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Copyright of Revista Española de Cardiología (18855857) is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

More than 90% of children born with heart defects reach adulthood. They continue to require specialized medical care. In most countries, their care has to be transferred from the pediatric care environment to specialized adult clinics. This transfer of care usually occurs at a time when adolescents become young adults. Supporting adolescents and emerging adults with congenital heart disease through transition has been recognized as an important task of their treating teams in recent years. An environment where adolescents feel welcome and where education and patient participation are fostered is crucial. For an optimal transition process, patients, their families and all health care providers need to be involved. Different models for transition programs have emerged, depending on local policies and resources. The authors offer insight into established transition programs in Bern and Zurich, Switzerland. Advantages and challenges of different models of care and transition programs are presented. [ABSTRACT FROM AUTHOR]

The aim of this study was to test the hypothesis that ear oximetry immediately after the release of a sustained Valsalva maneuver accurately detects patent foramen ovale (PFO). One hundred sixty-five scuba divers underwent transesophageal echocardiography (TEE; reference method) for PFO assessment. Ear oximetry of the right earlobe was performed in a different room within a time frame of 2 hours before or after TEE. The subject and the oximetry operator were unaware of the results of TEE. Oxygen saturation (SO2) measurements were obtained at baseline and during the release phase of 4 Valsalva maneuvers within 10 minutes, and the average SO2 change (SO2 at baseline minus SO2 at Valsalva release) was determined as the primary study end point. One hundred seventeen divers had no PFO, and 48 (29%) had PFO by TEE (mean age 39 ± 8 years). The average SO2 change was 0.79 ± 1.13% (i.e., a slight absolute SO2 decrease in response to the Valsalva maneuver) in the group without PFO and 1.67 ± 1.19% in the PFO group (p <0.0001). Using receiver-operating characteristic curve analysis, a PFO as defined by TEE could be detected at a threshold of a Valsalva-induced decrease in SO2 of ≥0.825 percentage points in comparison to baseline (sensitivity 0.756, specificity 0.706, area under the receiveroperating characteristic curve 0.763, p <0.0001, negative predictive value 0.882). In conclusion, the entirely noninvasive method of ear oximetry in response to repetitive Valsalva maneuvers is accurate and useful as a screening method for the detection of a PFO, as shown in this study of divers. [ABSTRACT FROM AUTHOR]

Abstract: The steadily growing number of adults with congenital heart disease has led with some delay to an increase of specialized programs dedicated to the care of these patients. Still, in many countries the prevalence of congenital heart disease in adults is not known, nor the number of adults requiring specialized care. By extrapolating the results of epidemiological studies on the population in Europe and North America, only half or even less of all adults with moderate or complex congenital heart disease is currently under follow-up in specialized centers. Lapse of appropriate medical care in adults with congenital heart disease is associated with unnecessary morbidity and an increased likelihood of the need for urgent interventions. In order to improve medical care for adults with moderate or complex congenital heart disease, the number of patients with specialized follow-up in congenital cardiac clinics has to increase. One approach is to avoid lapses of care in patients already followed by a pediatric or adult congenital heart disease center. Therefore, congenital heart disease clinics should try to identify among their patients the ones at risk for lost to follow-up, and focus some of their efforts on measures to increase the awareness for life-long medical care among these patients. Characteristics of the adult congenital cardiac patient at high risk of inappropriate medical follow-up include having few interventions in early life despite a complex cardiac defect, being born in an earlier decade, receiving no specific follow-up recommendations, and having no education about defect- or surgery-related sequelae or residual lesions. Implementation of a structured transition program, teaching patients about their heart defect and the consequences of repair or palliation in earlier life, establishing a continuity of care within the congenital cardiac clinic, optimizing organization and communication between different care-givers, and encouraging patients to play an active role regarding their personal health care, are some of the recommendations on how we should try to avoid another generation of adult congenital heart disease patients from being lost to follow-up. [Copyright &y& Elsevier]

Objectives: We sought to assess the safety and clinical efficacy of patent foramen ovale (PFO) closure under fluoroscopic guidance only, without intraprocedural echocardiography. Background: Percutaneous PFO closure has been shown to be safe and feasible using several devices. It is generally performed using simultaneously fluoroscopic and transesophageal or intracardiac echocardiographic guidance. Transesophageal echocardiography requires sedation or general anesthesia and intubation to avoid aspiration. Intracardiac echocardiography is costly and has inherent risks. Both lengthen the procedure. The Amplatzer PFO Occluder (AGA Medical Corporation, Golden Valley, Minnesota) can be safely implanted without echocardiographic guidance. Methods: A total of 620 patients (51 ± 12 years; 66% male) underwent PFO closure using the Amplatzer PFO Occluder for secondary prevention of presumed paradoxical embolism. Based on size and mobility of the PFO and the interatrial septum, an 18-mm device was used in 50 patients, a 25-mm device in 492, and a 35-mm device in 78. Results: All procedures were successful, with 5 procedural complications (0.8%): 4 arteriovenous fistulae requiring elective surgical correction, and 1 transient ischemic attack. Contrast transesophageal echocardiography at 6 months showed complete closure in 91% of patients, whereas a minimal, moderate, or large residual shunt persisted in 6%, 2%, and 1%, respectively. During a mean follow-up period of 3.0 ± 1.9 years (median: 2.6 years; total patient-years: 1,871), 5 ischemic strokes, 8 transient ischemic attacks, and no peripheral emboli were reported. Freedom from recurrent ischemic stroke, transient ischemic attack, or peripheral embolism was 99% at 1 year, 99% at 2 years, and 97% at 5 years. Conclusions: The Amplatzer PFO Occluder affords excellent safety and long-term clinical efficacy of percutaneous PFO closure without intraprocedural echocardiography. [Copyright &y& Elsevier]

Background: Sex differences in patients with patent foramen ovale (PFO) and cryptogenic stroke have not been systematically analyzed. We aimed to determine sex influences on demographics, vascular risk factors, clinical manifestations, stroke location, and clinical outcome. Methods: One thousand two hundred eighty-eight consecutive patients with ischemic stroke or transient ischemic attack (TIA) were admitted to a single stroke center. All patients underwent a complete stroke workup including clinical examination, standard blood tests, cerebral and vascular imaging, transesophageal echocardiography, and 24-hour electrocardiography. In 500 patients, no definite etiology could be established (cryptogenic stroke/TIA). Of them, 167 patients (107 men and 60 women, mean age 52 +/- 13 years) had an PFO. Results: The prevalence of PFO in patients with cryptogenic stroke or TIA was higher in men than in women (38% vs 28%, P = .014). Stroke severity and the prevalence of risk factors did not differ between the 2 sexes. There was an independent association between male sex and stroke location in the posterior cerebral circulation (OR 3.0, 95% CI 1.4-6.5, P = .006). Men and women did not differ in respect to PFO grade, prevalence of right-to-left shunt at rest, or coexistence of atrial septal aneurysm. Clinical outcome at 3 months was similar in both sexes. Conclusion: Patent foramen ovale was more prevalent in men than in women with cryptogenic stroke. There were no sex influences on age, risk factors, echocardiographic characteristics of PFO, or clinical outcome. Male sex was independently associated with stroke in the posterior cerebral circulation. [ABSTRACT FROM AUTHOR]

Background: Adult patients with repaired tetralogy of Fallot (rTOF) often have diminished exercise capacity. The primary objective of this study was to examine whether abnormalities of biventricular function play a role in exercise limitation in patients with rTOF. Methods: This was a retrospective review of 99 adult patients with rTOF. Right ventricular (RV) and left ventricular (LV) function were assessed echocardiographically using the myocardial performance index (MPI). Maximal oxygen consumption (VO2 Max) was measured during a level 1 cardiopulmonary exercise test. Results: The mean age of the cohort was 34 ± 11 years (50% females). Although most of the patients reported good functional capacity, the peak Vo 2max was decreased at 22 ± 6 mL/kg per minute (66% ± 13% predicted Vo 2max for age and sex). The mean RV and LV MPI were 0.30 ± 0.07 and 0.42 ± 0.09, respectively. In the multivariate model, higher RV MPI (P = .04) and LV MPI (P = .005) values, representing impaired ventricular function, were associated with diminished Vo 2max. There was a significant correlation between the RV and LV MPI (r = 0.54, P = .001). Conclusions: Impairment of RV and LV function, as measured by MPI, is associated with diminished exercise capacity in patients with repaired tetralogy of Fallot. Furthermore, there is a linear relationship between the RV and LV function suggesting that ventricular interactions are contributing to the limited exercise capacity in this group of patients. Strategies aimed at preserving biventricular function or improving adverse ventricular interactions could help to improve functional capacity in these patients. [Copyright &y& Elsevier]

Background: Despite advances in surgical and interventional techniques, the optimal surgical treatment of severe aortic (re) coarctation and hypoplastic aortic arch is still controversial. Anatomic repair may require extensive dissection, cardiopulmonary bypass, and deep hypothermic circulatory arrest with their inherent risks. The aim of this study was to analyze the outcome of off-pump extraanatomic aortic bypass as a surgical alternative to local repair. Methods: From February 2000 to December 2005, ten consecutive patients (median age 20 years; range, 11 to 38 years) with severe aortic (re) coarctation (n = 4) and (or) hypoplastic aortic arch (n = 7) underwent off-pump extraanatomic aortic bypass through median sternotomy. All but three patients had undergone previous surgery for coarctation and angioplasty or stenting. Three patients underwent concomitant replacement of the ascending aorta because of an aneurysm using cardiopulmonary bypass. Results: Postoperative hospital course was uneventful in all patients. There was no perioperative mortality or significant morbidity. During a mean follow-up of 48 ± 22 months no patient required additional procedures. All patients were free of symptoms; no patient showed signs of heart failure after follow-up. At last follow-up, no patient presented with claudication, nor any patient experienced orthostatic problems due to a steal phenomenon. During follow-up, hypertension resolved in all patients with residual mild hypertension in two patients. Conclusions: Off-pump extraanatomic aortic bypass is an attractive treatment option for complex aortic (re) coarctation and hypoplastic aortic arch. Perioperative risks are minimized, hypertension is influenced favorably, and midterm survival is event-free. [Copyright &y& Elsevier]

Adults with repaired tetralogy of Fallot and significant chronic pulmonary regurgitation are at risk for progressive right ventricular (RV) dilatation and dysfunction. The assessment of RV function is important in the management in these patients. There is still a lack of an adequate geometric model to quantify RV function by echocardiography. The myocardial performance index (MPI) is a nonvolumetric method to quantify global ventricular function. In this study, the accuracy of MPI obtained by echocardiography to quantify RV function was assessed in 57 adults with repaired tetralogy of Fallot. The MPI measurement was compared with the RV ejection fraction (EF) derived by cardiac magnetic resonance imaging. There was a negative linear correlation between the MPI and the RVEF (r = 0.73, p <0.001). A MPI cutoff of ≥0.40 had a sensitivity of 81% and a specificity of 85% to diagnose a RVEF <35%. A MPI cutoff of <0.25 had a sensitivity of 70% and a specificity of 89% to identify patients with RVEFs ≥0.50. In a multivariate regression model, the MPI was not affected by the degree of pulmonary regurgitation, the presence of tricuspid regurgitation, or the QRS duration. In conclusion, the Doppler-derived MPI is a simple and reliable method for the evaluation of RV systolic function in adults with repaired tetralogy of Fallot. [Copyright &y& Elsevier]

Abstract: The presence of irreversible pulmonary hypertension in patients with atrial septal defect (ASD) is thought to preclude shunt closure. We report the case of a woman with plexiform pulmonary arteriopathy secondary to an ostium secundum ASD who was able to successfully undergo percutaneous shunt closure following therapy with chronic intravenous prostacyclin (Flolan). One year after closure, the patient was weaned off Flolan over a period of 7 months following the institution of oral Bosentan therapy. Our case illustrates how aggressive vasodilator therapy with prostaglandins may be capable of reducing pulmonary artery pressure and permitting shunt closure in a patient once considered to have “inoperable” pulmonary arteriopathy. [Copyright &y& Elsevier]

Dysfunction of the systemic right ventricle is common after the atrial switch procedure for transposition of the great arteries. Cardiac transplantation remains the only long-term solution in terminal systemic right ventricular (RV) failure, but concomitant pulmonary hypertension (PHT) may preclude it. The increasing number of such patients, together with the concerns related to combined heart-lung transplantation (HLTx), urge us to consider other therapeutic options. [ABSTRACT FROM AUTHOR]

Background: Dilatative aortopathy is a feature of tetralogy of Fallot (TOF). Structural abnormalities of the medial aorta have also been described for other conotruncal defects, like d-transposition of the great arteries (d-TGA). This study aims to elucidate the severity of aortopathy assessed by cardiac magnetic resonance imaging (CMR) in adults with repaired TOF compared to adults with repaired d-TGA (atrial switch procedure) and normal individuals (C). Methods: In 3 groups of 20 individuals each (d-TGA, TOF, C), aortic diameters were retrospectively measured with CMR at the following levels: aortic sinus (Si), sinotubular junction (STJ), ascending and descending aorta at the level of the bifurcation of the pulmonary artery (aAP, dAP), and diaphragm (D). As a measure of aortic elasticity the aortic distensibility of the ascending and descending aorta (aAD, dAD) at the level of the pulmonary bifurcation was determined from cine images: (maximal cross sectional area (CSA) – minimal CSA)/minimal CSA)/(systolic blood pressure - diastolic blood pressure). Results: There were no differences in age and gender between groups. Diameters of Si and STJ were significantly increased in TOF as compared to d-TGA and C: Si mm/m2: 21.6 vs. 18.2 vs. 17.2, p = .001. STJ mm/m2: 18 vs. 15 vs. 13.7 p = .001. Diameters of aPB were significantly increased in TOF as compared to d-TGA and C: aPB mm/m2: 16.8 vs. 15.8 vs 14.4, p = .008. aAD was significantly reduced in TOF compared to C, and showed a trend to a lower value in d-TGA: aAD 10^-3 mmHg^-1: 3.3 vs. 3.6 vs. 5.3, p = .014 (figure). dAD showed a trend to a lower value in TOF compared to d-TGA and C: dAD 10^-3 mmHg^-1: 5.2 vs. 6.2 vs. 6.9, p = .113 The diameters of the descending aorta (dAP, D) did not differ significantly between the groups. Conclusions: The intrinsic aortopathy in repaired TOF is evident by increased diameters and reduced elasticity of the ascending aorta. In d-TGA patients, the severity of aortopathy is less evident and primarily expressed by a reduced distensibility. Nevertheless, aortic dimensions in this patient group should also be carefully followed, especially with increasing age. Display Omitted [Copyright &y& Elsevier]

Abstract: Altered myocardial perfusion is frequent in patients with congenital heart disease. Measurement of myocardial blood flow per gram of myocardium at rest and during hyperemia is the most robust method to evaluate myocardial perfusion in absolute terms. So far, most studies in congenital heart disease patients used positron emission tomography to evaluate absolute myocardial blood flow. With the recent advances in contrast echocardiography, we have now a more easily applicable but similarly solid method at hand to assess myocardial perfusion in different clinical scenarios. Myocardial contrast echocardiography has proven its clinical and scientific value in coronary artery disease patients. In this review, we discuss the merits of myocardial perfusion assessment in patients with a systemic right ventricle, a univentricular heart physiology, and tetralogy of Fallot. We outline how myocardial contrast echocardiography can improve our understanding of ventricular function. We have to admit that its clinical value remains to be elucidated. For most congenital defects, the impact of impaired myocardial perfusion on ventricular function and clinical outcome is still poorly investigated. Myocardial contrast echocardiography can contribute to fill this gap. [Copyright &y& Elsevier]

Abstract: Background: Systolic right ventricular (RV) function is an important predictor in the course of various congenital and acquired heart diseases. Its practical determination by echocardiography remains challenging. We compared routine assessment of lateral tricuspid annular systolic motion velocity (TVlat, cm/s) using pulsed-wave tissue Doppler imaging from the apical 4-chamber view with cardiac magnetic resonance (CMR) as reference method. Methods and results: 254 individuals (43±18years) underwent both CMR (contiguous short axis slices; retrogated cine steady state free precession technique; manual contour tracing) and echocardiography within 2±2months. Seventy-five had coronary artery disease, 87 congenital heart disease, 17 dilated cardiomyopathy, 15 pulmonary artery hypertension, and 47 normal findings. RV ejection fraction (EF) by CMR was 51±12% (range 17–78%). There was a linear correlation between RVEF and TVlat (r =0.60; p <0.0001). A TVlat cut-off of 12cm/s identified patients with normal EF (≥50%) with 81% sensitivity and 68% specificity, and a threshold of TVlat <9cm/s identified patients with severely reduced RVEF (<30%) with 82% sensitivity and 86% specificity. Conclusions: Systolic long-axis velocity measurements of the lateral tricuspid annulus allow a reliable assessment of RVEF in clinical routine. A threshold of TVlat <9cm/s identifies patients with severely reduced RVEF (<30%) with high sensitivity and specificity. [Copyright &y& Elsevier]

Abstract: Aims: The goal of this study was to assess the prevalence of left ventricular (LV) hypertrophy in patients with aortic stenosis late (>6 months) after aortic valve replacement and its impact on cardiac-related morbidity and mortality. Methods and results: In a single tertiary centre, echocardiographic data of LV muscle mass were collected. Detailed information of medical history and angiographic data were gathered. Ninety-nine of 213 patients (46%) had LV hypertrophy late (mean 5.8±5.4 years) after aortic valve replacement. LV hypertrophy was associated with impaired exercise capacity, higher New York Heart Association dyspnoea class, a tendency for more frequent chest pain expressed as higher Canadian Cardiovascular Society class, and more rehospitalizations. 24% of patients with normal LV mass vs. 39% of patients with LV hypertrophy reported cardiac-related morbidity (p =0.04). In a multivariate logistic regression model, LV hypertrophy was an independent predictor of cardiac-related morbidity (odds ratio 2.31, 95% CI 1.08 to 5.41), after correction for gender, baseline ejection fraction, and coronary artery disease and its risk factors. Thirty seven deaths occurred during a total of 1959 patient years of follow-up (mean follow-up 9.6 years). Age at aortic valve replacement (hazard ratio 1.85, 95% CI 1.39 to 2.47, for every 5 years increase in age), coexisting coronary artery disease at the time of surgery (hazard ratio 3.36, 95% CI 1.31 to 8.62), and smoking (hazard ratio 4.82, 95% CI 1.72 to 13.45) were independent predictors of overall mortality late after surgery, but not LV hypertrophy. Conclusions: In patients with aortic valve replacement for isolated aortic stenosis, LV hypertrophy late after surgery is associated with increased morbidity. [Copyright &y& Elsevier]

Objectives: We set out to study the effect of transcatheter closure of atrial septal defect (ASD) on right ventricular (RV) and left ventricular (LV) function assessed by myocardial performance index (MPI), as well as left atrial (LA) volumes. Background: The hemodynamic response to the closure of ASD is well-documented in surgically treated patients. However, few studies have documented echocardiographic evaluation of ventricular function in patients undergoing transcatheter closure of ASDs. Methods: Pre- and post-ASD device closure echocardiograms of 25 consecutive patients were retrospectively reviewed. Measurements of RV and LV MPI and LA volumes were made. Results: Twenty-five patients with an average age of 45.5 ± 16.3 years underwent transcatheter closure of ASD. There was statistically significant improvement in RV MPI (0.35 to 0.28, p = 0.004), LV MPI (0.37 to 0.31, p = 0.04), and LA volume index (25.7 to 21.8 ml/m2, p < 0.001) after closure of ASD. Conclusions: Device closure of ASDs leads to improvement of both RV and LV function as well as reduction in LA volume. These hemodynamic improvements provide insights into the symptomatic benefits gained in closure of ASDs using the transcatheter approach. [Copyright &y& Elsevier]

Purpose Patients with Fontan circulation have reduced heart rate variability (HRV) in supine position. However, neither cardiac autonomic nervous activity (CANA) in response to orthostatic challenge nor vagal reactivation by means of heart rate (HR) recovery after cessation of exercise have previously been investigated in Fontan patients. The aim of this study was to compare HRV in supine and standing position, as well as HR recovery between Fontan patients and healthy controls. Methods Eight Fontan patients (4 male/4 female) without pacemakers and 12 healthy volunteers (5 m/7 f) with minimum age of 18 years were recruited. HR was measured by Holter-electrocardiogram. HRV was measured in supine position and after orthostatic challenge. The power of the high frequency (HF: 0.15 Hz–0.4 Hz) and low frequency (LF: 0.04 Hz–0.15 Hz) bands was analysed by fast-Fourier transformation. HR recovery was determined at 30 s and 60 s after termination of a maximal exercise test. Results In both supine and standing position, total power, HF and LF power were reduced in Fontan patients compared to controls (by approximately a factor of 10) while there was no differences in LF/HF power ratio. Response to orthostatic challenge was blunted in absolute power but normal in relative power. HR recovery was not different between groups. Conclusion Fontan patients have greatly reduced HRV, a blood-pressure dependent marker of CANA, but normal HR recovery, a blood pressure independent marker of vagal reactivation, suggesting that vagal activity may be normal, and only vascular capacitance reduced. [ABSTRACT FROM AUTHOR]

The number of adults with congenital heart disease (CHD) has increased markedly over the past few decades as a result of astounding successes in pediatric cardiac care. Nevertheless, it is now well understood that CHD is not cured but palliated, such that life-long expert care is required to optimize outcomes. All countries in the world that experience improved survival in CHD must face new challenges inherent to the emergence of a growing and aging CHD population with changing needs and medical and psychosocial issues. Founded in 1992, the International Society for Adult Congenital Heart Disease (ISACHD) is the leading global organization of professionals dedicated to pursuing excellence in the care of adults with CHD worldwide. Recognizing the unique and varied issues involved in caring for adults with CHD, ISACHD established a task force to assess the current status of care for adults with CHD across the globe, highlight major challenges and priorities, and provide future direction. The writing committee consisted of experts from North America, South America, Europe, South Asia, East Asia, and Oceania. The committee was divided into subgroups to review key aspects of adult CHD (ACHD) care. Regional representatives were tasked with investigating and reporting on relevant local issues as accurately as possible, within the constraints of available data. The resulting ISACHD position statement addresses changing patterns of worldwide epidemiology, models of care and organization of care, education and training, and the global research landscape in ACHD. [ABSTRACT FROM AUTHOR]

Background Data on pharmacological management during pregnancy are scarce. The aim of this study was to describe the type and frequency of cardiac medication used in pregnancy in patients with cardiovascular disease and to assess the relationship between medication use and fetal outcome. Methods and results Between 2007 and 2011 sixty hospitals in 28 countries enrolled 1321 pregnant women. All patients had structural heart disease (congenital 66%, valvular 25% or cardiomyopathy 7% or ischemic 2%). Medication was used by 424 patients (32%) at some time during pregnancy: 22% used beta-blockers, 8% antiplatelet agents, 7% diuretics, 2.8% ACE inhibitors and 0.5% statins. Compared to those who did not take medication, patients taking medication were older, more likely to be parous, have valvular heart disease and were less often in sinus rhythm. The odds ratio of fetal adverse events in users versus non-users of medication was 2.6 (95% CI 2.0-3.4) and after adjustment for cardiac and obstetric parameter was 2.0 (95% CI 1.4-2.7). Babies of patients treated with beta-blockers had a significantly lower adjusted birth weight (3140 versus 3240 g, p = 0.002). The highest rate of fetal malformation was found in patients taking ACE inhibitors (8%). Conclusion One third of pregnant women with heart disease used cardiac medication during their pregnancy, which was associated with an increased rate of adverse fetal events. Birth weight was significantly lower in children of patients taking beta-blockers. A randomized trial is needed to distinguish the effects of the medication from the effects of the underlying maternal cardiac condition. [ABSTRACT FROM AUTHOR]

In adults with congenital heart disease and a systemic right ventricle, subaortic ventricular systolic dysfunction is common. Echocardiographic assessment of systolic right ventricular (RV) function in these patients is important but challenging. The aim of the present study was to assess the reliability of conventional echocardiographic RV functional parameters to quantify the systolic performance of a subaortic right ventricle. We compared 56 contemporary echocardiograms and cardiac magnetic resonance studies in 37 adults, aged 26.9 - 7.4 years, with complete transposition and a subaortic right ventricle. The fractional area change (FAC), lateral tricuspid annular plane systolic excursion, lateral RV systolic motion velocities by tissue Doppler, RV myocardial performance index, and the rate of systolic RV pressure increase (dp/dt) measured across the tricuspid regurgitant jet were assessed by echocardiography and correlated with the cardiac magnetic resonance-derived RV ejection fraction (EF). The mean RVEF was 48.0 - 7.8%. FAC (r2 [ 0.206, p [ 0.001) and dp/dt (r2 [ 0.173, p [ 0.009) significantly correlated with RVEF, and the other nongeometric echocardiographic parameters failed to show a significant correlation with RVEF by linear regression analysis. FAC <33% and dp/dt <1,000 mm Hg/s identified a RVEF of <50% with a sensitivity of 77% and 69% and a specificity of 58% and 87%, respectively. In conclusion, in patients with a systemic right ventricle, routine nongeometric echocardiographic parameters of RV function correlated weakly with cardiac magnetic resonance-derived EF. RV FAC and the measurement of the rate of systolic RV pressure increase (dp/dt) should be preferentially used to assess systemic systolic function in adult patients with a subaortic right ventricle. [ABSTRACT FROM AUTHOR]

Structural abnormalities of the medial aorta have been described for conotruncal defects (e.g., tetralogy of Fallot [TOF] and complete transposition of the great arteries (dextrotransposition [d]-TGA). In TOF, progressive aortic dilation is a frequent finding. In patients with d-TGA with an atrial switch, this problem is less often described. The aim of the present study was to compare the extent of dilative aortopathy and aortic distensibility in adults with an atrial switch procedure (n = 39) to that in adults with repaired TOF (n = 39) and controls (n = 39), using cardiac magnetic resonance imaging. The groups were matched for age and gender. Diameters of the aorta indexed to the body surface area were significantly increased in the patients with d-TGA and TOF compared to that of the controls at the aortic sinus up to the level of the right pulmonary artery. On multivariate testing, the diagnosis of a conotruncal defect (β = 0.260; p = 0.003) and aortic regurgitant fraction (β = 0.405; p <0.001) were independent predictors of an increased aortic sinus diameter. Ascending aorta distensibility was significantly reduced in those with d-TGA and TOF compared to controls: 3.6 (interquartile range 1.5 to 4.4) versus 2.8 (interquartile range 2.0 to 3.7) versus 5.5 (interquartile range 4.8 to 6.9) ×10−3 mm Hg−1 (p <0.001). The independent predictors of ascending aorta distensibility were the diagnosis of a conotruncal defect (p <0.001) and age (p = 0.028). In conclusion, intrinsic aortopathy, manifested as increased ascending aortic diameters and reduced ascending aortic distensibility, is not only evident in adults with TOF, but also in adults with d-TGA and an atrial switch procedure. Long-term follow-up is needed to monitor the aortic size in both patient groups. [Copyright &y& Elsevier]

Objectives: This study sought to investigate the safety and efficacy of transcatheter treatment of atrial septal aneurysm (ASA) associated with patent foramen ovale (PFO). Background: Patients with both ASA and PFO are at high risk for recurrent paradoxical embolism. Methods: The procedural, echocardiographic, and clinical outcomes of 141 patients with ASA + PFO and ≥1 paradoxical embolic event undergoing transcatheter treatment were compared with 220 patients with PFO alone. Results: Device success (ASA + PFO, 99.3%; PFO alone, 99.5%; p = 0.75) and procedural complications (ASA + PFO, 0.7%; PFO alone, 3.2%; p = 0.12) were similar in both groups. Maximal atrial septal excursion in patients with ASA + PFO decreased from 16 ± 4 mm before to 4 ± 3 mm after the intervention (p < 0.0001). At 6 months follow-up, right-to-left shunt was abolished in 120 (86%) patients with ASA + PFO, compared to 187 (85%) patients with PFO alone (p = 0.80). Freedom from recurrent transient ischemic attack, stroke, and peripheral embolism at 4 years was 95% (ASA + PFO) and 94% (PFO alone, p = 0.70), respectively. A residual right-to-left shunt after the intervention was the only predictor for recurrence (hazard ratio [HR] 6.9; 95% confidence interval [CI] 1.3 to 36.9, p < 0.03) in patients with ASA + PFO. Conclusions: Transcatheter treatment of ASA + PFO is safe and effective in patients with paradoxical embolism. The procedure effectively abolishes right-to-left shunt and decreases atrial septal mobility. Long-term prevention of recurrent events appears favorable when compared to patients with PFO alone. [Copyright &y& Elsevier]

The purpose of this study was to compare the efficacy of medical treatment with percutaneous closure of patent foramen ovale (PFO).Patients with cryptogenic stroke and PFO are at risk for recurrent cerebrovascular events.We compared the risk of recurrence in 308 patients with cryptogenic stroke and PFO, who were treated either medically (158 patients) or underwent percutaneous PFO closure (150 patients) between 1994 and 2000.Patients undergoing percutaneous PFO closure had a larger right-to-left shunt (p < 0.001; 95% confidence interval [CI] 1.38 to 3.07) and were more likely to have suffered more than one cerebrovascular event (p = 0.03; 95% CI 1.04 to 2.71). At four years of follow-up, percutaneous PFO closure resulted in a non-significant trend toward risk reduction of death, stroke, or transient ischemic attack (TIA) combined (8.5% vs. 24.3%; p = 0.05; 95% CI 0.23 to 1.01), and of recurrent stroke or TIA (7.8% vs. 22.2%; p = 0.08; 95% CI 0.23 to 1.11) compared with medical treatment. Patients with more than one cerebrovascular event at baseline and those with complete occlusion of PFO were at lower risk for recurrent stroke or TIA after percutaneous PFO closure compared with medically treated patients (7.3% vs. 33.2%; p = 0.01; 95% CI 0.08 to 0.81, and 6.5% vs. 22.2%; p = 0.04; 95% CI 0.14 to 0.99, respectively).Percutaneous PFO closure appears at least as effective as medical treatment for prevention of recurrent cerebrovascular events in cryptogenic stroke patients with PFO. It might be more effective than medical treatment in patients with complete closure and more than one cerebrovascular event. [Copyright &y& Elsevier]