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To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.ahj.2009.02.019 Byline: Alexander R. Opotowsky (a), Michael J. Landzberg (b), Stephen E. Kimmel (a)(c), Gary D. Webb (a) Abstract: Percutaneous closure of patent foramen ovale/atrial septal defect (PFO/ASD) is an increasingly common procedure perceived as having minimal risk. There are no population-based estimates of in-hospital adverse event rates of percutaneous PFO/ASD closure. Author Affiliation: (a) Department of Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA (b) Department of Cardiology, Children's Hospital and Department of Medicine, Brigham and Women's Hospital, Boston, MA (c) Department of Biostatistics and Epidemiology, University of Pennsylvania School of Medicine, Philadelphia, PA Article History: Received 9 December 2008; Accepted 26 February 2009

Background: Survival prospects for adults with repaired tetralogy of Fallot (TOF) are now excellent. Attention should therefore shift to assessing and improving functional health status and quality of life. We aimed to assess late functional health status of adults surviving TOF repair by matched comparison to their healthy siblings. Methods: All 1,693 TOF repairs performed at our institution between 1946 and 1990 were reviewed. A matched comparison was undertaken whereby presumed survivors and their healthy sibling were contacted and asked to complete the Ontario Health Survey 1990 and the 36-Item Short Form Health Survey (SF-36) questionnaire. Results: Both questionnaires were completed by 224 adult survivors and their sibling closest in age. Adults with repaired TOF had lower scores for self-perceived general health status ( p < 0.001), were less likely to rate their health as good or excellent ( p < 0.001), and had lower SF-36 scores for physical functioning and general health ( p = 0.001) than their siblings. However, patients reported similar satisfaction with their lives, similar levels of social participation and support, and were as likely to be in long-term partnerships. Worse physical and mental health scores were associated with older age at surgery and at time of questionnaire completion and recent requirement for noncardiac medication. Conclusions: Although reporting lower functional health status then their siblings, quality of life and life satisfaction for adults who underwent surgery for TOF during childhood is comparable to that of their siblings without heart defects. Follow-up of younger adults is required to understand current health outcomes attributable to improvements in the management of TOF. [ABSTRACT FROM AUTHOR]

Background: Late cardiac outcomes in women with aortic stenosis (AS) who have undergone pregnancy have not been well defined. Methods: We examined 51 consecutive women with congenital AS who underwent 70 pregnancies. Late cardiac events (pulmonary edema, cardiac arrhythmia, cardiac death, cardiac interventions >1 year since baseline evaluation) were the outcome of interest. The frequency of late cardiac events in the postpregnant group were compared to age- and lesion-matched women with congenital AS who have never been pregnant. Results: During the follow-up period (6 + 4 years), 43% of women underwent cardiac interventions which comprised all late cardiac events. Independent baseline predictors of late cardiac events were (1) moderate or severe AS (hazard ratio = 4.5, P = .045) and (2) New York Functional Class II (hazard ratio = 4.6, P = .014). When outcomes in 26 women from the postpregnant group were compared to 26 age- and lesion-matched women who have never been pregnant, the postpregnant group had a higher late cardiac event rate than the never-pregnant group (31% vs 0%, P = .021). Conclusion: Women with moderate or severe AS and symptomatic during pregnancy are at high likelihood of requiring cardiac interventions late after pregnancy. Women with congenital AS who have undergone pregnancy have a higher frequency of late cardiac events compared to those who have never been pregnant. Late cardiac outcomes after pregnancy should be considered in the counseling of women with AS who are contemplating pregnancy. [Copyright &y& Elsevier]

Objectives: This study sought to determine what adolescents and young adults with moderate to complex congenital heart disease (CHD) believe their life span to be and to examine correlates of their beliefs. Background: Patients with moderate to complex CHD have a shortened life expectancy. Patients’ perceptions of their life expectancy have not been examined. Methods: Young adults and older adolescents with moderate or complex CHD (n = 296) estimated their own life expectancy and that of healthy peers, and rated their health status and risk of CHD complications. Adults with CHD discussed reasons for life expectancy ratings in an interview. Results: Patients with CHD expected to live to age 75 ± 11 years, only 4 years less than their healthy peers. Over 85% of patients expected to live longer than our estimates of their life expectancy. Poorer health status and higher perceived risk of CHD complications related to shorter perceived life expectancy. Young adults lacked awareness and understanding of CHD-specific risks, and their life expectancy perceptions often related to risk factors for coronary artery disease. Conclusions: Patients with moderate to complex CHD expect to live almost as long as their healthy peers. For most patients, this is unlikely. The implications of these beliefs on health behaviors and life choices are unknown, but should be examined. Nevertheless, patients need accurate information delivered in a sensitive manner to make informed life choices regarding education, careers, and family. [Copyright &y& Elsevier]

Background: We evaluated outcomes in patients requiring late reoperation after tetralogy of Fallot (ToF) repair to identify risk factors for arrhythmia and determine whether arrhythmia surgery decreased the risk of subsequent death or recurrent arrhythmia. Methods: Review was performed of all ToF patients from 1969 to 2005 undergoing reoperation late (>1 year) after repair. Patients with associated lesions, except pulmonary atresia, were included. A total of 249 patients had 278 reoperations. Procedures at initial reoperation included pulmonary valve replacement (PVR) in 217, ablation in 63, and tricuspid valve repair/replacement in 46. Pre-reoperative arrhythmias were present in 75, including supraventricular tachycardia (SVT) in 31, ventricular tachycardia (VT) in 34, and SVT+VT in 10 patients. Results: Median age at reoperation was 23 years (range, 1 to 63). Ten-year survival after reoperation was 93%, and was independent of arrhythmia status (p = 0.86). Arrhythmia patients were characterized by older age at initial repair and at late reoperation, tricuspid and pulmonary regurgitation, and longer QRS duration (p < 0.001 for all). Risk factors for post-reoperative recurrent arrhythmia were longer QRS duration and not having PVR. Longer QRS duration, with a cut-point of more than 160 msec, was associated with recurrent SVT (p = 0.004). Supraventricular tachycardia ablation improved arrhythmia-free survival (75% versus 33%, p < 0.001) but VT ablation did not (96% versus 95%, p = 0.50). However, recurrent VT occurred in only 3 patients (10%). Conclusions: Late mortality in patients undergoing reoperation after ToF repair is not impacted by pre-reoperative arrhythmia. Prolongation of QRS identifies patients at risk for recurrent VT and SVT, but recurrent VT is uncommon. Early PVR, and surgical ablation in patients with SVT, decreases arrhythmic risk. [Copyright &y& Elsevier]

: BackgroundPalliative atrial switch (PAS) procedures that reroute pulmonary and systemic venous drainage and leave a ventricular septal defect (VSD) open have been used in the treatment of deeply cyanotic patients who have severe pulmonary vascular obstructive disease (PVOD). Palliative atrial switch is beneficial for patients with transposition of the great arteries or other complex lesions with VSD who show higher arterial oxygen saturation in the pulmonary artery than in the aorta (transposition hemodynamics/unfavorable streaming). We reviewed the early and late results of PAS (Mustard, n = 25; Senning, n = 3) in patients at two institutions.: MethodsBetween April 1965 and March 2000, PAS was performed in 28 cyanotic patients (18 male, 10 female). Median age was 10 years (range, 1 to 27). Mean preoperative pulmonary arterial pressure was 68 mm Hg (range, 30 to 121 mm Hg). Mean systemic arterial oxygen saturation was 65% (range, 47% to 80%). The majority of patients (95%) were in New York Heart Association (NYHA) functional class III or IV preoperatively.: ResultsOverall early mortality was 21%; for patients after 1972 (n = 23), the early mortality was 8.7%. Mean follow-up was 8.3 years (maximum 20). Mean postoperative systemic arterial oxygen saturation was increased significantly to 88% (p < 0.0001). Late survival for early survivors at 5, 10, and 15 years respectively was 84% (59%, 97%), 64% (39%, 88%), and 54% (15%, 72%). The NYHA functional class was significantly improved; 94% of late survivors (n = 17) were in functional class I or II (p = 0.002).: ConclusionsThe PAS operation significantly improves systemic arterial oxygen saturation and quality of life in selected patients with transposition hemodynamics, VSD, and severe PVOD. [Copyright &y& Elsevier]

Background: A study was conducted to determine the long-term results of aortic valve reimplantation to treat aortic root aneurysm. Methods: Prospective follow-up with clinical assessments and echocardiography was done of 167 consecutive patients who had reimplantation of the aortic valve as treatment of aortic root aneurysm. Their mean age was 45 ± 15 years, 78% were men, 38% had Marfan syndrome, 14% had aortic dissection, and 7% had bicuspid aortic valve. The aortic valve was reimplanted into a straight Dacron (Dupont, Wilmington, DE) tube in 89 patients and in a Dacron tube with creation of neoaortic sinuses in 78. Aortic cusp repair was performed in 66 patients, and the free margin was reinforced with a fine Gore-Tex suture (W.L. Gore & Assoc, Flagstaff, AZ) in 36. The mean follow-up was 5.1 ± 3.8 years and was 100% complete. Results: There were two operative and six late deaths. Survival at 10 years was 92% ± 3%. Moderate aortic insufficiency developed in 3 patients, and severe developed in 2. Freedom from moderate or severe aortic insufficiency was 94% ± 4% at 10 years. Two patients required aortic valve replacement. Freedom from aortic valve replacement was 95% ± 4% at 10 years. At the latest follow-up, 90% of the patients were in New York Heart Association functional class I and 10% were in class II. Conclusions: Reimplantation of the aortic valve to treat patients with aortic root aneurysm is associated with excellent long-term survival and low rates of valve-related complications. Reimplantation of the aortic valve is a durable type of aortic valve repair. [Copyright &y& Elsevier]

: BackgroundAortic valve-sparing operations are an alternative to aortic root replacement in patients with aortic root aneurysms, or aortic valve replacement and supracoronary replacement of the ascending aorta in patients with ascending aorta aneurysms and dilated sinotubular junctions with consequent aortic insufficiency.: MethodsFrom 1988 to 2001, 230 patients underwent aortic valve-sparing operations for aortic root aneurysms (151 patients) or ascending aortic aneurysms with aortic insufficiency (79 patients). Two types of aortic valve-sparing operations were performed in patients with aortic root aneurysms: reimplantation of the aortic valve and remodeling of the aortic root. Mean follow-up was 3.8 ± 2.8 years.: ResultsPatients with aortic root aneurysms were younger, had less severe aortic insufficiency, less extensive vascular disease, and better left ventricular function than patients with ascending aorta aneurysms. The 8-year survival was 83% ± 5% for the first group and 36% ± 14% for the second. The freedom from aortic valve reoperation at 8 years was 99% ± 1% for the first group and 97% ± 2% for the second. In patients who had aortic root aneurysms, 3 developed severe aortic insufficiency (AI), and 15 developed moderate AI, for an 8-year freedom from significant AI of 67% ± 7%. But freedom from AI was 90% ± 3% after the technique of reimplantation, and 55% ± 6% after the technique of remodeling (p = 0.02). In patients with ascending aortic aneurysms, the freedom from AI greater than 2+ at 8 years was 67% ± 11%.: ConclusionsThe long-term results of aortic valve sparing for aortic root aneurysms are excellent, and reimplantation of the aortic valve may provide a more stable repair of the aortic valve than remodeling of the aortic root. [Copyright &y& Elsevier]

Objectives: The purpose of this study was to better define the epidemiology of hospitalizations for adults with congenital heart disease (ACHD) in the U.S. Background: There is a growing population of ACHD as the result of advances in pediatric care and diagnostic testing. Methods: We used nationally representative data from the 1998 to 2005 Nationwide Inpatient Sample to identify patients ≥18 years of age admitted to an acute care hospital with an International Classification of Diseases-9th Revision code designating a CHD diagnosis. National estimates of hospitalizations and total hospital charges by year were calculated. Results: The number of ACHD hospitalizations increased 101.9% from 35,992 ± 2,645 in 1998 to 72,656 ± 5,258 in 2005. During this period, the annual number of admissions grew for both simple (19,448 ± 1,614 to 44,707 ± 3,644) and complex (12,507 ± 1,172 to 19,973 ± 1,624) diagnoses. The percentage of admissions originating in the emergency department (41.7 ± 0.8%) or involving cardiac surgery (17.7 ± 0.7%) remained stable during the study period. The average patient age (52.3 ± 0.8 years to 53.8 ± 0.6 years, p < 0.0001) and proportion of patients with ≥2 medical comorbidities (23.3 ± 0.9% to 33.0 ± 0.7%, p < 0.0001) increased. Mean hospital charges per hospitalization increased 127% from $19,186 ± $803 to $43,496 ± $2,166, and the estimated total national charges for these hospitalizations increased 357% from $691 million in 1998 to $3.16 billion in 2005 (in inflation-adjusted 2005 dollars). Conclusions: The number of hospital admissions for ACHD in the U.S. more than doubled between 1998 and 2005. Hospital charges attributable to these admissions have grown even more dramatically. [Copyright &y& Elsevier]

Abstract: Background and objectives: 22q11.2 Deletion Syndrome (22q11.2DS) is an important genetic syndrome to cardiologists yet remains under-recognized in adults. There is no evidence-based guideline for genetic testing referrals. Feasibility issues in many jurisdictions preclude testing for 22q11.2 deletions in every congenital cardiac patient. We aimed to determine an optimal combination of extracardiac features that could be clinically helpful in identifying adults with tetralogy of Fallot (TOF) and related conotruncal anomalies at highest risk for 22q11.2DS. Methods: Adults (n =103) at a congenital cardiac clinic (86 with TOF) had a brief clinical screening assessment and genetic testing for 22q11.2 deletions using standard fluorescence in-situ hybridization; 31 had a 22q11.2 deletion. Discriminant ability (DA), defined as (sensitivity+specificity)/2, was used to measure performance of 18 (17 clinical and one demographic) features in predicting 22q11.2DS (DA>80%=a good screening test). Results: Combining two features was required for a good test: a global impression of 22q11.2DS dysmorphic facies, with either learning difficulties (DA=82.4%) or voice abnormalities such as hypernasality (DA=81.6%). A four-feature combination (suggestive dysmorphic facies, voice abnormalities, learning difficulties and age <30 years) yielded maximal sensitivity (100%) and DA>85% at a cut-off of three features. Neither rates of right aortic arch or cardiac surgery differed between patients with and without 22q11.2 deletions. Conclusions: Clinicians who consider as few as two extracardiac features readily detectable in a brief clinical encounter could help identify those with 22q11.2DS among adults with congenital heart disease. Diagnosis of 22q11.2DS is important for optimizing management of these complex patients. [Copyright &y& Elsevier]

Background: Specialized tertiary care facilities developed in response to the increasing numbers of adults with congenital heart disease (CHD). Because this patient population comprises a relatively new area of specialized cardiovascular interest, the first facilities necessarily evolved without preexisting guidelines or interaction. Objectives: To characterize the major features of the six original and largest tertiary adults CHD facilities. Methods: Written questionnaire sent to six participating facilities in North America and Europe. Information was analyzed centrally. Results: All but one facilities was established over 20 years ago, and each cares for over 1500 patients. Hospital admissions ranged from 100 to 660 patients/unit/year. Of the total number of registered patients, 52–81% had undergone one or more reparative surgeries. Reoperations constituted 25–80% of the 50–170 operations/unit/year. Overall mean surgical mortality was 1.9%/year. Inpatient and outpatient care was provided in adult (n=4) or both adult and pediatric (n=2) settings. All six facilities enjoyed close collaboration between adult and pediatric cardiologists, cardiac surgeons, nurse specialists and cardiac and non-cardiac consultants. Training and research were pivotal activities. Conclusions: Provision of comprehensive care by multidisciplinary teams including adult and pediatric cardiologists, cardiac surgeons, specialized nurses and other cardiac and non-cardiac consultants was the unifying feature for all six tertiary care facilities reported here. There were minor differences among them based on available resources, local expertise and national health care policies. There appears to be a significant shortfall in tertiary care provision for the adult with CHD that requires further planning and resource allocation. These data may be useful for new and evolving adult CHD services. [Copyright &y& Elsevier]

Objectives: The purpose of this study was to determine the prevalence of valvular aortic stenosis requiring surgery in patients with a pre-existing diagnosis of subaortic stenosis. Background: Classic teachings emphasize aortic regurgitation as the most common complication associated with discrete subaortic stenosis. We hypothesized that significant aortic stenosis may also be an important valve lesion associated with this condition. Methods: Clinical outcomes in patients with subaortic stenosis were examined. The primary outcome of interest was the prevalence of valvular aortic stenosis requiring surgery (surgical valvotomy or valve replacement). Logistic regression was used to identify variables associated with the need for surgery for aortic stenosis. Results: One hundred twenty-one adults with subaortic stenosis (median age 32 years) were evaluated in our clinic. Associated lesions were common: 23% had bicuspid valves and 21% had coarctation of the aorta. Seventy-nine percent of the patients had at least 1 surgical resection of subaortic tissue (median age 12 years). Moderate to severe aortic regurgitation was present in 16% of patients (19 of 121), 3 of whom required surgical intervention in adulthood. Twenty-six percent of patients (32 of 121) required surgery for valvular aortic stenosis. Valve surgery for aortic stenosis was more common in patients with concomitant bicuspid aortic valve disease (p = 0.008), coarctation of the aorta (p = 0.03), and supravalvular stenosis (p = 0.02). Conclusions: Valvular aortic stenosis is a surprisingly common finding in patients with discrete subaortic stenosis. Careful clinical follow-up of this population to monitor aortic valve status continues to be warranted even after a successful surgical resection. [Copyright &y& Elsevier]

Hypertension is a common consequence of coarctation of the aorta. The frequency of hypertensive complications of pregnancy in women with coarctation in the general population is undefined. In this study, we used the 1998 to 2007 Nationwide Inpatient Sample, a nationally representative data set, to identify patients admitted to an acute care hospital for delivery. The frequency of hypertensive complications of pregnancy was compared between women with and without coarctation. Secondary outcomes, including length of stay, hospital charges, Caesarean delivery, and adverse maternal outcomes, were also assessed. There were an estimated 697 deliveries among women with coarctation, compared to 42,601,409 deliveries by women without coarctation. The frequency of hypertensive complications of pregnancy was 24.1 ± 3.3% for women with coarctation compared to 8.0 ± 0.1% for women without coarctation (multivariate odds ratio [OR] 3.6, 95% confidence interval [CI] 2.5 to 5.2). Preexisting hypertension complicating pregnancy (10.2 ± 2.5% vs 1.0% ± 0.02%, multivariate OR 10.8, 95% CI 5.9 to 19.8) and pregnancy-induced hypertension (13.9 ± 3.0% vs 7.0% ± 0.1%, multivariate OR 2.1, 95% CI 1.3 to 3.3) were more common in women with coarctation. Women with coarctation were more likely to deliver by Caesarean section (41.6 ± 3.3% vs 26.4% ± 0.2%, multivariate OR 2.0, 95% CI 1.4 to 2.8), have adverse cardiovascular outcomes (4.8 ± 2.2% vs 0.3 ± 0.01%, multivariate OR 16.7, 95% CI 6.7 to 41.5), have longer hospital stays, and incur higher hospital charges (both p values <0.0001) than women without coarctation. In conclusion, women with coarctation are more likely to have hypertensive complications of pregnancy, deliver by Caesarean section, have adverse cardiovascular outcomes, have longer hospitalizations, and incur higher hospital charges than women without coarctation. [Copyright &y& Elsevier]

Even after successful repair, hypertension is one of the main determinants of cardiovascular morbidity and mortality in patients with aortic coarctation (CoA). We compared the effect of candesartan (angiotensin II receptor blockade) and metoprolol (β-adrenergic receptor blockade) on blood pressure, large artery stiffness, and neurohormonal status in hypertensive patients after repair of CoA. In the present open-label, crossover study, hypertensive patients after CoA repair were first randomly assigned to treatment with candesartan 8 mg or metoprolol 100 mg once per day. After 8 weeks of treatment with one of the drugs, the other treatment was given for 8 weeks. The treatment effects were assessed with 24-hour ambulatory blood pressure monitoring, measurement of large artery stiffness, and neurohormonal plasma levels at baseline and after 8 weeks of either treatment. Sixteen patients (mean age 37 ± 12 years, 26 ± 15 years after repair, 63% men) completed the study. The 24-hour mean arterial pressure at baseline was 97.7 ± 6.2 mm Hg. Metoprolol (mean dose 163 ± 50 mg/day) decreased the mean arterial pressure (7.0 ± 4.2 and 4.1 ± 3.6 mm Hg, respectively) more than did candesartan (mean dose 13 ± 4 mg/day; p = 0.018, 95% confidence interval 0.6 to 5.5). Large artery stiffness did not change with either treatment. With metoprolol, plasma B-type natriuretic peptide increased and plasma renin decreased. With candesartan, the plasma renin and noradrenaline levels increased and aldosterone levels decreased. In conclusion, in adult hypertensive patients after CoA repair, metoprolol had more of an antihypertensive effect than did candesartan. Moreover, the neurohormonal outcome did not support a significant role for the renin-angiotensin system in the causative mechanism of hypertension after CoA. [Copyright &y& Elsevier]

Abstract: Objectives: To examine the sexual behaviors and reproductive concerns among patients with moderate to complex congenital heart disease (CHD). Background: There is a growing need to understand and address the psychosocial issues for older adolescents and young adults with CHD. Emerging sexuality is an issue for this age group and pregnancy for many women with CHD is risky. But, patients'' sexual behavior and reproductive concerns have not been studied. Methods: Young adults (19–20 years old; n =212) and adolescents (16–18 years old; n =144) with moderate to complex CHD reported their sexual behaviors and reproductive concerns. Data were compared to normative samples from Canada and the United States. Results: Few adolescents (14%) but many young adults (48%) with CHD were sexually active (at least one partner in the previous 3 months). These rates were lower than those of their healthy peers. Among the sexually active patients, 36% of the young adults and 72% of the adolescents engaged in one or more types of potentially risky sexual behavior (i.e., two or more partners in the past 3 months, questionable birth control, using drugs or alcohol before sex at least sometimes). Women with complex CHD had the highest levels of concern regarding their fertility and risk of genetic transmission of CHD, as well as concerns about adverse effects of pregnancy on their own health. Conclusions: Sexual health should be discussed with adolescents and young adults with CHD. Particular attention should be given to discussing sexual health with women who have complex CHD. [Copyright &y& Elsevier]

Atrial tachyarrhythmias (ATs) contribute substantially to morbidity in adult patients with secundum atrial septal defects (ASDs). The purpose of this study was to prospectively determine the incidence of AT in adults with an ASD and identify predictors of AT occurrence after closure. This was a prospective study of 200 adult patients undergoing closure of a secundum ASD. Arrhythmic events were defined as sustained or symptomatic AT requiring treatment. Twenty percent of patients (mean age 50 ± 17 years; 26% men) referred for ASD closure had a history of AT. Early follow-up was available for 90% of patients, and the prevalence of AT was 17%. Of 171 patients with late follow-up (mean 1.9 ± 0.9 years), data were available for 90%. AT was detected in 16% of these patients. Closure resulted in alleviation of symptoms (p <0.001), but symptoms alone did not identify patients at risk of recurrent AT. After closure of the ASD, the likelihood of remaining arrhythmia free was highest in patients without a history of AT (p = 0.001) and those <40 years at closure (p = 0.04). In conclusion, transcatheter ASD closure in patients without a history of arrhythmias and those <40 years of age conferred the highest likelihood of a patient remaining arrhythmia free in follow-up. An arrhythmia-specific treatment strategy should be considered for patients with documented established AT before ASD closure, in addition to shunt relief. [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]

Objectives: The purpose of this retrospective study was to define long-term outcomes after pacemaker therapy in adults with congenital heart disease (CHD).Background: Adults with CHD represent a unique and expanding population. Many will require pacemaker or implantable defibrillator therapy, with a lifelong need for re-intervention and follow-up. They pose technical and management challenges not encountered in other groups receiving pacing, and the complication and re-intervention rates specific to this population are not well-defined.Methods: We reviewed outcomes of 168 adults with CHD, 89 females, mean age 40 years, in whom a pacemaker or anti-tachycardia device was implanted.Results: Mean age at implant was 28 years with mean pacing duration 11 years at follow-up (range, 0.5 to 38.0). Seventy-two (42%) received initial dual-chamber devices and remained in this mode, while 23 (14%) went from ventricular to dual-chamber pacing in follow-up. Initial mode of pacing did not have a significant effect on subsequent atrial arrhythmia. Patients receiving an initial epicardial system were younger than those paced endocardially (17 ± 12 years vs. 35 ± 16 years, p < 0.001) and more likely to undergo re-intervention (p = 0.019). Difficulty with vascular access was encountered in 25 patients (15%), while 45 (27%) experienced lead-related complications. No significant predictors of lead complications were identified.Conclusions: Lead complications were not significantly different for epicardial versus endocardial, nor physiologic versus ventricular pacing, but a trend toward improved lead survival in patients receiving endocardial leads at first implant was observed. Adults with CHD remain at risk for atrial arrhythmias regardless of pacing mode. [Copyright &y& Elsevier]

: ObjectivesWe sought to determine the prevalence of pulmonary artery thrombosis in patients with Eisenmenger syndrome and to identify individuals at highest risk.: BackgroundEisenmenger syndrome is associated with pulmonary arterial thrombus formation. Both the prevalence and the determinants of pulmonary arterial thrombosis are unknown.: MethodsThis is a review of patients with Eisenmenger syndrome seen at the Toronto Congenital Cardiac Centre for Adults, Canada. Patients underwent a contrast-enhanced computed tomographic (CT) scan of the thorax.: ResultsForty-nine consecutive patients with Eisenmenger syndrome were seen in our hospital. Fifteen patients did not undergo CT angiograms; therefore, 34 patients (mean age 42 ± 10 years) were included in the study. Responsible shunts included ventricular septal defect (65%), atrial septal defect (15%), patent ductus arteriosus (9%), and other (11%). The prevalence of proximal pulmonary artery thrombus was 21% (7/34) of patients. Evidence of more distal vessel thrombosis was observed in 43% (3/7) of the patients who had visible thrombus in the proximal pulmonary arteries. Patients with thrombus were more likely to be female (86% vs. 37%, p = 0.04) and to have lower oxygen saturations (72% ± 9% vs. 85% ± 6%, p = 0.01). Differences in functional status did not identify patients at highest risk for thrombosis.: ConclusionsPatients with Eisenmenger syndrome have a substantial risk of pulmonary artery thrombus formation. Women and patients with lower oxygen saturations are at the highest risk of developing thrombosis. In the context of an increased bleeding tendency in these patients, the role of anticoagulation treatment needs to be determined. [Copyright &y& Elsevier]

Objectives: The study was done to determine the prevalence of pulmonary emboli (PE) in asymptomatic adult Fontan patients and to identify the risk factors associated with PE.Background: Right atrial thrombi and systemic thromboembolic complications have been reported after the Fontan procedure. However, the frequency of silent PE in this patient population is not known.Methods: All consecutive adult Fontan patients attending the adult congenital clinic over a six-month period underwent ventilation-perfusion (VQ) scanning and blood testing for thrombophilia tendency. If the VQ scan showed an intermediate or high probability for PE, a computerized tomography (CT) pulmonary angiogram was performed to confirm the presence of PE.Results: Thirty patients (mean age 26 +/- 7 years, 57% men) were included in this study. Five (17%) adult Fontan patients had an intermediate or high probability for PE on VQ scan, all of which were confirmed on CT pulmonary angiography. No patient had a thrombophilia tendency. Pulmonary emboli were not present in any patients (30%) taking warfarin. Late age at time of Fontan operation (19 +/- 6 years vs. 11 +/- 6 years, p = 0.012) and type of Fontan anatomy (p = 0.001) were associated with increased risk of silent PE.Conclusions: Seventeen percent of adult patients with Fontan procedure have clinically silent PE. The long-term hemodynamic implications of this with respect to Fontan attrition over time are unknown. Large randomized prospective studies looking at anticoagulation therapy in all Fontan patients are urgently needed. [ABSTRACT FROM AUTHOR]

: ObjectivesThe purpose of this study was to determine if left ventricular (LV) systolic dysfunction was also a predictor of sudden cardiac death (SCD) in adults late after repair of tetralogy of Fallot (TOF).: BackgroundPrevious studies looking at risk factors for SCD in adults with repair of TOF have focused on the right ventricle (RV).: MethodsA retrospective chart review of patients assessed at the Toronto Congenital Cardiac Centre for Adults was performed. Twelve adult patients with repaired TOF and SCD were identified (SCD group). A total of 125 living adult patients with repaired TOF were randomly selected for comparison (control group).: ResultsPatients with SCD were more likely to exhibit moderate or severe pulmonary regurgitation (92% vs. 51%, p = 0.02), have a history of sustained ventricular tachycardia (42% vs. 6%, p < 0.01), and have a QRS ≥180 ms (56% vs. 13%, p = 0.02). Moderate or severe LV systolic dysfunction was also significantly more common in patients with SCD than in the control group (42% vs. 9%, p < 0.01) with a positive predictive value of 29%. The combination of moderate or severe LV systolic dysfunction and QRS ≥180 ms had a positive and negative predictive value for SCD of 66% and 93%, respectively.: ConclusionsModerate or severe LV systolic dysfunction is significantly more common in adult patients with repaired TOF and SCD. The combination of QRS ≥180 ms and significant LV systolic dysfunction has high positive and negative predictive value for SCD. The implication of the role of prophylactic antiarrhythmic implantable cardiac defibrillator insertion in these patients needs further elucidating. [Copyright &y& Elsevier]

We determined the prevalence of sustained atrial tachyarrhythmia (AT) in adults late after repair of tetralogy of Fallot (ToF) and examined its impact on subsequent heart failure, reoperation, and mortality. Ventricular arrhythmias are associated with increased morbidity and mortality in patients with repair of ToF. The clinical impact of AT in this population has not been established. A retrospective cohort study of 242 patients with repaired ToF identified 29 patients (prevalence of 12%) with sustained episodes of AT. Patients with repaired ToF but without sustained arrhythmia (n = 213) constituted a comparison group. Baseline characteristics and clinical outcomes in the 2 groups were compared. An echocardiographic analysis compared 15 patients with AT and 15 matched for age at operation and timing of echocardiography. The development of AT was associated with substantial morbidity including congestive heart failure, reoperation, subsequent ventricular tachycardia, stroke, and death (combined events, 20 of 29 patients [69%]). The rate of combined events (congestive heart failure, stroke, and deaths) in the 213 "arrhythmia-free" patients was 30% (64 of 213 patients). Event-free survival after repair was 18 +/- 2 years for the AT group and 28 +/- 1 years for the arrhythmia-free group (p < 0.001). Patients with AT were older at surgical repair (25 +/- 16 vs 10 +/- 9 years, p = 0.001), and at most recent assessment were aged 48 +/- 12 vs 32 +/- 10 years (p = 0.001). The AT group had a higher mean right atrial volume and proportion of significant pulmonary regurgitation than matched controls. The development of AT in the adult late after ToF repair identifies patients at risk and is associated with older age at repair, a higher frequency of hemodynamic abnormalities, and increased morbidity. [ABSTRACT FROM AUTHOR]

Right ventricular (RV) dysfunction has adverse effects on long-term outcome in patients with repaired tetralogy of Fallot (TOF). We employed serial radionuclide angiography (RNA) to examine RV and left ventricular (LV) systolic function in adults late after TOF repair and its relation to clinical outcome. We reviewed 10-year records of 95 patients (53 men) with TOF followed in our clinic (mean age at repair 12.6 +/- 10.5 years, mean age at last follow-up 37.7 +/- 9.8 years) who underwent at least 2 RNAs between 1987 and 1997. Most patients were well by the end of the study (80% were New York Heart Association class I, 17% were class II, and 3% were in class III). Sixteen patients experienced sustained tachyarrhythmias (8 had atrial; 8 patients had ventricular). One patient died suddenly. Fifteen patients underwent RV outflow reoperations (15 underwent pulmonary valve replacement; 7 had relief of RV outflow obstruction); RV systolic function during exercise in these 15 patients was significantly impaired before and returned to similar levels after surgery, compared with the rest of the patients. Overall, RV and LV function remained stable in the whole group at a mean interval of 5.7 +/- 2.2 years between first and last RNA. This group of closely followed adults with TOF remained well over 10 years with a low incidence of sudden death and stable RV and LV systolic function, despite a relatively large number of RV outflow reoperations. Aggressive intervention for right-sided hemodynamic abnormalities may have contributed to this outcome. Preserved ventricular function may herald a favorable long-term outlook in this group. [ABSTRACT FROM AUTHOR]