The recommendations outlined constitute the first clinical practice guidelines of the Korean Heart Rhythm Society regarding catheter ablation of ventricular arrhythmias (VA). This is a guideline PART 2, which includes VA in the structurally normal heart, inherited primary arrhythmia syndromes, VA related to congenital heart disease, as well as VA and sudden cardiac death observed in specific populations. In the structurally normal heart, treatment is guided by the occurrence of symptoms or the frequency of arrhythmias that cause ventricular dysfunction over time. Catheter ablation can be recommended in patients in whom anti-arrhythmic medications are ineffective. The sites of origin of arrhythmic activity are known to be the outflow tract, fascicles, papillary muscle, or the annulus. Specific cardiac channelopathies include congenital long QT and Brugada syndrome. This guideline discusses the diagnostic criteria, risk stratification, and treatment of these syndromes. We have included recommendations for adult congenital heart disease. Moreover, we have discussed the management of VA occurring in specific populations such as in patients with psychiatric and neurological disorders, pregnant patients, those with obstructive sleep apnea or drug-related pro-arrhythmias, athletes, and elderly patients.
Adult ; Aged ; Arrhythmias, Cardiac ; Athletes ; Brugada Syndrome ; Catheter Ablation ; Catheters ; Channelopathies ; Death, Sudden, Cardiac ; Heart ; Heart Defects, Congenital ; Humans ; Nervous System Diseases ; Papillary Muscles ; Sleep Apnea, Obstructive ; Ventricular Dysfunction

Maternal diabetes is known to have teratogenic effects which increase the risk for congenital anomalies, such as caudal dysplasia, cardiac defects, hydronephrosis, and small left colon syndrome. Infants of diabetic mothers have a 10-fold higher frequency of anomalies in the central nervous system and a 5-fold higher frequency of congenital heart defects. However, jejunal atresia combined with multiple anomalies of the face, ears, and hands has rarely been reported. Herein we report a neonate born to a diabetic mother, who had hemifacial microsomia, displacement of the lacrimal ducts, polydactyly of the right hand, microtia of the right ear and proximal jejunal atresia presenting as bile regurgitation on the 1st day of life.
Bile ; Central Nervous System ; Colon ; Congenital Abnormalities ; Diabetes, Gestational ; Displacement (Psychology) ; Ear ; Facial Asymmetry ; Female ; Hand ; Heart Defects, Congenital ; Humans ; Hydronephrosis ; Infant ; Infant, Newborn ; Intestinal Atresia ; Mothers ; Polydactyly ; Pregnancy

PURPOSE: To report a case of bilateral Marcus Gunn syndrome, which can be easily overlooked. CASE SUMMARY: A 6-year-old boy visited our clinic complaining of right ptosis. On ocular examination, the palpebral fissure width was 4 mm in the right lid and 7 mm in the left lid. Marginal reflex distance 1 (MRD 1) was -1 mm in the right lid and 2 mm in the left lid. Bilateral levator muscle function was good. When the patient moved the jaw to the right side, the left eyelid was retracted by 2 mm, and when the jaw was moved to the left side, the right upper eyelid retracted by 4 mm, which showed a moderate bilateral jaw-winking phenomenon. There was no change in bilateral palpebral fissure width when only opening the mouth without jaw movement. CONCLUSIONS: Asymmetrical bilateral ptosis can be easily misdiagnosed for unilateral ptosis, and concurrent Marcus Gunn phenomenon can exist. Therefore, a thorough examination is necessary when examining a ptosis patient.
Blepharoptosis ; Child ; Eyelids ; Heart Defects, Congenital ; Humans ; Jaw ; Jaw Abnormalities ; Mouth ; Muscles ; Nervous System Diseases ; Reflex ; Reflex, Abnormal

Background: Dioxin affects nervous system via the system of nervous communicative hormones and endocrine hormones, causing disorders and nervous behavior wrongs. Reproductive health is one of the serious effects of exposing Agent Organe/Dioxin. Objectives: To evaluate the birth defects of the nervous system of children, from the generation of veterans exposed to Agent Orange/Dioxin during wartime and to assess congenital nervous defects related factors, characteristics in these subjects. Subject and methods: A retrospective cohort, interview and examine 12.369 exposed and 7.659 unexposed Hanoi veterans, their birth defective children were examined by the pediatrics. During the 2002-2003 timescale. Results: A statistically high significant rate of reproductive failures and birth defects for exposed veterans, compared to unexposed ones, spontaneous abortion with relative risk RR = 4.18, hydatidiform mole with RR = 3.0, still births with RR = 2.2, birth defects with RR = 2.12. Nervous birth defects were highest; representing more than 40% of live births; the relative risk of the exposed group is 2.3 fold higher than the unexposed group, with p < 0.01. The birth defects increased significantly: mild mental retardation with RR = 3.5, severe mental retardation with RR = 3.5, Down's syndrome with RR = 2.16. Conclusions: Nervous birth defects of children of the Hanoi veterans exposed to Agent Orange during wartime accounted for the highest rate. Congenital nervous defects in male were higher than female. Exposed time, exposed levels and direct exposure to Agent Orange were significant related factors in this study. The increase of birth defects was in correlation with exposed levels.
congenital nervous defects ; birth defects ; veterans

BACKGROUND: Brain abscess is a important infectious disease of the central nervous system, although the mortality has been reduced due to new antibiotics therapy and improved imaging techniques. MATERIALS amp; METHODS: Over a period of 17 years, from March 1986 to Feburary 2003, 110 patients were identified as having brain abscess at the Severance Hospital. Based on medical records, we reviewed these cases and investigated retrospectively the epidemiology, clinical findings, therapeutic modalities and prognostic factors of brain abscess. RESULTS: There were 78 males and 32 females, and the mean age was 34.4 years. The most common location of brain abscess was the parietal lobe (24.5%), followed by temporal (20.9%) and frontal lobes (20.0%). Gram positive cocci were most frequently isolated as the causative microorganism. Tuberculous brain abscess were diagnosed in 6 (8.8%) cases. The common predisposing conditions were neurosurgery (21.8%), otitis media (10%) and congenital heart disease (8.2%). Headache (63.6%), fever (55.5%) and focal neurologic deficits (51.8%) were the common symptoms. Aspiration or open drainage was performed in 77 cases (70.0%), and excision was performed in 17 cases(15.5%). Thirty five (31.9%) patients had neurologic sequelae after treatment, and the mortality rate was 11.8%. Factors influencing mortality of brain abscess were mental status and multiple abscesses. CONCLUSION: Brain abscess is still a critical infectious disease of the central nervous system with high mortality and morbidity in Korea. Active surgical procedures including aspiration, open drainage or excision, as well as antibiotic therapy, are needed for the proper management.
Abscess ; Anti-Bacterial Agents ; Brain Abscess* ; Brain* ; Central Nervous System ; Communicable Diseases ; Drainage ; Epidemiology ; Female ; Fever ; Frontal Lobe ; Gram-Positive Cocci ; Headache ; Heart Defects, Congenital ; Humans ; Korea ; Male ; Medical Records ; Mortality ; Neurologic Manifestations ; Neurosurgery ; Otitis Media ; Parietal Lobe ; Retrospective Studies

BACKGROUND: Brain abscess is a important infectious disease of the central nervous system, although the mortality has been reduced due to new antibiotics therapy and improved imaging techniques. MATERIALS amp; METHODS: Over a period of 17 years, from March 1986 to Feburary 2003, 110 patients were identified as having brain abscess at the Severance Hospital. Based on medical records, we reviewed these cases and investigated retrospectively the epidemiology, clinical findings, therapeutic modalities and prognostic factors of brain abscess. RESULTS: There were 78 males and 32 females, and the mean age was 34.4 years. The most common location of brain abscess was the parietal lobe (24.5%), followed by temporal (20.9%) and frontal lobes (20.0%). Gram positive cocci were most frequently isolated as the causative microorganism. Tuberculous brain abscess were diagnosed in 6 (8.8%) cases. The common predisposing conditions were neurosurgery (21.8%), otitis media (10%) and congenital heart disease (8.2%). Headache (63.6%), fever (55.5%) and focal neurologic deficits (51.8%) were the common symptoms. Aspiration or open drainage was performed in 77 cases (70.0%), and excision was performed in 17 cases(15.5%). Thirty five (31.9%) patients had neurologic sequelae after treatment, and the mortality rate was 11.8%. Factors influencing mortality of brain abscess were mental status and multiple abscesses. CONCLUSION: Brain abscess is still a critical infectious disease of the central nervous system with high mortality and morbidity in Korea. Active surgical procedures including aspiration, open drainage or excision, as well as antibiotic therapy, are needed for the proper management.
Abscess ; Anti-Bacterial Agents ; Brain Abscess* ; Brain* ; Central Nervous System ; Communicable Diseases ; Drainage ; Epidemiology ; Female ; Fever ; Frontal Lobe ; Gram-Positive Cocci ; Headache ; Heart Defects, Congenital ; Humans ; Korea ; Male ; Medical Records ; Mortality ; Neurologic Manifestations ; Neurosurgery ; Otitis Media ; Parietal Lobe ; Retrospective Studies

The 13q deletion syndrome was described firstly by Allderdice et al. in 1969. Common findings include fetal growth restriction, microcephaly, other central nervous system malformations, eye abnormalities, characteristic facial appearance, congenital heart defects, gastrointestinal anomalies, vertebral, limb, and perineal defects, and varying degrees of mental retardation. We report a case of terminal deletion of chromosome 13q. Conventional cytogenetic result from amniocytes was 46,XY,del(13)(q32). The prenatal ultrasonographic findings were fetal growth restriction, oligohydramnios, microcephaly, ventriculomegaly and clubfoot. Gross anomalies after delivery included microcephaly, low set ears, absent thumbs, club foot, partial syndactyly of 4th and 5th toes, ambiguous genitalia, and imperforate anus. In addition to characteristic features of 13q terminal deletion, cleft lip and palate was found in our case. Cytogenetic abnormality probably results from phenotypically normal mother, whose karyotype was 46,XX,inv(3)(p26q13.1),t(13;20)(q32;p13) from lymphocytes.
Anus, Imperforate ; Central Nervous System ; Chromosome Aberrations ; Chromosomes, Human, Pair 13 ; Cleft Lip ; Clubfoot ; Cytogenetics ; Disorders of Sex Development ; Ear ; Extremities ; Eye Abnormalities ; Female ; Fetal Development ; Foot ; Heart Defects, Congenital ; Humans ; Intellectual Disability ; Karyotype ; Lymphocytes ; Microcephaly ; Mothers ; Oligohydramnios ; Palate ; Pregnancy ; Syndactyly ; Thumb ; Toes

Fryns syndrome is a lethal syndrome of multiple congenital anomalies first described by Fryns et al in 1979. A recently developed major diagnostic criteria includes abnormal face, small thorax with widely spaced hypoplastic nipples, distal limb and nail hypoplasia, lung hypoplasia with diaphragmatic hernia, central nervous system anomalies and congenital heart disease. The pathogenesis of Fryns syndrome is not clear. Of the major immediate life-threatening abnormalities of this syndrome, lung hypoplasia associated with diaphragmatic hemia has usually proven to be fatal. We report a case of Fryns syndrome, which has the prenatal ultrasonographic findings of Dandy-Walker malformation and renal hypoplasia.
Central Nervous System ; Dandy-Walker Syndrome ; Extremities ; Heart Defects, Congenital ; Hernia, Diaphragmatic ; Lung ; Nipples ; Thorax

Purpose: The diagnostic usefulness of fetal echocardiography in congenital heart disease is well known. One of the indications for fetal echocardiography is presence of extracardiac anomalies and such coexistent congenital heart disease may have important implications for obstetric and neonatal management. MATERIALS AND METHODS: 496 women with singleton pegnancies with amgenital anomalies that underwent penatal care and follow-up in Severance Hospital for 5 years from 1991 to 1995 were evaluated for the incidence of congenital heart disease. RESULTS: The incidence of congenital heart disease in fetuses with the associated extracardiac anomalies were as follows; central nervous system 6.9%(11/160), gastro-intestinal system 10.7%(8/75), genito-urinary sysem 4.7%(4/85), others 5.6%(4/72). Thirty-one of eighty-ane fetuses with congenital heart disease were found to have the associated extracardidac anomalies and ten of them chenoscenal abnormalities. CONCLUSION: We recommend that fetuses with congenital extracardiac anomalies sbould be checked for the presence of congenital heart disease and chmmosomal abnormalities, if needed.
Central Nervous System ; Echocardiography ; Female ; Fetus ; Follow-Up Studies ; Heart Defects, Congenital* ; Humans ; Incidence

Incontinentia pigmenti(IP) is an X-linked dominantly inherited disorder with female predominance. Skin lesions are characterized by three or four stages; vesicobullous, verrucous, hyperpigmented and hypopigmented lesions. About 80% of patients with incontinentia pigmenti have one or more associated ectodermal or mesodermal anomalies involving teeth, nail, hair, eye, breast, bones and nervous system. A newborn girl had erythematous based vesicles and bullae on her trunk and extremities with peripheral eosinophilia. Within several days, she showed linear verrucous plaques. A skin biopsy specimen showed eosinophilic spongiosis in the epidermis and numerous eosinophils in the dermis. The diagnosis of IP was made. She was revealed to have some congenital heart anomalies; atrial septal defect (ASD) and patent ductus arteriosus(PDA). Cases of IP with congenital heart disease have been reported very rarely. Therefore, we report this unique case of IP associated with ASD and PDA.
Biopsy ; Breast ; Dermis ; Diagnosis ; Ectoderm ; Eosinophilia ; Eosinophils ; Epidermis ; Extremities ; Female ; Hair ; Heart Defects, Congenital ; Heart Diseases* ; Heart Septal Defects, Atrial ; Heart* ; Humans ; Incontinentia Pigmenti* ; Infant, Newborn ; Mesoderm ; Nervous System ; Skin ; Tooth