Communication between the aortic sinus and a cardiac chamber is a rare anomaly that can be diagnosed in children and young adults. We describe two cases with a tunnel between the aortic sinus and... Show moreCommunication between the aortic sinus and a cardiac chamber is a rare anomaly that can be diagnosed in children and young adults. We describe two cases with a tunnel between the aortic sinus and right atrium, discuss diagnostic modalities, and review the literature on this anomaly. Show less
BACKGROUND Type D personality, characterized by high levels of negative affectivity and social inhibition, is related to mortality, morbidity, poor health status, quality of life (QoL) and less... Show moreBACKGROUND Type D personality, characterized by high levels of negative affectivity and social inhibition, is related to mortality, morbidity, poor health status, quality of life (QoL) and less healthcare utilization in various cardiovascular patient groups. To date, studies in patients with congenital heart disease (CHD) are lacking. AIMS (1) To examine the prevalence of type D personality in CHD patients; (2) to compare type D to non-type D patients with regard to disease severity, functional status, health status and QoL; and (3) to examine the extent to which type D personality is independently related to healthcare utilization. METHODS A total of 1109 adult CHD patients were included in a questionnaire survey. Due to missing data, 302 patients were excluded. RESULTS The prevalence of Type D personality was 20.4%. Type D patients reported a poorer functional status, health status and QoL than non-type D patients (p<0.05). Type D patients reported less healthcare use than non-type D patients (primary and cardiac outpatient healthcare: adjusted OR=0.56, 95% CI=0.35-0.90; inpatient healthcare: adjusted OR=0.38, 95% CI=0.17-0.83). Results of a post-hoc analysis showed a high prevalence of type D personality in patients with a poor functional status who did not consult their cardiologist. CONCLUSION type D patients report a poorer functional status, health status and QoL, but less healthcare utilization. In clinical practice, patients should be screened for type D personality, since social inhibition may prevent them from contacting a healthcare provider in the event of symptom aggravation. Show less
BACKGROUND: In patients with Marfan syndrome (MFS), increased aortic wall stiffening may lead to progressive aortic dilatation. Aortic Pulse Wave Velocity (PWV), a marker of wall stiffness can be... Show moreBACKGROUND: In patients with Marfan syndrome (MFS), increased aortic wall stiffening may lead to progressive aortic dilatation. Aortic Pulse Wave Velocity (PWV), a marker of wall stiffness can be assessed regionally, using in-plane multi-directional velocity-encoded MRI. This study examined the diagnostic accuracy of regional PWV for prediction of regional aortic luminal growth during 2-year follow-up in MFS patients. METHODS: In twenty-one MFS patients (mean age 36±15years, 11 male) regional PWV and aortic luminal areas were assessed by 1.5T MRI. At 2-year follow-up, the incidence of luminal growth, defined as mean luminal diameter increase >2mm was determined for five aortic segments (S1, ascending aorta; S2, aortic arch; S3, thoracic descending aorta, S4, supra-renal and S5, infra-renal abdominal aorta). Regional PWV at baseline was considered increased when exceeding age-related normal PWV (healthy volunteers (n=26; mean age 30±10years, 15 male)) by two standard-errors. Sensitivity and specificity of regional PWV-testing for prediction of regional luminal growth were determined. RESULTS: Regional PWV at baseline was increased in 17 out of 102 segments (17%). Significant luminal growth at follow-up was reported in 14 segments (14%). The specificity of regional PWV-testing was ≥78% for all aortic segments, sensitivity was ≤33%. CONCLUSIONS: Regional PWV was significantly increased in MFS patients as compared to healthy volunteers within similar age range, in all aortic segments except the ascending aorta. Furthermore, regional PWV-assessment has moderate to high specificity for predicting absence of regional aortic luminal growth for all aortic segments in MFS patients. Show less
BACKGROUND: Patients with moderate pulmonary valve restenosis late after relief of severe pulmonary stenosis (PS) may show decreased exercise tolerance. To elucidate the mechanism of decreased... Show moreBACKGROUND: Patients with moderate pulmonary valve restenosis late after relief of severe pulmonary stenosis (PS) may show decreased exercise tolerance. To elucidate the mechanism of decreased exercise tolerance, we evaluated cardiac response to physical and pharmacological stress in these patients and compared results with those of patients with native moderate PS. METHODS: Twenty asymptomatic patients with moderate PS were divided into 2 groups: Group I (late after relief of severe PS, n=9), and Group II (no previous intervention, n=11). All patients underwent an exercise test, dobutamine stress (DS) MRI, and delayed contrast enhanced MRI. The response to physical and pharmacological stress was compared between both groups. RESULTS: Group I showed impaired exercise capacity compared to Group II (VO(2)max=72.8%±3.5% vs. 102.5%±16.3%, p<0.001). During DS-MRI, RV-SV increased in Group II, but not in Group I (+13±8ml, -5±8ml, p<0.001). RV end-diastolic volume decreased significantly in Group I patients (p=0.006) while it did not significantly change in Group II patients. The amount of RV-SV increase (∆ RV-SV) correlated negatively with the period of moderate PS existence and the current PG in Group I (r=-0.82, p=0.007, and r=-0.68, p=0.04, respectively) but not in Group II (r=0.45, p=0.1, and r=0.40, p=0.2, respectively). Furthermore, ∆ RV-SV correlated negatively with the PG before valvuloplasty (r=-0.76, p=0.02). CONCLUSION: Impaired exercise capacity in patients with moderate pulmonary restenosis after relief of severe PS is probably caused by inability to increase RV-SV. Disturbed RV filling properties, worsening in time, might play a role. Show less
PURPOSE: Aortic valve dysfunction is common in coarctation patients(CoA). Bicuspid aortic valve (BAV) in CoA is associated with aortic valve stenosis (AS), aortic valve regurgitation (AR), and... Show morePURPOSE: Aortic valve dysfunction is common in coarctation patients(CoA). Bicuspid aortic valve (BAV) in CoA is associated with aortic valve stenosis (AS), aortic valve regurgitation (AR), and ascending aortic dilatation. The aim of this study was to evaluate the progression of and predictors for aortic valve dysfunction in CoA. METHODS: 96 CoA patients prospectively underwent echocardiography twice between 2001 and 2010. AS was defined as an aortic valve gradient ≥20mmHg, AR as none/minor, or moderate/severe. Aortic dilatation as an ascending aortic diameter ≥37mm. RESULTS: All patients (median age 28.0years, range 17-61years; male 57%) were followed with a median follow-up of 7.0years. Sixty patients (63%) had BAV. At baseline 10 patients had AS (10%, 9 BAV), 6 patients AR (6%, 3 BAV) and 11 patients aortic dilatation (11%, 11 BAV). At follow-up 15 patients had AS (15%, 13 BAV) and 12 patients AR. (13%, 8 BAV). Median AS progression was 1.1mmHg/5years (range - 13-28). Determinants for AS at follow-up were age (ß=0.20, P=0.01), aortic dilatation (ß=4.6, P=0.03), and baseline aortic valve gradient (ß=0.93, P<0.001). BAV was predictive for AR. (ß=0.91, P=0.049). CONCLUSION: Progression of AS in adult CoA patients is mild in this young population. Older age, aortic dilatation and the baseline aortic valve gradient are determinants for AS at follow-up. BAV is predictive for AR. These findings point towards a common embryological pathway of both valvular and aortic disease in CoA. Show less
Romeih, S.; Groenink, M.; Plas, M.N. van der; Spijkerboer, A.M.; Hazekamp, M.G.; Luijnenburg, S.; ... ; Blom, N.A. 2012
BACKGROUND We hypothesized 1) that long-term ventricular outcome and exercise capacity would be better in patients with isolated pulmonary valve stenosis (PS) treated with balloon pulmonary... Show moreBACKGROUND We hypothesized 1) that long-term ventricular outcome and exercise capacity would be better in patients with isolated pulmonary valve stenosis (PS) treated with balloon pulmonary valvuloplasty (BPV) than in patients operated for tetralogy of Fallot (TOF), and 2) that ventricular outcome and exercise capacity would not be different in PS patients and healthy controls. METHODS We included 21 PS patients after BPV (16.2 ± 5.2 years) and 21 patients operated for TOF (16.6 ± 5.6 years), matching them for gender, age at treatment, and age at study. Patients underwent cardiovascular magnetic resonance (CMR) imaging, exercise testing, 12-lead ECG and 24-hour Holter monitoring for assessment of right ventricular (RV) size and function, pulmonary regurgitation (PR), exercise capacity and electrocardiographic status. Healthy controls for CMR imaging and exercise testing were matched for gender and age at study. RESULTS RV volumes and PR percentage were significantly larger in TOF patients than in PS patients; biventricular ejection fraction (EF) was not different. PR was mild in most PS patients. RV end-systolic volume was significantly larger in PS patients than in healthy controls; RVEF was significantly lower. Both patient groups had similar exercise test results. Peak workload and VO(2) max. were significantly lower in PS patients than in healthy controls. CONCLUSIONS Longstanding mild PR in PS patients can lead to an enlarged RV, reduced RV function and reduced exercise capacity. Despite more PR and larger RV volumes in TOF patients, exercise capacity and biventricular function are similar in both patient groups. Show less
