussed, particularly with regard to sudden cardiac death. Finally, recommendations for exercise training are provided, with consideration for the type of congenital heart disease, the nature (ie, static vs dynamic) and intensity (ie, low, medium, high) of the physical activity, and associated factors such as systemic ventricular dysfunction and residual defects. Further research is required to determine optimal exercise regimens and to identify effective strategies to implement exercise training as a key determinant of healthy living.

Aortic imaging is an essential part of a surveillance program for patients with a confirmed or suspected aortopathy because aortic size is crucial for predicting the risk of death, aortic rupture, or aortic dissection. Noninvasive aortic imaging relies on transthoracic and transesophageal echocardiography, cardiovascular magnetic resonance and computed tomography (CT) imaging. Echocardiography and cardiovascular magnetic resonance offer comprehensive anatomical and functional evaluation of the heart, aortic valve, and aorta, and CT is more limited to anatomical data. However, CT is fast, available, and less operator-dependent. There is general consensus that the aorta should be measured at reproducible anatomical landmarks on electrocardiogram-gated images, perpendicular to the blood flow and using multiplanar reconstruction if possible. The method of measurement must be included in the clinical report. Normal aortic size depends on age, sex, and body size. Serial measurements should use identical methods. There is controversy about the inclusion of the aortic wall in the vessel diameter and the trigger time in the cardiac cycle for measurement, although diastole is more reproducible. The best method to measure the diameter of the sinuses of Valsalva remains unclear. Imagers and clinicians should pay close attention to the aorta measurement techniques and weigh the clinical implications of modification of their institutional protocols.

Medical and surgical breakthroughs in the care of children born with heart defects have generated a growing population of adult survivors and spawned a new subspecialty of cardiology: adult congenital heart disease. The prevalence of adult congenital heart disease is escalating at a rampant rate, outpacing the relatively static prevalence of pediatric congenital heart disease, because adults now surpass children in numbers by a ratio of 2:1. As such, congenital heart disease can no longer be considered primarily a pediatric specialty. Most congenital heart defects are not curable and require lifelong specialized care. Health care systems worldwide are challenged to meet the unique needs of this increasingly complex patient population, including the development of supraregional centres of excellence to provide comprehensive and multidisciplinary specialized care. In this review, we explore the incidence and prevalence of congenital heart disease and their changing patterns, address organization and delivery of care, highlight the importance of appropriate training and dedicated research, summarize the high burden of health care resource utilization, and provide an overview of common issues encountered in adults with congenital heart disease.

Background Aortic coarctation has been associated with generalized vascular disease, yet little is known about retinal vascular patterns and their changes over time. Objectives The aim of this study is to characterize the nature and extent of retinal vascular disease in adults with aortic coarctation, and explore age-related effects and associations with cardiovascular outcomes. Methods A prospective cross-sectional seroepidemiological study was conducted on 60 consecutive adults with repaired aortic coarctation, age 42.4±14.1years, 61.7% male. In addition to detailed questionnaires, imaging studies, and laboratory testing, high-quality retinal images were acquired by 45° nonmydriatic digital funduscopy. Results No patient had evidence of hypertensive retinopathy. A distinctive vascular pattern characterized by bilaterally symmetric tortuosity of retinal arteries and veins was observed. Arterial tortuosity was abnormal in 98.3% of patients and decreased with age ( P =0.0005). In patients ≥45years, a 1-point increase in the arterial tortuosity score was associated with a 1.5-fold higher risk of cardiovascular complications (i.e., acute coronary syndrome, stroke, cerebral aneurysm, aortic dissection/rupture) [odds ratio 1.50, 95% CI (1.01, 2.24), P =0.0496]. Abnormal venous tortuosity was present in 75.0% of patients and non-significantly correlated with higher levels of serum inflammatory markers (C-reactive protein, fibrinogen, interleukin-6, and tumor necrosis factor-alpha). A higher venous tortuosity score was likewise associated with an increased risk of cardiovascular complications [odds ratio 1.86, 95% CI (1.03, 3.35), P =0.0392]. Conclusions Adults with repaired aortic coarctation exhibit a unique retinal vascular pattern characterized by excessive arterial and venous tortuosity that regresses with age. Greater tortuosity is associated with adverse cardiovascular outcomes in patients ≥45years.

Aims To describe the initial experience with the GORE® septal occluder (GSO), a new septal occluder for the treatment of atrial septal defects (ASD) and patent foramen ovale (PFO). Methods and results This was a prospective single-centre review of patients undergoing percutaneous closure for a PFO or ASD with the GSO. A clinical evaluation and follow-up echocardiography were performed at three months with transoesophageal echocardiography (TEE) in case of PFO and transthoracic echocardiography (TTE) in ASDs. Between July 2011 and February 2012, thirty-eight patients underwent PFO (n=29) or ASD (n=9) closure with the GSO using TEE (n=36, 94.7%) or intracardiac echocardiography (n=2, 5.3%) guidance. In PFOs, three-month TEE was available in 24 patients and showed no residual shunt in 18 (75%), bubble shunt in two (8.3%) and bubble shunt after Valsalva in four (16.7%). In ASDs, three-month TTE showed no shunt in eight patients (88.8%) and residual shunt in one patient (11.2%). There was no device embolisation, air embolism, procedure-related stroke or pericardial effusion. No neurological events occurred during the follow-up period. Conclusions This initial experience with the new GSO device has demonstrated acceptable safety with no procedural complications and acceptable efficacy with low rates of residual shunting at three-month follow-up.

Symptoms consistent with myocarditis are a frequent cause of medical visits, especially in young and middleaged patients. Moreover, myocarditis was found to be the most frequent disease in patients with acute coronary syndrome yet normal coronary arteries. 1 Although many causes have been identified, acute cases are mostly because of myocardial involvement in systemic viral disease. 2,3 During the first days of viral myocarditis, there is direct cardiomyocyte injury, accompanied by edema, necrosis, and, depending on its spatial extent, regional, or even global contractile dysfunction. The tissue is typically cleared from the virus within 5 days; yet, reactive inflammation (clean-up) may last for several weeks. In uncomplicated disease, there is full tissue and functional recovery within 3 to 4 weeks, whereas more severe disease necrosis results in myocardial scarring. Prolonged autoimmune response or virus persistence may lead to chronic inflammation and is considered a frequent cause of dilated cardiomyopathy. 3 Symptoms are not specific; patients may present with chest pain, fatigue, dyspnea, or arrhythmia. ECG findings may include AV block, ventricular or supraventricular arrhythmia, and ST changes, including severe elevation mimicking acute myocardial infarction. Except for more severe cases, echocardiography typically shows normal systolic wall motion or just mild regional dysfunction. Serological markers for cardiomyocyte injury, such as troponin, may be normal. Because of the nonspecificity of its symptoms, signs and test findings, myocarditis is often diagnosed by exclusion of other cardiac diseases. The specific identification of an active nonischemic inflammatory process, therefore, is a clinical challenge, especially in patients presenting with acute chest pain and heart failure. Invasive endomyocardial biopsy is only recommended in patients with evidence for heart failure in combination with acute disease (

Designing a manufacturing system or, more generally, modelling an existing system in order to make it evolve to provide better performance leads to the use of an integrated methodology. This methodology enables one to understand the system studied for several points of view in a consistent way. This paper presents GIM (GRAI Integrated Methodology) which has been developed within the framework of several ESPRIT projects based on the research work developed by the GRAI research group. This integrated methodology proposes a conceptual model, several modelling formalisms and a structured approach supporting the project. The main part of this paper aims at showing a real application of GIM, the purpose of which was to re-engineer an assembly shop.

Many adults with repaired tetralogy of Fallot have had prior Blalock-Taussig shunts. These shunts may theoretically hinder growth and development of the ipsilateral arm.We prospectively enrolled consecutive patients with tetralogy of Fallot in a cross-sectional study to measure arm length and assess handgrip strength. Bilateral handgrip strength was quantified by a dynamometer in a standing position after instructing patients to clench each hand tightly in succession. The maximum force achieved, in kilograms, was measured.A total of 80 consecutive adults with tetralogy of Fallot, aged 36.0 ± 12.5 years, 49% female, were prospectively enrolled. Thirty-eight (47.5%) patients had prior Blalock-Taussig shunts at a median age of 1.0 year. Twenty-one (55.3%) were left-sided and 23 (60.5%) were classic shunts. All but six patients with right-sided shunts and one without a prior shunt were right-handed. The shunts were present for a median of 4.0 years prior to takedown during corrective surgery. The arm ipsilateral to the shunt was significantly shorter than the contralateral arm (71.5 ± 6.1 versus 73.6 ± 5.6 cm, P0.0001). Handgrip strength was significantly weaker on the ipsilateral versus contralateral side (median [IQR], 26.5 [14.0-41.5] versus 31.0 [18.0-46.0] kg, P0.0001) and the ipsilateral-to-contralateral handgrip ratio was lower with classic versus modified shunts (median [IQR], 1.05 [1.02-1.14] versus 1.19 [1.07-1.33] kg, P=0.0541).In patients with tetralogy of Fallot, Blalock-Taussig shunts may impair normal development of the ipsilateral arm with repercussions in adulthood that include shorter limb length and reduced handgrip strength. These changes are most pronounced in patients with classic end-to-side anastomoses.

The purpose of this paper is to review the usefulness of multiplanar transesophageal echocardiography before, during and after percutaneous transcatheter closure of secundum atrial septal defects. Transesophageal echocardiography imaging techniques,including their role in patient selection, procedural guidance and immediate assessment of technical success and complications are described and discussed in this review. Percutaneous transcatheter closure is indicated for ostium secundum atrial septal defects of less than 40 mm in maximal diameter. The defect must have a favorable anatomy, with adequate rims of at least 5 mm to anchor the prosthesis. Transesophageal echocardiography plays a critical role before the procedure in identifying potential candidates for percutaneous closure and to exclude those with unfavorable anatomy or associated lesions, which could not be addressed percutaneously. Transesophageal echocardiography is also important during the procedure to guide the deployment of the device. After device deployment, the echocardiographer must assess the device (integrity, position and stability), residual shunt, atrio-ventricular valve regurgitation, obstruction to systemic or venous return and pericardial effusion, in order to determine procedural success and diagnose immediate complications.

Advances in the care of patients with a univentricular heart have enabled the majority to survive well into adulthood. Consequently, an increasing number of women with Fontan palliation are contemplating pregnancy. This review summarizes physiologic modifications related to pregnancy in the setting of single-ventricle physiology, discusses potential maternal cardiovascular concerns and describes commonly encountered obstetrical and neonatal complications. Management issues are addressed, including counseling, anesthetic considerations and the need for multidisciplinary specialized care.

Echocardiography has been and remains an invaluable tool for cardiac evaluation, including characterisation of cardiac masses. Even if most cardiac masses are considered benign, significant morbidity related to obstruction, infiltration, thromboembolism, arrhythmias and even death may occur. Therefore, a rapid and precise diagnosis is mandatory. Echocardiography enables key questions regarding the mass to be answered, such as: location, size, mobility, haemodynamic consequences and differentiation with extra-cardiac disease, embryologic remnants or artefacts. Recent advances in computed tomography (CT) and cardiac magnetic resonance (CMR) may help in the further characterisation of cardiac masses. This article presents challenging cases encountered at the Montreal Heart Institute between 2005 and 2008; we discuss the differential diagnosis of each mass and the current role of echocardiography, cardiac CT, and cardiac MRI (table 1). View this table: Table 1 Evaluation of seven cardiac masses through transthoracic echocardiography (TTE), transoesophageal echocardiography (TOE), cardiac magnetic resonance imaging (CMR), and computed tomography (CT) scan A middle age person underwent an uneventful left atrial myxoma resection many years ago, without any residual mass on follow-up transthoracic echocardiography (TTE) performed 1 year after. A few months later' however, she complained of non-specific symptoms of fatigue, shortness of breath, and atypical chest pain. Symptoms such as fever, weight loss or neurological problems were denied. Physical examination, ECG and chest x-ray were normal. Repeat TTE (figure 1, video 1) depicted a sessile bi-lobar, non-obstructive and immobile mass in the left atrium, with suspected right atrium extension. A transoesophageal echocardiogram (TOE) confirmed the 11×9 mm slightly mobile mass attached posteriorly to the interatrial septum, on the left atrial side. A recurrent myxoma was suspected, but neither a thrombus nor a sarcoma could be excluded, given the rapid growth of this mass. CMR further delineated this left atrial irregular mass, originating from the interatrial septum, hypointense on T1 weighted and hyperintense …

We present a case of digoxin toxicity in the context of dehydration, renal dysfunction and concomitant use of the macrolide antibiotic, clarithromycin, which is known to inhibit P-glycoprotein-mediated efflux mechanisms of digoxin. A focused review of the literature is presented. Health care providers need to be vigilant of this mechanism of drug-drug interaction, which is also relevant to other commonly used cardiovascular drugs.

ObjectivesThis study sought to describe and compare a novel fluoroscopic method and a 2-dimensional transesophageal echocardiographic (TEE) method to localize mitral periprosthetic leaks (PPLs) for transcatheter reduction.BackgroundTranscatheter reduction of significant regurgitation represents a modern and attractive alternative to surgery for the treatment of mitral PPL in high-risk patients. Accurate localization and precise communication between the echocardiographer and the interventional cardiologist are essential for procedural success.MethodsWe analyzed TEE and fluoroscopic studies of patients with mitral PPL who underwent multiplane 2-dimensional TEE–guided transcatheter reduction in our institution. Periprosthetic leaks were routinely localized using the “surgeon's-view” time-clock method during periprocedural TEE assessments. The 2-dimensional TEE examinations were later retrospectively reviewed by an echocardiographer blinded to procedural TEE findings. A corresponding surgeon's-view time-clock method was plotted for fluoroscopic PPL localization. Using this fluoroscopic method, offline fluoroscopic images were reviewed by an independent interventional cardiologist blinded to TEE results. Agreement between methods was evaluated.ResultsComplete imaging data were available for analysis in 20 patients who, between 2002 and 2009, underwent transcatheter reduction in which the defect was successfully crossed. There was excellent agreement between procedural TEE and retrospective TEE review for PPL localization (100%; p < 0.0001) and between fluoroscopic and procedural TEE localization (90%; 95% confidence interval [CI]: 77% to 100%; p = 0.0003). In the 2 cases where there was disagreement, fluoroscopic PPL localization was adjacent to TEE localization.ConclusionsThe surgeon's-view time-clock method of localizing PPL using 2-dimensional TEE is highly reproducible and allows fluoroscopic localization using the same reference system with very good agreement.

As a major success story of an early surgical era, corrective surgery transformed the prognosis of patients born with tetralogy of Fallot. With lifelong care, healthy survival well into adulthood is now the norm, albeit somewhat blemished by long-term sequelae. Arrhythmias are an important source of morbidity and a leading cause of mortality. This succinct review provides a contemporary summary of the prevalence and types of arrhythmias encountered in tetralogy of Fallot, including conduction disorders, atrial and ventricular tachyarrhythmias, and sudden death. Current prognostic markers and existing diagnostic tools are discussed, and available therapeutic options are elaborated.

It has been proposed that dyssynchrony assessment before cardiac resynchronization therapy (CRT) implantation could help predict response to CRT. It is known that up to 40% of patients who receive a CRT device for established indications do not respond to CRT. Great expectations came from the Predictors of Response to Cardiac Resynchronization Therapy (PROSPECT) study, which would finally identify the ultimate echocardiographic dyssynchrony criteria to help select responders. The recently published PROSPECT trial failed to identify an ideal parameter of dyssynchrony. Patient selection for CRT should involve a multimodal approach, and new promising tools are being investigated in that view. The present review integrated new data coming from the exciting field of imaging with currently available evidence to generate a stepwise approach to patient selection.

This paper gives an overview on the concepts, models, methods and commercial software packages for production management systems (PMS). We start by presenting the concepts and explaining what is production management, its position and role in CIM, and the structure of PMS. Then we will talk about some concepts relating to MRP, kanban and JIT. The economic aspects and performance indicators of PMS are not ignored. Next we will present the state-of-the-art in the field of PMS models ( cam-i, icam, nbs, mmcs, pac , etc.), as well as simulation models. Then we give a state-of-the-art on PMS design methods, in particular the sadt, ssad, merise, grai, ecograi and gim methods. We also present what we consider the three most promising approaches for the PMS design in the future: CIM-OSA, impacs and FOF, which are currently developed within the European ESPRIT programme. Finally a brief summary on the packages used in France for production management is given at the end of the paper.

A currently asymptomatic 31-year-old man was referred for recent atypical short stabbing chest discomfort occurring at rest. One month earlier, he had complained of a prolonged chest pain episode lasting ≈1 day, temporally related to the use of inhaled cocaine, for which he did not seek medical consultation. He had smoked half a pack of cigarettes a day for 15 years and suffered 2 remote episodes of blunt chest trauma. The first occurred at 12 years of age when he was in a low-velocity automobile accident as a …

Coronary artery aneurysms, stenoses, and thromboses are significant complications of Kawasaki disease (KD). While appearing in childhood, coronary complications are often left unrecognized until early and mid-adulthood. Along with the increasing capacity of noninvasive coronary artery imaging modalities, especially computed tomography and magnetic resonance, radiologists are more likely to face the diagnosis of KD in adults. This article will review the clinical aspects of KD for radiologists and will compare coronary imaging modalities in the diagnosis of KD.