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.

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.

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.

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.

Functional health status of adults with tetralogy of Fallot: matched comparison with healthy siblings.

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.

Maternal cardiovascular events during childbirth among women with congenital heart disease.

OBJECTIVES: To define the epidemiology of adverse cardiovascular events among women with congenital heart disease (CHD) hospitalised for childbirth in the USA. DESIGN AND SETTING: The 1998-2007 Nationwide Inpatient Sample, an administrative dataset representative of overall US hospital admissions, was used to identify hospitalisations for delivery. MAIN OUTCOME MEASURES: Logistic regression was used to estimate ORs for cardiovascular outcomes (arrhythmia, heart failure, cerebrovascular accident, embolism, death or a combined outcome) for women with and without CHD. Covariates included age, number of medical comorbidities, pulmonary hypertension, hospital teaching status, insurance status and method of delivery. RESULTS: Annual deliveries for women with CHD increased 34.9% from 1998 to 2007 compared with an increase of 21.3% in the general population. Women with CHD were more likely to sustain a cardiovascular event (4042/100,000 vs 278/100,000 deliveries, univariate OR 15.1, 95% CI 13.1 to 17.4, multivariable OR 8.4, 95% CI 7.0 to 10.0). Arrhythmia, the most common cardiovascular event, was more frequent among women with CHD (2637/100,000 vs 210/100,000, univariate OR 12.9, 95% CI 10.9 to 15.3, multivariable OR 8.3, 95% CI 6.7 to 10.1). Death occurred in 150/100,000 patients with CHD compared with 8.2/100,000 patients without CHD (multivariable OR 6.7, 95% CI 2.9 to 15.4). Complex CHD was associated with greater odds of having an adverse cardiovascular event than simple CHD (8158/100,000 vs 3166/100,000, multivariable OR 2.0, 95% CI 1.4 to 3.0). CONCLUSIONS: Maternal CHD is associated with a markedly increased risk of adverse cardiovascular events and death during admission for delivery.

Valvular aortic stenosis as a major sequelae in patients with pre-existing subaortic stenosis changing spectrum of outcomes.

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.

Comparison of risk of hypertensive complications of pregnancy among women with versus without coarctation of the aorta.

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.

Successful pregnancies in two women with hypoplastic left heart syndrome.

OBJECTIVE: Hypoplastic left heart syndrome (HLHS) is a relatively common complex congenital heart defect. Prior to development of staged reconstruction (i.e., Norwood procedure), HLHS was almost universally fatal within months of birth. Early survivors of the Norwood procedure are now reaching reproductive age. We report successful pregnancies in two such women. PATIENTS: The first patient was a 20-year-old woman transferred from a community hospital at 33 3/7 weeks gestation because of preterm labor, suspected preeclampsia, and mild chronic hypoxemia. She had normal systemic ventricular shortening without significant valvar regurgitation but severe neoaortic dilatation. A fetal ultrasound demonstrated intrauterine growth restriction. An urgent Cesarean section was performed at 33 6/7 weeks gestation, given breech position and intractable preterm labor. The second patient, a 23-year-old woman followed at this institution through pregnancy, presented with preterm labor at 36 weeks gestation. Her systemic ventricular shortening was normal, with mild tricuspid regurgitation but without neoaortic dilation or regurgitation. She developed active labor at 36 3/6 weeks, and had a spontaneous vaginal delivery of a small for gestational age infant. Both women tolerated labor and childbirth without complication. Neither infant had evidence of structural heart disease on fetal echocardiography or physical examination. CONCLUSIONS: These cases, the first reported successful pregnancies in mothers with HLHS, highlight the challenges of pregnancy among women with complex congenital heart disease in general and raise several considerations specific to HLHS.

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.