Smoking among adult congenital heart disease survivors in the United States: Prevalence and relationship with illness perceptions.

The relationship between smoking and illness perceptions among congenital heart disease (CHD) survivors is unknown. The primary aims of the present study were to compare the smoking prevalence among CHD survivors to a nationally representative U.S. sample and examine the relationship between smoking and illness perceptions. CHD survivors (N = 744) from six U.S. sites participated in the study. The smoking prevalence among CHD survivors (9.3%) was lower than the general population (15.3%). However, 23.3% of CHD survivors with severe functional limitations smoked. Smoking prevalence differed by U.S. region, with a greater proportion of those attending CHD care in the Midwest reporting smoking (11.8%). The illness perception dimensions of Concern and Emotional Response were independently associated with smoking. Differences in illness perceptions enhance our understanding of smoking among CHD survivors and may guide interventions promoting positive health behaviors. The protocol for the study from which the present analyses were conducted was recorded at ClinicalTrials.gov: NCT02150603.

Patient-Reported Outcomes in Adults With Congenital Heart Disease Following Hospitalization (from APPROACH-IS).

In this international study, we (1) compared patient-reported outcomes (PROs) in adults with congenital heart disease (CHD) who had versus had not been hospitalized during the previous 12 month, (2) contrasted PROs in patients who had been hospitalized for cardiac surgery versus nonsurgical reasons, (3) assessed the magnitude of differences between the groups (i.e., effect sizes), and (4) explored differential effect sizes between countries. APPROACH-IS was a cross-sectional, observational study that enrolled 4,028 patients from 15 countries (median age 32 years; 53% females). Self-report questionnaires were administered to measure PROs: health status; anxiety and depression; and quality of life. Overall, 668 patients (17%) had been hospitalized in the previous 12 months. These patients reported poorer outcomes on all PROs, with the exception of anxiety. Patients who underwent cardiac surgery demonstrated a better quality of life compared with those who were hospitalized for nonsurgical reasons. For significant differences, the effect sizes were small, whereas they were negligible in nonsignificant comparisons. Substantial intercountry differences were observed. For various PROs, moderate to large effect sizes were found comparing different countries. In conclusion, adults with CHD who had undergone hospitalization in the previous year had poorer PROs than those who were medically stable. Researchers ought to account for the timing of recruitment when conducting PRO research as hospitalization can impact results.

ACC/AHA/ASE/HRS/ISACHD/SCAI/SCCT/SCMR/SOPE 2020 Appropriate Use Criteria for Multimodality Imaging During the Follow-Up Care of Patients With Congenital Heart Disease: A Report of the American College of Cardiology Solution Set Oversight Committee and Appropriate Use Criteria Task Force, American Heart Association, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Pediatric Echocardiography.

The American College of Cardiology (ACC) collaborated with the American Heart Association, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and the Society of Pediatric Echocardiography to develop Appropriate Use Criteria (AUC) for multimodality imaging during the follow-up care of patients with congenital heart disease (CHD). This is the first AUC to address cardiac imaging in adult and pediatric patients with established CHD. A number of common patient scenarios (also termed "indications") and associated assumptions and definitions were developed using guidelines, clinical trial data, and expert opinion in the field of CHD.1 The indications relate primarily to evaluation before and after cardiac surgery or catheter-based intervention, and they address routine surveillance as well as evaluation of new-onset signs or symptoms. The writing group developed 324 clinical indications, which they separated into 19 tables according to the type of cardiac lesion. Noninvasive cardiac imaging modalities that could potentially be used for these indications were incorporated into the tables, resulting in a total of 1,035 unique scenarios. These scenarios were presented to a separate, independent panel for rating, with each being scored on a scale of 1 to 9, with 1 to 3 categorized as "Rarely Appropriate," 4 to 6 as "May Be Appropriate," and 7 to 9 as "Appropriate." Forty-four percent of the scenarios were rated as Appropriate, 39% as May Be Appropriate, and 17% as Rarely Appropriate. This AUC document will provide guidance to clinicians in the care of patients with established CHD by identifying the reasonable imaging modality options available for evaluation and surveillance of such patients. It will also serve as an educational and quality improvement tool to identify patterns of care and reduce the number of Rarely Appropriate tests in clinical practice.

Aortic Coarctation.

Aortic coarctation is a discrete narrowing of the thoracic aorta. In addition to anatomic obstruction, it can be considered an aortopathy with abnormal vascular properties characterized by stiffness and impaired relaxation. There are surgical and transcatheter techniques to address the obstruction but, despite relief, patients with aortic coarctation are at risk for hypertension, aortic complications, and abnormalities with left ventricular performance. This review covers the etiology, pathophysiology, diagnosis, and management of adults with aortic coarctation, with emphasis on multimodality imaging characteristics and lifelong surveillance to identify long-term complications.

Health behaviours reported by adults with congenital heart disease across 15 countries.

BACKGROUND: Health behaviours are essential to maintain optimal health and reduce the risk of cardiovascular complications in adults with congenital heart disease. This study aimed to describe health behaviours in adults with congenital heart disease in 15 countries and to identify patient characteristics associated with optimal health behaviours in the international sample. DESIGN: This was a cross-sectional observational study. METHODS: Adults with congenital heart disease (n = 4028, median age = 32 years, interquartile range 25-42 years) completed self-report measures as part of the Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study (APPROACH-IS). Participants reported on seven health behaviours using the Health Behaviors Scale-Congenital Heart Disease. Demographic and medical characteristics were assessed via medical chart review and self-report. Multivariate path analyses with inverse sampling weights were used to investigate study aims. RESULTS: Health behaviour rates for the full sample were 10% binge drinking, 12% cigarette smoking, 6% recreational drug use, 72% annual dental visit, 69% twice daily tooth brushing, 27% daily dental flossing and 43% sport participation. Pairwise comparisons indicated that rates differed between countries. Rates of substance use behaviours were higher in younger, male participants. Optimal dental health behaviours were more common among older, female participants with higher educational attainment while sports participation was more frequent among participants who were younger, male, married, employed/students, with higher educational attainment, less complex anatomical defects and better functional status. CONCLUSIONS: Health behaviour rates vary by country. Predictors of health behaviours may reflect larger geographic trends. Our findings have implications for the development and implementation of programmes for the assessment and promotion of optimal health behaviours in adults with congenital heart disease.

Prevalence and Effects of Cigarette Smoking, Cannabis Consumption, and Co-use in Adults From 15 Countries With Congenital Heart Disease.

BACKGROUND: The prevalence and effects of cigarette smoking and cannabis use in persons with congenital heart disease (CHD) are poorly understood. We (1) described the prevalence of cigarette smoking, cannabis consumption, and co-use in adults with CHD; (2) investigated intercountry differences; (3) tested the relative effects on physical functioning, mental health, and quality of life (QOL); and (4) quantified the differential effect of cigarette smoking, cannabis use, or co-use on those outcomes. METHODS: APPROACH-IS was a cross-sectional study, including 4028 adults with CHD from 15 countries. Patients completed questionnaires to measure physical functioning, mental health, and QOL. Smoking status and cannabis use were assessed by means of the Health Behaviour Scale-Congenital Heart Disease. Linear models with doubly robust estimations were computed after groups were balanced with the use of propensity weighting. RESULTS: Overall, 14% of men and 11% of women smoked cigarettes only; 8% of men and 4% of women consumed cannabis only; and 4% of men and 1% of women used both substances. Large intercountry variations were observed, with Switzerland having the highest prevalence for smoking cigarettes (24% of men, 19% of women) and Canada the highest for cannabis use (19% of men, 4% of women). Cigarette smoking had a small negative effect on patient-reported outcomes, and the effect of cannabis was negligible. The effect of co-use was more prominent, with a moderate negative effect on mental health. CONCLUSIONS: We found significant intercountry variability in cigarette and cannabis use in adults with CHD. Co-use has the most detrimental effects on patient-reported outcomes.

Clinical Experience With the Subcutaneous Implantable Cardioverter-Defibrillator in Adults With Congenital Heart Disease.

BACKGROUND: Sudden cardiac death is a major contributor to mortality for adults with congenital heart disease. The subcutaneous implantable cardioverter-defibrillator (ICD) has emerged as a novel tool for prevention of sudden cardiac death, but clinical performance data for adults with congenital heart disease are limited. METHODS AND RESULTS: A retrospective study involving 7 centers over a 5-year period beginning in 2011 was performed. Twenty-one patients (median 33.9 years) were identified. The most common diagnosis was single ventricle physiology (52%), 9 palliated by Fontan operation and 2 by aortopulmonary shunts: d-transposition of the great arteries after Mustard/Senning (n=2), tetralogy of Fallot (n=2), aortic valve disease (n=2), and other biventricular surgery (n=4). A prior cardiac device had been implanted in 7 (33%). The ICD indication was primary prevention in 67% and secondary in 33% patients. The most common reason for subcutaneous ICD placement was limited transvenous access for ventricular lead placement (n=10) followed by intracardiac right-to-left shunt (n=5). Ventricular arrhythmia was induced in 17 (81%) and was converted with ≤80 Joules in all. There was one implant complication related to infection, not requiring device removal. Over a median follow-up of 14 months, 4 patients (21%) received inappropriate and 1 (5%) patient received appropriate shocks. There was one arrhythmic death related to asystole in a single ventricle patient. CONCLUSIONS: Subcutaneous ICD implantation is feasible for adults with congenital heart disease patients. Most candidates have single ventricle heart disease and limited transvenous options for ICD placement. Despite variable anatomy, this study demonstrates successful conversion of induced ventricular arrhythmia and reasonable rhythm discrimination during follow-up.

Takotsubo Cardiomyopathy in an Adult Woman With Repaired Tetralogy of Fallot.

Takotsubo cardiomyopathy is a reversible form of cardiomyopathy rarely reported in the adult congenital patient. We describe a case of a 49-year-old woman with repaired tetralogy of Fallot who presented with acute dyspnea. A 12-lead electrocardiogram revealed diffuse anterolateral T-wave inversion suggestive of myocardial ischemia. Cardiac catheterization was performed, demonstrating angiographically normal coronary arteries. A cardiovascular magnetic resonance examination showed apical akinesis with associated myocardial edema, but no myocardial damage on late gadolinium enhancement imaging, which is a characteristic of Takotsubo cardiomyopathy. The patient was treated medically. A follow-up echocardiogram demonstrated normalization of left ventricular systolic function and apical wall motion abnormalities.

Coarctation of the Aorta: Strategies for Improving Outcomes.

Coarctation of the aorta is a common congenital heart defect through which management has rapidly evolved over the last few decades. The role of transcatheter-based therapies is expanding and seems to be an effective treatment option for coarctation, especially in adults. Patients with prior coarctation repair are at risk of long-term complications related to prior surgeries and associated congenital heart defects, in particular, the risk of restenosis and aortic aneurysm development related to the timing and mode of prior intervention. This article outlines the evaluation and management of adults with unrepaired coarctation and patients after coarctation repair.

Evaluation by MRA of aortic dilation late after repair of tetralogy of Fallot.

OBJECTIVES: This study evaluated predictors for aortic dilation (AD) in patients with repaired tetralogy of Fallot (rTOF) using magnetic resonance angiography (MRA). BACKGROUND: AD is common in patients with rTOF and may result in increased morbidity and mortality. There are no guidelines for evaluation of AD for rTOF patients. METHODS: All adults with rTOF who previously underwent MRA had retrospective aortic measurements at the sinuses of Valsalva (SoV) and ascending aorta (AsAo). Rate of change in diameter was determined in patients with multiple MRAs. Chart review identified risk factors for AD. Univariate and multivariate analyses tested predictors of AD. RESULTS: Of the 87 patients who met the inclusion criteria, 12 (14%) had AD. At baseline, mean diameter was 3.6 ± 0.6 cm and 3.1 ± 0.6 cm at the SoV and AsAo, respectively. The AsAo was larger than the SoV in 17%. Predictors of AD included male gender, age, right aortic arch, pregnancy, older age at complete repair, smoking, and systemic hypertension. Serial studies were available in 55 patients; the rate of growth was slow: 0.4 ± 0.9 mm/year (SoV) and 0.1 ± 0.8mm/year (AsAo). CONCLUSIONS: AD is common in rTOF at the SoV and AsAo. Transthoracic echocardiography, which does not always image the AsAo as well as MRA, may not image AD in rTOF in cases in which the AsAo is dilated. Although several risk factors correlate with AD in rTOF, the rate of aortic growth is slow, suggesting that rTOF patients may not require frequent aortic imaging.

Correlation of serum biomarkers in adults with single ventricles with strain and strain rate using 2D speckle tracking.

BACKGROUND: With advances in cardiac care, patients with congenital heart disease, including single ventricle (SV) physiology, now survive into adulthood. These patients often suffer from congestive heart failure (CHF) with overexpression of serum biomarkers. Strain and strain rate (SR) may better describe the myocardial mechanics of a failing SV. Our objective was to determine the correlation between strain/SR and biomarkers in adult patients with SV and CHF. METHODS: Adult patients (age ≥8 years) with a SV were enrolled. Strain/SR in a 16-segment model of a SV was measured using 2D speckle echocardiography. Serum levels of interleukin 6, interleukin 8, matrix metalloproteinase 9, procollagen I C-terminal peptide (PCIP), cross-linked carboxy-terminal telopeptide of type I collagen (ICTP), pro-B-type natriuretic peptide, nitrotryrosine, tissue growth factor beta (TGF-ß), tumor necrosis factor alpha, vascular endothelial growth factor, and creatinine (Cr) were measured. Patients underwent a complete 6 Minute Walk Test (MWT). Pearson correlation coefficient was used. P <.05 was considered significant. RESULTS: Ten patients with SV (LV = 7, RV = 3) were enrolled. Mean age was 35.5 years (25-42 years). Mean single ventricular ejection fraction (SVEF) was 47%. ICTP correlated with the basal, mid, and apical anterolateral SR, as well as apical anterior and inferior SR. PCIP correlated with mid anterolateral, basal anteroseptal, and mid inferolateral SR. TGF-ß correlated with apical inferior SR. Cr correlated with mid inferior-septal and apical lateral SR. 6 MWT negatively correlated with the apical anterior septum SR. CONCLUSIONS: ICTP, Cr, and PCIP correlated best with segmental SR values. Our results provide a preliminary platform for future studies to follow the results of treatment modalities using strain/SR and biomarkers for CHF in this population.

The impact of obesity on early postoperative outcomes in adults with congenital heart disease.

BACKGROUND: As the prevalence of obesity continues to increase, it now includes the growing number of patients with congenital heart disease (CHD). This particular obese patient population may pose additional intraoperative as well as postoperative challenges that may contribute to poor outcomes. Our aims were to determine the influence of obesity on morbidity and mortality in adults with CHD undergoing surgical repair at a free standing children's hospital. METHODS: A retrospective analysis of adult (≥18 years) CHD surgery cases from 2002 to 2008 was performed. Congenital heart lesions were defined as mild, moderate, or complex. Patients were categorized by body mass index (BMI): underweight (BMI < 20 kg/m(2)), normal (BMI 20-24.9 kg/m(2)), overweight (BMI 25-29.9 kg/m(2)), and obese (BMI ≥ 30 kg/m(2)). Demographics, incidence of mortality, or specific morbidities were statistically compared using Fisher's exact test and analyses of variance (anovas). RESULTS: In this population (n = 165), overweight (29%) and obese (22%) patients were prevalent. Hypertension (HTN) and pre-HTN were more prevalent in obese and overweight patients. Postoperative renal dysfunction was observed in obese patients with complex CHD (P = .04). Mortality was not different among groups. CONCLUSIONS: Obesity is becoming increasingly common among adults with CHD. Despite marginal evidence of postoperative renal complications in obese patients with CHD of severe complexity, the overall presence of obesity did not influence mortality or short term postoperative morbidities.

Cardiovascular computed tomography and MRI in clinical practice: aortopathy.

The aorta serves as a lifeline for arterial supply throughout the body, the site of potentially fatal mechanical complications, and a mechanistic partner in hypertension and numerous cardiovascular diseases, thus meriting our careful attention. The following clinical vignette illustrates a case of aortic disease, paying homage to the aorta as we begin a series on cardiovascular imaging in the Journal of Cardiovascular Medicine. This series seeks to present contemporary approaches to diagnosis and management in cardiovascular medicine that include state-of-the-art imaging techniques guided by bedside clinical assessment. Incorporating volumetric multidetector computed tomography and dynamic MRI at various stages of this patient's care afforded the timely detection and management of her aortopathy.