Isolated interrupted aortic arch in an adult: A case report.

BACKGROUND: Interrupted aortic arch (IAA) is a rare congenital heart disease defined by an interruption of the lumen and anatomical continuity between the ascending and descending major arteries. It is usually found within a few hours or days of birth. Without surgery, the chances of survival are low. If IAA patients have an effective collateral circulation established, they can survive into adulthood. However, IAA in adults is extremely rare, with few reported cases. CASE SUMMARY: A 27-year-old woman presented with a 6-year history of progressively worsening shortness of breath and chest tightness on exertion. She had cyanotic lips and clubbing of the fingers. A transthoracic echocardiogram revealed an enlarged heart and dilation of the main pulmonary artery. There was an abnormal 9 mm passage between the descending aorta and pulmonary artery. The ventricular septal outflow tract had a 14 mm defect. Doppler ultrasound suggested a patent ductus arteriosus and computed tomographic angiography showed the absence of the aortic arch. The diagnoses were ventricular septal defect, patent ductus arteriosus, and definite interruption of the aortic arch. Although surgical correction was recommended, the patient declined due to the surgical risks and was treated with medications to reduce pulmonary artery pressure and treat heart failure. Her condition has been stable for 12 mo of follow-up. CONCLUSION: Although rare, IAA should be considered in adults with refractory hypertension or unexplained congestive heart failure.

[MRI diagnosis of multiple cerebral sclerosis].

OBJECTIVE: To investigate the magnetic resonance imaging (MRI) feature of multiple cerebral sclerosis (MS) for better understanding and diagnosis of this disease. METHODS: The MRI data of 32 patients with MS were reviewed. Conventional scanning with T1WI, T2WI, Flair sequence was performed, and 26 patients underwent Gd-DTPA enhanced scanning. The MS plaques were analyzed for their locations, sizes, shapes, MR signals and enhanced features, space-occupying signs, and the related corpus callosum changes and brain atrophy. Descriptive statistical method was used for all the data. RESULTS: MRI identified MS lesions in the brain in 30 cases, with the sensitivity of 93.75%. All the MS patients had multiple lesions with predilection sites of the cortical/juxtacortical and periventricle areas, the centrum semiovale, and the corpus callosum. Most of the MS plaques were round or oval of different sizes. Bilateral lesions were almost symmetrical in distribution. Twenty patients had "rectangular demyelination" and 12 had "dirty white matter" signs, and 11 had both manifestations. The lesions were isointense, slightly hypointense or hypointense on T1WI, and hyperintense on T2WI and Flair sequences. Most of the MS plaques presented no enhancement, with occasional nodular or circular enhancement. No or slight space-occupying effect was found in the plaques. Of the 28 MS patients undergoing sagittal scanning of the corpus callosum, 17 presented with abnormal signals, with the sensitivity of 60.71% (17/28). Five patients had corpus callosum atrophy, and 10 had brain atrophy of different degrees. CONCLUSION: These results suggest that the corpus callosum is often compromised by the MS lesions to present diffusive, nodular, radiating signal abnormalities and irregular ependymal thickening, which can be most obvious with sagittal FLAIR imaging.

[Hemodynamic study of primary hepatocellular carcinoma evolved from viral-induced cirrhosis using CT perfusion imaging].

OBJECTIVE: To investigate the hemodynamic changes of primary hepatocellular carcinoma (HCC) evolved from hepatic cirrhosis using CT perfusion imaging. METHODS: Thirty-two patients with primary hepatocellular carcinoma evolved from virus-induced fibrosis or cirrhosis underwent dynamic CT scanning of the target slices for 60 min. The perfusion parameters of the hepatic parenchyma and HCC including the blood flow (BF), blood volume (BV), mean transit time (MTT), permeability-surface area product (PS), hepatic arterial fraction (HAF), IRF time of arrival (IRF TO) were obtained. Paired-sample t test was used to determine the differences in the perfusion parameters between the hepatic parenchyma and the primary HCC mass. RESULTS: Compared with hepatic BF (117.13-/+31.05 ml/100 mg/min), BV (14.73-/+3.91 ml/100 mg), PS (31.93-/+5.91 ml/100 mg/min), HAF (25.02-/+8.19%), MTT (12.79-/+3.31 s), IRF TO (3.14-/+1.09 s), the primary HCC mass showed significant increments in the BF (239.69-/+96.07 ml/100 mg/min), BV (20.26-/+6.73 ml/100 mg), PS (37.50-/+9.50 ml/100 mg/min), HAF (68.97-/+15.22%) with decreased MTT (7.17-/+1.38 s) and IRF TO (2.42-/+0.94 s). Significant differences were found in all the perfusion parameters between the hepatic parenchyma and HCC (P<0.05). CONCLUSION: Liver perfusion parameters can represent the hemodynamic changes in the HCC derived from hepatic cirrhosis.

Assessment of regional myocardial blood flow with myocardial contrast echocardiography: an experimental study.

OBJECTIVES: The aim of this study was to verify the accuracy of using myocardial contrast echocardiography (MCE), to quantify regional myocardial blood flow (MBF), and to evaluate myocardial viability in comparison to that measured by radiolabeled microsphere and pathologic examination. METHODS: Epicardial MCE was obtained in five myocardial ischemic dogs with constant microbubble intravenous infusion. After the video intensity (VI, y) versus pulsing interval plots derived from each myocardial pixel were fitted to an exponential function: y = A(1 - e(-beta t)), the MBF was calculated as the product of A (microvascular cross-sectional area or myocardial blood volume) and beta (mean myocardial microbubble velocity). The MBF was also obtained by radiolabeled microsphere method. RESULTS: The MBF derived by radiolabeled microsphere method in the normal, ischemic, and infarcted region was 1.5 +/- 0.3, 0.7 +/- 0.3, and 0.3 +/- 0.2 ml/min per gram, respectively; P < 0.01. The product of A and beta in those regions was 52.5 +/- 15.1, 24.4 +/- 3.9, and 3.7 +/- 3.8, respectively; P < 0.01. The normalized product of A and beta correlated well with normalized MBF (r = 0.81, P = 0.001). CONCLUSION: Our initial study demonstrated that MCE has an ability to assess MBF in ischemic myocardium in the experimental model. It may provide a potential capability to detect viable myocardium noninvasively after total persistent coronary occlusion in the clinical setting.