Congenital heart surgery (CHS) is technically demanding, and its training is extremely complex and challenging. Training of the surgeon’s technical skills has relied on a preceptorship format in which the trainees are gradually exposed to patients in the operating room under the close tutelage of senior staff surgeons. Training in the operating room is an inefficient process and the concept of a learning curve is no longer acceptable in terms of patient outcomes. The benefits of surgical simulation in training of congenital heart surgeons are well known and appreciated. However, adequate surgical simulation models and equipment for training have been scarce until the recent development of three-dimensionally (3D) printed models. Using comprehensive 3D printing and silicone-molding techniques, realistic simulation training models for most congenital heart surgical procedures have been produced. Newly developed silicone-molded models allow efficient CHS training in a stressfree environment with instantaneous feedback from the proctors and avoids risk to patients. The time has arrived when all congenital heart surgeons should consider surgical simulation training before progressing to real-life operating in a similar fashion to the aviation industry where all pilots are required to complete simulation training before flying a real aircraft. It is argued here that simulation training is not an option anymore but should be a mandatory component of CHS training.

Diagnosis ; Heart Defects, Congenital ; Humans ; Image Processing, Computer-Assisted ; Imaging, Three-Dimensional ; Printing, Three-Dimensional

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.
Anesthesia, General ; Arteries ; Cardiac Output ; Child ; Female ; Fontan Procedure ; Humans ; Magnetic Resonance Imaging ; Male ; Protein-Losing Enteropathies ; Reference Values ; Veins

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.
Adult ; Child ; Compliance ; Death, Sudden, Cardiac ; Dilatation ; Fertilization ; Fibrosis ; Gadolinium ; Humans ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy ; Magnetics ; Magnets ; Myocardium ; Reading ; Ventricular Dysfunction ; Ventricular Function

BACKGROUND/AIMS: The International Prognostic Index (IPI) and absolute lymphocyte count (ALC) are prognostic factors in diffuse large B cell lymphoma (DLBCL). Nevertheless, in the Rituximab era, a new predictive marker related to Rituximab might be needed. We evaluated prognostic factors for survival in patients with early stage DLBCL after R-CHOP (Rituximab, cyclophosphamide, adriamycin, vincristine, prednisolone) treatment. METHODS: From Aug 2003 to Nov 2007, 78 patients with early stage DLBCL, who finished R-CHOP as scheduled, were reviewed retrospectively. Survival analyses were performed according to clinical parameters (age, performance status, lactate dehydrogenase (LDH), extra-nodal involvement, stage, ALC, and the rates of reduction of the white blood count (WBC) and ALC). RESULTS: Of the 78 patients with early stage DLBCL, 26 (33.3%) were classified as stage I. Seventy-three patients (93.6%) presented with a good performance status, while LDH was elevated in 20 patients (25.6%). According to the IPI, 67 (85.9%), 8 (10.3%), and 3 (3.8%) patients were classified in the low, low-intermediate, and high-intermediate risk groups, respectively. The overall response rate was 100%, including a 94.8% complete response. Survival analysis demonstrated that the rate of reduction of ALC following the first cycle of the R-CHOP regimen was the only factor associated with time-to-progression (p=0.037), whereas age was the single most important prognostic factor for overall survival (p=0.006). CONCLUSIONS: In our study, the rate of reduction of ALC in addition to age and IPI was found to be a significant prognostic factor in patients with early stage DLBCL treated with the R-CHOP regimen.
Antibodies, Monoclonal, Murine-Derived ; Cyclophosphamide ; Doxorubicin ; Humans ; L-Lactate Dehydrogenase ; Lymphocyte Count ; Lymphocytes ; Lymphoma, B-Cell ; Retrospective Studies ; Vincristine ; Rituximab

OBJECTIVES: Delayed sleep phase syndrome (DSPS) is characterized by difficulties in falling asleep and waking up at a desired time. Dawn simulation is a technique using a light that gradually increases in intensity before awakening in the morning, to imitate a natural sunrise. It has been found to be effective in decreasing both morning drowsiness and difficulty in awakening as well as treating symptoms of seasonal affective disorder. The aim of this study was to determine whether dawn simulation is helpful in decreasing difficulty in morning awakening and daytime sleepiness in adolescents with DSPS. METHODS: Twelve adolescents with DSPS participated in a 2-week dawn simulation trial. Each subject self-assessed level of difficulty in awakening, morning drowsiness and daytime sleepiness during a 2-week baseline period and a following 2-week trial period with dawn simulator. Subjects used Stanford Sleepiness Scale (SSS) for measuring morning drowsiness and Epworth Sleepiness Scale (ESS) for measuring daytime sleepiness. Difficulty in awakening was assessed by a single-item questionnaire. RESULTS: Dawn simulation trial decreased morning drowsiness (p=0.016) and daytime sleepiness (p=0.013) significantly compared to baseline. It also seemed to improve difficulty in awakening, but the effect was not statistically significant (p=0.092). CONCLUSION: Dawn simulation may help waking up in the morning and may improve daytime functioning by deceasing both morning drowsiness and daytime sleepiness in adolescents with DSPS.
Adolescent* ; Humans ; Surveys and Questionnaires ; Seasonal Affective Disorder ; Sleep Disorders, Circadian Rhythm* ; Sleep Stages

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.
Adult ; *Chromosome Deletion ; *Chromosomes, Human, Pair 22 ; Echocardiography ; Female ; Fetal Diseases/*diagnosis/genetics ; Humans ; In Situ Hybridization, Fluorescence/methods ; Pregnancy ; *Prenatal Diagnosis/methods ; Tetralogy of Fallot/*diagnosis/genetics

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.
Central Nervous System ; Chungcheongnam-do ; Corpus Callosum ; Demyelinating Diseases ; Diagnosis ; Encephalomyelitis, Acute Disseminated* ; Follow-Up Studies ; Gadolinium ; Herpesvirus 4, Human ; Humans ; Internal Capsule ; Magnetic Resonance Imaging* ; Measles virus ; Mycoplasma pneumoniae ; Pediatrics ; Pneumonia, Mycoplasma ; Prednisolone ; Steroids ; Tegmentum Mesencephali ; Vaccination

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.
Arrhythmias, Cardiac ; Cardiomegaly ; Diagnosis ; Diagnostic Errors ; Ebstein Anomaly* ; Echocardiography* ; Fetus* ; Follow-Up Studies ; Gestational Age ; Karyotyping ; Pregnancy ; Pregnancy, Twin ; Prognosis ; Pulmonary Artery ; Pulmonary Valve Stenosis ; Referral and Consultation ; Tricuspid Valve ; Tricuspid Valve Insufficiency

No abstract available.
Cleft Lip* ; Diagnosis* ; Ultrasonography*