Advanced Medical Use of Three-Dimensional Imaging in Congenital Heart Disease: Augmented Reality, Mixed Reality, Virtual Reality, and Three-Dimensional Printing

Magnetic Resonance Imaging Assessment of Blood Flow Distribution in Fenestrated and Completed Fontan Circulation with Special Emphasis on Abdominal Blood Flow

OBJECTIVE: To investigate the regional flow distribution in patients with Fontan circulation by using magnetic resonance imaging (MRI). MATERIALS AND METHODS: We identified 39 children (18 females and 21 males; mean age, 9.3 years; age range, 3.3–17.0 years) with Fontan circulation in whom flow volumes across the thoracic and abdominal arteries and veins were measured by using MRI. The patients were divided into three groups: fenestrated Fontan circulation group with MRI performed under general anesthesia (GA) (Group 1, 15 patients; average age, 5.9 years), completed Fontan circulation group with MRI performed under GA (Group 2, 6 patients; average age, 8.7 years), and completed Fontan circulation group with MRI performed without GA (Group 3, 18 patients; average age, 12.5 years). The patient data were compared with the reference ranges in healthy controls. RESULTS: In comparison with the controls, Group 1 showed normal cardiac output (3.92 ± 0.40 vs. 3.72 ± 0.69 L/min/m2, p = 0.30), while Group 3 showed decreased cardiac output (3.24 ± 0.71 vs. 3.96 ± 0.64 L/min/m2, p = 0.003). Groups 1 and 3 showed reduced abdominal flow (1.21 ± 0.28 vs. 2.37 ± 0.45 L/min/m2, p < 0.001 and 1.89 ± 0.39 vs. 2.64 ± 0.38 L/min/m2, p < 0.001, respectively), which was mainly due to the diversion of the cardiac output to the aortopulmonary collaterals in Group 1 and the reduced cardiac output in Group 3. Superior mesenteric and portal venous flows were more severely reduced in Group 3 than in Group 1 (ratios between the flow volumes of the patients and healthy controls was 0.26 and 0.37 in Group 3 and 0.63 and 0.53 in Group 1, respectively). Hepatic arterial flow was decreased in Group 1 (0.11 ± 0.22 vs. 0.34 ± 0.38 L/min/m2, p = 0.04) and markedly increased in Group 3 (0.38 ± 0.22 vs. −0.08 ± 0.29 L/min/m2, p < 0.0001). Group 2 showed a mixture of the patterns seen in Groups 1 and 3. CONCLUSION: Fontan circulation is associated with reduced abdominal flow, which can be attributed to reduced cardiac output and portal venous return in completed Fontan circulation, and diversion of the cardiac output to the aortopulmonary collaterals in fenestrated Fontan circulation.

Magnetic Resonance Imaging Assessment of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia in Children

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a genetically determined disease that progresses continuously from conception and throughout life. ARVC/D manifests predominantly in young adulthood. Early identification of the concealed cases in childhood is of utmost importance for the prevention of sudden cardiac death later in life. Magnetic resonance imaging (MRI) is routinely requested in patients with a confirmed or suspected diagnosis of ARVC/D and in family members of the patients with ARVC/D. Although the utility of MRI in the assessment of ARVC/D is well recognized in adults, MRI is a low-yield test in children as the anatomical, histological, and functional changes are frequently subtle or not present in the early phase of the disease. MRI findings of ARVC/D include morphologic changes such as right ventricular dilatation, wall thinning, and aneurismal outpouchings, as well as abnormal tissue characteristics such as myocardial fibrosis and fatty infiltration, and functional abnormalities such as global ventricular dysfunction and regional wall motion abnormalities. Among these findings, regional wall motion abnormalities are the most reliable MRI findings both in children and adults, while myocardial fibrosis and fat infiltration are rarely seen in children. Therefore, an MRI protocol should be tailored according to the patient's age and compliance, as well as the presence of other findings, instead of using the protocol that is used for adults. We propose that MRI in children with ARVC/D should focus on the detection of regional wall motion abnormalities and global ventricular function by using a cine imaging sequence and that the sequences for myocardial fat and late gadolinium enhancement of the myocardium are reserved for those who show abnormal findings at cine imaging. Importantly, MRI should be performed and interpreted by experienced examiners to reduce the number of false positive and false negative readings.

Prenatal Diagnosis of Tetralogy of Fallot Associated with Chromosome 22q11 Deletion

Microdeletion of 22q11 is responsible for DiGeorge syndrome, velocardiofacial syndrome, congenital conotruncal heart defects, and related disorders. We report our experiences on prenatal diagnosis by fluorescence in situ hybridization (FISH) for 22q11 deletion in two fetuses with tetralogy of fallot. Karyotyping and FISH of the parents revealed that one fetus inherited the disease from maternal microdeletion. These findings suggest the importance of performing FISH in pregnancies with prenatally detected tetralogy of Fallot.

Ebstein's Anomaly: Echocardiographic and Clinical Features in the Fetus

BACKGROUND: Ebstein's anomaly is characterized by various degrees of apical displacement of the proximal attachment of the tricuspid valve. The disease has an extremely variable course in presenting itself. Therefore, we examined the morphologic and clinical features of Ebstein's anomaly as presented in the fetus to define the factors that determine its outcome. METHODS: We reviewed the history and echocardiographic studies of 10 fetuses (mean gestational age 28.0 weeks) diagnosed in utero with Ebstein's anomaly. The reason for referral was cardiomegaly on routine obstetric scanning in 5 cases, fetal arrhythmia in 3 cases, small main pulmonary artery in 3 cases, vessel view in 1, and twin pregnancy complicated by polyhydramniosis in 1. RESULTS: Of the 10 cases, pregnancy was terminated after diagnosis in 5, 2 cases were carried to term, and 1 case was at 34 weeks of gestation. We were unable to follow-up 2 cases. There were no intrauterine deaths and no misdiagnoses. Massive cardiomegaly and severe tricuspid regurgitation were detected in 7 cases; mild cases were detected in two, and one was nearly normal. Associated cardiac lesions included pulmonary artresia in 4 cases, pulmonary stenosis in 3, and pulmonary hypoplasia in 1. The cases with right ventricular outflow tract (RVOT) obstruction showed a tendency to develop more cardiomegaly and tricuspid regurgitation than with normal RVOT. There was no abnormality in other organ systems and karyotyping. CONCLUSION: Although increased cardiothoracic ratio and associated lesions of the right ventricular outflow tract contribute to the poor outcome in the prenatally detected cases, the absence of these features does not always indicate a good prognosis because progression of the disease can occur with advancing gestational age. No absolute measurement or single echocardiographic feature emerged as a consistent predictive factor for prognosis.

Clinical and MRI Findings of Acute Disseminated Encephalomyelitis Associated with Preceding Infections Confirmed Serologically

PURPOSE: Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system. The pathogenesis is not well known, but it is thought to be an immune-mediated disease and may follow a viral infection or vaccinations. MRI demonstrates decreased signal on T1 weighting and increased signal on T2-weighted image. We report clinical features and characteristic MRI findings in seven patients with ADEM who confirmed the preceding infections serologically. METHODS: Patients admitted to the Department of Pediatrics at Asan Medical Center as having ADEM were evaluated for the preceding infections : CSF findings, MRI findings, effects of high-dose steroids and follow-up on changes of neurological symtoms and MRI findings. RESULTS: The onset of neurological symptoms from preceding infection was between 1-3 weeks. The etiologies were Epstein-Barr virus in two, mycoplasma pneumoniae in three, and measles virus in two cases. All lesions found in MRI were cerebral white matter lesions of which there were subcortical white matter in three, corpus callosum in three, deep white matter in two, periventricular white matter in one, internal capsule in one, and cerebral peduncle in one case. There were a total of 3 cases with gray matter lesions. Two cases were treated with high- dose methyl prednisolone, one which showed a good clinical response with much improved lesions on follow-up T2-weighted image, but no clinical response in the other with aggravated MRI lesions. There was 1 patient with gadolinium enhancement lesion in acute phase and the lesion was completely resolved after steroid treatment. CONCLUSION: MRI shows various features in ADEM and may be useful in diagnosis and evaluation of clinical course.

Prenatal Diagnosis and Sonographic Findings of Down Syndrome; Review of 30 Cases / 대한산부인과학회잡지

No abstract available.

PRENATAL SONOGRAPHIC DIAGNOSIS OF CLEFT LIP * PLATE

No abstract available.

A Case Report of Gallbladder Duplication

Congenital duplication of the gallbladder, including true duplication and septated gallbladder, is an uncommon but potentially complicated malformation. It presents biliary colic associated with acute or chronic cholecystitis. It can be diagnosed preoperatively by various imaging tools such as real-time ultrasonography, biliary scintigraphy, oral cholecystography, or endoscopic retrograde cholangiography. It can be safely managed by either laparoscopic or open procedures. This report describes a 6-year-old girl with true duplication of gallbladder diagnosed preoperatively by real-time ultrasonography and treated by open cholecystectomy.

Delayed-Onset Adrenoleukodystrophy after Cerebral Contusion: Progressive Pattern of Demyelination on Serial MR Imaging

We described serial MR findings in a 20-year-old male with adrenoleukodystrophy who presented progressive neurologic deterioration after cerebral contusion. On MR imaging, progressive demyelination was predominant in the white matter of the right temporal lobe as well as in the parietal lobe at the site of prior trauma and extendedinto the contralateral hemisphere through the an terior commissure.

MR Findings of ADEIVI in Children

PURPOSE: To evaluate MR characteristics of acute disseminated encephalomyelitis (ADEM) in children, which was confirmed by clinical findings. MATERIALS AND METHODS: The subjects were six patients, who were diagnosed by clinical findings. One subject had recurrence one year after clinical improvement leading to one additional care with the total of seven. The modes of viral infections were as follows;four cases of non-specific upper respiratory tract infection, one of E-B virus, one of Japanese-B-encephalitis vaccination, and one of upper respiratory infection in Bruton's disease. The Gd-DTPA enhanced scan was performed in all cases. MR findings were evaluated in anatomic location of the lesions, presence or absence of contrast enhancement, and the temporal changes were also evaluated on follow-up MRI. RESULT: There were multifocal high signal intensity lesions on T2WI in all cases. The location of lesions were basal ganglia in five, thalamus and brain stem in four, and cerebral gray and white matter and cerebellar white matter in three. Bilaterality was 77%. There were contrast enhancement in two of three cerebral cortical lesions and one of three white matter lesions. The size of lesions decreased on the first follow-up MRI which were done after 1 month in 4 cases, but new lesions were developed in two cases. On the second follow-up MRI which were done 2 months after, all lesions were decreased in size and there was no newly developed lesion. However, in one case who had recurrent similar symptom after 1 year, several new lesions developed on follow-up MRI, and it was comidened as a recurrence. CONCLUSION: The characteristic MR findings of ADEM were multifocal bilateral white and gray matter lesions which were high signal intensities on T2WI. The majority of lesions improved on follow-up MRI, but occasionally showed multiphasic pattern.

Generalized Meconium Peritonitis Diagnosed with Antenatal Ultrasonography

Meconium peritonitis is defined as an aseptic, chemical or foreign-body peritonitis caused by spill of meconium in the abdominal cavity related to the prenatal perforation of the intestine. Perforation is usually caused by obstruction from meconium ileus, intestinal atresia, stenosis, volvulus, internal hernia, congenital pertoneal bands, intussusception, or gastroschisis. Less commonly, no evidence of distal obstruction exists. Here, we present two cases of generalized meconium peritonitis of antenatal diagnosis. The first case, detected at 8 months of gestational age, had a perforation of the proximal blind pouch of jejunal atresia, associated with respiratory distress due to severe abdominal distension. This case was successfullY treated with resection and anastomosis and brief period of postoperative ventilatory support. The second case had a distal ileal perforation with thick meconium in the terminal ileum. In this case, there was no dilatation of ileum proximal to the perforation site. Resection and anastomosis was performed and postoperative course was uneventful.

Bacterial meningitis in newborn and infant: Correlation between organism, CT findings and clinical outcome

Bacterial meningitis results in significant neurologic dificits despite in spite of much effort in the treatment of the disease. This study was performed to determine the incidence of caustive organisms and to correlate between the organisms and computed tomographic (CT) findings with clinical outcome of bacterial meningitis in newborns and infants. We analyzed the brain CT and clinical records of 15 infants who had been diagnosed as bactrial meningitis by CSF culture. We found that the most common organisms were Group B streptococcus in neonates withou no neurologic complications in all but one and Hemophilus influenza in infants whose clinical outomes were poor in all except one. CT findings related with poor prognosis in this study were cerebral edema, basal cistermal obliteration & enhancement, and cerebral infarction on initial CT and ventriculomegaly on follow-up CT. We concluded that CT diagnosed intracranial complications of bacterial meningitis well and could contributed to better treatment of bacterial meningitis.

Comparative value of cardiac MRI and echocardiography in the assessment of congenital heart lesions

To examine the usefulness of cardiac MRI in assessing patients (pt) with congenital heart diseases(CHD), informations obtained from MRI and echocardiogrphy (echo) were compared in 91 consecutive pt with CHD and was correlated with findigs at cardiac catheterization (53pt) and at surgery (71pt). Pt were studied with 1.5 Tesla MRI unit and multiplanar images of the heart and great vessels were obtained using ECG-gated multislice spin-echo technique. Age ranged from newborn to 22 years. We obtained the following results. MRI was vary useful in providing important diagnostic informations in 19pt, provided informations which was not crucial to the clinical decision in 28pt, and did not provide additional informations in 44pt. MRI was very useful in assessing complex lesions, particularly in identifying atrial situs, rudimentary ventricular chamber, criss-cross atrioventricular connection, total anomalous pulmonary venous connection, anatomy of ventricular septal defect in double outlet right ventricle, anomalous ventricular muscles, aortopulmonary collateral artery and distal pulmonary artery anatomy. En face view of the ventricular septum was very useful in clearly outlining the ventricular septal defect. MRI gave false information in 17pt. Diagnostic accuracy of MRI was poor for coarctation of the aorta in neonates and small infants, patent ductus arteriosus and pulmonary stenosis. Cardiac MRI is recommended for preoperative planning in selected pt with CHD, particularly with complex lesions.

P MR spectroscopy study of the brain at 4.7 T in new borns

In vivo 31P NMR spectra were obtained in eight infant brain at 4.7T. Each phosphorus metabolite and its ratio were analyzed to evaluate the brain damage and maturity, and compared with the reported data obtained at the lower field strength. Measurement of T1 relaxation time at 4.7T was done in an infant and a cat brain in vivo. PCr/Pi and PCr/β-ATP ratio were used as a marker of brain damage. PME/PDE revealed higher values than those of the reported data obtained at the lower field strength and the difference was partly attributed to the long T1 relaxation time of PME rather than the brain immaturity. Although the resolution of the spectrum was improved at 4.7T, a long repetition time is recommended to minimize T1difference of phosphorus metabolites of brain at 4.7T.

Various appearances of rib companion shadow mimicking a pathologic condition

We have observed that the companion shadow of the upper rib may be misinterpreted as a small pnemothorax or pleural plaque associated with asbestosis. To observe the radiographic characteristics of the normal companion shadow, we analyzed, on the posteroanterior(PA) chest radiographs, the companion shadow of 50 normal cases. Factors such as occurrence on each rib, the sharpness of the margin, the relative position to the rib, the shape and the thickness were observed. Also, we analyzed the displaced pleura of 4 pneumothorax cases to differentiate their frndings from the findings of normal companion shadows. On 50 normal chest radiographs, 192 compaion shadows were observed on the first to fourth ribs. In 173 of those shadows, the visceral margin of the companion shadow on the second rib simulated pneumothorax more closely than those on any othe rivs due to its apical location and thinness. In six of 50 normal cases, the companion shadow on the first or second rib showed an inw rdly convex lower margin, mimicking pleural plaque. The compaion shadow was suggested on the plain chest radiograph by the following characteristics imultiplicity(47/50), thicker than normal pleura(3/4), persistent on serial filma with the same shape and specific location(4/4).

Initial palliation of the pulmonary atresia with interventricular communication / 대한흉부외과학회지

No abstract available.

Balloon atrial septostomy through umbilical vein approach using flexible guide wire and sheath technique

No abstract available.

Tricuspid atresia associated with double orifice mitral valve and coronary sinus septal defect

No abstract available.

2 Case of Scimitar syndrome

No abstract available.