The association between cardiac magnetic resonance-derived aortic stiffness parameters and aortic dilation in young adults with bicuspid aortic valve: With and without coarctation of aorta.

Background: Bicuspid aortic valve (BAV) is associated with progressive aortic dilation. Studies in aortopathies have shown a correlation between increased aortic stiffness and aortic dilation. We aimed to evaluate aortic stiffness measures as predictors of progressive aortic dilation by cardiac magnetic resonance (CMR) in BAV patients. Methods: This is a retrospective study of 49 patients with BAV (median age 21.1 years at first CMR visit) with ≥2 CMR at the Wisconsin Adult Congenital Heart Disease Program (WAtCH). Circumferential aortic strain, distensibility, and ß-stiffness index were obtained from CMR-derived aortic root cine imaging, and aortic dimensions were measured at aortic root and ascending aorta. A linear mixed-model and logistic regression were used to identify important predictors of progressive aortic dilation. Results: Over a median of 3.8 years follow-up, the annual growth rates of aortic root and ascending aorta dimensions were 0.25 and 0.16 mm/year, respectively. Aortic strain and distensibility decreased while ß-stiffness index increased with age. Aortic root strain and distensibility were associated with progressive dilation of the ascending aorta. Baseline aortic root diameter was an independent predictor of >1 mm/year growth rate of the aortic root (adjusted OR 1.34, 95 % CI 1.03-1.74, p = 0.028). Most patients (61 %) had coexisting coarctation of aorta. Despite the higher prevalence of hypertension in patients with aortic coarctation, hypertension or coarctation had no effect on baseline aorta dimensions, stiffness, or progressive aortic dilation. Conclusion: Some CMR-derived aortic stiffness parameters correlated with progressive aortic dilation in BAV and should be further investigated in larger and older BAV cohorts.

Cardiac Arrest Secondary to Traumatic Aortopulmonary Window During Transcatheter Pulmonary Valve Implantation in Supported Ross.

We present a patient with a supported Ross procedure and severe pulmonary homograft stenosis who developed cardiac arrest while undergoing transcatheter pulmonary valve replacement and was found to have a large iatrogenic aortopulmonary window. Cardiopulmonary resuscitation was initiated followed by covered stent placement, extracorporeal membrane oxygenation support, and ultimately emergent surgery with a good outcome. (Level of Difficulty: Advanced.).

Impact of cardiac rehabilitation and exercise training programs in coronary heart disease.

Cardiovascular rehabilitation (CR) is the process of developing and maintaining an optimal level of physical, social, and psychological well-being in order to promote recovery from cardiovascular (CV) illness. It is a multi-disciplinary approach encompassing supervised exercise training, patient counseling, education and nutritional guidance that may also enhance quality of life. Beneficial CV effects may include improving coronary heart disease risk factors; particularly exercise capacity, reversing cardiac remodeling, and favorably modifying metabolism and systemic oxygen transport. We review the historical basis for contemporary CR, the indications and critical components of CR, as well as the potential salutary physiological and clinical effects of exercise-based CR.

Comparing SF-36® scores versus biomarkers to predict mortality in primary cardiac prevention patients.

BACKGROUND: Risk stratification plays an important role in evaluating patients with no known cardiovascular disease (CVD). Few studies have investigated health-related quality of life questionnaires such as the Medical Outcomes Study Short Form-36 (SF-36®) as predictive tools for mortality, particularly in direct comparison with biomarkers. Our objective is to measure the relative effectiveness of SF-36® scores in predicting mortality when compared to traditional and novel biomarkers in a primary prevention population. METHODS: 7056 patients evaluated for primary cardiac prevention between January 1996 and April 2011 were included in this study. Patient characteristics included medical history, SF-36® questionnaire and a laboratory panel (total cholesterol, triglycerides, HDL, LDL, ApoA, ApoB, ApoA1/ApoB ratio, homocysteine, lipoprotein (a), fibrinogen, hsCRP, uric acid and urine ACR). The primary outcome was all-cause mortality. RESULTS: A low SF-36® physical score independently predicted a 6-fold increase in death at 8years (above vs. below median Hazard Ratio [95% confidence interval] 5.99 [3.86-9.35], p<0.001). In a univariate analysis, SF-36® physical score had a c-index of 0.75, which was superior to that of all the biomarkers. It also carried incremental predictive ability when added to non-laboratory risk factors (Net Reclassification Index=59.9%), as well as Framingham risk score components (Net Reclassification Index=61.1%). Biomarkers added no incremental predictive value to a non-laboratory risk factor model when combined to SF-36 physical score. CONCLUSION: The SF-36® physical score is a reliable predictor of mortality in patients without CVD, and outperformed most studied traditional and novel biomarkers. In an era of rising healthcare costs, the SF-36® questionnaire could be used as an adjunct simple and cost-effective predictor of mortality to current predictors.

Correlation of the Rutgeerts score and recurrence of Crohn's disease in patients with end ileostomy.

BACKGROUND: Recurrence of Crohn's disease (CD) can occur after surgery, including end ileostomy (EI). The Rutgeerts score (RS) was developed to predict postsurgical CD recurrence via ileocolonoscopy in patients having ileocolonic resection. The role of ileoscopic evaluation via stoma for assessing recurrence of CD has not been investigated. The aim of this study was to evaluate the role of ileoscopy for predicting disease recurrence in CD patients after EI with the use of RS. METHODS: A total of 73 eligible CD patients with at least two ileoscopies in our institution following EI were included. Mucosal inflammation of the neo-terminal ileum was graded based on the RS. The primary outcomes were the need for endoscopic stricture dilation and subsequent surgery due to recurrence of disease. The secondary outcomes were CD-related hospitalization and the need to escalate CD-associated medications. RESULTS: The median duration of CD until EI was 9 years (interquartile range: 4-13 years), and the median duration from EI to the first ileoscopy was 28 months (interquartile range: 11-93 months). The RSs in the neo-terminal ileum close to EI were calculated, and subjects were divided into two groups: the normal RS group with the score being zero (n = 25) and the abnormal RS group with the RS score being ≥1 (n = 48). Patients in the abnormal RS group were more likely to have recurrence of CD (92% vs 27%) and need endoscopic dilation of stricture (40% vs 10%), subsequent bowel surgery (68% vs 15%), disease-related hospitalizations (80% vs 23%) and escalation of CD medications (64% vs 25%) than those in the normal RS group. Time-to-event analysis showed that patients in the abnormal RS group were at a higher risk of endoscopic dilation (odds ratio (OR) = 1.5; 95% CI: 1.09-1.9), need of second bowel surgery (OR = 1.5; 95%CI: 1.2-1.8) and disease-related hospitalizations (OR = 1.3; 95%CI: 1.1-1.6) after adjusting for factors such as duration from surgery to sensor, duration of disease and the patient's sex (all P < 0.001). Further multivariable analysis showed that patients in the abnormal RS group were more likely to need escalation of CD-related medications after adjusting for duration from surgery and age (OR = 5.3; 95% CI: 1.7-16.5; P = 0.004). CONCLUSION: RS can be used to predict the recurrence of CD in patients with EI. A high RS score based on ileoscopy appeared to be associated with poor outcomes. This may be considered a useful decision-making tool for monitoring disease after ileostomy surgery.

Reactive oxygen species production and MAPK activation are implicated in tetrahydrobiopterin-induced SH-SY5Y cell death.

Tetrahydrobiopterin (BH4), an obligatory cofactor for dopamine (DA) synthesis, has been shown to produce reactive oxygen species (ROS) upon its autoxidation and induce selective dopaminergic cell death in many in vivo and in vitro models of Parkinson's disease (PD). The precise molecular mechanisms underlying neuronal death upon BH4 exposure, however, have not yet been well elucidated. The present study aims to examine the intracellular ROS production and the signal transduction pathways underlying the toxic effects of BH4 on human dopaminergic SH-SY5Y cells. The results show that BH4 treatment at concentrations ranging from 50microM to 400microM induces neuronal death in a dose-dependent manner. In concomitant with the elevation of intracellular ROS formation, BH4-induced activation of MAPK, p38 and ERK1/2 in SH-SY5Y cells is attenuated by pretreatment with MAPK inhibitors, SB203580 or PD98059. These data indicate that MAPK activation and oxidative stress are involved in BH4-induced dopaminergic cell death, possibly through the autoxidation of BH4 and subsequent ROS production.