Cardiac Risk Score to Predict Small for Gestational Age Infants in Pregnant Women With Heart Disease.

BACKGROUND: One of the most common fetal complications in pregnant women with cardiovascular disease is a small for gestational age (SGA) neonate, which is associated with a higher risk of perinatal morbidity/mortality and poor long-term health outcomes. The objective of this study was to identify cardiac determinants and derive a risk score for clinically relevant SGA < 5th percentile (SGA-5th). METHODS: A prospective cohort of 1812 pregnancies in women with heart disease were studied. SGA-5th was the outcome of interest, defined as birth weight < 5th percentile for gestational age and sex. Multivariable logistic regression analysis was used to identify predictors for SGA-5th. Based on the regression coefficients, a weighted risk score was created. RESULTS: SGA-5th complicated 10% of pregnancies, 11 predictors of SGA-5th were identified, and each was assigned a weighted score: maternal cyanosis (8), Fontan palliation (7), smoking (3), moderate or severe valvular regurgitation (3), ß-blocker use throughout pregnancy (4) or only in the 2nd and 3rd trimesters (2), high baseline ß-blocker dose (4), body mass index < 18.5 kg/m2 (3) or 18.5-24.9 kg/m2 (1), Asian/other ethnicity (2), and significant outflow tract obstruction (1). In the absence of these identified risk factors, the risk of SGA-5th was approximately 4%. Pregnancies with risk scores of 1 had a rate of 5%; 2, 7%; 3, 9%; 4, 12%; 5, 14%; 6, 18%; 7, 23%; 8, 28%; and ≥ 9, 34%. CONCLUSIONS: There are a number of cardiac predictors that are associated with increased risk of SGA-5th. This is a prognostically important outcome, and consideration should be given to routinely predicting and modifying the risk whenever possible.

The American Association for Thoracic Surgery consensus guidelines on bicuspid aortic valve-related aortopathy: Executive summary.

Bicuspid aortic valve disease is a common congenital cardiac disorder, being present in 1% to 2% of the general population. Associated aortopathy is a common finding in patients with bicuspid aortic valve disease, with thoracic aortic dilation noted in approximately 40% of patients in referral centers. Several previous consensus statements and guidelines have addressed the management of bicuspid aortic valve-associated aortopathy, but none focused entirely on this disease process. The current document is an executive summary of "The American Association for Thoracic Surgery Guidelines on Bicuspid Aortic Valve-Related Aortopathy." All major aspects of bicuspid aortic valve aortopathy, including natural history, phenotypic expression, histology and molecular pathomechanisms, imaging, indications for surgery, surveillance, and follow-up, and recommendations for future research are contained within these guidelines. The current executive summary serves as a condensed version of the guidelines to provide clinicians with a current and comprehensive review of bicuspid aortic valve aortopathy and to guide the daily management of these complex patients.

The American Association for Thoracic Surgery consensus guidelines on bicuspid aortic valve-related aortopathy: Full online-only version.

Bicuspid aortic valve disease is the most common congenital cardiac disorder, being present in 1% to 2% of the general population. Associated aortopathy is a common finding in patients with bicuspid aortic valve disease, with thoracic aortic dilation noted in approximately 40% of patients in referral centers. Several previous consensus statements and guidelines have addressed the management of bicuspid aortic valve-associated aortopathy, but none focused entirely on this disease process. The current guidelines cover all major aspects of bicuspid aortic valve aortopathy, including natural history, phenotypic expression, histology and molecular pathomechanisms, imaging, indications for surgery, surveillance, and follow-up, and recommendations for future research. It is intended to provide clinicians with a current and comprehensive review of bicuspid aortic valve aortopathy and to guide the daily management of these complex patients.

Cardiac magnetic resonance imaging characteristics and pregnancy outcomes in women with Mustard palliation for complete transposition of the great arteries.

BACKGROUND: Women with transposition of the great arteries (TGA) following atrial redirection surgery are at risk of pregnancy-associated arrhythmia and heart failure. The cardiovascular magnetic resonance imaging (CMR) characteristics of these women and the relationship of CMR findings to pregnancy outcomes have not been described. METHODS: We included 17 women with atrial redirection surgery and CMR within 2 years of delivery. RESULTS: All women were asymptomatic at baseline (New York Heart Association Class 1). CMR studies were completed pre-pregnancy in 3, antepartum/peripartum in 2, and postpartum in 12 women. Three women (3/17, 18%) experienced major cardiovascular events related to pregnancy: cardiac arrest (n = 1) and symptomatic atrial arrhythmia (n = 2). Median gestational age at delivery was 38 weeks (24-39 weeks) and birth weight was 2770 g (2195-3720 g). Complications were seen in 3 offspring (3/17, 18%): death (n = 1) and prematurity (n = 2). CMR characteristics included median right ventricular end diastolic volume 119 mL/m2 (range 85-214 mL/m2) and median right ventricular ejection fraction (RVEF) 37% (range 30-51%). All women with cardiovascular complications had an RVEF < 35% (range 32-34%). The association between RVEF < 35% and cardiovascular complications trended towards statistical significance (p = 0.05). No statistically significant differences in CMR measurements were found between those with and without neonatal complications. CONCLUSIONS: While the majority of women in this cohort had successful outcomes following pregnancy, important cardiovascular complications were seen in a significant minority, all of whom had an RVEF < 35%. The preliminary findings of our study provide impetus for a larger prospective study to evaluate the prognostic role of CMR in pregnant women with atrial redirection surgery.

Canadian Cardiovascular Society position statement on the management of thoracic aortic disease.

This Canadian Cardiovascular Society position statement aims to provide succinct perspectives on key issues in the management of thoracic aortic disease (TAD). This document is not a comprehensive overview of TAD and important elements of the epidemiology, presentation, diagnosis, and management of acute aortic syndromes are deliberately not discussed; readers are referred to the 2010 guidelines published by the American Heart Association, American College of Cardiology, American Association for Thoracic Surgery, and other stakeholders. Rather, this document is a practical guide for clinicians managing adult patients with TAD. Topics covered include size thresholds for surgical intervention, emerging therapies, imaging modalities, medical and lifestyle management, and genetics of TAD. The primary panel consisted of experts from a variety of disciplines that are essential for comprehensive management of TAD patients. The methodology involved a focused literature review with an emphasis on updates since 2010 and the use of Grading of Recommendations Assessment, Development, and Evaluation methodology to arrive at specific recommendations. The final document then underwent review by a secondary panel. This document aims to provide recommendations for most patients and situations. However, the ultimate judgement regarding the management of any individual patients should be made by their health care team.

New directives in cardiac imaging: imaging the adult with congenital heart disease.

Advances in pediatric surgical and interventional techniques and medical care over the past 50 years have revolutionized the care of children with congenital heart disease. Survival to adulthood is now expected and, as such, there is a growing population of adults which is exceeding the pediatric population with congenital heart disease. Noninvasive cardiac imaging with modalities such as echocardiography, computed tomography, and cardiac magnetic resonance imaging are integral to the care of adults with congenital heart disease. These modalities are used for diagnosis, surveillance for complications late after surgery and catheter-based interventions, and in decision-making for medical, interventional, and surgical therapies. In this review we will discuss noninvasive imaging modalities used to assess congenital cardiac lesions, imaging strategies for select congenital lesions, and comment on the future of cardiac imaging in congenital heart disease.

Bicuspid aortic valve disease.

Bicuspid aortic valve (BAV) disease is the most common congenital cardiac defect. While the BAV can be found in isolation, it is often associated with other congenital cardiac lesions. The most frequent associated finding is dilation of the proximal ascending aorta secondary to abnormalities of the aortic media. Changes in the aortic media are present independent of whether the valve is functionally normal, stenotic, or incompetent. Although symptoms often manifest in adulthood, there is a wide spectrum of presentations ranging from severe disease detected in utero to asymptomatic disease in old age. Complications can include aortic valve stenosis or incompetence, endocarditis, aortic aneurysm formation, and aortic dissection. Despite the potential complications, 2 large contemporary series have demonstrated that life expectancy in adults with BAV disease is not shortened when compared with the general population. Because BAV is a disease of both the valve and the aorta, surgical decision making is more complicated, and many undergoing aortic valve replacement will also need aortic root surgery. With or without surgery, patients with BAV require continued surveillance. Recent studies have improved our understanding of the genetics, the pathobiology, and the clinical course of the disease, but questions are still unanswered. In the future, medical treatment strategies and timing of interventions will likely be refined. This review summarizes our current understanding of the pathology, genetics, and clinical aspects of BAV disease with a focus on BAV disease in adulthood.

Pulmonary valve replacement in repaired tetralogy of Fallot: determinants of early postoperative adverse outcomes.

OBJECTIVE: We sought to examine the frequency of early postoperative complications and preoperative predictors of prolonged hospitalization in adults with repaired tetralogy of Fallot undergoing pulmonary valve replacement. METHODS: This was a retrospective study of consecutive adult patients undergoing surgical intervention between 1995 and 2006. A multivariate logistic regression model was used to identify determinants of prolonged hospitalization. RESULTS: One hundred sixteen patients (mean age, 36 +/- 11 years) underwent 118 pulmonary valve replacements. Most (95%) operations included additional procedures, such as pulmonary artery/outflow tract reconstruction or tricuspid valve annuloplasty. The early postoperative mortality (<30 days) was 2.5%. The majority of the patients (60%) had no postoperative complications. The postoperative adverse events included postoperative arrhythmias (19%), respiratory complications (13%), reoperation during admission (13%), renal dysfunction (13%), and myocardial infarction (3%). Postoperative adverse events were associated with prolonged hospitalization (14 +/- 12 vs 7 +/- 3 days, P = .001). In the multivariate analysis age at reoperation of greater than 45 years (odds ratio, 6.1; 95% confidence interval, 1.6-23.6; P = .009), the number of previous sternotomies (odds ratio, 3.8; 95% confidence interval, 1.4-10; P = .007), and the need for urgent surgical intervention (odds ratio, 5.7; 95% confidence interval, 1.1-27.8; P = .03) were predictors of prolonged hospitalization. CONCLUSION: Pulmonary valve replacement in adults with repaired tetralogy of Fallot has a low mortality risk. The most common early postoperative complications are arrhythmias and respiratory and renal complications. Although most early postoperative complications do not result in long-term sequelae, they are associated with prolonged hospitalization. Patients undergoing urgent interventions, older patients, and those with multiple previous sternotomies are at the highest risk for prolonged hospitalization.

Health behaviors among adolescents and young adults with congenital heart disease.

OBJECTIVES: Document the frequency of substance use and oral hygiene among adolescents and young adults with moderate to complex congenital heart disease (CHD). BACKGROUND: Patients' knowledge of health behaviors, including substance use and oral hygiene, has been examined among patients with CHD, but patients' actual behavior has not been studied. Understanding patients' behavior is needed to inform interventions that enhance healthy lifestyles, and in turn encourage patients to better care for their own health. METHODS: Young adults (19 or 20 years old) and older adolescents (16-18 years old) with moderate or complex CHD (n = 328) reported their substance use (i.e., smoking, marijuana, alcohol, other illicit drugs) and oral hygiene. Rates of these health behaviors were contrasted with comparison samples of peers of the same age. RESULTS: Just over half of the young adults (54%) and over one-quarter of the adolescents (28%) reported significant substance use (i.e., smoking cigarettes on more than 2 days, using marijuana or other illicit drugs at least once, or binge drinking) during the previous 30 days. Rates of significant substance use among the patients with CHD were either comparable to, or lower than, rates in comparison samples of similar aged peers. Only about 15% of the patients with CHD had excellent oral hygiene (i.e., self-reported annual teeth cleaning by dentist, brushing and flossing daily); however, virtually all patients (>95%) brushed daily. Patients with CHD generally had comparable oral hygiene to comparison samples in previously published data. CONCLUSIONS: Many older adolescents and young adults with CHD are engaging in behaviors that may compromise their health. The impact of substance use and poor dental hygiene warrants further investigation. Nevertheless, health behaviors should be routinely discussed in this population.

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.

Mitral annular disjunction in advanced myxomatous mitral valve disease: echocardiographic detection and surgical correction.

Mitral annular disjunction is a structural abnormality of the mitral annulus fibrosus described by pathologists in association with mitral leaflet prolapse and defined as a separation between the atrial wall-mitral valve (MV) junction and the left ventricular attachment allowing for hypermobility of the MV apparatus. The transesophageal echocardiographic characteristics of this abnormality have not been previously described. In patients undergoing MV repair for myxomatous MV degeneration and evaluated using a standardized transesophageal echocardiographic protocol, annular disjunction (mean value 10 +/- 3 mm) was seen at the base of the posterior leaflet in 98% of patients with advanced, and in 9% of patients with mild/moderate MV degeneration. There was a significant correlation between the magnitude of disjunction and the number of segments with prolapse/flail (r = 0.397, P = .001). We found annular disjunction to be a common component of MV apparatus in advanced MV degeneration. Its recognition on transesophageal echocardiography is important to facilitate optimal MV repair. The modification of the repair technique allows surgical correction of the annular disjunction, which seems to optimize long-term results in these challenging cases.

Transthoracic echocardiography does not improve prediction of outcome over APACHE II in medical-surgical intensive care.

PURPOSE: To examine the hypothesis that transthoracic echocardiographic findings predict mortality in critically ill patients. METHODS: A retrospective analysis of concurrently collected data for consecutive patients from May 1996 to May 1998 who had transthoracic echocardiography on or within six months of admission to the medical surgical intensive care (MSICU). We examined the role of physiologic, clinical, and echocardiography variables in predicting the mortality of patients admitted to the MSICU. Three logistic regression models were developed: 1) clinical; 2) echocardiographic; and 3) combined clinical with echocardiographic. Univariate and multivariate analyses were performed and the relative strength of clinical and echocardiographic predictors was compared using odds ratio (OR) and receiver-operator-characteristic (ROC). RESULTS: Of 4,070 MSICU patient admissions, 1,093 patients had transthoracic echocardiography; the study group comprised 942 patients with complete clinical and echocardiographic data. The MSICU mortality was 28%. For the combined model, analyses identified left ventricular systolic function (LVSF), [OR 1.26; confidence interval (CI) 1.01-1.57], severe tricuspid regurgitation (TR) (OR 3.72; CI 1.04-13.24), medical diagnosis (OR 1.91; CI 1.15-3.19), and acute physiology and chronic health evaluation (APACHE) II score (OR 1.27; CI 1.23-1.31), as predictors of MSICU mortality. The combined model yielded an area under ROC curve of 0.913. For the clinical model, analyses identified age (OR 1.04; CI 1.02-1.05) and APACHE II (OR 1.32; 1.26-1.35) as predictors of mortality with an area under ROC curve of 0.917. For the echocardiography model, TR (OR 2.40; 1.08-5.38), severe aortic insufficiency (AI) (OR 4.13; CI 1.17-16.29) and pulmonary hypertension (OR 2.05; 1.01-4.09) were identified as predictors of outcome with an ROC curve of 0.536 for this model. CONCLUSION: Statistical models utilizing clinical variables are predictive of mortality in MSICU. Models that include diagnostic transthoracic echocardiography variables do not provide incremental value to predict ICU mortality. These findings may have implications for non-invasive hemodynamic assessment of critically ill patients, and raise the hypothesis that echocardiography-guided interventions may not alter outcome in ICU.