Heart University: a new online educational forum in paediatric and adult congenital cardiac care. The future of virtual learning in a post-pandemic world?

Online learning has become an increasingly expected and popular component for education of the modern-day adult learner, including the medical provider. In light of the recent coronavirus pandemic, there has never been more urgency to establish opportunities for supplemental online learning. Heart University aims to be "the go-to online resource" for e-learning in CHD and paediatric-acquired heart disease. It is a carefully curated open access library of paedagogical material for all providers of care to children and adults with CHD or children with acquired heart disease, whether a trainee or a practising provider. In this manuscript, we review the aims, development, current offerings and standing, and future goals of Heart University.

Outcome of pregnancy in patients with structural or ischaemic heart disease: results of a registry of the European Society of Cardiology.

AIMS: To describe the outcome of pregnancy in patients with structural or ischaemic heart disease. METHODS AND RESULTS: In 2007, the European Registry on Pregnancy and Heart disease was initiated by the European Society of Cardiology. Consecutive patients with valvular heart disease, congenital heart disease, ischaemic heart disease (IHD), or cardiomyopathy (CMP) presenting with pregnancy were enrolled. Data for the normal population were derived from the literature. Sixty hospitals in 28 countries enrolled 1321 pregnant women between 2007 and 2011. Median maternal age was 30 years (range 16-53). Most patients were in NYHA class I (72%). Congenital heart disease (66%) was most prevalent, followed by valvular heart disease 25%, CMP 7%, and IHD in 2%. Maternal death occurred in 1%, compared with 0.007% in the normal population. Highest maternal mortality was found in patients with CMP. During pregnancy, 338 patients (26%) were hospitalized, 133 for heart failure. Caesarean section was performed in 41%. Foetal mortality occurred in 1.7% and neonatal mortality in 0.6%, both higher than in the normal population. Median duration of pregnancy was 38 weeks (range 24-42) and median birth weight 3010 g (range 300-4850). In centres of developing countries, maternal and foetal mortality was higher than in centres of developed countries (3.9 vs. 0.6%, P < 0.001 and 6.5 vs. 0.9% P < 0.001) CONCLUSION: The vast majority of patients can go safely through pregnancy and delivery as long as adequate pre-pregnancy evaluation and specialized high-quality care during pregnancy and delivery are available. Pregnancy outcomes were markedly worse in patients with CMP and in developing countries.

Canadian Cardiovascular Society 2022 Guidelines for Cardiovascular Interventions in Adults With Congenital Heart Disease.

Interventions in adults with congenital heart disease (ACHD) focus on surgical and percutaneous interventions in light of rapidly evolving ACHD clinical practice. To bring rigour to our process and amplify the cumulative nature of evidence ACHD care we used the ADAPTE process; we systematically adjudicated, updated, and adapted existing guidelines by Canadian, American, and European cardiac societies from 2010 to 2020. We applied this to interventions related to right and left ventricular outflow obstruction, tetralogy of Fallot, coarctation, aortopathy associated with bicuspid aortic valve, atrioventricular canal defects, Ebstein anomaly, complete and congenitally corrected transposition, and patients with the Fontan operation. In addition to tables indexed to evidence, clinical flow diagrams are included for each lesion to facilitate a practical approach to clinical decision-making. Excluded are recommendations for pacemakers, defibrillators, and arrhythmia-directed interventions covered in separate designated documents. Similarly, where overlap occurs with other guidelines for valvular interventions, reference is made to parallel publications. There is a paucity of high-level quality of evidence in the form of randomized clinical trials to support guidelines in ACHD. We accounted for this in the wording of the strength of recommendations put forth by our national and international experts. As data grow on long-term follow-up, we expect that the evidence driving clinical practice will become increasingly granular. These recommendations are meant to be used to guide dialogue between clinicians, interventional cardiologists, surgeons, and patients making complex decisions relative to ACHD interventions.

Recommendations for cardiovascular magnetic resonance in adults with congenital heart disease from the respective working groups of the European Society of Cardiology.

This paper aims to provide information and explanations regarding the clinically relevant options, strengths, and limitations of cardiovascular magnetic resonance (CMR) in relation to adults with congenital heart disease (CHD). Cardiovascular magnetic resonance can provide assessments of anatomical connections, biventricular function, myocardial viability, measurements of flow, angiography, and more, without ionizing radiation. It should be regarded as a necessary facility in a centre specializing in the care of adults with CHD. Also, those using CMR to investigate acquired heart disease should be able to recognize and evaluate previously unsuspected CHD such as septal defects, anomalously connected pulmonary veins, or double-chambered right ventricle. To realize its full potential and to avoid pitfalls, however, CMR of CHD requires training and experience. Appropriate pathophysiological understanding is needed to evaluate cardiovascular function after surgery for tetralogy of Fallot, transposition of the great arteries, and after Fontan operations. For these and other complex CHD, CMR should be undertaken by specialists committed to long-term collaboration with the clinicians and surgeons managing the patients. We provide a table of CMR acquisition protocols in relation to CHD categories as a guide towards appropriate use of this uniquely versatile imaging modality.

Tetralogy of Fallot.

Tetralogy of Fallot is the most common form of cyanotic congenital heart disease, and one of the first to be successfully repaired by congenital heart surgeons. Since the first procedures in the 1950s, advances in the diagnosis, perioperative and surgical treatment, and postoperative care have been such that almost all those born with tetralogy of Fallot can now expect to survive to adulthood. The startling improvement in outcomes for babies born with congenital heart disease in general-and for those with tetralogy of Fallot in particular-is one of the success stories of modern medicine. Indeed, in many countries adults with tetralogy of Fallot outnumber children. Consequently, new issues have emerged, ranging from hitherto unpredicted medical complications to issues with training for caregivers and resource allocation for this population of survivors. Therefore, evolution of treatment, recognition of late complications, research on disease mechanisms and therapies-with feedback to changes in care of affected children born nowadays-are templates on which the timely discussion of organisation of care of those affected by congenital heart diseases from the fetus to the elderly can be based. Here, we focus on new developments in the understanding of the causes, diagnosis, early treatment, and late outcomes of tetralogy of Fallot, emphasising the continuum of multidisciplinary care that is necessary for best possible lifelong treatment of the 1% of the population born with congenital heart diseases.

Ventricular arrhythmias and sudden death in adults after a Mustard operation for transposition of the great arteries.

AIMS: To examine the prevalence of sustained ventricular tachycardia (VT) and sudden death (SD) in adults with atrial repair of transposition of the great arteries (TGA) and to determine associated risk factors. METHODS AND RESULTS: In a single-centre review, we studied the outcome of 149 adults (mean age 28 +/- 7 years) who had undergone a Mustard operation for TGA. During a mean follow-up of 9 +/- 6 years, sustained VT and/or SD occurred in 9% (13/149) of the cohort. Sustained VT/SD was more likely to occur in patients with associated anatomic lesions [hazard ratio (HR) 4.9, 95% CI 1.5-16.0], with NYHA class >or=III (HR 9.8, 95% CI 3.0-31.6) and with an impaired subaortic right ventricular (RV) ejection fraction (EF) (HR 2.2, 95% CI 1.2-4.0 per 10% decrease in EF). There was an inverse correlation between the RV-EF and both age and QRS duration. Patients with a QRS duration >or=140 ms were at highest risk of sustained VT/SD (HR 13.6, 95% CI 2.9-63.4). Atrial tachyarrhythmia was detected in 66 (44%) patients, but was not a statistically significant predictor of sustained VT/SD in our adult population (HR 2.7, 95% CI 0.6-13.0). CONCLUSION: Sustained VT/SD in adults after a Mustard operation for TGA are more common than previously described. Age, systemic ventricular function, and QRS duration are interrelated and are associated with VT/SD. A QRS duration >or=140 ms helps to identify the high risk patient.

Percutaneous closure of patent foramen ovale and atrial septal defect in adults: the impact of clinical variables and hospital procedure volume on in-hospital adverse events.

BACKGROUND: 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. METHODS: We used nationally representative data from the 2001-2005 Nationwide Inpatient Sample to identify patients >or-=20 years old admitted to an acute care hospital with an International Classification of Diseases, Ninth Revision code designating percutaneous PFO/ASD closure on the first or second hospital day. Variables analyzed included age, sex, number of comorbidities, year, same-day use of intracardiac or other echocardiography, same-day left heart catheterization, hospital size and teaching status, PFO/ASD procedural volume, and coronary intervention volume. Outcomes of interest included length of stay, charges, and adverse events. RESULTS: The study included 2,555 (weighted to United States population: 12,544 +/- 1,987) PFO/ASD closure procedures. Mean age was 52.0 +/- 0.4 years, and 57.3% +/- 1.0% were women. Annual hospital volume averaged 40.8 +/- 7.7 procedures (range, 1-114). Overall, 8.2 +/- 0.8% of admissions involved an adverse event. Older patients and those with comorbidities were more likely to sustain adverse events. Use of intracardiac echocardiography was associated with fewer adverse events. The risk of adverse events was inversely proportional to annual hospital volume (odds ratio [OR] 0.91, 95% confidence interval [CI] 0.86-0.96, per 10 procedures), even after limiting the analysis to hospitals performing >or=10 procedures annually (OR 0.91, 95% CI 0.85-0.98). Adverse events were more frequent at hospitals in the lowest volume quintile as compared with the highest volume quintile (13.3% vs 5.4%, OR 2.42, 95% CI 1.55-3.78). CONCLUSIONS: The risk of adverse events of percutaneous PFO/ASD closure is inversely correlated with hospital volume. This relationship applies even to hospitals meeting the current guidelines, performing >or=10 procedures annually.

Late cardiac outcomes after pregnancy in women with congenital aortic stenosis.

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.

Predictors of atrial arrhythmias after device closure of secundum type atrial septal defects in adults.

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.

Outcomes in adults with bicuspid aortic valves.

CONTEXT: Bicuspid aortic valve is the most common congenital cardiac anomaly in the adult population. Cardiac outcomes in a contemporary population of adults with bicuspid aortic valve have not been systematically determined. OBJECTIVE: To determine the frequency and predictors of cardiac outcomes in a large consecutive series of adults with bicuspid aortic valve. DESIGN, SETTING, AND PARTICIPANTS: Cohort study examining cardiac outcomes in 642 consecutive ambulatory adults (mean [SD] age, 35 [16] years; 68% male) with bicuspid aortic valve presenting to a Canadian congenital cardiac center from 1994 through 2001 and followed up for a mean (SD) period of 9 (5) years. Frequency and predictors of major cardiac events were determined by multivariate analysis. Mortality rate in the study group was compared with age- and sex-matched population estimates. MAIN OUTCOME MEASURES: Mortality and cause of death were determined. Primary cardiac events were defined as the occurrence of any of the following complications: cardiac death, intervention on the aortic valve or ascending aorta, aortic dissection or aneurysm, or congestive heart failure requiring hospital admission during the follow-up period. RESULTS: During the follow-up period, there were 28 deaths (mean [SD], 4% [1%]). One or more primary cardiac events occurred in 161 patients (mean [SD], 25% [2%]), which included cardiac death in 17 patients (mean [SD], 3% [1%]), intervention on aortic valve or ascending aorta in 142 patients (mean [SD], 22% [2%]), aortic dissection or aneurysm in 11 patients (mean [SD], 2% [1%]), or congestive heart failure requiring hospital admission in 16 patients (mean [SD], 2% [1%]). Independent predictors of primary cardiac events were age older than 30 years (hazard ratio [HR], 3.01; 95% confidence interval [CI], 2.15-4.19; P<.001), moderate or severe aortic stenosis (HR, 5.67; 95% CI, 4.16-7.80; P<.001), and moderate or severe aortic regurgitation (HR, 2.68; 95% CI, 1.93-3.76; P<.001). The 10-year survival rate of the study group (mean [SD], 96% [1%]) was not significantly different from population estimates (mean [SD], 97% [1%]; P = .71). At last follow-up, 280 patients (mean [SD], 45% [2%]) had dilated aortic sinus and/or ascending aorta. CONCLUSIONS: In this study population of young adults with bicuspid aortic valve, age, severity of aortic stenosis, and severity of aortic regurgitation were independently associated with primary cardiac events. Over the mean follow-up duration of 9 years, survival rates were not lower than for the general population.

Strength in numbers: Crowdsourcing the most relevant literature in pediatric cardiology.

OBJECTIVE: The growing body of medical literature in pediatric cardiology has made it increasingly difficult for individual providers to stay abreast of the most current, meaningful articles to help guide practice. Crowdsourcing represents a collaborative process of obtaining information from a large group of individuals, typically from an online or web-based community, and could serve a potential mechanism to pool individual efforts to combat this issue. This study aimed to utilize crowdsourcing as a novel way to generate a list of the most relevant, current publications in congenital heart disease, utilizing input from an international group of professionals in the field of pediatric cardiology. DESIGN AND SETTING: All members of the PediHeartNet Google group, an international email distribution list of medical professionals with an interest in pediatric cardiology, were queried in 2017 to submit literature that they considered to be most relevant to their current practice. A Google Form submission platform was used. The articles were evaluated by a multi-institutional panel of four experts in pediatric cardiology using the Delphi method via an electronic evaluation form until a consensus was reached regarding whether the article merited inclusion in the final list. RESULTS: In total, 260 articles were submitted by members of the PediHeartNet Google group. Expert review using the Delphi method resulted in a list of 108 articles. The final collection of articles was published on a publicly available educational website. CONCLUSIONS: Crowdsourcing represents a novel approach for generating a high-yield, comprehensive, yet practical list of the most relevant recent publications in pediatric cardiology. The same techniques could be easily applied to any medical subspecialty. By enlisting the input of frontline providers, the value and relevance of such a list will be significant. A web-based platform for publication of the list allows for real-time updates to ensure continued relevance.

Comparison of echocardiographic and cardiac magnetic resonance imaging for assessing right ventricular function in adults with repaired tetralogy of fallot.

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 > or =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 > or =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.

The impact of pulmonary valve replacement after tetralogy of Fallot repair: a matched comparison.

OBJECTIVE: While there are no controlled studies, pulmonary valve replacement (PVR) after late tetralogy of Fallot repair is performed for patients with symptoms, arrhythmia or ventricular dysfunction. We sought to determine the impact of PVR on clinical outcomes. METHODS: In a matched cohort study, 82 patients with PVR after tetralogy of Fallot repair without a history of ventricular arrhythmia were matched and compared with similarly followed non-PVR control subjects. A propensity-score adjusted analysis using repeated measures regression techniques was performed. RESULTS: For the PVR subjects, the mean age was 28 years with a mean duration follow-up of 9 years. Before PVR, these subjects were significantly more likely than matched non-PVR subjects to have had non-ventricular arrhythmias, symptoms, lower functional class, longer QRS duration, lower right ventricular ejection fraction and higher right ventricular pressure, and reduced exercise duration but not aerobic capacity. During follow-up, sudden death occurred in four non-PVR subjects compared with no PVR subjects, with three episodes of ventricular tachycardia in the PVR subjects versus none in the non-PVR subjects (p=0.49). Symptoms and functional class improved in the PVR subjects with no change in the non-PVR subjects (p<0.001). The change in QRS duration was not significantly different between PVR and non-PVR subjects (p=0.48). Oxygen consumption at peak exercise did not significantly change in either group. For PVR subjects, there was a significant qualitative reduction in pulmonary (p<0.001) and tricuspid valve regurgitation (p=0.009) and right ventricular size (p<0.001) and dysfunction (p<0.001) noted on echocardiography. CONCLUSIONS: Symptoms and functional status are improved after late PVR, with a reduction in pulmonary and tricuspid valve regurgitation and right ventricular size and dysfunction. While a significant impact on arrhythmia was not detected, there were no sudden deaths in the PVR subjects.

Transition and transfer from pediatric to adult care of the young adult with complex congenital heart disease.

This article focuses first on the process of transition and transfer of care of young adults with complex congenital heart disease. It defines the transition process and briefly discusses its history. It reviews the important aspects of transition, outlines the key elements of a successful transition program, and provides a curriculum appropriate for the young adult with congenital heart disease. Finally, it identifies the barriers to transfer of care, discusses the importance of a policy on timing, outlines the components of adult provider services that may be needed, and reviews the steps to an orderly transfer process.

Heart failure and ventricular dysfunction in patients with single or systemic right ventricles.

BACKGROUND: Previous studies suggested a high incidence of congestive heart failure in patients with single and/or systemic right ventricles. The corresponding risk in an adult population is unknown. METHODS AND RESULTS: A cohort of 188 consecutive adult patients with single or systemic right ventricles was prospectively assessed with gated radionuclide angiography (n=135) or 2D echocardiography (n=188) and followed up clinically. Clinical assessment showed 82.4% of the patients were in New York Heart Association class I or II, 13.3% were in class III, and 4.3% were in class IV. Heart failure occurred in 22.2% of patients with transposition of the great arteries and a Mustard procedure, 32.3% of patients with congenitally corrected transposition of the great arteries, and 40% of Fontan-palliated patients. Symptomatic patients had significantly lower anaerobic thresholds (10.3 +/- 2.8 versus 13.2 +/- 4.8 mL center dot kg(-1) center dot min(-1), P=0.006) and peak (center dot)VO(2) (15.2 +/- 4.8 versus 20.3 +/- 6.8 mL center dot kg(-1) center dot min(-1), P<0.00029). Systemic ventricular ejection fraction in symptomatic versus asymptomatic patients at rest was 34.8 +/- 15.7% versus 46.7 +/- 13.4% (P=0.00001). Mortality was 47.1% among symptomatic patients and 5% among asymptomatic patients at 15.7 years of postoperative follow-up. Seven of 12 patients with potentially correctable surgical lesions died or persisted in heart failure despite surgery. Best predictors for mortality were New York Heart Association class, systemic ejection fraction, and age at operation. CONCLUSIONS: Patients with single or systemic right ventricles have significant risk for heart failure accompanied by high mortality. This study suggests the importance of identifying this group of patients who are at risk for heart failure and considering strategies to preserve ventricular function.

Progressive aortic root dilatation in adults late after repair of tetralogy of Fallot.

BACKGROUND: Aortic valve or aortic root (AoRo) replacement is occasionally required because of AoRo dilatation and aortic regurgitation (AR) in repaired tetralogy of Fallot (TOF). We evaluated AoRo size and possible factors associated with its dynamic nature in adults with repaired TOF. METHODS AND RESULTS: Of 216 patients with TOF repair who underwent echocardiography in 1997, we identified 32 patients (mean age, 36+/-8.0 years) with AoRo dilatation, defined as ratio of observed to expected AoRo size by standard nomogram >1.5 (group A), and 54 TOF controls, matched for age with AoRo ratio <1.5 (group B), who underwent at least 1 previous echocardiogram in the preceding 10 years. Mean indexed AoRo size (cm/m2) in 1997 was 2.5+/-0.5 in group A and 1.7+/-0.2 in group B (P<0.0001). AoRo rate of change (mm/year) from the first to 1997 study (mean interval, 5.2+/-3.8 years) was 1.7+/-3.8 in group A and 0.03+/-1.6 in group B (P=0.001). Patients from group A had a longer shunt-to-repair interval (P=0.048) with a higher prevalence of pulmonary atresia (P<0.0001), right aortic arch (P=0.03), moderate to severe AR (P=0.002), aortic valve replacement (P=0.02), larger cardiothoracic ratio (P=0.02), and increased left ventricular end-diastolic dimensions (P=0.002). CONCLUSIONS: A subset of adult TOF exhibits ongoing dilatation of AoRo late after repair. This dilatation relates to previous long-standing volume overload of AoRo and possibly to intrinsic properties of AoRo and may lead to AR. Meticulous follow-up of AoRo after TOF repair is recommended.

Assessment of systemic right ventricular function in patients with transposition of the great arteries using the myocardial performance index: comparison with cardiac magnetic resonance imaging.

BACKGROUND: Assessment of systemic right ventricular (RV) function is a key point in the follow-up of patients with transposition of the great arteries (TGA). Current echocardiographic assessment of RV function is at best an estimate, and cardiac magnetic resonance (CMR) is considered the gold standard. However, this technique is expensive, has limited availability, and requires significant expertise to acquire and interpret the images. The myocardial performance index (MPI) has recently been studied for assessment of pulmonary RV function and shows promise as a simple yet powerful tool for assessing patients with RV dysfunction of various origins. We set out to compare MPI and CMR assessment of systemic RV function in patients with TGA. METHODS AND RESULTS: Data from patients with TGA (11 with congenitally corrected TGA, 18 with surgically corrected TGA) who had CMR within 6 months of their echocardiogram were reviewed. The average systemic RV ejection fraction (RVEF) by CMR was 39.4+/-11.4%, and the systemic RVMPI for this group was 0.56+/-0.21. There was a strong negative correlation between the systemic RVMPI and systemic RVEF by CMR (r=-0.82, P<0.01). The systemic RVEF can be estimated from this formula: RVEF=65%-(45.2xMPI). CONCLUSIONS: MPI can be used in patients with systemic RVs to assess global function and to estimate an EF with good accuracy.

Left ventricular dysfunction is a risk factor for sudden cardiac death in adults late after repair of tetralogy of Fallot.

OBJECTIVES: The 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). BACKGROUND: Previous studies looking at risk factors for SCD in adults with repair of TOF have focused on the right ventricle (RV). METHODS: A 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). RESULTS: Patients 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 > or =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 > or =180 ms had a positive and negative predictive value for SCD of 66% and 93%, respectively. CONCLUSIONS: Moderate or severe LV systolic dysfunction is significantly more common in adult patients with repaired TOF and SCD. The combination of QRS > or =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.