Assessment of atrial septal defect size and residual rim using real-time 3D transesophageal echocardiography

Background

Accurate preoperative determination of defect location and size is important for successful transcatheter closure of atrial septal defects (ASD). Real-time 3D transesophageal echocardiography (3DTEE) has the potential to delineate the shape of ASD in 3D space.

Methods

Full volume and 3D zoom datasets by 3DTEE were acquired in 17 ASD patients. Using quantitative software, maximal/minimal diameter, defect area and residual rim length were measured and compared to the standard 2D measurements.

Results

Real-time 3DTEE allowed delineation of the en-face view of the ASDs. The defect typically had an oval shape, and its size changed dynamically, having its minimal size at end-diastole and maximal at end-systole. A good correlation was noted between the maximal defect area by 3DTEE and 2DTEE (r = 0.93, p < 0.001). Successful delineation of rim length to the specific cardiac structure was 100% by 3DTEE and 88% by 2DTEE. There was a fair correlation of residual rim length between 3DTEE and 2DTEE (r = 0.69, p < 0.001). Eight patients underwent transcatheter closure of the ASD. Excellent correlation was noted between 3D-derived maximal defect diameter and device diameter (r = 0.97, p < 0.001).

Conclusions

Real-time 3DTEE allows measurements of the temporal and spatial changes of ASD size and shape. This methodology provides detailed information on defect dynamics.     

Surgical treatment of thoracic aortic aneurysm in patients with concomitant coronary artery disease

Objective: Surgical treatment of thoracic aortic surgery in patients with coronary artery disease was investigated. Methods: Between 1990 and April 2003, 330 patients underwent elective thoracic aortic surgery. Fifty-six patients who underwent aortic root reconstruction were excluded and 274 patients were examined. Fifty-four (20%) patients showed concomitant coronary artery disease. Ten had undergone coronary revascularization previously; and 3 underwent coronary revascularization [2 coronary artery bypass grafting (CABG), 1 percutaneous transluminal coronary angioplasty (PTCA)] before aortic surgery. Twenty-three patients underwent elective CABG simultaneously and 2 patients had additional coronary artery bypass because of cardiac ischemia during operation. The number of patients who underwent thoracic aortic surgery including Asc Ao+AVR was 2, hemi arch 1, total arch 15, distal arch 5, distal arch+LV aneurysmectomy 1, and thoracoabdominal Ao 1. Two patients underwent coronary revascularization with arterial grafts and the others with SVG grafts. Results: There was one hospital death (4%). In patients without coronary bypass, 2 patients suffered cardiac ischemic events. Conclusion: Our thoracic aortic operations with concomitant CABG using SVG were overall successful. Our current strategies for thoracic aortic surgery in patients with concomitant coronary artery disease include conducting a dipyridamole myocardial perfusion-imaging test first in patients not at risk of coronary artery disease, and if the test is positive, coronary angiography is performed and aggressive coronary revascularization is conducted where possible.     

Prediction of iopromide reduction rates during haemodialysis using an in vitro dialysis system.

BACKGROUND: In clinical studies, it has been difficult to evaluate the influence of haemodialysis (HD) parameters on HD clearance (CL(HD)) and reduction rate (RR) of non-ionic contrast medium during HD sessions. We therefore predicted clinical values of CL(HD) and RR of iopromide, a non-ionic contrast medium, from findings obtained from in vitro experiments, and confirmed that these predictive values were comparable with the actual values in clinical cases. METHODS: We developed a correlation equation for predicting CL(HD) on the basis of in vitro HD experiments by varying blood flow rates between 100 and 200 ml/min with a cuprammonium rayon dialyser (AM-SD-10H). Total body clearance of iopromide (CL(PT)) was estimated by the Cockroft-Gault equation. The volume of distribution (V(d)) was obtained from the reported value. By using the HD and three pharmacokinetic parameters (CL(HD), CL(PT) and V(d)), we predicted CL(HD) and RR for seven patients undergoing HD after the administration of iopromide. RESULTS: In the in vitro study, the mean values (+/-SD) of iopromide clearance at blood flow rates of 100, 150 and 200 ml/min were 45.35 (2.54), 53.88 (6.46) and 57.61 (4.72) ml/min, respectively. There were highly significant correlations between clearance and blood flow rate (r = 0.975). Although the predicted CL(HD) showed a tendency towards underestimation, a good correlation was found. Predicted RR values were similar to observed values except for one case. CONCLUSION: The in vitro model used in the present study provides pertinent information about CL(HD) and is helpful for predicting RR during HD in individual patients undergoing HD.     

Surgical treatment of ventricular and pericardial perforation by a permanent pacing lead: a case report

An 83-year-old woman was transferred to our hospital because of pacing failure and suspected ventricular perforation by a permanent pacing lead. She had undergone permanent pacemaker implantation 5 months previously. Chest radiography showed the pacing lead running out of the cardiac shadow. Computed tomography and echocardiography confirmed the diagnosis of ventricular perforation by the pacing lead. No evidence of cardiac tamponade was found. The lead was surgically removed through a median sternotomy. Intraoperatively, the lead was found perforating the ventricle and the pericardium, and reaching into the left pleural cavity but not injuring the left lung. A pacing lead may potentially injure the heart or the lung. Regular check-up of lead position and pacing status is recommended.     

Novel ACOX1 mutations in two siblings with peroxisomal acyl-CoA oxidase deficiency

Peroxisomal acyl-CoA oxidase (ACOX1) deficiency is a rare autosomal recessive single enzyme deficiency characterized by hypotonia, seizures, failure to thrive, developmental delay, and neurological regression starting from approximately 3 years of age.Here, we report two siblings with ACOX1 deficiency born to non-consanguineous Japanese parents. They showed mild global developmental delay from infancy and began to regress at 5 years 10 months and 5 years 6 months of age respectively. They gradually manifested with cerebellar ataxia, dysarthria, pyramidal signs, and dysphasia. Brain MRI revealed T2 high-intensity areas in the cerebellar white matter, bilateral middle cerebellar peduncle, and transverse tracts of the pons, followed by progressive atrophy of these areas.Intriguingly, the ratios of C24:0, C25:0, and C26:0 to C22:0 in plasma, which usually increase in ACOX1 deficiency were within normal ranges in both patients. On the other hand, whole exome sequencing revealed novel compound heterozygous variants in ACOX1: a frameshift variant (c.160delC:p.Leu54Serfs*18) and a missense variant (c.1259 T > C:p.Phe420Ser). The plasma concentration of individual very long chain fatty acids (C24:0, C25:0, and C26:0) was elevated, and we found that peroxisomes in fibroblasts of the patients were larger in size and fewer in number as previously reported in patients with ACOX1 deficiency. Furthermore, the C24:0 β-oxidation activity was dramatically reduced.Our findings suggest that the elevation of individual plasma very long chain fatty acids concentration, genetic analysis including whole exome analysis, and biochemical studies on the patient’s fibroblasts should be considered for the correct diagnosis of ACOX1 deficiency.