An 18-years-old male presented to emergency department after a car accident with the diagnosis of femoral bone fracture. Arterial blood pressure was 160/90 mmHg in both arms. Bilateral femoral and popliteal pulses were extremely weak and there was systolic ejection murmur on the left second intercostals area. Chest X-ray showed rib notching with normal cardiac silhouette. Transthoracic echocardiography showed the aortic interruption just below the left subclavian artery. Aortography showed a complete interruption of the aortic arch (IAA) just distal to the origin of the left subclavian artery. Femoral bone fracture was treated by conservative strategy. A gadolinium contrast-enhanced magnetic resonance angiogram (1.5 T scanners) clearly reaffirmed a complete interruption of the descending aorta, 3.6 cm from the left subclavian artery with extensive collateralizations. Mild degree hypertension was controlled by a long acting calcium channel blocker. Later the patient has been scheduled for elective surgical repair. We aimed to discuss the diagnostic and treatment options of the interrupted aortic arch as being a rare anomaly.
SUMMARYThe present study was designed to investigate the incidence of benign joint hypermobility syndrome (BJHMS) in mitral valve prolapse (MVP) and the correlation between the echocardiographic features of the mitral valve and elastic properties of the aortic wall and Beighton hypermobility score (BHS) in patients with MVP and BJHMS.Fourty-six patients with nonrheumatic, uncomplicated, and isolated mitral anterior leaflet prolapse (7 men and 39 women, mean age; 26.1 ± 5.9) and 25 healthy subjects (3 men and 22 women, mean age, 25.4 ± 4.3) were studied. Patients were divided into two groups according to their BHS (group I, MVP+BJHMS; group II, MVP-BJHMS). Individuals with accompanying cardiac or systemic disease were excluded. Echocardiographic examination was performed in all subjects. The presence of BJHMS was evaluated according to Beighton's criteria.The incidence of BJHMS in patients with MVP was found to be significantly higher than that of controls (45.6%, (21/46) vs 12% (3/25), P < 0.0001). Group I (MVP + BJHMS) had significantly increased anterior mitral leaflet thickness (AMLT, 3.4 ± 0.4 vs 3.1 ± 0.3; P < 0.005), maximal leaflet displacement (MLD, 2.4 ± 0.4 vs 1.7 ± 0.4; P < 0.005), and degree of mitral regurgitation (DMR, 17.1 ± 7.2 vs 11.2 ± 4.4; P < 0.01) compared to group II. However, the index of aortic stiffness (IAOS) was found to be lower (17.6 ± 6.9 vs 23.9 ± 7.6; P < 0.005) and aortic distensibility (AOD) to be higher (0.0035 ± 0.007 vs 0.0024 ± 0.005; P < 0.005) in group I. There was a significant correlation between AMLT, MLD and DMR, and BHS (r = 0.57/P = 0.007, r = 0.55/P < 0.009, r = 0.51/P < 0.01, respectively). In addition, AOD correlated positively with BHS (r = 0.53/ P < 0.005), but the index of aortic stiffness correlated inversely with BHS (r = -0.49/P < 0.007).The incidence of BJHMS in patients with MVP was more frequent than the normal population and there was a significant correlation between the severity of BJHMS From the
Aims: Although there are plenty of data about the differences in left ventricular tissue Doppler (TD) velocities by preload reduction, only a few studies regarding right ventricular function are found in the literature. We investigated the effect of intravascular volume reduction on right ventricular function by ultrafiltration in dialysis patients. Methods: 27 end-stage renal failure patients who were hypervolemic and undergoing hemodialysis were included in the study. TD studies of the right ventricle were performed before and 1 h after dialysis. These data were compared. Results: The mean age of the patients was 41 ± 15 years and mean volume of ultrafiltration was 3.8 ± 1.8 liters. Systolic, early and late diastolic lateral annular TD velocities before dialysis were 0.109 ± 0.029, 0.088 ± 0.039, 0.111 ± 0.039 m/s, and after dialysis were 0.099 ± 0.028, 0.078 ± 0.036, 0.106 ± 0.037 m/s, respectively (p = 0.216, p = 0.112, p = 0.350). Myocardial early diastolic velocity decreased significantly (p = 0.049) but systolic and late diastolic velocities did not change significantly (p = 0.579, p = 0.146). Conclusion: Right ventricular systolic and diastolic velocities detected by TD were not or only minimally affected by preload reduction in hemodialysis patients and the TD early/late ratio is the most valuable variable that can predict right ventricular diastolic function. The right ventricular systolic and early diastolic TD velocities were positively correlated with left ventricle ejection fraction.
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