The authors present a 67-year-old man who developed trigeminal neuralia caused by a dolichoectatic vertebrobasilar artery. Brain magnetic resonance imaging showed a tubular structure traversed the anterior surface of the sbrainstem, which compressed the left ven trilateral pons in the region of the trigeminal root entry zone. Vertebral angiography demonstrated a tortuous dilated vertebrobasilar artery. Microvascular decompression of the trigeminal nerve from the dolichoectatic vertebral artery and simultaneous selective trigeminal rhizotomy were performed. Postoperatively, the patient was relieved of pain but suffered a hearing deficit in the ipsilateral side.
Aged ; Angiography ; Arteries ; Brain ; Hearing ; Humans ; Magnetic Resonance Imaging ; Microvascular Decompression Surgery ; Pons ; Rhizotomy ; Trigeminal Nerve ; Trigeminal Neuralgia* ; Vertebral Artery*

BACKGROUND: A management protocol for hepatitis C virus (HCV) after liver transplantation (LT) has not been established in Korea. We therefore investigated HCV transplant protocols and post-transplant results from liver transplant centers in Korea. METHODS: The HCV protocol and medical data of individual cases from eight major liver transplant centers were compiled and analyzed. RESULTS: A post-transplant protocol biopsy was performed in only three centers. In these centers, HCV treatment was considered when pathological abnormalities were confirmed on the protocol biopsy (irrespective of liver function). In the other five centers, biopsies were performed when biochemical parameters were aggravated. Only two out of the eight centers performed preemptive or prophylactic therapy. A total of 5,663 adult LTs were performed between 2000 and 2010. HCV-related liver disease was responsible for 277 LTs (4.9%). Pre-transplant data were not available in many patients, including HCV genotype and serum HCV RNA level. Tacrolimus was more frequently used for initial maintenance immunosuppression than cyclosporine A (61.7% vs. 36.8%). Post-transplant HCV treatment was performed in 135 patients (48.7%). Sixty-seven recipients (24.2%) died during follow-up after LT and 11 HCV-related graft loss (4.0%) developed. The cumulative patient survival rate was 74.7% at 5 years and 67.9% at 10 years after LT. CONCLUSIONS: The HCV management protocol after LT varied markedly between the eight Korean transplant centers and a standard protocol did not exist. A nationwide multicenter study is required to investigate the most effective treatment for HCV after LT, with the goal of establishing the most effective standard protocol.
Adult ; Biopsy ; Cyclosporine ; Follow-Up Studies ; Genotype ; Hepacivirus ; Hepatitis ; Hepatitis C ; Humans ; Immunosuppression ; Korea ; Liver ; Liver Diseases ; Liver Transplantation ; RNA ; Survival Rate ; Tacrolimus ; Transplants

Cordyceps bassiana has long been used as an oriental medicine and reported to possess diverse biological activities. The fruiting bodies of Cordyceps bassiana was extracted with ethanol and then further fractionated with n-hexane, ethyl acetate, n-butanol and water. The butanol fraction from Cordyceps bassiana (CBBF) exhibited the most effective in anti-inflammatory activity in RAW 264.7 macrophages and the roles of CBBF on the anti-inflammation cascade in LPS-stimulated RAW 264.7 cells were studied. To investigate the mechanism by which CBBF inhibits NO, iNOS and COX-2, the activation of IκB and MAPKs in LPS-activated macrophage were examined. Our present results demonstrated that CBBF inhibits NO production and iNOS expression in LPS-stimulated RAW 264.7 macrophage cells, and these effects were mediated through the inhibition of IκB-α, JNK and p38 phosphorylation. Also, CBBF suppressed activation of MAPKs including p38 and SAPK/JNK. Furthermore, CBBF significantly suppressed LPS-induced intracellular ROS generation. Its inhibition on iNOS expression, together with its antioxidant activity, may support its anti-inflammatory activity. Thus Cordyceps bassiana can be used as a useful medicinal food or drug for further studies.
1-Butanol ; Cordyceps* ; Ethanol ; Fruit ; Inflammation* ; Macrophages ; Medicine, East Asian Traditional ; Phosphorylation ; RAW 264.7 Cells* ; Water

Management of severely dilated pulmonary artery (PA) associated with severe pulmonary hypertension from congenital heart disease remains controversial, primarily due to its rare nature and concern for perioperative unpredictable complications. Herein, we report a 25 year-old female with a severely dilated PA (up to 73 mm), who was successfully treated by a PA graft replacement by creating a Y-shaped conduit using a 28 mm hemashield tube in the main PA and a 20 mm hemashield tube in both proximal parts of the branch PA.
Female* ; Heart Defects, Congenital ; Humans ; Hypertension, Pulmonary* ; Pulmonary Artery* ; Pulmonary Surgical Procedures ; Transplants

PURPOSE: We assumed preoperative duplex examination may be helpful for determination of the site of hemodialysis fistula operation and result in decreased early failures. METHOD: From January 1999 through October 1999, 25 operations were performed in 23 patients (historical control, Group I). From November 1999 through June 2000, 30 operations were performed in 29 patients (Group II). During the latter period, preoperative duplex examination was selectively introduced for patients whose forearm veins were not prominent or who were suspected to have stenosis or obstruction of outflow. Forearm veins with a diameter more than 2.5 mm were sought and their continuity up to the elbow level was confirmed. When an adequate forearm vein was not detectable, veins on antecubital area with the diameter more than 4 mm was sought for graft fistula (GF). RESULT: Group I consisted of 24 autogenous fistulas (AF) and 1 GF, and group II consisted of 26 AF and 4 GF. Among the group II patients, 19 preoperative duplex examinations were done (18 patients). The mean diameter of forearm veins used for AF were 3.27mm (0.69 SD). Diameter of veins used for GF ranged from 3.7 to 6.0 mm. Early failure rate in group I was 16% (4/25), whereas it was 6.7% in group II (2/30). The two failures in group II were associated with sclerotic veins on physical examination. CONCLUSION: Our early results show that preoperative duplex scan may decrease early failure rate of fistula operation. Findings of duplex scan combined with avoidance of sclerotic veins on physical examination may significantly decrease the rate of early failure.
Constriction, Pathologic ; Elbow ; Fistula* ; Forearm ; Humans ; Physical Examination ; Preoperative Period ; Renal Dialysis* ; Transplants ; Veins

BACKGROUND AND OBJECTIVES: We studied the results of patient management for left isomerism (LI) and sought to determine factors that may influence survival and prognosis. SUBJECTS AND METHODS: We reviewed the medical records of 76 patients who were compatible with LI criteria between 1982 and 2014. RESULTS: Of the total study population, 29 patients (38.1%) had functional univentricular heart disease, 43 patients (56.5%) had cardiac anomalies suitable for biventricular hearts, and four patients (5.2%) had normal heart structure. Extracardiac anomalies were noted in 38.1% of the study population, including biliary atresia in 7.8% of all patients. Of the 25 patients who underwent Kawashima procedures, 24.0% developed pulmonary arteriovenous fistulas (PAVFs). During the median follow-up period of 11.4 years (range: 1 day to 32 years), 14 patients died. The 10-year, 20-year, and 30-year survival rates were 87%, 84%, and 76%, respectively. Preoperative dysrhythmia and uncorrected atrioventricular valve regurgitation were significantly associated with late death. There was no significant difference in the number of surgical procedures and in survival expectancy between patients in the functional single-ventricle group and in the biventricular group. However, late mortality was higher in functional single-ventricle patients after 18 years of age. CONCLUSION: Patients with LI need to be carefully followed, not only for late cardiovascular problems such as dysrhythmia, valve regurgitation, and the development of PAVFs, but also for noncardiac systemic manifestations.
Arrhythmias, Cardiac ; Arteriovenous Fistula ; Biliary Atresia ; Follow-Up Studies ; Heart ; Heart Diseases ; Heterotaxy Syndrome ; Humans ; Isomerism* ; Medical Records ; Mortality ; Prognosis ; Survival Rate

BACKGROUND AND OBJECTIVES: This study aimed to evaluate the usefulness of cardiopulmonary exercise test (CPET) in children with various congenital heart diseases (CHDs). SUBJECTS AND METHODS: Forty-eight children and adolescents (18 girls and 30 boys; mean+/-SD age, 12.6+/-0.5 years) with CHD who had undergone corrective surgery performed CPET using a programmable treadmill. The participants were divided into 4 groups based on the surgery performed: Fontan operation (12 patients), total correction for tetralogy of Fallot (13 patients), repair for coarctation of aorta (11 patients), and corrective surgery for simple CHDs (12 patients). Patients with simple CHDs who had undergone an early surgery were selected as controls. RESULTS: Progressive exercise time was the longest in the simple CHD group than in the other groups (10.6+/-2 versus 8.2-8.7 min, p=0.122). Absolute and predicted values of mean peak oxygen consumption were the lowest in the Fontan operation group (p=0.220 and 0.091, respectively). The coarctation of aorta group exhibited a prominent blood pressure response, that is, an elevation in the systolic blood pressure relative to that in the other groups at rest (131.7+/-18.8 versus 114.4-121.3 mmHg) and at peak exercise (196.5+/-41.5 versus 146.8-184.3 mmHg). CONCLUSION: CPET can be a useful tool to clarify the exercise capacity and reflect the hemodynamic status of patients who have undergone surgery for various CHDs and other heart diseases.
Adolescent ; Aortic Coarctation ; Blood Pressure ; Child ; Exercise Test* ; Female ; Fontan Procedure ; Heart Defects, Congenital* ; Heart Diseases ; Hemodynamics ; Humans ; Oxygen Consumption ; Tetralogy of Fallot

Takayasu arteritis is a chronic inflammatory disease of unknown etiology primarily affecting the aorta and its major branches and usually occurring in the second or third decade of life. Here, we report a case of Takayasu arteritis in a 10-month-old patient. The infant presented with signs of congestive heart failure and severe aortic regurgitation. Echocardiography and computed tomography angiography showed an abnormally dilated thoracic and abdominal aorta. The infant was initially treated with prednisolone, followed by commissuroplasty of the aortic valve but neither approach ameliorated the heart failure. The patient was eventually treated with a mechanical aortic valve replacement surgery at the age of 12 months, and her condition stabilized. Although unusual, this case indicates that the diagnosis of Takayasu arteritis should be considered in children with unexplained systemic symptoms, aortic valve regurgitation, and heart failure. Because severe aortic regurgitation may be a fatal complication of Takayasu arteritis, early aortic valve replacement surgery should be considered, even in very young children.
Angiography ; Aorta ; Aorta, Abdominal ; Aortic Valve ; Aortic Valve Insufficiency ; Child ; Echocardiography ; Heart Failure ; Humans ; Infant ; Prednisolone ; Takayasu Arteritis

PURPOSE: The use of implantable cardioverter defibrillators (ICDs) to prevent sudden cardiac death is increasing in children and adolescents. This study investigated the use of ICDs in children with congenital heart disease. METHODS: This retrospective study was conducted on the clinical characteristics and effectiveness of ICD implantation at the department of pediatrics of a single tertiary center between 2007 and 2011. RESULTS: Fifteen patients underwent ICD implantation. Their mean age at the time of implantation was 14.5+/-5.4 years (range, 2 to 22 years). The follow-up duration was 28.9+/-20.4 months. The cause of ICD implantation was cardiac arrest in 7, sustained ventricular tachycardia in 6, and syncope in 2 patients. The underlying disorders were as follows: ionic channelopathy in 6 patients (long QT type 3 in 4, catecholaminergic polymorphic ventricular tachycardia [CPVT] in 1, and J wave syndrome in 1), cardiomyopathy in 5 patients, and postoperative congenital heart disease in 4 patients. ICD coils were implanted in the pericardial space in 2 children (ages 2 and 6 years). Five patients received appropriate ICD shock therapy, and 2 patients received inappropriate shocks due to supraventricular tachycardia. During follow-up, 2 patients required lead dysfunction-related revision. One patient with CPVT suffered from an ICD storm that was resolved using sympathetic denervation surgery. CONCLUSION: The overall ICD outcome was acceptable in most pediatric patients. Early diagnosis and timely ICD implantation are recommended for preventing sudden death in high-risk children and patients with congenital heart disease.
Adolescent ; Cardiomyopathies ; Channelopathies ; Child ; Convulsive Therapy ; Death, Sudden ; Death, Sudden, Cardiac ; Defibrillators ; Defibrillators, Implantable ; Early Diagnosis ; Follow-Up Studies ; Heart ; Heart Arrest ; Heart Defects, Congenital ; Heart Diseases ; Humans ; Korea ; Long QT Syndrome ; Pediatrics ; Retrospective Studies ; Shock ; Sympathectomy ; Syncope ; Tachycardia, Supraventricular ; Tachycardia, Ventricular

Unguarded tricuspid regurgitation (TR) due to a flail tricuspid leaflet is a rare condition of newborn cyanosis. A high perinatal mortality has been associated with this fatal condition. But, there are feasible surgical repairs to improve survival. We report the case of a male full-term neonate with intractable hypoxia. He had profound tricuspid insufficiency and leaflet prolapse caused by a ruptured papillary muscle supporting the anterior leaflet of the tricuspid valve. He presented with severe cyanosis and respiratory distress immediately after birth. Despite medical management, the pulmonary vascular resistance was not decreased and a low cardiac output persisted. Initial stabilization was accomplished with nitric oxide and extracorporeal membrane oxygenation. The tricuspid valve repair surgery was successfully performed subsequently. TR resulting from papillary muscle rupture is a potentially lethal condition. Timely diagnosis and proper surgical treatment can be lifesaving.
Anoxia ; Cardiac Output, Low ; Cyanosis ; Diagnosis ; Extracorporeal Membrane Oxygenation ; Female ; Humans ; Infant, Newborn* ; Male ; Nitric Oxide ; Papillary Muscles* ; Parturition ; Perinatal Mortality ; Persistent Fetal Circulation Syndrome* ; Prolapse ; Rupture ; Thoracic Surgery ; Tricuspid Valve Insufficiency ; Tricuspid Valve* ; Vascular Resistance