Variability in the Echocardiographic Evaluation of the Systemic Right Ventricle.

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.

Late Onset Postcapillary Pulmonary Hypertension in Patients With Transposition of the Great Arteries and Mustard or Senning Baffles.

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.

Shone Complex: An Under-recognized Congenital Heart Disease With Substantial Morbidity in Adulthood.

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.

The impact of exercise on ventricular arrhythmias in adults with tetralogy of Fallot.

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.

Risks and Benefits of Exercise Training in Adults With Congenital Heart Disease.

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.

Adult congenital heart disease: a growing epidemic.

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.

Cardiac, obstetric, and fetal outcomes during pregnancy after biological or mechanical aortic valve replacement.

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.

Aortic coarctation and the retinal microvasculature.

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.

Initial clinical experience with the GORE septal occluder for the treatment of atrial septal defects and patent foramen ovale.

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 circulation: success or failure?

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.

Tetralogy of Fallot and aortic root disease.

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.

Impaired arm development after Blalock-Taussig shunts in adults with repaired tetralogy of Fallot.

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.

Multiplanar transesophageal echocardiography for the evaluation and percutaneous management of ostium secundum atrial septal defects in the adult.

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.

Multiplanar transesophageal echocardiography for the evaluation and percutaneous management of ostium secundum atrial septal defects in the adult / Ecocardiografía transesofágica multiplanar para la evaluación y el tratamiento percutáneo de la comunicación interauricular tipo ostium secundum en el adulto

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.

El propósito de esta revisión es analizar la utilidad de la ecocardiografía transesofágica multiplanar antes, durante y después del cierre percutáneo de la comunicación interauricular tipo ostium secundum. Las consideraciones técnicas de imagen durante la ecocardiografía transesofágica multiplanar, su utilidad en la evaluación de los pacientes, la guía peri-procedimiento, la evaluación del éxito técnico y las complicaciones son descritas y discutidas en esta revisión. El cierre percutáneo está indicado en la comunicación interauricular tipo ostium secundum con diámetro máximo de 40 mm. El defecto debe tener una anatomía favorable con bordes de al menos 5 mm. La ecocardiografía transesofágica multiplanar tiene un papel determinante antes del procedimiento para identificar a candidatos potenciales para el cierre percutáneo y para excluir aquéllos con anatomía no favorable o lesiones asociadas que no pueden ser manejados vía percutánea. La ecocardiografía transesofágica multiplanar es importante durante el procedimiento para guiar la liberación del dispositivo. Después de la liberación del dispositivo el ecocardiografista debe evaluar la posición y estabilidad del dispositivo, la presencia de corto-circuito residual, la regurgitación de las válvulas A-V, el retorno venoso sistémico y pulmonar, y el pericardio, a fin de determinar el éxito del procedimiento y descartar complicaciones asociadas.

Structure and process measures of quality of care in adult congenital heart disease patients: a pan-Canadian study.

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.

Pregnancy in women with Fontan physiology.

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.

Recent advances in adult congenital heart disease.

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.

Prediction of complications in pregnant women with cardiac diseases referred to a tertiary center.

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.

Clinical assessment of arrhythmias in tetralogy of Fallot.

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.

Pulmonary arterial hypertension in patients with transcatheter closure of secundum atrial septal defects: a longitudinal study.

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.