No abstract available.
Atrial Fibrillation* ; Mitral Valve*

Meningoencephalocele in the anterior part of the cranium is a rare congenital anomaly and has been sparsely reported in the neurologic literature. Recently we encountered a case of bilateral frontoethmoidal meningoencephalocele in a 3-year-old otherwise healthy boy. The cranial ends of the tumors were amputated by bifrontal craniotomy and dural defect was tightly repaired with Neodura. The external portions of the tumors were excised at the second stage and double-limbed YV shaped canthoplasty was performed. Postoperatively, the patient recovered uneventfully without any complication.
Child, Preschool ; Craniotomy ; Humans ; Male ; Skull

The congenital diverticulum of ventricle is a rare cardiac malformation arising from the left or right ventricle, the former being more common. Ventricular diverticulum is usually associated with other anomalies including intracardiac, midline thoracic, diaphragmatic and abdominal wall defect. The authors experienced a case of congenital diverticulum of left ventricle in nine month-old female infant. Left Blolock-Taussig shunt operation was done and total correction will be done later. Thereafter we presented a case with brief review of the related literatures.
Abdominal Wall ; Diverticulum* ; Female ; Heart Ventricles ; Humans ; Infant ; Pulmonary Atresia*

No abstract available.
Myasthenia Gravis* ; Thymectomy*

The 8 cases of left ventricular thrombus detected by the 2 D echocardiography or left ventriculography, after acute transmural anterior myocardial infarction were effectively lysed by the thrombolytic agents and heparin therapy. The thrombolytic agents were either urokinase or tissue plasminogen activator. Urokinase was infused intravenously at a dose of 1.0 million unit for three days. And tissue plasminogen activator was infused at a dose of 100mg for a day. In all cases, the thrombi were completely lysed. At follow up, no recurrence of left ventricular thrombus was found. We have experienced 2 cases of peripheral embolization in which, left ventricular thrombi were protruding nonmobile type. The one was the embolic cerebral infarction, the other was transient hoarseness and paresthesia on the left foot, which may be transient ischemic attack. These results show that left ventricular thrombi can be treated by intravenous thrombolytic agents without life-threatening complication. However, for the better establishment of the risk and benefit of therapy further investigation is needed.
Cerebral Infarction ; Echocardiography ; Fibrinolytic Agents ; Follow-Up Studies ; Foot ; Heparin ; Hoarseness ; Ischemic Attack, Transient ; Myocardial Infarction* ; Paresthesia ; Recurrence ; Thrombolytic Therapy* ; Thrombosis* ; Tissue Plasminogen Activator ; Urokinase-Type Plasminogen Activator

Bilateral coronary artery fistula is very uncommon congenital anomaly which occupy small percentage of all congenital coronary artery fistula. About 20% of the cases associated with additional congenital heart disease. And it may cause congestive heart failure, especially severely in neonate. We experienced a case of neonate who was 1 day-old-male with bilateral coronary artery-right ventricular fistula associated with atrial septal defect and congestive heart failure. The Patient was admitted because dyspnea, cyanosis since birth. 2D ecgicardiogram and cardiac catheterization revealed that a fistulous communicating ; forming a large aneurysm, was noted from bilateral coronary artery emptied into the right ventricle, and there was oxygen step-up in right ventricle.
Aneurysm ; Cardiac Catheterization ; Cardiac Catheters ; Coronary Vessels ; Cyanosis ; Dyspnea ; Fistula* ; Heart Defects, Congenital ; Heart Failure ; Heart Septal Defects, Atrial* ; Heart Ventricles ; Humans ; Infant, Newborn* ; Oxygen ; Parturition

Double outlet right ventricle, a complex of congenital cardiac anomalies in which both great arteries arise wholly or in large part from the morphologic right ventricle presents diverse clinical manifestations according to the morphological characteristics. Eighty cases with DORV were diagnosed and operated at Yonsei Cardiovascular Center from 1988 to 1992. The analysis of the morphological characteristics of this anomaly and comparative study of operative methods and mortality according to the morphological classification led to the following results: 1) The location of VSD varied to be subaortic in 40 cases, subpulmonic in 15, doubly committed in 6 and noncommitted in 19 cases. The great arteries were interrelated in D-malposition in 43 cases and L-malposition in 18 and 26 cases among above mentioned 61 cases revealed the side-by-side relationship. Ten of the cases showed normal position and the rest 9, A-malposition. 2) Pulmonary stenosis was found in 60 cases, and when associated with subpulmonic VSD, occurring less frequently. PDA and ASD secundum were associated in successional order and only 3 cases were verified to carry aortic arch anomalies such as coarctation and interruption, all with subpulmonic VSD. 3) Intraventricular tunnel repair was possible in every case associated with subaortic VSD and the postoperative prognosis was excellent. In comparison, most of the cases associated with other types of VSD in which only palliative surgery or various types of intraventricular repair were performed, the general outcome was poor. But total cavopulmonary connection, one of the physiological corrective methods, showing a better postoperative prognosis is being carefully considered for its broad adoptation in future. In conclusion, double outlet right ventricle, a pathophysiological complex of various anomalies, should be thoroughly evaluated for the morphological characteristics to decide the most appropriate types of operation and for consequent improvement of prognosis.
Aorta, Thoracic ; Arteries ; Classification ; Double Outlet Right Ventricle* ; Heart Ventricles ; Mortality ; Palliative Care ; Prognosis ; Pulmonary Valve Stenosis

BACKGROUND: It is well known that ischemic preconditioning protects the heart against infarction or arrhythmias from a subsequent ischemic injury. Recent laboratory data indicate that the adenosine during the ischemic period may trigger protection via A1 or A3 adenosine receptor and also protein kinase C(PKC) plays a central role. This study was designed to determine the role of adenosine receptor subtypes and PKC in the preconditioning protection. METHODS: All cat heart groups were subjected to 40min ischemia and 30min reperfusion. The preconditioning protocol consists of 4min ischemia and then 10min of reperfusion 4 times. The effects of ischemic preconditioning, nonselective adenosine receptor blocker(SPT), an A1 specific antagonist(DPCPX) and protein kinase C inhibitor(Polymyxin B), on ischemic preconditioning were determined by infarction size. There were 5 groups : (1) control group (Group 1, n=10)(2) Ischemic preconditioned group(Group 2, n=9)(3) DPCPX pretreatment group(Group 3, n=6)(4) SPT preteatment group(Group 3, n=6)(5) Polymyxin B pretreatment group(Group 5, n=6). SPT and DPCPX were given intravenously 5 min before ischemic preconditioning. Polymyxin B was administered to cats for 30min during ischemic preconditioning period. RESULTS: Ischemic preconditioning only or pretreatment with DPCPX prior to preconditioning demonstrated a significant reduction in infarct size(22.6+/-1.5, 25.4+/-0.9% infarction of the risk zone, respectively, p<0.05) with respect to control, SPT-pretreatment, and polymyxin B-pretreatment groups(44.0+/-1.7, 43.0+/-2.0 and 40.3+/-0.4% infarction of the risk zone, respectively). CONCLUSIONS: Ischemic preconditioning protects heart from subsequent ischemia. Protection was blocked by SPT and protein kinase C inhibitor(polymyxin B), but not by A1 antagonist DPCPX. The cardioprotective effects by ischemic preconditioning in the in vivo cat heart appear to be dependent on A3 adenosine receptors and activation of protein kinase C.
Adenosine* ; Animals ; Arrhythmias, Cardiac ; Cats* ; Heart* ; Infarction ; Ischemia ; Ischemic Preconditioning* ; Polymyxin B ; Polymyxins ; Protein Kinase C* ; Protein Kinases* ; Receptors, Purinergic P1* ; Reperfusion

BACKGROUND: The prostaglandin E1(PGE1) is a well known protent dilator of arteriosus. Maintaining of the patency of ductus arteriosus is crucial for the survival of patients suffering from ductus-dependent cyanotic congenital heart disease. We aimed to analyse the efficacy and the influencing factors upon PGE1 in patients suffering from this disease. METHODS: Between May 1991 and April 1993, 26 neonates and infants with ductus- dependent cyanotic congenital heart disease received on intravenous infusion of PGE1 in the Division of Pediatric Cardiology. Yonsei Cardiovascular Center. The result was a dramatic improvement in systemic arterial oxygen tension and oxygen saturation during infusion of PGE1with a dependency on the infusion of PGE1. We evaluated the arterial blood gas analysis both at the immediate pre-infusion stage and 2 hours after infusion. We aimed to analyse the factors which may influence the intravenous of PGE1to infant suffers of ducts-dependent cyanotic congenital heart disease, such as pulmonary atresia(n=14), severe pulmonary stenosis(n=7) or complete transposition of the great arteries(n=5). RESULTS: 1) There was a significant increase in PaO2 and Oxygen saturation 2 hours after the infusion of PGE1. This appeared to be unrelated to the different forms of the disease when compared with the pre-infusion values. 2) The infants' responsiveness of the ductus arteriosus appeared to be age related with significant differences emerging between the 2 group(p<.05). In infants younger than 9 hours old, the differences in PaO2 changes between pre-infusion and post-infusion of PGE1 were 16.3+/-3.7mmHg compared to just 10.4+/-0.4mmHg in infants older than 96 hours. 3) No significant difference emerged between an increase in PaO2or oxygen saturation relating to the shape of ductus arteriosus ; or the level of PaO2prior to the infusion. 4) The side effects of PGE1were as follows ; fever(84.6%),loose stool(61.5%), apnea(30.8%) and hypotension(15.4%), etc.. CONCLUSION: PGE1provides excellent medical palliation for infants suffering from ductus-dependent cyanotic congenital heart disease until the pulmonary arteries are large enough for a modified Blalock-Taussig shunt ; or until corrective surgery is possible.
Alprostadil ; Blalock-Taussig Procedure ; Blood Gas Analysis ; Cardiology ; Ductus Arteriosus ; Heart Defects, Congenital* ; Humans ; Infant* ; Infant, Newborn ; Infusions, Intravenous ; Oxygen ; Pulmonary Artery

Aneurysms of the coronary arteries are rare. They may be due to atheroselerosis, mucocutaneous lymph node syndrome, mycotic emboli, syphilis or trauma and occasionally they are congenital. The prognosis appears to be poor and death can occur suddenly from rupture of the aneurysm, peripheral coronary embolism or bacterial endocarditis. Recently surgical treatment has been successful. In a 5 year old patient with ventricular septal defect, two aneurysmal sacs located at the cardiac crux and just posterior to it was proved by coronary angiography and MRI, and successfully excised at operation.
Aneurysm* ; Child, Preschool ; Coronary Angiography ; Coronary Vessels* ; Embolism ; Endocarditis, Bacterial ; Heart Septal Defects, Ventricular ; Humans ; Magnetic Resonance Imaging ; Mucocutaneous Lymph Node Syndrome ; Prognosis ; Rupture ; Syphilis