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Many patients (20%-30%) experience suboptimal weight loss (WL) after bariatric surgery (BS), and unrealistic preoperative WL expectations may be a contributing factor. This study aimed to describe the nature of patients' general expectations of BS during the pre-surgical period, and how patients determined whether their expectations and WL goals (WLGs) were realistic. The extent to which patients' expectations and WLGs were met and/or changed during the post-surgical period was also assessed. Semi-structured interviews were conducted with 15 preoperative patients recruited approximately 6-months before surgery. Focus groups were also conducted with 14 post-operative patients recruited approximately 6-months after surgery. Interviews and focus groups were audio-recorded, transcribed verbatim and analysed using qualitative content analysis. Preoperative patients reported expectations that BS would positively impact physical and psychological health, social relationships, as well as quality of care. Preoperative patients perceived that they and their health care professionals had unrealistically high expectations of WL. Post-operative patients reported being generally satisfied with the outcomes of surgery, even though many did not reach their expected WL. Finally, most post-operative patients reported changing their expectations from pre- to post-surgery. This study provides data that may help inform the development of preoperative interventions focusing on helping patients set realistic expectations for WL and related outcomes, which could better prepare patients for the challenges they will face after surgery., (© 2023 The Authors. Clinical Obesity published by John Wiley & Sons Ltd on behalf of World Obesity Federation.)

Metabolic and bariatric surgery (MBS) yields unprecedented clinical outcomes, though variability is high in weight change and health benefits. Behavioral weight management (BWM) interventions may optimize MBS outcomes. However, there is a lack of an evidence base to inform their use in practice, particularly regarding optimal delivery timing. This paper evaluated the efficacy of BWM conducted pre- versus post- versus pre- and post-MBS. The review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement and included pre- and/or post-operative BWM interventions in adults reporting anthropometric and/or body composition data. Thirty-six studies (2,919 participants) were included. Post-operative BWM yielded greater decreases in weight (standardized mean difference [SMD] = -0.41; 95% confidence interval [CI]: -0.766 to -0.049, p < 0.05; I 2 = 93.5%) and body mass index (SMD = -0.60; 95% CI: -0.913 to -0.289, p < 0.001; I 2 = 87.8%) relative to comparators. There was no effect of BWM delivered pre- or joint pre- and post-operatively. The risk of selection and performance bias was generally high. Delivering BWM after MBS appears to confer the most benefits on weight, though there was high variability in study characteristics and risk of bias across trials. This provides insight into the type of support that should be considered post-operatively., (© 2021 World Obesity Federation.)

Background: Echocardiographic evaluation of the systemic right ventricle (sRV) remains challenging in patients with transposition of the great arteries (TGA) corrected by an atrial switch (AS) and with congenitally corrected TGA (ccTGA). The aim of this study was to determine the interobserver and intraobserver variability of echocardiographic parameters for sRV size and systolic function., Methods: Six independent observers retrospectively interpreted 44 previously acquired echocardiograms (25 patients with TGA/AS and 19 patients with ccTGA). Quantitative parameters included inlet and longitudinal diameters, systolic and diastolic areas, fractional area change (FAC), and wall thickness. sRV dilatation and systolic function were qualitatively graded as normal, mild, moderate, or severe. sRV hypertrophy was graded as present or absent. Intraclass correlation coefficients (ICCs) and Kappa statistics were computed to assess interobserver variability. Images from 10 patients (5 TGA/AS and 5 ccTGA) were reinterpreted at a 1-month interval, and ICC and Kendall tau b statistics were computed to assess intraobserver variability., Results: Interobserver and intraobserver agreement were good to excellent for sRV diameters, areas and FAC (ICC, 0.49-0.97), except for the sRV wall thickness (ICC < 0) and the FAC for 1 observer. Interobserver agreement was poor for the qualitative assessment of sRV size and systolic function (Kappa < 0.25), but with a good to excellent intraobserver agreement., Conclusions: These findings suggest that overall appreciation of sRV size and systolic function relies on variable interpretation of measurements by observers. Readers experienced in CHD and with clear thresholds for quantitative parameters, along with a validated algorithm, are required to guide the evaluation of sRV., (Copyright © 2018 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

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 <0.001) were independently associated with 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 <0.001), and undergo tricuspid valve surgery ( P <0.001). Mortality was significantly higher in patients with PH (hazard ratio 9.4, 95% confidence interval 2.1-43.0], P <0.001)., 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., (© 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.)

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., (Copyright © 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

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 (ß coefficient=-0.031, p=0.408). Runs of NSVT decreased significantly over time (ß 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., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

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., (Copyright © 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

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., (Copyright © 2014 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Background: The aim of this study was to assess pregnancy-related cardiac, maternal, and fetal outcomes in women who underwent aortic valve replacement (AVR)., Methods: From 1978-2011, 67 women < 40 years of age underwent 74 isolated AVRs (52 mechanical prostheses and 22 bioprostheses). All patients were prospectively followed at our dedicated valve clinic. Patients with Turner syndrome, previous hysterectomy, or tubal ligation were excluded. Cardiovascular, obstetric, and fetal outcomes were gathered from medical records and telephone interviews., Results: A total of 27 pregnancies were reported in 14 patients (bioprosthetic AVR, n = 20; mechanical AVR, n = 7). In the bioprosthetic AVR group, the following adverse events occurred: hospitalizations for syncope (n = 2), prosthetic valve deterioration after pregnancy necessitating reintervention 6 months postpartum (n = 1), miscarriages (n = 9), and preterm birth (n = 1). In the mechanical AVR group, the following adverse events occurred: embolic myocardial infarctions with a decrease in systolic function (n = 2; 1 pregnancy was terminated and 1 was completed), miscarriage (n = 1), postpartum bleeding (n = 1), urgent cesarean section for placental abruption (n = 1), and preterm birth (n = 1)., Conclusions: Findings from this study suggest that pregnancies in women with mechanical AVRs are associated with a higher risk of cardiac and obstetric adverse events. Thus, from this limited cohort, it appears that pregnancies in women with bioprostheses are safer than those in patients with mechanical AVRs., (Copyright © 2014 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

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±14.1 years, 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≥45 years, 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≥45 years., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Background: 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., Methods: 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., Results: 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, P<0.0001). 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, P<0.0001) 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)., Conclusion: 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., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

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.

Fontan surgery represents a milestone in the evolution of congenital heart disease management. It achieved the seemingly improbable (ie, restoration of a noncyanotic state by entirely bypassing the subpulmonary ventricle). In so doing, it has allowed a generation of children who may have otherwise succumbed to their severe congenital heart defect to survive to adulthood. Perfect univentricular physiology is, however, an elusive goal. The Fontan circulation inherently represents a hemodynamic compromise that results in a catalog of potential multiorgan complications. In this review, we explore current knowledge regarding pathophysiology of the failing Fontan, its varied clinical manifestations, and potential therapeutic options. Failure of the Fontan circulation is broadly divided into 3 overlapping categories: ventricular dysfunction, systemic complications of Fontan physiology, and chronic Fontan failure. As long as the Fontan operation continues to serve as the paradigm of care for patients with univentricular hearts, efforts must be directed toward supporting this dynamic circulation that progressively declines in efficiency with age. Continued research in therapies is needed for univentricular dysfunction and systemic complications of Fontan palliation, including potential uses of mechanical support as a bridge to transplantation or as a neosubpulmonary ventricle. Fontan patients remain a major challenge to the medical and surgical community as a whole. Multicentre and multidisciplinary efforts to improve the density and depth of experiences might lead to a better appreciation for, and management of, Fontan failure and its ramifications., (Copyright © 2013 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

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: There are more adults than children with congenital heart disease. Of over 96,000 ACHD patients in Canada, approximately 50% require ongoing expert care. In spite of published recommendations, data on the quality of care for ACHD patients are lacking., Methods: Survey methodology targeted all Canadian Adult Congenital Heart (CACH) network affiliated ACHD centers. Clinics were asked to prospectively collect outpatient and procedural volumes for 2007. In 2008, centers were surveyed regarding infrastructure, staffing, patient volumes and waiting times., Results: All 15 CACH network registered centers responded. The total number of patients followed in ACHD clinics was 21,879 (median per clinic=1132 (IQR: 585, 1816)). Of the total 80 adult and pediatric cardiologists affiliated to an ACHD clinic, only 27% had received formal ACHD training. Waiting times for non-urgent consultations were 4 ± 2 months, and 4 ± 3 months for percutaneous and surgical procedures. These were beyond Canadian recommended targets at 11 sites (73%) for non-urgent consultations, at 8 sites (53%) for percutaneous interventions and 13 sites (87%) for surgery., Conclusions: Of a minimum number of 96,000 ACHD patients in Canada, only 21,879 were being regularly followed in 2007. At most sites waiting times for ACHD services were beyond Canadian recommended targets. In spite of universal health care access, published guidelines for ACHD patient structure and process measures of health care quality are not being met., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

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.

Background: 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., Methods: 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., Results: 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, p<0.0001). 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., Conclusion: 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., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

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.

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.

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, <40 mm Hg), mild (II, 40 to 49 mm Hg), moderate (III, 50 to 59 mm Hg), or severe (IV, >or=60 mm Hg) PAH. Independent predictors of moderate or severe PAH were older age (odds ratio [OR], 1.10 per year; P<0.0001), larger ASD (OR, 1.13 per millimeter; 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 >or=5-mm Hg decrease (33.7%, 73.9%, 79.2%, and 100.0% in groups I to IV, P<0.0001), with a larger magnitude of reduction (0, 8, 17, and 22 mm Hg; P<0.0001). However, normalization of pressures (<40 mm Hg) occurred less frequently in patients with more advanced PAH (90.2%, 71.7%, 66.7%, and 23.5%, P<0.0001). Among patients with moderate or severe PAH, independent predictors of normalization were lower baseline pressures (OR, 0.91 per mm Hg; P=0.0418) and no more than mild tricuspid regurgitation (OR, 0.14; P=0.0420)., Conclusions: 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.

Cardiovascular magnetic resonance (CMR) imaging plays an essential role in the evaluation and follow-up of adult congenital heart disease (ACHD), providing safe, high-resolution imaging of some of the most complex anatomies encountered. Unlimited by acoustic windows and capable of tissue characterization, CMR is devoid of ionizing radiation and provides superior three-dimensional spatial resolution to transthoracic echocardiography and superior temporal resolution to computed tomography, making it the gold standard for various cardiac and great vessel imaging indications in ACHD. In this state-of-the art review, we provide an overview of CMR examination methods and detail the various approaches and classical findings in the more common forms of ACHD. Although this review touches upon technical aspects of CMR imaging in ACHD, it is primarily geared toward the adult congenital caregiver (i.e., clinical, interventional, or surgical), highlighting relevant practical considerations. To enhance the clinical utility of this review, numerous examples with intraoperative correlates are provided to highlight our imaging approaches for various defects. As CMR image acquisition may be time consuming and requires patient collaboration (e.g., intermittent breath holding), a systemic approach is required to maximize efficiency. A thorough knowledge of ACHD anatomy and natural history is essential in maximizing image interpretation. Proficient scanning is further enabled by clearly outlined study objectives with prior documentation of interventional and surgical procedures, where applicable.

Background: Transposition of the great arteries with intra-atrial baffle repair is among the congenital heart defects at highest risk of sudden death. Little is known about mechanisms of sudden death and the role of implantable cardioverter defibrillators., Methods and Results: We conducted a multicenter cohort study in patients with transposition of the great arteries to determine actuarial rates of implantable cardioverter defibrillator shocks, identify risk factors, assess underlying arrhythmias, and characterize complications. Overall, 37 patients (age, 28.0+/-7.6 years; 89.2% male) were enrolled from 7 sites. Implantable cardioverter defibrillators were implanted for primary prevention in 23 (62.1%) patients and secondary prevention in 14 patients (37.8%). Annual rates of appropriate shocks were 0.5% and 6.0% in primary and secondary prevention, respectively (P=0.0366). Independent predictors were a secondary prevention indication (hazard ratio, 18.0; P=0.0341) and lack of beta-blockers (hazard ratio, 16.7; P=0.0301). In patients with appropriate shocks, intracardiac electrograms documented supraventricular tachycardia preceding or coexisting with ventricular tachycardia in 50%. No patient with inducible ventricular tachycardia received an appropriate shock in comparison with 37.5% of noninducible patients (P=0.0429). Inappropriate shocks occurred in 6.6% per year, more so in patients of lesser weight (hazard ratio, 0.91 per kg; P=0.0168). Additionally, 14 patients (37.8%) experienced complications: 5 (13.5%) acute, 1 (2.7%) late generator related, and 12 (32.4%) late lead related., Conclusions: In patients with transposition of the great arteries, high rates of appropriate shocks are noted in secondary but not primary prevention. Supraventricular arrhythmias may be implicated in the etiology of ventricular tachyarrhythmias; beta-blockers seem protective, and inducible ventricular tachycardia does not seem to predict future events. Inappropriate shocks and late lead-related complications are common.

Background: Sleep disordered breathing is frequently observed in patients with cardiovascular disease. Even in the absence of heart disease, acute and chronic hypoxia have been shown to promote sleep-related periodic breathing with central apnea characterized by a repetitive reduction or lack of respiratory activity. Cyanotic congenital heart disease (CCHD) is associated with chronic hypoxia, regardless of whether an increase in pulmonary artery pressures coexists. Sleep aggravated hypoxia has been observed in many such patients, but it remains to be determined whether sleep disordered breathing is contributory. We, therefore, sought to assess sleep-related breathing pattern in patients with CCHD., Methods: Adults with CCHD, resting arterial oxygen saturation <90%, and systemic ejection fraction >40% were prospectively enrolled in a cross-sectional study. To assess sleep and respiratory indices, subjects underwent a standardized clinical appraisal that included arterial blood gas analysis and a comprehensive sleep study with an ambulatory device. An apnea-hypopnea index (AHI) >or=5/h was considered to indicate sleep apnea., Results: Ten adults with CCHD, aged 38+/-11 years, completed the study. Seven patients had elevated pulmonary artery pressures, with a mean systolic pressure of 86.3+/-18.1 mm Hg. All patients demonstrated normal sleep parameters. Oxygen saturation further declined in 5 patients during sleep. However, no associated alteration in respiratory parameters was observed and no significant arrhythmia. The mean AHI was 1.1+/-1.0/h. No subject met the pre-defined criterion for sleep apnea., Conclusion: Although further oxygen desaturation may be observed during sleep in patients with CCHD, it occurs in the absence of sleep disordered breathing.

Background: In adults with congenital heart disease (ACHD), lead extraction procedures are expected to parallel increasing transvenous pacemaker and defibrillator implantations. We sought to assess the safety and feasibility of laser lead extraction in ACHD., Methods and Results: All laser lead extractions (Spectranectics, Colorado Springs, CO, USA) performed at the Montreal Heart Institute between September 2000 and August 2005 were prospectively registered. Efficacy and complications in patients with ACHD were compared to the larger cohort. Laser lead extraction was attempted on 270 leads in 175 patients. In ACHD, 23 (five atrial, 15 ventricular pacing, and three defibrillator) leads were targeted in 16 patients. Indications were: infection 44%, dysfunction 25%, upgrade 25%, and pain 6%. Patients with ACHD were younger (43.0 +/- 13.5 vs 63.7 +/- 14.7 years, P < 0.0001) and had a higher proportion of active fixation leads (74% vs 37%, P = 0.0013). Lead age in patients with and without ACHD was 9.0 +/- 5.2 vs 7.7 +/- 5.2 years (P = 0.2713). Overall, 21 of 23 leads (91%) were successfully extracted in ACHD compared with 220 of 247 leads (89%) (P = 0.7405). One major complication (6.3%) occurred in ACHD (tricuspid valve laceration) compared with five major (3.0%) and eight minor (5.0%) complications in patients without ACHD. Presence of ACHD did not modulate procedural success (OR 1.3, 95% CI [0.3, 5.8]) or complications (OR 1.0, 95% CI [0.2, 4.4]). Median procedural time was 27 minutes longer in ACHD (127 vs 100 minutes, P = 0.0595)., Conclusion: In selected patients with ACHD, laser lead extraction may be performed with a safety and efficacy profile comparable to patients without ACHD.

As early as 1699, Chemineau described a heart composed of 2 auricles but only 1 ventricle. The univentricular heart has since fascinated the medical community. Unique in its complexity and scope, the univentricular heart has sparked intense debates about embryology and nomenclature, challenged our understanding of cardiovascular physiology and hemodynamics, and inspired some of the most creative surgical and interventional approaches in human history. The present report provides an overview of the nomenclature and classification of the univentricular heart, epidemiology and pathological subtypes, genetic factors, physiology, clinical features, diagnostic assessment, therapy, and postoperative sequelae. Although the present report touches on issues applicable to neonates and children with univentricular hearts, the focus is on information of interest and relevance to the adult cardiologist.

Background and Aim of the Study: Low-molecular-weight heparin (LMWH) is considered a recommended anticoagulation option in pregnant women with prosthetic heart valves. However, few data are available regarding the efficacy and safety of LMWH in this setting., Methods: In 1999, the authors' institution developed a standardized anticoagulation protocol for pregnant women with prosthetic heart valves, which included LMWH administered between six and 12 weeks' gestation, and after 36 weeks, with prespecified target levels, and additional low-dose aspirin. Herein is presented the initial experience using this anticoagulation regimen., Results: Among five women with prosthetic heart valves treated with LMWH during part of their pregnancy, four had uneventful pregnancies while one suffered a coronary artery embolus. A review is provided of the current state of knowledge regarding anticoagulation in pregnancy, with emphasis placed on the importance of strict monitoring of anticoagulation levels., Conclusion: Given the drawbacks of other forms of anticoagulation, and within the constraints of available data, LMWH appears--when administered with caution--to be an acceptable alternative in pregnant women with prosthetic heart valves.

Recent advances in pediatric cardiology and cardiac surgery have allowed a rapidly expanding population of patients with congenital heart disease to thrive well into their adult years. Often after prolonged uneventful clinical courses as children and adolescents, arrhythmias may surface later in life. These arrhythmias are a major source of morbidity. In addition, sudden death is the leading cause of mortality in adult patients with congenital heart disease. This review highlights the various types of brady- and tachyarrhythmias encountered in the more common forms of adult congenital heart disease and explores prognostic implications and therapeutic options.

We present an unusual case of ruptured aneurysm of the left sinus of Valsalva discovered 41 years after a car accident. Echocardiography was a key element in establishing the diagnosis of this rare anomaly, which is often congenital but has also been reported as acquired or traumatic in origin.

Objectives: We sought to determine the impact of pregnancy on the systemic right ventricle (RV) after a Mustard operation for transposition of the great arteries., Background: Dysfunction of the RV remains a continuing problem after a Mustard operation. Concerns exist about the potentially deleterious effects of pregnancy on this ventricle., Methods: The records of 16 women who completed 28 pregnancies were reviewed for clinical status, echocardiographic evaluation of RV dimensions, RV function, and tricuspid regurgitation (TR) before, during, and after pregnancy., Results: Women were in New York Heart Association functional class I (n = 21) and II (n = 7) before pregnancy. The functional class deteriorated in six women, with no return to the pre-pregnancy level after delivery in two. Data on RV dimensions were available in 18 pregnancies, on RV function in 21, and on TR in 20. Before pregnancy, RV dilation was absent (n = 4), mild/moderate (n = 12), or severe (n = 2) and progressed in five women (31%), with no recovery in all patients at the last follow-up. Right ventricular systolic dysfunction was absent (n = 16), mild/moderate (n = 4), or severe (n = 1) before pregnancy and progressed in four women (25%), with no recovery in three cases. Tricuspid regurgitation was absent (n = 8), mild (n = 9), or moderate (n = 3) before pregnancy and deteriorated in eight women (50%), with no recovery in three patients., Conclusions: Pregnancy after a Mustard operation is clinically well tolerated but carries a risk of RV dysfunction, which is sometimes irreversible.

Even with continuing technical improvements in prosthesis design and the development of less thrombogenic materials, mechanical valve prostheses still carry a thromboembolic risk significant enough to warrant long-term anticoagulation therapy. Optimal anticoagulation is especially crucial during pregnancy due to the hypercoagulable state that rapidly develops after conception. Conventional anticoagulation therapy with coumarin derivatives is associated with risks of teratogenicity and hemorrhage for the fetus, and thromboembolic and hemorrhagic complications for the mother. As a result, other forms of anticoagulation, such as unfractionated or low molecular weight heparin, have been advocated as an alternative in selected cases. The present report describes a case of embolic myocardial infarction occurring in a pregnant woman with an aortic bileaflet mechanical valve prosthesis while on therapeutic low molecular weight heparin after only one dose was withheld before amniocentesis.

Objective: To evaluate the effects of inhibition of the Na+/H+ exchanger (NHE) on regional left ventricular dysfunction in patients with a non-ST elevation acute coronary syndrome (ACS) BACKGROUND: NHE inhibition protects against myocardial necrosis and stunning in ischemia-reperfusion models., Methods and Results: In a substudy of the GUARd During Ischemia Against Necrosis (GUARDIAN) trial, 141 patients with an ACS and wall motion abnormalities on a baseline echocardiogram underwent repeated studies after 48 h and 36 days to determine the extent of early and late recovery of ventricular function. Patients were randomized to cariporide 20 mg, 80 mg or 120 mg intravenously three times per day or placebo for two to seven days. The wall motion score index improved from baseline to day 36 by 0.13+/-0.21, 0.16+/-0.34 and 0.15+/-0.28, respectively, in the cariporide groups, and by 0.10+/-0.16 with placebo (P=0.57). From baseline to 48 h, the wall motion score index decreased by 0.04+/-0.16, 0.08+/-0.20 and 0.03+/-0.22, respectively, for the cariporide groups and by 0.03+/-0.11 with placebo (P=0.09); the number of abnormal chords (centreline method) decreased by 11.2+/-24.0, 9.5+/-24.3 and 8.1+/-27.9, respectively, in the cariporide groups compared with an increase of 0.6+/-20.0 with placebo (P=NS)., Conclusion: No significant benefit of NHE inhibition could be detected in ACS patients in the GUARDIAN trial.

Sudden death is the most frequent cause of late mortality in adults with congenital heart disease. This study reviews the experience of an Adult Congenital Heart Center with the use of implantable cardioverter defibrillators (ICDs). The charts of adults with congenital heart disease who had an ICD implantation were reviewed for diagnosis, residual lesions, reoperations, reason for implantation, complications, and recurrence of arrhythmias. Since 1995, 13 patients (mean age 43 years) had an ICD implantation for aborted sudden death (4), and spontaneous (6), or induced (3) ventricular tachycardia. Diagnosis were repaired (6) or palliated (1) tetralogy of Fallot, operated pulmonary stenosis (2), palliated complex pulmonary atresia (1), congenitally corrected transposition (1), operated ventricular (1), and atrial (1) septal defects. Significant residual lesions included severe pulmonary regurgitation (2), systemic ventricular dysfunction (2), and severe pulmonary hypertension (1). Five patients had a QRS > 180 ms, four had a QRS < 180 ms, four had a paced rhythm. Overall, four (31%) patients had no ventricular dysfunction, no residual lesion, and QRS < 180 ms. During a mean follow-up of 29 months, seven patients had recurrent ventricular tachycardia, three with normal ventricular function, no residual lesion, and QRS < 180 ms. ICD implantation is an important adjunct in the management of adults with congenital heart disease. As malignant arrhythmias occur even in patients with no residual lesion, no QRS prolongation and no ventricular dysfunction, the recognition of those who would benefit from an ICD remains a clinical challenge.

Background: To determine the rate of progression of dilation of the aortic root in adults with a bicuspid aortic valve., Methods: We reviewed retrospectively the transthoracic echocardiograms of 50 adults with a bicuspid aortic valve. Each patient had had at least two examinations made 12 months apart. Measurements were taken at four levels: at the basal attachment of the leaflets of the valve within the left ventricular outflow tract, at the widest point of the sinuses of Valsalva, at the sinutubular junction, and in the ascending aorta 1 cm beyond the sinutubular junction., Results: Progressive dilation occurred at all levels, ranging from 0.3 mm/yr at the basal attachment within the left ventricular outflow tract to 1.0 mm/yr, 1 cm beyond sinutubular junction. These rates of dilation were greater than the reported rate of 0.8 mm per decade in the normal population. The rate of dilation found in the ascending aorta 1 cm beyond the sinutubular junction was significantly greater than at the other sites (p = 0.005). The 21 patients with baseline measurements greater than 34 mm had a significantly higher rate of progression (p = 0.007). Sex, age, and the degree of valvar obstruction or regurgitation did not significantly influence the rate of progression of dilation., Conclusion: There is a significantly higher rate of dilation of the aortic root in adults with a bicuspid aortic valve when compared to the normal population. Periodic evaluation of the ascending aorta is essential in these patients, even after replacement of the aortic valve. Other imaging modalities should be considered if the region beyond the sinutubular junction is not well visualized by transthoracic echocardiography.

Percutaneous closure of atrial septal defects (ASD) is increasingly performed in pediatric and adult patients. This study reports a single-center experience with the use of the Amplatzer Septal Occluder for percutaneous ASD closure in adults. One hundred and seventeen consecutive patients were prospectively included in the database. The procedure was successful in 106 patients (90.6%). The mean age was 50 years. Eighty-four percent of the patients had a Qp:Qs > 1.5. Other indications included a history of thromboembolism, decreased exercise tolerance, atrial arrhythmias, evidence of pulmonary hypertension or right heart overload. Mean procedure time was 44 minutes, and mean fluoroscopy time was 13 minutes. The mean defect diameter was 17.2 mm (range, 5 30 mm). There was no residual shunting at the end of the procedure in 75% of the patients. Shunting was minimal in all but 2 of the remaining 25 patients (1.8%). No major complications were observed at a mean follow-up of 19 months. Patients in whom the procedure failed had larger defects (mean, 25.0 mm versus 18.3 mm; p < 0.001), larger right ventricles (44.1 mm versus 38.2 mm; p = 0.03) and more significant shunting (Qp:Qs, 3.5 versus 2.1; p < 0.001). New York Heart Association functional class improved in all but 1 patient. Percutaneous ASD closure using ASO is safe and mid-term results compare favorably with those reported with surgical ASD closure. It can therefore be recommended as the first line of treatment in adult patients with secundum ASD.

Cloth-covered Starr-Edwards valves were withdrawn in the 1970s because of complications of cloth tear. Nevertheless, many patients are still alive with their prosthesis in place. We evaluated the ability of transesophageal echocardiography (TEE) to detect cloth tear in 9 asymptomatic patients operated on with a cloth-covered Starr-Edwards aortic valve at our institution. An echogenic mobile structure attached to the prosthesis, suggestive of a tear, was detected in 5 patients (55%). A tear was diagnosed in a sixth patient when TEE was repeated during follow-up because of neurologic symptoms. In all cases, transthoracic echocardiography failed to detect any abnormality. Two patients had symptoms develop and underwent reoperation; cloth tear was more extensive than suspected by TEE. Cloth tear is a frequent finding that can be detected by TEE in asymptomatic patients with cloth-covered Starr-Edwards aortic prosthesis. TEE is recommended in these patients and reoperation should be considered as soon as symptoms appear.

Background: Controversy exists as to whether secundum atrial septal defects (ASDs) in asymptomatic or mildly symptomatic New York Heart Association (NYHA) class I or II adult patients should be closed., Methods and Results: Thirty-seven patients (24 females; mean age 49.4 years, range 19 to 76) with a mean pulmonary to systemic flow ratio (Qp:Qs) of 2.1 (1.2 to 3.4) had a maximal oxygen uptake (VO2max) determination and echocardiographic measurement of right ventricular dimensions before and 6 months after elective percutaneous closure of ASD. At baseline, mean VO2max was 23.5+/-6.4 mL/kg per minute and was higher in the 15 NYHA I patients than in the 22 NYHA II patients (27+/-6.9 versus 20.8+/-4.6 mL/kg per minute; P=0.0015). VO2max increased significantly at 6 months (23.5+/-6.4 to 26.9+/-6.9 mL/kg per minute; P<0.0001). Improvement was as marked in NYHA I (+22%; P<0.0001) as in NYHA II patients (+12%; P<0.0001), in patients with Qp:Qs 1.2 to 2.0 (+16%; P<0.0001) as in those with Qp:Qs >2 (+12%; P<0.0001), and in patients > or =40 years of age (+14%; P<0.0001) as in those <40 years of age (+16%; P<0.0001). Compared with 15 of 37 patients before closure, 35 of 37 patients were in NYHA I at 6 months. Right ventricular dimensions decreased significantly (P<0.0001)., Conclusions: Adult ASD patients significantly increase their functional capacity after percutaneous defect closure. This is observed even in patients classified as asymptomatic, in those with lesser shunts, and in older patients. These findings suggest that ASD closure in an adult population should be considered even in the absence of symptoms.

Background: The population of adults with congenital heart disease is growing rapidly, and the transition of care from the pediatric to the adult setting is often not optimal., Objectives: To assess the level of knowledge that adults with congenital heart disease have of their condition., Methods: All new patients referred to an adult congenital heart centre were asked to answer questions about their clinical diagnosis, their need and reason for anti-bioprophylaxis, and their risks of pregnancy when applicable. The patient's age at first visit, source of referral and time from last visit in cardiology (defined as the last visit with a cardiologist) were also noted., Results: From November 1999 to July 2000, 104 patients (42 men) were referred to the adult centre by pediatric cardiologists (56), medical cardiologists (26), general practitioners (11), dentists (two) or obstetricians (two), or they were self-referred (seven). The mean age at the time of referral was 28 11 years (range 16 to 72 years, median 24 years). The time from the last visit in cardiology varied widely from one month to 25 years (median three years), with 29 patients (28%) having had no follow-up for more than five years. Among these, 14 patients had no follow-up for more than 10 years, with six patients having been referred for complications related to their cardiac anatomy, such as heart failure (Eisenmenger, Ebstein), syncope (operated and unoperated tetralogy of Fallot) and arrhythmias (atrial septal defect, unoperated Fallot). The clinical diagnosis was completely unknown by 36 patients (34.6%), including patients with repaired Fallot (three patients), Mustard procedure (two), severe aortic stenosis (two), severe pulmonary stenosis (one), Eisenmenger (one), unoperated Fallot (one), ventricular septal defect (six) and bicuspid aortic valve (seven). Seventy-three patients (79%) knew about antibiotic prophylaxis, but 50% did not comprehend why they needed it. Many women (66%) had never discussed the risks of pregnancy with their physician., Conclusions: A large percentage of adults with congenital heart abnormalities show a poor level of knowledge about their heart condition. These observations suggest the importance of structured transitional programs, the impact of which will need to be validated in prospective studies.

Background: The maternal and neonatal risks associated with pregnancy in women with heart disease receiving comprehensive prenatal care have not been well defined., Methods and Results: We prospectively enrolled 562 consecutive pregnant women with heart disease and determined the outcomes of 599 pregnancies not ending in miscarriage. Pulmonary edema, arrhythmia, stroke, or cardiac death complicated 13% of pregnancies. Prior cardiac events or arrhythmia, poor functional class or cyanosis, left heart obstruction, and left ventricular systolic dysfunction independently predicted maternal cardiac complications; the cardiac event rate can be predicted using a risk index incorporating these predictors. Neonatal complications (20% of pregnancies) were associated with poor functional class or cyanosis, left heart obstruction, anticoagulation, smoking, and multiple gestations., Conclusions: Pregnancy in women with heart disease is associated with significant cardiac and neonatal complications, despite state-of-the-art obstetric and cardiac care. Maternal cardiac risk can be predicted with the use of a risk index.

Objective: To determine whether patients with cardiac tamponade are subject to delays and clinical deterioration before undergoing echocardiography and pericardial drainage., Design: Retrospective study., Setting: The Montreal Heart Institute, Montreal, Quebec, a cardiology referral centre., Patients: The charts of 50 patients who presented with tamponade were reviewed. Intervals between the appearance of symptoms, consultation, echocardiography and drainage were noted. The presence of clinical deterioration before drainage was evaluated. Causes for delays were investigated., Results: Previous cardiac surgery (74%) was the most common etiology of tamponade. Symptoms were present 6.6+/-5.8 days before consultation. The delay between consultation and echocardiography was 1.2+/-2.0 days (range 0 to 12), and that between echocardiography and drainage was 0.8+/-0.9 days (range 0 to four). Patients underwent drainage 1. 9+/-2.5 days (range 0 to 16) after the initial consultation. Deterioration of the clinical status was noted in 34% of patients before pericardial drainage. An error in the initial diagnosis was present in 36% of patients; the majority of these were incorrectly diagnosed with heart failure. Another 44% of patients had no mention of either a working diagnosis in the chart at admission or the desire to rule out tamponade on the request for echocardiography., Conclusion: The proper diagnosis does not appear to be initially considered in up to 80% of patients who present with cardiac tamponade. Clinical deterioration occurs in approximately a third of patients during the interval between consultation and pericardial drainage.

Idiopathic dilatation of the pulmonary artery (IDPA) is a rare congenital disease which is usually detected fortuitously on chest x-ray, thus radiologists must be aware of this clinical entity. This report describes four cases to which Magnetic Resonance Imaging (MRI) played a major role in diagnosing IDPA and in detecting the concomitant findings observed in this disease. MRI is a non-invasive procedure with many advantages for the accurate and reproducible measurement of artery structures, which makes it the preferred option for combined use with echocardiography in the diagnosis and follow-up of patients with IDPA.

There are few studies providing information on the natural course of hemodynamically insignificant atrial septal defect (ASD). To review the outcome of patients with secundum ASD, we retrospectively reviewed the charts of patients who had initially not been considered for surgical closure after age 1 year, and who had either a follow-up of at least 10 years or documented closure. Thirty patients, 22 females and 8 males, fulfilled our inclusion criteria. Mean age at diagnosis was 1.3 year and mean follow-up duration was 11.5 years. Seventeen patients had spontaneous closure of the ASD at a mean age of 8.4 years. There were 7 asymptomatic patients whose ASD was still patent at the last visit (mean age 14.1 years, mean follow-up 13.2), with defect dimensions on echocardiography ranging from 1 to 6 mm. The remaining 6 patients were considered to require surgical closure on the basis of an apparent increase in size of the ASD and secondary clinical and hemodynamic manifestations. These results (1) confirm that not all secundum ASDs need to be treated surgically because they can still spontaneously close past the age of 5, and (2) suggest that in a minority of cases the size of the defect could increase.