OBJECTIVES The aim of this study was to determine the relationship between aortic valve morphology and valve dysfunction. BACKGROUND The morphology of the bicuspid or bicommissural aortic valve (BAV) may predict the severity of valve dysfunction. Therefore, we assessed the relationship between BAV, aortic coarctation, and the degree of valve pathology in children. METHODS A retrospective review of 1,135 patients with BAV who were identified between 1986 and 1999 was performed. Patients younger than 18 years of age with BAV that was identifiable via echocardiography were included. The most recent or last study of each patient before intervention or endocarditis was reviewed. Mild stenosis was defined as a valve gradient 2 m/s, moderate or greater aortic stenosis as 3.5 m/s. Aortic regurgitation was quantified using standard criteria. RESULTS Median age was 3 years (range, 1 day to 17.9 years), and 67% of the patients were male. Right-coronary and left-coronary leaflet fusion were the most common types of BAV (70%). Aortic stenosis that was moderate or greater was observed most often in patients with right-coronary and non-coronary leaflet fusion (odds ratio 2.4, 95% confidence interval 1.6 to 3.6; p 0.001). Similarly, right-coronary and non-coronary leaflet fusion was more often associated with moderate aortic regurgitation or greater (odds ratio 2.4, 95% confidence interval 1.2 to 4.7; p 0.01). The majority of patients with aortic coarctation had fusion of the right-coronary and left-coronary leaflets (89%), and aortic coarctation was associated with lesser degrees of valve stenosis or regurgitation. CONCLUSIONS Analysis of BAV morphology is of clinical and prognostic relevance. Fusion of the right-coronary and non-coronary leaflets was associated with more significant valve pathology, whereas fusion of the right-coronary and left-coronary leaflets was associated overwhelmingly with aortic coarctation and less aortic valve pathology. (J Am Coll Cardiol ublished by Elsevier Inc. doi:10.1016/j.jacc.2004.05.063

Background: Little is known about how physician and parent perspectives compare regarding the prognosis and end-of-life (EOL) experience of children with advanced heart disease (AHD). Objective: The study's objective was to describe and compare parent and physician perceptions regarding prognosis and EOL experience in children with AHD. Methods: This was a cross-sectional survey study of cardiologists and bereaved parents. Study subjects were parents and cardiologists of children with primary cardiac diagnoses who died in a tertiary care pediatric hospital between January 2007 and December 2009. Inclusion required both physician and parent to have completed surveys respective to the same patient. A total of 31 parent/physician pairs formed the analytic sample. Perceptions were measured of cardiologists and bereaved parents regarding the EOL experience of children with AHD. Results: Nearly half of parents and physicians felt that patients suffered 'a great deal,' 'a lot,' or 'somewhat' at EOL, but there was no agreement between them. At diagnosis, parents more often expected complete repair and normal lifespan while the majority of physicians expected shortened lifespan without normal quality of life. Parents who expected complete repair with normal life were more likely to report 'a lot' of suffering at EOL (p=0.002). In 43% of cases, physicians reported that the parents were prepared for the way in which their child died, while the parents reported feeling unprepared. Conclusion: Both parents and physicians perceive suffering at EOL in patients who die of AHD. Moreover, parent expectations at diagnosis may influence perceptions of suffering at EOL. Physicians overestimate the degree of parent preparedness for their child's death.

Although conventional radiography is the method most frequently used for monitoring progression of osteoarthritis, it may not show osteoarthritic changes of the knee until late in the disease, and it may show involvement of only one or two compartments in patients who have tricompartmental disease. We compared radiography, CT, and MR imaging for assessing the extent and severity of osteoarthritis of the knee in 20 patients. Radiography included posteroanterior weight-bearing, true lateral, and sunrise patellar projections. Axial CT scans were reformatted in sagittal and coronal planes. MR imaging consisted of spin-echo (600-800/20; 2000/60, 120 [TR/TE]), and gradient-echo (600/30, theta = 30-degrees) sequences. The severity of osteoarthritic changes was graded from 0 to 3. MR frequently showed tricompartmental cartilage loss when radiography and CT showed only bicompartmental involvement in the medial and patellofemoral compartments. In the lateral compartment, MR showed a higher prevalence of cartilage loss (60%) than radiography (35%) and CT (25%) did. In the medial compartment, CT and MR showed osteophytes in 100% of the knees, whereas radiography showed osteophytes in only 60%. Notably, radiography often failed to show osteophytes in the posterior medial femoral condyle. On MR images, meniscal degeneration or tears were found in all 20 knees studied. Partial and complete tears of the anterior cruciate ligament were found in three and seven patients, respectively. MR is more sensitive than radiography and CT for assessing the extent and severity of osteoarthritic changes and frequently shows tricompartmental disease in patients in whom radiography and CT show only bicompartmental involvement. MR imaging is unique for evaluating meniscal and ligamentous disease related to osteoarthritis.

The long-term outcome after surgical reimplantation of a unilateral congenitally anomalous pulmonary artery (UCAPA) origin is inadequately described. We performed a single-center retrospective review of patients with pulmonary artery (PA) sling or anomalous origin of 1 PA from the ascending aorta who underwent surgical repair from 1980 to 2006. Baseline data were available for 20 patients with PA sling and 29 patients with anomalous origin of 1 PA from the ascending aorta. Follow-up data for 38 patients, representing 190 cumulative patient-years, were available. Of 28 (74%) patients who developed pulmonary artery stenosis (PAS), 17 (45%) patients underwent a first intervention for PAS, and 9 (24%) patients underwent a second intervention. Median time from surgical repair to diagnosis of PAS was 0.4 years (95% confidence interval [CI] 0, 0.9). Median time from diagnosis of PAS to first intervention was 2.7 years (95% CI 0.07, 5.4). In the 29 patients initially repaired at our center, no first intervention occurred later than 2.8 postoperative years. Median time from first to second intervention was 2 years (95% CI 0, 4.8). Percutaneous intervention for postoperative PAS resulted in acute hemodynamic and anatomic improvement with modest risk profile. On multivariate analysis, recent surgical repair (after 1993) was a risk factor for PAS (p = 0.03, hazard ratio [HR] 1.9), and operative first intervention (vs. percutaneous) was a risk factor for second intervention (p = 0.05, HR 11.2). After reimplantation of a UCAPA origin, PAS is frequent and occurs early postoperatively. Close follow-up after repair is necessary.

Unrestricted physiologic joint motion results in multidirectional displacement of the anatomic structures. When performing real-time magnetic resonance (MR) imaging of such a joint motion, continuous adjustment of the scan plane position may be required. The purpose of this study was to evaluate the clinical feasibility of a method to guide the scan plane position during dynamic-motion MR imaging of freely moving joints.

Background. Discontinuous pulmonary arteries (PAs) may develop in patients with single-ventricle heart disease from a variety of causes. We investigated factors associated with successful connection of nonconfluent PAs in patients with a cavopulmonary circulation.Methods. We reviewed 49 patients who underwent connection of discontinuous PAs with or after a bidirectional Glenn (n = 29) or Fontan (n = 20) procedure at a median age of 7.9 years. PA continuity was established by direct anastomosis in 27, interposition graft in 19, and transcatheter recanalization in 3. Survival was 92% +/- 4% at 1 year and 89% +/- 5% at 5 years.Results. Recurrent PA occlusion was documented in 7 patients, 5 within 10 days of PA connection. The only factor associated with shorter freedom from PA occlusion was sole supply of blood flow to 1 lung by systemic-to-PA collaterals before connection (66% +/- 14% vs 95% +/- 4% freedom from occlusion at 6 months, p = 0.03). Among the 45 early survivors, freedom from PA reintervention or occlusion was 83 +/- 6% at 1 year and 55 +/- 9% at 3 years.Conclusions. Discontinuous PAs can be successfully connected in most patients with a cavopulmonary circulation, although nonconfluent PAs appear to increase the risk of poor outcome after Fontan. Recurrent PA occlusion was usually diagnosed in the early postoperative period. In patients with sole supply to 1 lung through collaterals, shunt placement before PA connection may optimize outcome. A low threshold for investigation of the reconnected PA is warranted.

Background The slope of the minute ventilation versus CO2 production relationship (VE/VCO2 slope) is an index of gas exchange efficiency during exercise. In patients with repaired tetralogy of Fallot (rTOF), it correlates negatively with exercise capacity and is one of the best predictors of peak oxygen consumption (VO2). In these patients, the magnitude of the VE/VCO2 slope is related to the severity of pulmonary blood flow maldistribution (PBFM). The purpose of this study was to determine whether, in patients with rTOF, improvements in PBFM after a successful balloon angioplasty procedure (BAP) result in improvements in peak VO2 and gas exchange during exercise.Methods Seventeen patients with rTOF and residual pulmonary artery stenoses referred for BAP were recruited. Exercise tests were performed and PBFM determined before and after BAP.Results Nine patients (group 1) had a successful BAP (ie, improvement of >5 percentage points in PBFM); 8 did not (group 2). Patients in group 1 had significantly greater improvements in VE/VCO2 slope, peak VO2, and peak oxygen pulse (an index of forward stroke volume at peak exercise) than did patients in group 2. A significant correlation existed between the improvement in PBFM and the decline in the VE/VCO2 slope (r = -070, P = .002). Changes in peak oxygen pulse accounted for 89% of the improvement in peak VO2.Concliusions In these patients, a successful BAP resulted in improved peak VO2 and more efficient gas exchange during exercise. The improvement in peak VO2 appeared to be mediated by an increase in forward stroke volume.

BACKGROUND:Bone marrow edema (BME) is a condition detectable with magnetic resonance imaging (MRI) and is present in different stages of osteoarthritis (OA). Its pathogenesis is still not completely known.PURPOSE:To evaluate the longitudinal occurrence and persistence of BME in early OA of the knee.MATERIAL AND METHODS:Twenty-three patients (eight females, 15 males; mean age 55.5+/-10.3 years) were scanned with a 1.5T MR imaging unit (sagittal fat-suppressed intermediate-weighted fast spin echo; 4-mm section thickness, 1-mm intersection gap, 256 x 192 matrix, 120-mm field of view). Images were obtained in all 23 patients at two time points (TPs) and in 12 patients at three TPs. Images were evaluated by two readers independently; discrepancies in image grading were reviewed and evaluated in consensus. A four-point image-grading scale was used (absence of BME to severe BME). Four main anatomical regions were evaluated (medial femur, lateral femur, medial tibia, lateral tibia), which were subcategorized into anterior, central, and posterior regions.RESULTS:One hundred five areas of BME in the 23 patients were found at all three TPs. In 16 areas, the BME was consistent at the same location over time, in seven locations the BME became larger, in six areas the BME became smaller, and in 16 locations it could not be detected in follow-up MRIs. In one case, the BME was smaller at TP2 but increased at TP3. In eight cases, only at the last time point could a BME be detected.CONCLUSION:BME is not a static phenomenon but changes over time. Correlation to physical activity and local inflammatory reaction should be evaluated.

Introduction: For patients undergoing Fontan procedures for palliation of single ventricle physiology the addition of a fenestration to the procedure, which permits intracardiac right-to-left shunting, mitigates perioperative morbidity. While some fenestrations may close spontaneously, many remain patent and subject patients to arterial hypoxemia and risk for paradoxical emboli. For this reason fenestration closure is routinely performed post-operatively in the cardiac catheterization laboratory. Fenestration closure typically results in improved arterial oxygenation, but decreased cardiac index. We assessed the hypothesis that sum effect of these physiologic changes would have not improve exercise capacity. Methods: Nineteen consecutive patients, age 4.8–46 (median 10) years underwent pre and post-fenestration closure exercise testing with expiratory gas analysis. Results: Prior to fenestration closure, peak oxygen consumption (VO2) was low. Arterial desaturation was present at rest and worsened with exercise. The ventilatory response to exercise was excessive, characterized by elevation of the minute ventilation vs. CO2 production slope (VE/VCO2 slope) and a low end tidal pCO2 at the ventilatory anaerobic threshold (pETCO2). Following fenestration closure, arterial saturation (at rest and at peak exercise) improved significantly. However, there was no change in VO2, heart rate, or oxygen pulse at peak exercise. In contrast, ventilatory abnormalities improved considerably. The VE/VCO2 slope fell 25% and pETCO2 rose from 27.9+/−4.2 to 34.1+/−3.2 mmHg. Conclusion: Elective Fontan fenestration closure does not improve peak VO2. However, the reduction in right-to-left intracardiac shunting improves arterial oxygen saturation during exercise and reduces the patient’s need to ventilate during exercise. These changes may reduce the Fontan patient’s perception of dyspnea during exercise. Pre and Post-Fenestration Exercise Data

A newborn with pulmonary atresia, intact ventricular septum, and a bicommissural aortic valve underwent percutaneous pulmonary valvotomy at 1 day of age. Severe coarctation of the aorta became apparent only after spontaneous closure of the ductus arteriosus at 5 weeks of age, requiring repair by surgical end-to-end anastomosis. This is the first report of a child with pulmonary atresia with intact ventricular septum in association with coarctation of the aorta.

Objective: To determine the prevalence of labral tears and cartilage lesions in patients with mechanical symptoms of the hip using magnetic resonance (MR) arthography.Methods: A total of 100 patients with mechanical symptoms of the hip such as pain, clicking, locking and giving way underwent MR arthrography of the hip to rule out a labral tear. Hip joints were evaluated for labral alterations, bone marrow edema (BME), and other pathology using a novel semi-quantitative scoring system. Cartilage changes were graded separately for the acetabulum and femoral head in the anterior, posterior, central, medial, and lateral hip joint regions (10 locations) on a 6-point scale: 0 = normal 1 = signal heterogeneity; 2 =fissuring, 3 =thinning <50% of the normal thickness; 4=thinning >50% of the normal thickness; 5=full thickness cartilage loss. Labral tears were categorized into normal intrasubstance degeneration, simple tear or complex tear with two or more tear regions. Statistical methods were contingency tables, Fisher's exact test for discrete outcomes (based on grade scores), Spearman's rank correlation (r) and t tests for continuous outcomes, using pooled and stratified analyses.Results: On MR arthrography, labral tears were found in 66 patients (66% prevalence) with 13 having more than one location involved. Locations were 19 lateral (13 simple, six complex), 54 anterior (39 simple, 15 complex), eight posterior (six simple, two complex). Cartilage lesions were found in 76 patients (76% prevalence) with 53 demonstrating involvement of more than one compartment. The most common finding was cartilage :thinning <50% in 99 regions in 44 patients. Cartilage thinning >50% was shown in 34 patients in 67 areas. When we correlated the grade of cartilage abnormality with the grade of labral tear, we found a statistically significant correlation (r= 0.29; P < 0.01). The size of cartilage abnormality was correlated with the grade of labral tear in the lateral acetabulum (r= 0.38; P < 0.001), anterior acetabulum (r= 0.22; P < 0.002), and lateral femoral head (r= 0.29; P < 0.004). The grade of cartilage loss was correlated with the grade of BME (r=0.44; P<0.001).Conclusiorr. Labral tears and cartilage loss are common in patients with mechanical symptoms in the hip. Cartilage loss, labral tears and BME appear interrelated and may represent important risk factors that may affect the development and progression of osteoarthritis in the hip joint. (c) 2007 Published by Elsevier Ltd on behalf of Osteoarthritis Research Society International.

OBJECTIVE:The aim of this study was to evaluate the longitudinal reproducibility of cartilage volume and surface area measurements in moderate osteoarthritis (OA) of the knee.MATERIALS AND METHODS:We analysed 5 MRI (GE 1.5T, sagittal 3D SPGR) data sets of patients with osteoarthritis (OA) of the knee (Kellgren Lawrence grade I-II). Two scans were performed: one baseline scan and one follow-up scan 3 months later (96 +/- 10 days). For segmentation, 3D Slicer 2.5 software was used. Two segmentations were performed by two readers independently who were blinded to the scan dates. Tibial and femoral cartilage volume and surface were determined. Longitudinal and cross-sectional precision errors were calculated using the standard deviation (SD) and coefficient of variation (CV%=100x[SD/mean]) from the repeated measurements in each patient. The in vivo reproducibility was then calculated as the root mean square of these individual reproducibility errors.RESULTS:The cross-sectional root mean squared coefficient of variation (RMSE-CV) was 1.2, 2.2 and 2.4% for surface area measurements (femur, medial and lateral tibia respectively) and 1.4, 1.8 and 1.3% for the corresponding cartilage volumes. Longitudinal RMSE-CV was 3.3, 3.1 and 3.7% for the surface area measurements (femur, medial and lateral tibia respectively) and 2.3, 3.3 and 2.4% for femur, medial and lateral tibia cartilage volumes.CONCLUSION:The longitudinal in vivo reproducibility of cartilage surface and volume measurements in the knee using this segmentation method is excellent. To the best of our knowledge we measured, for the first time, the longitudinal reproducibility of cartilage volume and surface area in participants with mild to moderate OA.

Objective: Validation of a new fast software technique to segment the cartilage on knee magnetic resonance (MR) acquisitions. Large studies of knee osteoarthritis (OA) will require fast and reproducible methods to quantify cartilage changes for knee MR data. In this report we document and measure the reproducibility and reader time of a software-based technique to quantify the Volume and thickness of articular cartilage on knee MR images.Methods: The software was tested on a set of duplicate sagittal three-dimensional (3D) dual echo steady state (DESS) acquisitions from 15 (8 OA, 7 normal) subjects. The repositioning, inter-reader, and intra-reader reproducibility of the cartilage volume (VC) and thickness (ThC) were measured independently as well as the reader time for each cartilage plate. The root-mean square coefficient of variation (RMSCoV) was used as metric to quantify the reproducibility of VC and mean ThC.Results: The repositioning RMSCoV was as follows: VC = 2.0% and ThC = 1.2% (femur), VC = 2.9% and ThC = 1.6% (medial tibial plateau), VC = 5.5% and ThC = 2.4% (lateral tibial plateau), and VC = 4.6% and ThC = 2.3% (patella). RMSCoV values were higher for the inter-reader reproducibility (VC: 2.5-8.6%) (ThC: 1.9-5.2%) and lower for the intra-reader reproducibility (VC: 1.6-2.5%) (ThC: 1.2-1.9%). T he method required an average of 75.4 min per knee.Conclusions: We have documented a fast reproducible semi-automated software method to segment articular cartilage on knee MR acquisitions. (C) 2006 Osteoarthritis Research Society International. Published by Elsevier Ltd. All rights reserved.

Background: Stenting for aortic coarctation has been shown to be effective in the short term. The safety and longer term efficacy of transcatheter therapy, however, must be well established if the technique is to be widely accepted as an alternative to surgery. In order to determine the frequency, spectrum, and outcome of injury to the aortic wall caused by angioplasty or stenting of aortic coarctation, the nomenclature of mural injury in these patients must be adapted to the conditions of transcatheter therapy. Methods and Results: Between 1989 and July 2005, we inserted stents in 153 patients with aortic coarctation, their median age being 15.8 years. Prior aortic interventions had been performed in 98 patients, and preexisting aneurysms were observed in 19. Stenting resulted in a significant reduction of the gradient across the site of coarctation, from a median of 30 millimetres of mercury to zero (p less than 0.001), with a residual gradient within the aortic arch of 20 millimetres of mercury or more in 5% of patients. Acute injuries to the aortic wall, other than therapeutic tears, were observed in 3 patients (2%), none of whom required surgery. At median follow-up of 2.5 years, this being more than 5 years in 30 patients, 4 patients had died, albeit none from complications relating to stenting or catheterization. Acute injuries to the aortic wall did not progress, and new aneurysms were observed in 6% of patients subsequent to follow-up imaging. Stent fractures, and jailed or partially covered brachiocephalic vessels, were observed in 12, and 49, patients, respectively, but did not result in haemodynamic or embolic complications. Conclusions: Stenting for aortic coarctation results in consistent relief of the gradient, and few serious complications in the short and intermediate term. Serious injuries to the aortic wall are uncommon in our experience, and can be minimized with a focus on technical measures, such as pre-dilation before stenting.

Background. Arthroscopic partial meniscectomy (APM) is the most frequently performed orthopedic procedure. Functional outcomes of APM are variable, particularly among patients with underlying knee osteoarthritis. While most patients undergoing APM have knee magnetic resonance imaging (MRI) performed preoperatively, the prognostic value of knee MRI in predicting the functional outcomes of APM has not been evaluated.Methods: We studied patients who had APM performed by one of five participating surgeons at one institution in 2002. The preoperative MRI scans of these patients were assessed using a standardized rating system by an independent observer who was not involved in the care of the patients and who was blinded to patient outcomes. Patients completed a questionnaire in the summer of 2003, 6-18 months postoperatively. The questionnaire included the Knee Injury and Osteoarthritis Outcome Score (KOOS) and items on satisfaction with the results of surgery. We used bivariate and multivariate techniques to evaluate the associations between MRI findings, other preoperative findings, and the functional status and pain scales of the KOOS.Results: Eighty-three patients were included in the analyses. The outcome of surgery was variable with average KOOS functional score of 77 and range of 15-100. One-quarter of patients were somewhat or very dissatisfied with the results of surgery and 17% were using a cane at the time of follow-up. In bivariate analyses, preoperative predictors of KOOS function score at follow-up included preoperative functional status and several MRI findings including the extent of cartilage damage, bone marrow edema in the medial compartment, and length of the tear. Multivariate analyses showed that after adjusting for preoperative functional status, the extent of cartilage signal abnormality in the medial compartment on MRI remained an independent predictor of functional status, 6-18 months following surgery. Specifically, preoperative functional status explained 21% of the variability in follow-up KOOS functional status score and the extent of medial tibial cartilage damage on MRI explained an additional 16%. Analyses of knee pain 1 year following APM yielded similar findings, with preoperative functional status accounting for 17% of the variability in pain scores and medial tibial cartilage damage accounting for an additional 13%.Conclusions: Preoperative MRI findings of cartilage damage have independent prognostic value in predicting the functional outcome of APM. This study was limited by a cross-sectional design with retrospective recall of preoperative functional status. Thus, the findings need to be confirmed in prospective investigations. (c) 2005 OsteoArthritis Research Society International. Published by Elsevier Ltd. All rights reserved.

Molecular imaging is an upcoming field in radiology as a result of great advances in imaging technology, genetics, and biochemistry in the recent past. Early-stage imaging of molecular pathological changes in cells opens the gates to new methods in medical treatment of diseases that otherwise would only be detected in advanced stages. Methods of imaging biochemical pathways with molecular agents are currently an issue of intensive research. This article reviews current modalities of molecular imaging in arthritis that should offer future perspective on early disease detection, diagnosis, and monitoring of treatment efficiency and how they can pave the way to optimized therapy.

M.H. Brem1, P.M. Schlechtweg2, G. Neumann2, R. Jackson3, J. Yu3, C. Eaton4, H. Yoshioka2, P. Lang2, J. Duryea2 1Friedrich Alexander University Erlangen-Nuremberg, Dept. of Surgery/Orthopedic Surgery, Erlangen, Germany, 2Brigham and Womens’ Hospital, Dept. of Radiology, Boston, MA, Boston, MA, 3The Ohio State University, Dept. Endocrinology, Diabetes and Metabolism and Radiology, Columbus, OH, 4Memorial Hospital of Rhode Island, Center for Primary Care and Prevention and Brown Medical School, Providence, RI