• Korean Journal of Radiology
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• v.2 no.3
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• pp.121-131
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• 2001

Echocardiography and catheterization angiography suffer certain limitations in the evaluation of congenital heart diseases in adults, though these are overcome by MRI, in which a wide field-of view, unlimited multiplanar imaging capability and three-dimensional contrast-enhanced MR angiography techniques are used. In adults, recently introduced fast imaging techniques provide cardiac MR images of sufficient quality and with less artifacts. Ventricular volume, ejection fraction, and vascular flow measurements, including pressure gradients and pulmonary-to-systemic flow ratio, can be calculated or obtained using fast cine MRI, phase-contrast MR flow-velocity mapping, and semiautomatic analysis software. MRI is superior to echocardiography in diagnosing partial anomalous pulmonary venous connection, unroofed coronary sinus, anomalies of the pulmonary arteries, aorta and systemic veins, complex heart diseases, and postsurgical sequelae. Biventricular function is reliably evaluated with cine MRI after repair of tetralogy of Fallot, and Senning's and Mustard's operations. MRI has an important and growing role in the morphologic and functional assessment of congenital heart diseases in adolescents and adults.

• Journal of Chest Surgery
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• v.39 no.6 s.263
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• pp.440-448
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• 2006

Background: Surgery of descending thoracic or thoracoabdominal aorta has the potential risk of causing neurological injury including spinal cord damage. This study was designed to find out the risk factors leading to spinal cord and brain damage after surgery of descending thoracic and thoracoabdominal aorta. Material and Method: Between October 1995 and July 2005, thirty three patients with descending thoracic or thoracoabdominal aortic disease underwent resection and graft replacement of the involved aortic segments. We reviewed these patients retrospectively. There were 23 descending thoracic aortic diseases and 10 thoracoabdominal aortic diseases. As an etiology, there were 23 aortic dissections and 10 aortic aneurysms. Preoperative and perioperative variables were analyzed univariately and multivariately to identify risk factors of neurological injury. Result: Paraplegia occurred in 2 (6.1%) patients and permanent in one. There were 7 brain damages (21%), among them, 4 were permanent damages. As risk factors of spinal cord damage, Crawford type II III(p=0.011) and intercostal artery anastomosis (p=0.040) were statistically significant. Cardiopulmonary bypass time more than 200 minutes (p=0.023), left atrial vent catheter insertion (p=0.005) were statistically significant as risk factors of brain damage. Left heart partial bypass (LHPB) was statistically significant as a protecting factor of brain (p=0.032). Conclusion: The incidence of brain damage was higher than that of spinal cord damage after surgery of descending thoracic and thoracoabdominal aorta. There was no brain damage in LHPB group. LHPB was advantageous in protecting brain from postoperative brain injury. Adjunctive procedures to protect spinal cord is needed and vigilant attention should be paid in patients with Crawford type II III and patients who have patent intercostal arteries.

• Journal of the Korean Society of Radiology
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• v.83 no.1
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• pp.70-83
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• 2022

Acute coronary syndrome (ACS) is mainly caused by atherosclerotic coronary artery disease (CAD); however, it can also occur in patients with non-atherosclerotic CAD. Conventional coronary angiography only shows the lumen of arteries, indicating the presence of stenosis or dilatation. Thus, it has limited value in evaluating the coronary artery wall and offers low specificity for diagnosing CAD. Coronary CT angiography provides additional information, including the depiction of the concerned vessel and the aorta, as well as the pulmonary artery, which permits the diagnosis of non-atherosclerotic CAD and the differentiation of various causes of the disease. In this review, we present the pathophysiology and CT imaging features of non-atherosclerotic CAD.

• Journal of the Korean Society of Radiology
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• v.82 no.2
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• pp.487-492
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• 2021

Immunoglobulin G4 (IgG4)-related disease is a systemic disease characterized by dense lymphoplasmacytic infiltrates with abundant IgG4-positive plasma cells and fibroblast proliferation. The retroperitoneal involvement of IgG4-related disease usually appears as a soft-tissue mass covering the abdominal aorta or entrapping the ureters, resulting in hydronephrosis. Here, we present a case of IgG4-related disease with retroperitoneal involvement in a 75-yearold woman with an unusual manifestation. A preoperative computed tomography (CT) scan revealed an irregular infiltrative retroperitoneal mass invading the normal anatomic barriers, raising the suspicion of malignancy or inflammation. Contrast-enhanced CT revealed a homogeneous progressive enhancement of the mass.

• Clinical and Experimental Pediatrics
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• v.51 no.9
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• pp.1012-1017
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• 2008

Purpose : This study aimed to assess the mechanical properties of the abdominal aorta in school-aged patients treated for Kawasaki disease and in normal, healthy children. Methods : This study examined 28 children with Kawasaki disease who had been followed up on and 30 healthy subjects of the same age and gender. We recorded systolic (Ps) and diastolic (Pd) blood pressure values and the aortic diameter at both minimum diastolic (Dd) and maximum systolic (Ds) expansion using two-dimensional echocardiography. These measurements were used to determine 1) aortic strain: S=(Ds-Dd)/Dd; 2) pressure strain elastic modulus: Ep=(Ps-Pd)/S; and 3) normalized Ep: $Ep^*=Ep/Pd$. Results : Ep (P=0.008) and $Ep^*$ (P=0.043) of the Kawasaki disease group were relatively high compared to those of the control group. Ep (P=0.002) and $Ep^*$ (P=0.015) of patients with coronary aneurysm were also relatively high compared with those of patients without coronary aneurysm, but lipid profiles did not differ, except for homocysteine (P=0.008). Therefore, in patients with coronary aneurysm, aortic stiffness was higher, compared to not only the control group but also patients without coronary aneurysm. However, in patients without coronary aneurysm, aortic stiffness was not significant, different compared to the control group. Conclusion : Measuring aortic distensibility may be helpful in assessing the risk of early atheroscletic change in the long-term management of Kawasaki disease.

• Clinical and Experimental Pediatrics
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• v.46 no.1
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• pp.86-90
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• 2003

Alagille syndrome is a autosomal dominant disorder characterized by intrahepatic bile duct paucity and resultant chronic cholestasis in combination with cardiac(mainly peripheral pulmonary stenosis), skeletal, ocular, and facial abnormalities. In addition to the pulmonary stenosis, in large series, anecdotal reports of vascular lesions have concerned the renal artery, aorta, hepatic artery, carotid artery, celiac artery or subclavian artery. Theses diffuse vascular abnormalities, which appear to be a feature of Alagille syndrome, suggest Notch signaling pathway defects affect angiogenesis. The associations of Alagille syndrome with moyamoya disease, the chronic cerebrovascular occlusive disease, were reported and suggested as additional evidence of vasculopathy of Alagille syndrome. We report another 25 month-old Alagille syndrome girl who presented with acute left hemiparesis and was diagnosed with moyamoya disease through the cerebral angiographic study.

• Korean Journal of Radiology
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• v.19 no.6
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• pp.1031-1041
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• 2018

Objective: To compare image quality and radiation dose of high-pitch dual-source spiral cardiothoracic computed tomography (CT) between non-electrocardiography (ECG)-synchronized and prospectively ECG-triggered data acquisitions in young children with congenital heart disease. Materials and Methods: Eighty-six children (${\leq}3$ years) with congenital heart disease who underwent high-pitch dual-source spiral cardiothoracic CT were included in this retrospective study. They were divided into two groups (n = 43 for each; group 1 with non-ECG-synchronization and group 2 with prospective ECG triggering). Patient-related parameters, radiation dose, and image quality were compared between the two groups. Results: There were no significant differences in patient-related parameters including age, cross-sectional area, body density, and water-equivalent area between the two groups (p > 0.05). Regarding radiation dose parameters, only volume CT dose index values were significantly different between group 1 ($1.13{\pm}0.09mGy$) and group 2 ($1.07{\pm}0.12mGy$, p < 0.02). Among image quality parameters, significantly higher image noise ($3.8{\pm}0.7$ Hounsfield units [HU] vs. $3.3{\pm}0.6HU$, p < 0.001), significantly lower signal-to-noise ratio ($105.0{\pm}28.9$ vs. $134.1{\pm}44.4$, p = 0.001) and contrast-to-noise ratio ($84.5{\pm}27.2$ vs. $110.1{\pm}43.2$, p = 0.002), and significantly less diaphragm motion artifacts ($3.8{\pm}0.5$ vs. $3.7{\pm}0.4$, p < 0.04) were found in group 1 compared with group 2. Image quality grades of cardiac structures, coronary arteries, ascending aorta, pulmonary trunk, lung markings, and chest wall showed no significant difference between groups (p > 0.05). Conclusion: In high-pitch dual-source spiral pediatric cardiothoracic CT, additional ECG triggering does not substantially reduce motion artifacts in young children with congenital heart disease.

• The Journal of the Korean life insurance medical association
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• v.29 no.2
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• pp.29-32
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• 2010

Moyamoya disease (MMD) is a progressive occlusive disease of the cerebral vasculature with particular involvement of the circle of Willis and the arteries that feed it. MMD is one of cerebrovacular accident,which is treated with sugical maeuver in pediatic neurosurgery. Moyamoya (ie, Japanese for "puff of smoke") characterizes the appearance on angiography of abnormal vascular collateral networks that develop adjacent to the stenotic vessels. The steno-occlusive areas are usually bilateral, but unilateral involvement does not exclude the diagnosis. The exact etiology of moyamoya disease is unknown. Some genetic predisposition is apparent because it is familial 10% of the time. The disease may be hereditary and multifactorial. It may occur by itself in a previously healthy individual. However, many disease states have been reported in association with moyamoya disease, including the following: 1) Immunological - Graves disease/thyrotoxicosis 2) Infections - Leptospirosis and tuberculosis 3) Hematologic disorders - Aplastic anemia, Fanconi anemia, sickle cell anemia, and lupus 4) Congenital syndromes - Apert syndrome, Down syndrome, Marfan syndrome, tuberous sclerosis, Turner syndrome, von Recklinghausen disease, and Hirschsprung disease 5) Vascular diseases - Atherosclerotic disease, coarctation of the aorta and fibromuscular dysplasia, 6)cranial trauma, radiation injury, parasellar tumors, and hypertension etc. These associations may not necessarily be causative but do warrant consideration due to impact on treatment.(Mainly neurosurgical operation.) The incidence of moyamoya disease is highest in Japan. The prevalence of MMD is 1 person per 100,000 population. The prevalence and incidence of moyamoya disease in Japan has been reported to be 3.16 cases and 0.35 case per 100,000 people, respectively. With regard to sex, the female-to-male ratio is 1.4:1. A bimodal peak of incidence is noted, with symptoms occurring either in the first decade(5-10yr) or in the third and fourth decades (30-40yr)of life. Mortality rates of moyamoya disease are approximately 10% in adults and 4.3% in children. Death is usually from hemorrhage. In aspect of life insurance, MR is 1700%, EDR is 16 per 1000 persons. Children and adults with moyamoya disease (MMD) may have different clinical presentations. The symptoms and clinical course vary widely from asymptomatic to transient events to severe neurologic deficits. Adults experience hemorrhage more commonly; cerebral ischemic events are more common in children. Children may have hemiparesis, monoparesis, sensory impairment, involuntary movements, headaches, dizziness, or seizures. Mental retardation or persistent neurologic deficits may be present. Adults may have symptoms and signs similar to those in children, but intraventricular, subarachnoid, or intracerebral hemorrhage of sudden onset is more common in adults. Recently increasing diagnosis of MMD with MRI, followed by surgical operation is noted. MMD needs to be considered as the "CI" state now in life insurance fields.

• Journal of Chest Surgery
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• v.40 no.2 s.271
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• pp.83-89
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• 2007

Background: The surgical repair of an isolated coarctation of the aorta, without complex cardiac anomalies, has improved, with very good results. However, despite the success of surgical repair, many long-term complications, such as hypertension, re-coarctation and an aortic aneurysm, still exist. Material and Method: Between 1991 and 2006, 50 patients diagnosed with an isolated coarctation of the aorta were reviewed retrospectively. The incidence of re-coarctation and hypertension were compared with respect to age and surgical methods. Result: There were no early & late mortality, or post operative aortic aneurysms. Hypertension developed in 11 patients (22%). A greater number of patients in the child/adult group had hypertension (52.4%) than in the neonate/infant group (0%). With respect to the surgical methods, the patients in the graft interposition group suffered more hypertension (88.9%) than those in the EEEA (extended end to end anastomosis) group (5.3%). Post operative re-coarctation developed in 2 out of the 29 patients (6.9%) in the neonate/infant group and 2 out of the 21 patients (9.5%) in the child/adult group, but without any statistical difference. There were no statistical differences between the operative type-related groups. Conclusion: Even though the surgical outcomes have greatly improved, an isolated coarctation of the aorta still has many long-term problems, such as hypertension and re-coarctation. An isolated coarctation is accepted as a systemic vascular dysfunction, and often progresses to other cardiovascular diseases. Therefore, patients with a coarctation of the aorta have to be carefully followed-up, and aggressive management must be given when required.

• Journal of Chest Surgery
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• v.25 no.3
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• pp.276-282
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• 1992

From Jan. 1981 to Dec. 1989 eleven cases of Funnel Chest, ten were males and one was female, were underwent an operation at the Department of Thoracic and Cardiovascular Surgery. Cheonbuk National University Hospital. The age of patients ranged from 3 to 29 years old. They all had symptoms of feeling inferiority about chest deformity. The degree of concavity on the funnel chest varied in extent, and the severity which was measured by water volume filled into it varied from 20 ml to 140 ml. Nine patients were corrected by Ravitch method and two patients were corrected by the Modified sternal turnover method. The Ravitch method was more effective in asymmetrical, severe depression deformity and in children. On the other hand the Modified sternal turnover method with preservation of vascular supply of repair was more simple and more effective in case of relatively less severe, wide symmetrical depression deformity of the chest and in case of associated with congenital or acquired heart disease and disease of the ascending aorta. This method has the advantage of maintaining chest wall stability in postoperative period.