Cyanotic congenital heart defect is one of the most important causes to evaluate the newborn presenting cyanosis because some of these defects may be fatal during the neonatal period. Recognizing cyanotic and critical congenital heart defects and providing appropriate supportive care before transport to a cardiac center are crucial in promoting survival and positive outcomes of affected newborns. This article will discuss the physiology and signs of cyanotic congenital heart defect, the evaluation process of the cyanotic newborn who has possibility of the cyanotic congenital heart defect and the proper management to stabilize these patients in neonate intensive care unit before transport or surgery.
Cyanosis ; Heart Defects, Congenital* ; Humans ; Infant, Newborn ; Intensive Care Units ; Intensive Care, Neonatal* ; Physiology

Posterior Reversible Encephalopathy Syndrome(PRES) mainly develops in patients under immunosuppressive therapy after transplantation, or patients who suffer hemato-oncologic diseases, eclampsia, acute hypertensive encephalopathy related with nephrotic disease. Sudden headache, nausea, mental derangement, convulsion, vision problems are the main symptoms shown in PRES. It typically shows edema of occipito-parietal area of cortex or subcortex in Magnetic Resonance Imaging(MRI). A 10-year-old male was hospitalized with the chief complaint of headache, vomiting and dizziness for 3 days. He was treated for upper respiratory infection for 1 week before the hospitalization. Initial blood pressure was 145/95 mmHg, which was high for his age. Uninalysis showed microscopic hematuria(3+). The hypertension persisted even after the hospitalization, and re-checked blood pressure was 175/115 mmHg. The patient complained of headache, and after that, he suffered from alteration of mental status with dysarthria and generalized type of seizure events. The MRI showed lesions suggestive of PRES. He recovered to alertmental status after the blood pressure was controlled. Antistreptolysin-O(ASO) titer was increased and complement 3(C3) titer was decreased. The follow up MRI taken 1 month after the event became normal. We report a case of PRES related with contemporary hypertensive event in Acute Post-Streptococcal Glomerulonephritis(APSGN).
Blood Pressure ; Child ; Complement System Proteins ; Dizziness ; Dysarthria ; Eclampsia ; Edema ; Female ; Follow-Up Studies ; Glomerulonephritis ; Headache ; Hospitalization ; Humans ; Hypertension ; Hypertensive Encephalopathy ; Magnetic Resonance Spectroscopy ; Male ; Nausea ; Posterior Leukoencephalopathy Syndrome ; Pregnancy ; Seizures ; Transplants ; Vision, Ocular ; Vomiting

PURPOSE: We investigated the spontaneous seroconversion rate of hepatitis B viral markers and predictive factors affecting seroconversion in children with chronic hepatitis B. METHODS: The study population included 214 children diagnosed as chronic hepatitis B, with positive HBsAg, HBeAg and HBVDNA over six months, and all patients had a family history of chronic Hepatitis B. They were followed between May 1982 and Febrary 2003 in the Department of Pediatrics, Yonsei University College of Medicine. Serum HBsAg, HBeAg, anti-HBs, HBVDNA, and AST/ALT were measured every six months. RESULTS: The mean age of patients was 7.4+/-4.5 years. The loss of HBeAg, HBVDNA and HBsAg were observed in 44(19.2%), 34(15.9%) and 3(1.4%) children respectively. The patients with serum ALT levels over three times normal and with HBVDNA less than 1,000 pg/dL showed significantly higher seroconversion rates of HBeAg and HBVDNA(P<0.001). By analyzing with the life table method, the cumulative seroconversion rate of HBeAg was 11% at 10 and 35% at 19 years of age, while HBVDNA was 9% at 10 and 32% at 19 years of age. In cases of HBsAg, the cumulative seroconversion rate was only 1.5% at 19 years of age. The age of patients, serum ALT and HBVDNA levels were proven as signifincant factors influencing the seroconversion of HBeAg and HBVDNA(P<0.001). CONCLUSION: In children with chronic hepatitis B, the seroconversion rate of HBeAg and HBVDNA are expected to be 35% and 32%, respectively, by the age of 19 years. The age of patients, serum ALT and HBVDNA levels seem to have significant influence on HBeAg and HBVDNA seroconversion.
Biomarkers ; Child* ; Hepatitis B ; Hepatitis B Antibodies ; Hepatitis B Antigens ; Hepatitis B e Antigens ; Hepatitis B Surface Antigens ; Hepatitis B, Chronic* ; Hepatitis, Chronic* ; Humans ; Life Tables ; Natural History* ; Pediatrics

Kawasaki disease is an acute systemic vasculitis of unknown origin. Giant coronary aneurysm is one of the most serious complications, although peripheral artery vasculitis can produce life-threatening events. Myocardial ischemia and infarction can be caused by coronary artery stenosis, aneurysm, and stagnation of blood flow in coronary arteries which triggers thromboembolism. Atypical presentation in young infants often interferes with prompt diagnosis and timely treatment, resulting in poor outcomes. We describe a 3-month-old infant with multiple giant coronary aneurysms with flow stagnation, stenosis and large mural thrombus due to Kawasaki disease. He presented with a prolonged course of severe coronary involvement in spite of all measures to reduce coronary complications. Finally, surgical intervention was tried because of the worsening coronary artery abnormalities. The patient died of acute cardiorespiratory failure shortly after weaning from cardiopulmonary bypass.
Aneurysm ; Arteries ; Cardiopulmonary Bypass ; Constriction, Pathologic ; Coronary Aneurysm* ; Coronary Stenosis ; Coronary Thrombosis ; Coronary Vessels ; Diagnosis ; Humans ; Infant* ; Infarction ; Mucocutaneous Lymph Node Syndrome* ; Myocardial Ischemia ; Systemic Vasculitis ; Thromboembolism ; Thrombosis* ; Vasculitis ; Weaning

Kawasaki disease is an acute systemic vasculitis of unknown origin. Coronary aneurysm or ectasia is one of the most serious complications of Kawasaki disease. The major complication of Kawasaki coronary disease is myocardial infarction caused by thrombus formation inside the aneurysm or by organic obstructive lesion following the regression of aneurysm. Percutaneous balloon angioplasty, rotational ablation, directional coronary artherectomy, stent insertion and coronary artery bypass graft can be used to treat coronary artery stenosis or occlusion. We describe a 6-year old boy who had an episode of Kawasaki disease with giant coronary artery aneurysm diagnosed at the age of 3 years. Surveillance echocardiogram showed giant coronary aneurysm with stenosis and large mural thrombus in the proximal portion of left main coronary artery. So we inserted two polytetrafluoroethylene(PTFE) covered graft stent without complication.
Aneurysm ; Angioplasty, Balloon ; Child ; Constriction, Pathologic* ; Coronary Aneurysm* ; Coronary Artery Bypass ; Coronary Disease ; Coronary Stenosis ; Coronary Vessels ; Dilatation, Pathologic ; Humans ; Male ; Mucocutaneous Lymph Node Syndrome* ; Myocardial Infarction ; Stents* ; Systemic Vasculitis ; Thrombosis* ; Transplants*

PURPOSE: We reviewed the therapeutic results of various Duct-Occlud coils(pfm AG, Koln, Germany) to evaluate the efficacy of the most-recently modified Duct-Occlud coil(Nit-Occlud) in the transcatheter closure of patent ductus arteriosus(PDA), including large defects more than 4 mm in diameter. METHODS: Two hundred and five patients who underwent percutaneous PDA occlusion using Duct- Occlud devices from March 1996 to December 2003 were enrolled and four types of Duct-Occlud [Standard(S), Reinforced(R), Reinforced reverse cone(RR) and Nit-Occlud(N)] were used in this study. The patients were followed up by echocardiogram and physical examination before discharge, one month, six months and 12 months after the procedure. RESULTS: The rate of residual shunt according to the type of Duct-Occlud were as follows: S-54%, R-72%, RR-50%, N-14%(P<0.05 compared with other devices) at one month, S-25%, R-44%, RR- 37%, N-0%(P<0.05 compared with other devices) at six months, S-8%, R-8%, RR-4%, N-0%(P< 0.05 compared with S and R) at 12 months and later. Nit-Occlud coil showed the complete occlusion of PDA after six months of follow-up, even in 12 patients with relatively large PDA(>4 mm). CONCLUSION: The transcatheter closure of PDA using Duct-Occlud was an effective treatment and our study revealed that a Nit-Occlud coil which showed higher rate of occlusion even in PDA with large diameters over than 4 mm, was a more effective modality compared to previous devices.
Ductus Arteriosus, Patent* ; Follow-Up Studies ; Humans ; Physical Examination

PURPOSE: Accurate measurement of defect size is important in transcatheter closure of atrial septal defect (ASD). We performed this study to analyze the difference between the measured ASD size and balloon occlusive diameter (BOD) by transthoracic (TTE) or transesophageal echocardiography (TEE). METHODS: We investigated 78 patients who underwent transcatheter closure of ASD. The defect size and the distance between the surrounding structures were measured by TTE and TEE. The BOD was measured by TEE during cardiac catheterization. Clinical characteristics and echocardiographic data were compared and analyzed. RESULTS: The difference between BOD and diameter by TTE was 4.8+/-3.6 mm on short axis view, 5.4+/-3.2 mm on long axis view. The difference between BOD and diameter by TEE was 3.6+/-2.2 mm on short axis view, 4.2+/-3.1 mm on long axis view. The difference between BOD and the diameter of defects on TTE, TEE had statistically significant positive correlations with the age of the patients, distance between the, defect and posterior atrial septal wall, the distance between the defect and the mitral valve leaflet, and the diameter of defects and the length of the atrial septum on TTE (P<0.05). CONCLUSION: BOD of ASD can be estimated by the diameter on TTE and TEE. BOD is expected to measure larger, depending on the size of defects, the distance from surrounding structures and the location of defects on echocardiography. Our data offers important information on details of transcatheter ASD closure which can be helpful in predicting suitability and judging the procedural appropriateness during the procedure.
Atrial Septum ; Axis, Cervical Vertebra ; Cardiac Catheterization ; Cardiac Catheters ; Echocardiography ; Echocardiography, Transesophageal* ; Heart Septal Defects, Atrial* ; Humans ; Mitral Valve

PURPOSE: We will present our mid-term result of transcatheter closure of PDA with Duct-Occlud device(pfm. AG. Germany) after 12 months follow up and report the problems during the procedure. METHODS: In total 154 patients, the Duct-Occlud devices were inserted in our institute from March, 1996 to August, 2002. Three types of Duct-Occlud device, i.e standard, reinforced, reinforced reverse cone coil were used. Echocardiographic examination was performed at 1, 6, 12 months after procedure. RESULTS: The echocardiographic closure rate was 96% after 12 months. The rates of residual shunt in the standard coil, the reverse cone coil, and the reinforced reverse cone group were 8%, 4% and 3% respectively. In PDA with diameter less than 4 mm, the closure rate was up to 98% while in large PDA with more than 4 mm, it was 72% after 12 months. Embolization of the inserted coils had occurred in 5 cases with successful retrieval using snare catheter. The rupture of the core wire during the procedure and distortion of the original coil shape had occurred in 4 cases. CONCLUSION: The transcatheter occlusion with Duct Occlud is safe and effective method for small to moderate sized PDA less than 4 mm. The minimum diameter of the PDA seems to be the predictor of residual shunt. Further refinement of the device to overcome the procedure-related problems seems to be needed.
Catheters ; Ductus Arteriosus, Patent* ; Echocardiography ; Follow-Up Studies ; Humans ; Rupture ; SNARE Proteins