OBJECTIVE: Our purpose was to establish a birth defects monitoring system in Korea by multi-center study. This novel study was initiated in 1999, and extended to detect the frequencies and trends of birth defects in Korea. METHODS: Six centers participated in this study. The actively ascertained surveillance data was collected from May 1999 to November 2001. RESULTS: Of the 65,653 births included in this study, 1,143 (1.7%) had birth defects. About one third of them were terminated. While disease of the genitourinary tract was more frequent in isolated defects, cardiovascular disease was more frequent in combined defects (19.7% and 21.7%, respectively). Chromosomal anomalies were detected 23.5 per 10,000 births. And it showed increasing tendency for 3 years. CONCLUSION: We could establish multi-center monitoring system for birth defects successfully. But, many of the problems arising in the collection of accurate, valid, and comparable epidemiological data about birth defects have not yet been overcome. It appears that the development of joint projects at national level is essential for upgrading the quality and usefulness of this study.
Cardiovascular Diseases ; Congenital Abnormalities* ; Joints ; Korea* ; Parturition*

A pulmonary artery sling is a very rare congenital abnormality in which the left pulmonary artery rises from the posterior surface of the right pulmonary artery and then passes between the trachea and the esophagus, causing tracheal compression. It is associated with tracheo-bronchial abnormalities (50%) and cardiovascular abnormalities (30%). It may produce respiratory symptoms through the airway compression of the abnormal left pulmonary artery and congenital abnormalities associated with it. Because most (90%) pulmonary artery sling patients present symptoms during infancy, their condition is often diagnosed in the first year of life. However, a pulmonary artery sling is occasionally found in adults. It is usually asymptomatic and found incidentally. This is a very rare case of an asymptomatic pulmonary artery sling in an adult. A 38-year-old man presented symptoms of mild exertional dyspnea. His spiral computed tomography showed a pulmonary artery sling. He was discharged without specific treatment because his symptoms improved without specific treatment and might not have been associated with a pulmonary artery sling. We report an adult case of an asymptomatic pulmonary artery sling diagnosed via spiral computed tomography, accompanied by a literature review.
Adult* ; Cardiovascular Abnormalities ; Congenital Abnormalities ; Dyspnea ; Esophagus ; Humans ; Pulmonary Artery* ; Tomography, Spiral Computed ; Trachea

OBJECTIVE: A growing body of literature has documented that job stress is associated with the development of cardiovascular disease. Nevertheless, the pathophysiological mechanism of this association remains unclear. Therefore, we tried to elucidate the relationship between job stress, heart rate variability and metabolic syndrome. METHOD: The study design was cross-sectional, and 169 industrial workers were recruited. A structured-questionnaire was used to assess the general characteristics and job characteristics (work demand, decision latitude). Heart rate variability (HRV) was recorded using SA-2000 (medi-core). HRV was assessed by time-domain and by frequency-domain analyses. Time domain analysis was performed for SDNN (Standard Deviation of NN interval), and spectral analysis for low-frequency (LF), high-frequency (HF) and total frequency power. Metabolic syndrome was defined on the basis of clustering of risk factors, when three or more of the following cardiovascular risk factors were included in the fifth quintile: glucose, systolic blood pressure, HDL-cholesterol (bottom quintile), triglyceride and waist-hip ratio. RESULTS: The results showed that job characteristics were not associated with cardiovascular risk factors. The high strain group had a less favorable cardiovascular risk profile with higher levels of blood pressure, glucose, homocysteine, and clotting factor than the lower strain group (low strain+passive+active group), but the difference was not statistically significant. The SDNN of HRV was significantly lower in the high strain group than in the low strain group. The prevalence of metabolic syndrome in the low strain, passive, active and high strain groups was 9.7%, 13.9%, 14.9% and 23.8%, respectively. In the high strain group, the metabolic syndrome was significantly related to a decreased SDNN. However, we could not find a significant association in LF/HF ratio. CONCLUSION: This result suggests that decreased HRV does not play a role in the development of disease; however, it can induce cardiovascular abnormalities or dysfunctions related to the onset of heart disease among high risk groups.
Blood Pressure ; Cardiovascular Abnormalities ; Cardiovascular Diseases ; Glucose ; Heart Diseases ; Heart Rate* ; Heart* ; Homocysteine ; Prevalence ; Risk Factors ; Triglycerides ; Waist-Hip Ratio

Kommerell's diverticulum is a rare congenital disorder characterized by typical right sided aortic arch and aberrant left subclavian artery which are usually detected by accident in asymptomatic patients. However, some of patients complain of severe symptoms caused by compression of the adjacent organs or complicated aortic dissection by the diverticulum. Early detection of the disease can lead to elective surgical correction. In this article, we report a Kommerell's diverticulum case initially detected by transesophageal echocardiography.
Aneurysm ; Aorta, Thoracic ; Cardiovascular Abnormalities ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Deglutition Disorders ; Diverticulum ; Echocardiography, Transesophageal ; Humans ; Subclavian Artery

Although it is a rare developmental malformation, van der Woude syndrome is the most common form of syndromic orofacial clefting, accounting for approximately 2% of all cleft cases. The lower lip pits with or without a cleft lip or palate is characteristic of the syndrome. Findings, such as hypodontia, limb deformities, popliteal webs, ankylogossia, ankyloblepheron, and genitourinary and cardiovascular abnormalities, are rarely associated with the syndrome. This paper reports a rare case of van der Woude syndrome in a 10-year-old male patient with a single median lower lip pit and a repaired bilateral cleft lip and cleft palate that were associated with microstomia, hypodontia, and clubbing of the left foot with syndactyly of the second to fifth lesser toes of the same foot.
Anodontia ; Cardiovascular Abnormalities ; Child ; Cleft Lip ; Cleft Palate ; Congenital Abnormalities* ; Extremities* ; Foot ; Humans ; Lip* ; Male ; Microstomia ; Palate ; Syndactyly ; Toes

Aneurysm ; pathology ; Aneurysm, Dissecting ; diagnosis ; Cardiovascular Abnormalities ; pathology ; Deglutition Disorders ; pathology ; Humans ; Male ; Middle Aged ; Subclavian Artery ; abnormalities ; pathology

Fetal bilateral renal agenesis is a lethal congenital anomaly characterized by bilateral pulmonary hypoplasia, deformities and death due to severe oligohydramnios. This syndrome is associated with malformations of genitourinary tract, cardiovascular system, vertebral bodies or imperforated anus in more than half of the affected individuals. An early and reliable prenatal diagnosis is extremely important because it may offer options for pregnancy termination as early as possible. The criteria for the ultrasonographic diagnosis of bilateral renal agenesis are severe oligohydramnios, nonvisualization of the bladder, empty renal fossae. But poor sonographic resolution of severe oligohydramnios makes it difficult to diagnose the disease. We present a case of bilateral renal agenesis diagnosed at the 18th weeks gestation by using Transvaginal Ultrasonography and Color Doppler.
Anal Canal ; Cardiovascular System ; Congenital Abnormalities ; Diagnosis ; Female ; Oligohydramnios ; Pregnancy ; Prenatal Diagnosis ; Ultrasonography* ; Urinary Bladder

Unilateral absence of pulmonary artery is a rare congenital anomaly that is frequently associated with other cardiovascular abnormalities. Most patients who have no associated cardiac anomalies have only minor or absent symptoms in childhood and survive into adulthood. We experienced a case of unilateral absence of pulmonary artery in a 5 year-old female patient with the chief complaint of abnormal chest X-ray finding. Unilateral absence of pulmonary artery was diagnosed by lung perfusion scan, cardiac catheterization with pulmonary and aortic angiography. So we report this case with brief review of related literatures.
Angiography ; Cardiac Catheterization ; Cardiac Catheters ; Cardiovascular Abnormalities ; Child, Preschool ; Female ; Humans ; Lung ; Perfusion ; Pulmonary Artery* ; Thorax

OBJECTIVE: Our aim was to establish a multi-center birth defects monitoring system for evaluating the prevalence and serial occurrence of birth defects in Korea. METHODS: Seven centers participated in this monitoring system. Trained nurses actively collected the 42,015 delivery records obtained from delivery units and pediatric clinics in the participating hospitals every month. RESULTS: We observed 722 birth defects among 42,015 births including live births and stillbirths. The prevalence of birth defect was 1.7%. About one thirds of birth defects were terminated. The most frequent congenital anomalies were cleft lip and Down syndrome. The highest proportion of birth defects was 20.1% in urogenital system. The proportion of birth defects in cardiovascular system and gastrointestinal system were 17.1% and 14.1% respectively. Chromosomal anomalies and genetic syndromes represent 13.4% and 3.3% among birth defects respectively. Among chromosomal anomalies Down syndrome was at the first rank. CONCLUSION: We could establish multi-center active monitoring system for birth defects successfully. To check serial occurrence of birth defects, it is necessary to increase participating hospitals and to launch the nationwide multi-center study.
Cardiovascular System ; Cleft Lip ; Congenital Abnormalities* ; Down Syndrome ; Korea* ; Live Birth ; Parturition* ; Prevalence ; Stillbirth ; Urogenital System

Heart rate variability is significantly associated with cardiovascular complications in various neurological disorders with cardiac impairment. Measures of spontaneous heart rate variability might be different from provocating tests of heart rate variability such as deep breathing and Valsava maneuver. Methods for analysis are divided into time domain methods and frequency domain methods. There are standard deviation of NN interval, standard deviation of average NN interval, root mean square of the successive differences, NN50, and pNN50 in time domain methods. Frequency domain bands can be divided into very low, low, and high frequency. Each variables are influenced by sympathetic and/or parasympathetic activity.
Autonomic Nervous System ; Cardiovascular Abnormalities ; Heart Rate* ; Nervous System Diseases ; Respiration