Atenolol versus losartan in children and young adults with Marfan's syndrome

BACKGROUND : Aortic-root dissection is the leading cause of death in Marfan's syndrome. Studies suggest that with regard to slowing aortic-root enlargement, losartan may be more effective than beta-blockers, the current standard therapy in most centers. METHODS : We conducted a randomized trial comparing losartan with atenolol in children and young adults with Marfan's syndrome. The primary outcome was the rate of aortic-root enlargement, expressed as the change in the maximum aortic-root-diameter z score indexed to body-surface area (hereafter, aortic-root z score) over a 3-year period. Secondary outcomes included the rate of change in the absolute diameter of the aortic root; the rate of change in aortic regurgitation; the time to aortic dissection, aortic-root surgery, or death; somatic growth; and the incidence of adverse events. RESULTS : From January 2007 through February 2011, a total of 21 clinical centers enrolled 608 participants, 6 months to 25 years of age (mean [+/- SD] age, 11.5 +/- 6.5 years in the atenolol group and 11.0 +/- 6.2 years in the losartan group), who had an aorticroot z score greater than 3.0. The baseline-adjusted rate of change (+/- SE) in the aortic-root z score did not differ significantly between the atenolol group and the losartan group (-0.139 +/- 0.013 and -0.107 +/- 0.013 standard-deviation units per year, respectively; P = 0.08). Both slopes were significantly less than zero, indicating a decrease in the degree of aortic-root dilatation relative to body-surface area with either treatment. The 3-year rates of aortic-root surgery, aortic dissection, death, and a composite of these events did not differ significantly between the two treatment groups. CONCLUSIONS : Among children and young adults with Marfan's syndrome who were randomly assigned to losartan or atenolol, we found no significant difference in the rate of aorticroot dilatation between the two treatment groups over a 3-year period

International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes

This International Consensus Classification and Nomenclature for the congenital bicuspid aortic valve condition recognizes 3 types of bicuspid valves: 1. The fused type (right-left cusp fusion, right-non-coronary cusp fusion and left-non-coronary cusp fusion phenotypes); 2. The 2-sinus type (latero-lateral and antero-posterior phenotypes); and 3. The partial-fusion (forme fruste) type. The presence of raphe and the symmetry of the fused type phenotypes are critical aspects to describe. The International Consensus also recognizes 3 types of bicuspid valve-associated aortopathy: 1. The ascending phenotype; 2. The root phenotype; and 3. Extended phenotypes.Cardiolog

Lesson learned from the International Registry of Acute Aortic Dissection (IRAD)

Acute aortic dissection (AAD) is a life-threatening disorder that is challenging to diagnose and with defined optimal therapies and outcomes. It is associated with high morbidity and mortality rates, in spite of new management approaches developed over the past few decades. In this article we review lessons learned from the 20-year IRADRegistry experience and its nearly 100 publications. The International Registry of Acute Aortic Dissection (IRAD) is an ongoing registry, which was established in 1996 with the aim to collect data and provide information on this rare disease in order to provide better decision making on clinical practice and surgical management. Acute aortic dissection, including intramural hematoma, is defined as onset of symptoms within 14 days to hospital admission. After 20 years of enrollment, more than 8000 patients with AAD have been collected worldwide, roughly two-thirds with type Aand one-third with type B. Data shows trend for a reduced overall in hospital mortality over the years, in particular for those operated on for type AAAD, due to the improvement in operative technologies, organ perfusion and surgeons taking on even high-risk patients. In contrast, for type B AADendovascular repair has been more frequently adopted in recent years but without a significant demonstrable reduction of in hospital mortality, although an increased survival rate at 5 years was observed. Data suggest an increase in surgical intervention treatment for both type Aand B AAD, particularly in B due to expansion of endovascular treatment. While expanded surgical repair for type Ahas shown a trend for a better outcome, this was not observed for type B patients. In addition, debate continues about endovascular treatment of uncomplicated type B and IMH

The role of age in complicated acute type B aortic dissection

Background Complicated acute type B aortic dissection (cABAD) generally requires urgent intervention. Advanced age is a risk factor for mortality after thoracic aortic intervention, including surgery for aortic dissection. The purpose of this study was to investigate the exact impact of increasing age on the management and outcomes of cABAD. Methods We analyzed the outcomes of 583 patients with cABAD enrolled in the International Registry of Acute Aortic Dissection (IRAD) between 1996 and 2012. All patients with cABAD were categorized according to age by decade and management type (medical, surgical, or endovascular treatment), and outcomes were subsequently investigated in the different age groups. Results The mean age of the cohort was 63.4 \ub1 14.2 years, 36% of patients (n = 209) were greater than 70 years of age and 64% (n = 374) were less than 70 years. The utilization of surgery and endovascular techniques progressively decreased with patient age, while the rate of medical management significantly increased with age (p < 0.001). The in-hospital mortality rates for complicated patients younger than 70 years versus 70 years or more were 10.1% versus 30.0% for endovascular treatment (p = 0.001), 17.2% versus 34.2% for surgical treatment (p = 0.027), and 14.2% versus 32.2% for medical treatment (p = 0.001). Age 70 years or greater was a predictor of in-hospital mortality in multivariate analysis (odds ratio 2.37, 95% confidence interval: 1.23 to 4.54, p = 0.010). Conclusions Advanced age has a dramatic impact on the management and outcomes of patients with cABAD. A nonsignificant trend toward lower mortality after endovascular management was observed, both for younger patients and for elderly patients

Shock complicating type A acute aortic dissection: clinical correlates, management, and outcomes

Aims Shock is among the most dreaded and common complications of type A acute aortic dissection (TAAAD). However, clinical correlates, management, and short- and long-term outcomes of TAAAD patients presenting with shock in real-world clinical practice are not known. Methods and Results We evaluated 2,704 patients with TAAAD enrolled in the International Registry of Acute Aortic Dissection between January 1, 1996, and August 18, 2012. On admission, 407 (15.1%) TAAAD patients presented with shock. Most in-hospital complications (coma, myocardial or mesenteric ischemia or infarction, and cardiac tamponade) were more frequent in shock patients. In-hospital mortality was significantly higher in TAAAD patients with than without shock (30.2% vs 23.9%, P =.007), regardless of surgical or medical treatment. Most shock patients underwent surgical repair, with medically managed patients demonstrating older age and more complications at presentation. Estimates using Kaplan-Meier survival analysis indicated that most (89%) TAAAD patients with shock discharged alive from the hospital survived 5 years, a rate similar to that of TAAAD patients without shock (82%, P =.609). Conclusions Shock occurred in 1 of 7 TAAAD patients and was associated with higher rates of in-hospital adverse events and mortality. However, TAAAD survivors with or without shock showed similar long-term mortality. Successful early and aggressive management of shock in TAAAD patients has the potential for improving long-term survival in this patient population

Acute type B aortic dissection complicated by visceral ischemia

Objective Acute type B aortic dissection (ABAD) can lead to visceral malperfusion, a potentially life-threatening complication. The purpose of this study was to investigate the presentation, management, and outcomes of ABAD patients with visceral ischemia who are enrolled in the International Registry of Acute Aortic Dissection. Methods Patients with ABAD enrolled in the registry between 1996 and 2013 were identified and stratified based on presence of visceral ischemia at admission. Demographics, medical history, imaging results, management, and outcomes were compared for patients with versus without visceral ischemia. Results A total of 1456 ABAD patients were identified, of which 104 (7.1%) presented with visceral ischemia. Preoperative limb ischemia (28% vs 7%, P <.001) and acute renal failure (41% vs 14%, P <.001) were more common among patients with visceral ischemia. Endovascular treatment and surgery were offered to 49% and 30% of the visceral ischemia cohort, respectively; remaining patients were managed conservatively. The in-hospital mortality was 30.8% for patients with visceral ischemia and 9.1% for those without visceral ischemia (odds ratio [OR] 4.44; 95% confidence interval [CI], 2.8-7.0, P <.0001). Mortality rates were similar after surgical and endovascular management of visceral ischemia (25.8% and 25.5%, respectively, P = not significant). Among the visceral ischemia group, medical management was a predictor of mortality in multivariate analysis (OR, 5.91; 95% CI, 1.2-31.0; P =.036). Conclusions Patients with ABAD complicated by visceral ischemia have a high risk of mortality. We observed similar outcomes for patients treated by endovascular management versus surgery, whereas medical management was an independent predictor of mortality. Early diagnosis and intervention for visceral ischemia seems to be crucial