Search

ObjectiveLong-term prognosis of patients with coarctation of the aorta (CoA) is impaired due to the high prevalence of hypertension and consequent cardiovascular complications. Although stent implantation results in acute anatomical and haemodynamic benefit, limited evidence exists regarding the late clinical outcome. In this meta-analysis, we aimed to evaluate the medium-term effect of stent placement for CoA on systemic blood pressure (BP).MethodsPubMed, EMBASE and Cochrane databases were searched for non-randomised cohort studies addressing systemic BP ≥12 months following CoA stenting. Meta-analysis was performed on the change in BP from baseline to last follow-up using a random-effects model. Subgroup analyses and meta-regression were conducted to identify sources of heterogeneity between studies.ResultsTwenty-six studies with a total of 1157 patients and a median follow-up of 26 months were included for final analysis. Meta-analysis showed a 20.3 mm Hg (95% CI 16.4 to 24.1 mm Hg; pConclusionsStent implantation for CoA is associated with a significant decline in systolic and diastolic BP during medium-term follow-up. The degree of BP reduction appears to be dependent on baseline systolic BP, baseline peak systolic gradient, and whether stenting is performed for native or recurrent CoA.

This study aimed to determine aortic disease severity in patients with Loeys-Dietz syndrome (LDS). Thirty-three patients with LDS diagnosed and followed up at our unit were included. After reviewing all family trees, 25 deceased family members with clear clinical suspicion of having had LDS were also included. Clinical presentation, aortic dilation rate by echocardiography and age at aortic surgery, dissection or death were determined. Median aortic diameter at diagnosis was 36 mm, 43% of the patients aged >40 years had a z-score

Objective Variable coronary anatomy has been described in patients with bicuspid aortic valves (BAVs). This was never specified to BAV morphology, and prognostic relevance of coronary vessel dominance in this patient group is unclear. The purpose of this study was to evaluate valve morphology in relation to coronary artery anatomy and outcome in patients with isolated BAV and with associated aortic coarctation (CoA). Methods Coronary anatomy was evaluated in 186 patients with BAV (141 men (79%), 51±14 years) by CT and invasive coronary angiography. Correlation of coronary anatomy was made with BAV morphology and coronary events. Results Strictly bicuspid valves (without raphe) with left-right cusp fusion (type 1B) had more left dominant coronary systems compared with BAVs with left-right cusp fusion with a raphe (type 1A) (48% vs. 26%, p=0.047) and showed more separate ostia (28% vs. 9%, p=0.016). Type 1B BAVs had more coronary artery disease than patients with type 1A BAV (36% vs. 19%, p=0.047). More left dominance was seen in BAV patients with CoA than in patients without (65% vs. 24%, p Conclusions The incidence of a left dominant coronary artery system and separate ostia was significantly related to BAVs with left-right fusion without a raphe (type 1B). These patients more often had significant coronary artery disease. In patients with BAV and CoA, left dominancy is more common.

Background Congenital heart defects (CHD) are common in patients with Down9s syndrome; however, patients living in residential centres have not always been screened for CHD in the past. The aim of this study was to investigate the prevalence of CHD in patients with Down9s syndrome living in residential centres, and to determine whether cardiac screening should be recommended. Methods Between January 2007 and November 2009 Dutch residential centres nationwide were randomly sampled. Medical files of all patients with Down9s syndrome were investigated to retrieve documented information on known CHD. Echocardiography was performed on patients with unknown cardiac status. The main outcome measure was the number of newly diagnosed cases of CHD in adult patients with Down9s syndrome. Results Thirty-one centres and 1158 patients were included in the first stage of the study. Overall prevalence of known CHD was 16% (189 defects). Screening was performed in 138 patients without known CHD. In total, 24 new patients (17%) with a CHD were found, of which six patients needed semi-urgent care. Furthermore, 77% of the screened patients had mild to moderate regurgitation in one or more heart valves. Overall prevalence of CHD in adult Down9s syndrome patients living in residential centres would be estimated at 33%. Conclusions Seventeen per cent of patients with Down9s syndrome living in residential centres had undiagnosed CHD, and valvular regurgitation was present in the majority of patients. Cardiac screening is recommended in older Down9s syndrome patients, for whom new therapeutic options are available and for prevention of cardiac complications in old age.

AIM: To investigate the role of pulmonary arterial hypertension (PAH) in adult patients born with a cardiac septal defect, by assessing its prevalence and its relation with patient characteristics and outcome. METHODS AND RESULTS: From the database of the Euro Heart Survey on adult congenital heart disease (a retrospective cohort study with a 5-year follow-up), the relevant data on all 1877 patients with an atrial septal defect (ASD), a ventricular septal defect (VSD), or a cyanotic defect were analysed. Most patients (83%) attended a specialised centre. There were 896 patients with an ASD (377 closed, 504 open without and 15 with Eisenmenger's syndrome), 710 with a VSD (275, 352 and 83, respectively), 133 with Eisenmenger's syndrome owing to another defect and 138 remaining patients with cyanosis. PAH was present in 531 (28%) patients, or in 34% of patients with an open ASD and 28% of patients with an open VSD, and 12% and 13% of patients with a closed defect, respectively. Mortality was highest in patients with Eisenmenger's syndrome (20.6%). In case of an open defect, PAH entailed an eightfold increased probability of functional limitations (New York Heart Association class >1), with a further sixfold increase when Eisenmenger's syndrome was present. Also, in patients with persisting PAH despite defect closure, functional limitations were more common. In patients with ASD, the prevalence of right ventricular dysfunction increased with systolic pulmonary artery pressure (OR = 1.073 per mm Hg; p

Item does not contain fulltext OBJECTIVE: Patients with surgically repaired tetralogy of Fallot (rTOF) may develop functional tricuspid regurgitation (TR) due to annulus dilation. Guidelines suggest pulmonary valve replacement (PVR) in patients with rTOF with progressive TR, but data on clinical outcomes are lacking. Our objective was to determine whether TR was predictive for adverse events after PVR. METHODS: In this retrospective, multicenter cohort study, patients with rTOF who had undergone PVR after preoperative echocardiographic assessment of TR grade were included. Preoperative and postoperative imaging data and a composite of adverse clinical events (death, sustained ventricular tachycardia, heart failure, or supraventricular tachycardia) were collected. Multivariate Cox hazards regression analysis was used to determine which factors were predictive for adverse events after PVR. RESULTS: A total of 129 patients (61% men, age at PVR 32.9+/-10.4 years) were included. The composite endpoint occurred in 39 patients during 8.4+/-4.2 years of follow-up. In multivariate analysis, severe preoperative TR (HR 2.49, 95% CI 1.11 to 5.52), right ventricular end-systolic volume (HR 1.02/mL/m(2), 95% CI 1.01 to 1.03) and age at PVR (HR 1.07/year, 95% CI 1.04 to 1.09) were predictive for adverse events. Early postoperative TR was not predictive for adverse events (p=0.96). In patients without any risk factor (age >40 years, right ventricular end-systolic volume >90 mL/m(2) or severe TR), 5-year event-free survival was 100% as compared with 61% in patients with two or three risk factors. CONCLUSIONS: In patients with rTOF, severe preoperative TR was predictive for adverse events after PVR. Close surveillance is warranted in these patients irrespective of postoperative TR.

OBJECTIVE: To investigate dilatory changes of the aorta distal to the root in patients with Marfan's syndrome. METHODS AND RESULTS: Data of 268 patients with Marfan's syndrome who were enrolled in the Euro Heart Survey on adult congenital heart disease were analysed. Data used for this study were baseline characteristics, diameters at four levels of the aorta and events during follow up (dissection, aortic repairs and death). At inclusion, 26 patients had a previous dissection and 53 patients without a previous dissection had undergone elective aortic repair, thus leaving 189 patients without previous dissection or repair. During follow up (median 5.4 years), four patients died. A total of 46 aortic events (dissection or elective surgery) occurred in 45 patients, in the distal aorta in 14 patients (31%). Baseline aortic diameter at the levels distal to the root (arch, descending aorta and abdominal aorta) was greater in patients with than in those without a previous elective aortic root intervention (median 26 mm v 24 mm, p = 0.01; 25 mm v 20 mm, p < 0.01; and 20 mm v 17 mm, p < 0.01, respectively). Multivariate analysis showed that a previous elective aortic intervention was associated with a fourfold increased probability of dilatation of the distal aorta, after adjustment for age and sex (p < 0.01). In patients without a previous intervention, the baseline diameter of the descending aorta was an independent predictor of aortic events (hazard ratio 3.0 per quartile, 95% CI 1.5 to 5.9, p = 0.002). Cause for concern is that complete measurements of the aorta (at least one measurement at each level at baseline or during follow up) were available for only 38% of the patients. CONCLUSIONS: Almost one in every three aortic events occurring during follow up of these patients involved the distal aorta. After elective aortic root replacement, a dilated distal aorta is more common than before. Moreover, an increased diameter of the descending aorta is associated with a higher risk of aortic events in patients without previous dissection or aortic root replacement, independent of the diameter of the aortic root. Careful monitoring of the entire aorta is essential for the optimal management of patients with Marfan's syndrome, especially after elective surgery, but is insufficiently performed in Europe