We reviewed the clinical and hemodynamic studies in 10 patients with the isolated congenital mitral insufficiency. The patients ranged in age from 6 months to 16 years at the time of diagnosis and the incidence was 0.26% among the total congenital heart disease proven by cardiac catheterization and angiography. In two cases anterior mitral cleft was demonstrated by 2-D echocardiography and/or operation. In two cases left coronary artery was originated from pulmonary artery. Eight cases out of ten presented symptoms indicating varying degrees of left ventricular failure. Apical systolic murmur was heard in all except one, who had only triple rhythm. The electrocardiograms revealed left ventricular hypertrophy in 8 cases and biventricular hypertrophy in 2 cases. Roentgenographically left atrial enlargement was observed in 4 cases. Left arterial and ventricular dimension were increased in 7 cases by echocardiography. In 5 cases of them, pulmonary artery wedge pressure or left atrial pressure was increased. One patient died in congestive heart failure at the age of 10 months and one patient died postoperatively. Other 4 patients have been managed with medication without difficulty.
Angiography ; Atrial Pressure ; Cardiac Catheterization ; Cardiac Catheters ; Coronary Vessels ; Diagnosis ; Echocardiography ; Electrocardiography ; Heart Defects, Congenital ; Heart Failure ; Hemodynamics ; Humans ; Hypertrophy ; Hypertrophy, Left Ventricular ; Incidence ; Mitral Valve Insufficiency* ; Pulmonary Artery ; Pulmonary Wedge Pressure ; Systolic Murmurs

BACKGROUND: Diagnosis of aortic arch obstruction can be made with two-dimensional and Doppler echocardiography in most cases.However,not infrequently,clear imaging of the aortic arch can not be obtained,particularly in sick neonates and young infants from a number of reasons and heart catheterization and angiography carries significant risk in sick babies.Therefore it is the purpose of this study to assess the feasibility and safety of counter-current aortography through a peripheral artery in young infants and neotates with suspected aortic arch obstruction. METHOD: We studied 56 patients with suspected aortic arch anomaly at Asian Medical Center from Feburary 1990 to April 1997.First choice for the peripheral artery was radial artery on the same side as the aortic arch,followed by brachial artery and axillary artery.Small 24 gauge plastic cannula was inserted and special attention was given to ensure that the peripheral artery,plastic cannula,and a syringe containing contrast material are all in the same plane.1ml/kg of contrast material was injected by rapid hand injection and biplane cineangiograms were taken at 60 frame/second. RESULTS: Fifty six patients underwent 58 angiograms.Age ranged from 5-255 days(median 30 days) and body weight nanged from 2.1-5.4kg(mean3.4kg).There were 27 males and 29 females.Arteries used were:Radial artery in 37,brachal artery in 19,and axillary artery in 2 cases.Peripheral arteries were ipsilateral side as the aortic arch in 54,contralateral side in 2 and bilateral in 2 cases.In 8 patients heart catheterization was done because of inadequate visualization of aortic arch anatomy and/or need for evaluating other defects.In 48 patients who had periperal angiography only,fluoroscopic time ranged from 0.6 to 3.5 minutes and total procedure time ranged from 10 to 15 minutes.Among these 48 patients,only 11 patients(23%) were given intraveous sedation and 37 patients(77%) did not recieve any sedation.Diagnosis of aortic arch anomaly was aortic coarctation in 38,aortic interruption in 10 and nomal aortic arch in 8 patients.Aortic arch anatomy was well demonstrated in all cases where injected artery was on the same side as the aortic arch.In patients who had angiograms through peripheral arteries contralateral to the side of the aortic arch did not haveadequate visualization of the arch.Compression of the carotid artery did not enhance the imaging of the arch.Simultanous bilateral angiography did not improve the imaging quality as compared to ipsilateral artery angiography.Transient complication,related to cannulation,e.g.,prolonged bleeding was seen in only one patient with aortic interruption.Circulation on the upper extremities was normal after angiography in all patients. CONCLUSION: Counter-current aortography using 24 gauge plastic cannular through peripheral artery is feasible,rapid,safe,economic and relatevely non-invasive procedure and provides adquate imaging of aortic arch obstruction in infants and neonates without risk of heart catheterization and angiography.We,therefore,recommend this procedure in selected patients in whom echocardiographic imaging alone is not conclusive for planning corrective sursery.
Angiography ; Aorta, Thoracic* ; Aortic Coarctation ; Aortography* ; Arteries* ; Asian Continental Ancestry Group ; Axillary Artery ; Body Weight ; Brachial Artery ; Cardiac Catheterization ; Cardiac Catheters ; Carotid Arteries ; Catheters ; Diagnosis ; Echocardiography ; Echocardiography, Doppler ; Hand ; Heart Defects, Congenital ; Hemorrhage ; Humans ; Infant* ; Infant, Newborn* ; Male ; Plastics ; Radial Artery ; Syringes ; Upper Extremity

BACKGROUND: Idiopathic left ventricular tachycardia with a QRS pattern of right bundle branch block and left axis deviation that is sensitive to verapamil is electrophysiologically distinct arrhythmia entity but descriptions in pediatric ages are infrequent. This study attempted to describe the clinical presentation and dignostic clues from the surface ECG in children with idiopathic left venteicular tachycardia. METHODS: We retrospectively reviewed the medical records, electrocardiograms, Holter recordings, echocardiographic reports of 10 children with idiopathic left ventricular tachycardia who have been managed at Sejong General Hospital and Asan Medical Center, Seoul, Korea from January 1991 to July 1996. Follow-up periods are 2 months to 5 years(mean=26 months). RESULTS: 1) Age of tachycardia onset was 8 months to 14 years(median=4.3 years). In 3 children tachycardia began from infancy. The main complaint in older children was palpitation and chest discomfort but young childen complained abdominal pain during tachycardia attack. Tachycardia was paroxysmal and sustained. Tachycardia responded to intravenous use of verapamil in 9 patients and was controlled by chronic verapamil medication in 6 patients. 2 patients were well without medication. Radiofrequency catheter ablation was done in an adolescent patient successfully. 2) In sinus rhythm, surface ECG showed normal QRS axis and normal QRS morphologies in all patients. During ventricular tachycardia, the heart rate was 183 to 270 beats/min. The taxhycardia of RBBB morphology was observed in all patients. The mean QRS duration was 109+-12ms and the QRS axis was -50* to -90*. Themean RS interval was 60+-12ms. AVdissociation was found on surface RCG in all patients and confirmed in 4 patients by transesophageal ECG. We tried rapid transesophageal atrial pacing in 3 patients and could induce and terminate the ventricular tachycardia. CONCLUSIONS: Idiopathic left ventricular tachycaedia is rare in childhood and is seen from infancy. Idiopathic left ventricular tachycardia is generally benign but occasionally causesmyocardial dysfunction. Idiopathic ventricular tachycardia with a QRS pattern of right bundle branch block amd left axis deviation that is sensitive to verapamil in children should not be confused with supraventricular tachycardia with aberrancy and most helpful diagnostic clue is AV dissociation in surface ECG.
Abdominal Pain ; Adolescent ; Arrhythmias, Cardiac ; Axis, Cervical Vertebra ; Bundle-Branch Block ; Catheter Ablation ; Child ; Chungcheongnam-do ; Echocardiography ; Electrocardiography ; Follow-Up Studies ; Heart Rate ; Hospitals, General ; Humans ; Korea ; Medical Records ; Retrospective Studies ; Seoul ; Tachycardia ; Tachycardia, Supraventricular ; Tachycardia, Ventricular* ; Thorax ; Verapamil

BACKGROUND: Chaotic atrial rhythm (CAR) is characterized by the presence of three or more P-wave morphologic features on the surface electrogram, absence of a dominant atrial pacemaker, and variable P-P, R-R, and P-R intervals with an atrial rate of over 100 beats/min. CAR is infrequently seen in pediatric ages and its clinical course, management and underlying mechanism are uncertain. We report our recent experience with 11 infants with CAR and describe their clinical characteristics and reponse to treatment. METHODS: We retrospectively reveiwed the medical records, electrocardiograms, Holter recordings, echocrdiographic reports of 11 cases of CAR managed at Sejong general hospital and Asan medical center from January 1991 to June 1995. RESULTS: 1) All patients were < or =6 months old and 5 of 11 patients had symptoms at neonatal period. The duration of follow-up was 3-42 months(mean : 18 months). 2 patients had structural heart disease and 3 patients showed signs of ventricular dysfunction. In 10 of 11 patients tachycardia was sustained or recurrent. 1 patient died of severe congestive heart failure due to incessant rapid tachycardia. 2) 3 of 10 patients took digoxin only and others took more than 2 medications. Full control within 1 month after medication was in 2 patients, with digoxin only in one and digoxin and amiodarone in another patient. At discharge, state of arrhythmia control in 8 patients with medications were full control in 2, good control in 3, and partial control in 3. At last follow-up, full control in 5, good control in 1 were confirmed through Holter recordings and the other 4 patients showed sinus rhythm in surface electrocardiograms. The total duration of medications were < or =1 year except 1 patient. 3) In 3 patients with ventricular dysfunction, ventricular function was normalized after restoration of sinus rhythm. CONCLUSION: CAR in children usually occurs in the first month of life and genenally takes benign course, but sometimes it causes severe congestive heart failure or ventricular dysfunction. Frequently, the patients remain asymptomatic despite persistence of the tachycardia for weeks or months. CAR is difficult to convert to sinus rhythm with medications but tends to resolve spontaneously within 1 year. We think treatment is necessary only in the symptomatic patients with rapid ventricular response and it is enough to control the ventricular rate with antiarrhythmic agents.
Amiodarone ; Arrhythmias, Cardiac ; Child ; Chungcheongnam-do ; Digoxin ; Electrocardiography ; Follow-Up Studies ; Heart Diseases ; Heart Failure ; Hospitals, General ; Humans ; Infant ; Medical Records ; Retrospective Studies ; Tachycardia ; Ventricular Dysfunction ; Ventricular Function

PURPOSE: Dilated cardiomyopathy in children is usually progressive and leads to death in several years. Chronic tachyarrhythmia has been shown to cause dilated cardiomyopathy in human and animals and this ventricular dysfuntion is usually reversible after control of the arrhythmia. The uncontrolled chronic tachycardia may be a curable cause of dilated cardiomyopathy in some patients. We describe six children who had persistent chronic tachycardia and ventricular dysfunction that improve significantly after control of the arrhythmia. METHODS: We retrosepctively reveiwed the medical records, electrocardiograms, Holter recordings, Echcardiographic reports of 6 children with the tachycardia induced ventricular dysfunction who have been managed at Sejong general hospital and Asan medical center from January 1992 to June 1995. RESULTS: 1) The causes of referral were dilated cardiomyopathy in 2 and tachyarrhythmia in 4. The age at diagnosis was 4 to 36 months old and follow-up period was 6 to 29 months. The symptoms of congestive heart failure were seen in 3 children. 2) The mechanisms of thachyarrhythmia causing cardiomyopathy were atrial ectopic tachycardia in 2, chaotic atrial rhythm in 3 and junctional ectopic tachycardia in 1. Atrial ectopic tachycardia has improved after combined treatment with amidarone and atenonl. One case of chaotic atrial rhythm has improved spontaneously and other 2 cases of chaotic arial rthythm have improved after treatment with digoxin or after combined therapy with amidarone and digoxin. Junctional ectopic tachycardia is partially controlled with mexiletine. 3) The shortening fractions of left ventricle at diagnosis were 14-21% and improved to over 30% in all after 2 to 9 months of follow-up. CONCLUSIONS: We have shown that control of persistent tachycardia resolved the left ventricular dysfunction. We suggest that patients with dilated cardiomyopathy be carefully screened for tachyarrhythmia as a curable cause of ventricular dysfunction and that left ventricular function be assessed in the asymptomatic patients with persistent tachycardia.
Animals ; Arrhythmias, Cardiac ; Cardiomyopathies ; Cardiomyopathy, Dilated ; Child ; Child, Preschool ; Chungcheongnam-do ; Diagnosis ; Digoxin ; Electrocardiography ; Follow-Up Studies ; Heart Failure ; Heart Ventricles ; Hospitals, General ; Humans ; Medical Records ; Mexiletine ; Referral and Consultation ; Tachycardia* ; Tachycardia, Ectopic Atrial ; Tachycardia, Ectopic Junctional ; Ventricular Dysfunction ; Ventricular Dysfunction, Left ; Ventricular Function, Left

PURPOSE: Dilated cardiomyopathy in children is usually progressive and leads to death in several years. Chronic tachyarrhythmia has been shown to cause dilated cardiomyopathy in human and animals and this ventricular dysfuntion is usually reversible after control of the arrhythmia. The uncontrolled chronic tachycardia may be a curable cause of dilated cardiomyopathy in some patients. We describe six children who had persistent chronic tachycardia and ventricular dysfunction that improve significantly after control of the arrhythmia. METHODS: We retrosepctively reveiwed the medical records, electrocardiograms, Holter recordings, Echcardiographic reports of 6 children with the tachycardia induced ventricular dysfunction who have been managed at Sejong general hospital and Asan medical center from January 1992 to June 1995. RESULTS: 1) The causes of referral were dilated cardiomyopathy in 2 and tachyarrhythmia in 4. The age at diagnosis was 4 to 36 months old and follow-up period was 6 to 29 months. The symptoms of congestive heart failure were seen in 3 children. 2) The mechanisms of thachyarrhythmia causing cardiomyopathy were atrial ectopic tachycardia in 2, chaotic atrial rhythm in 3 and junctional ectopic tachycardia in 1. Atrial ectopic tachycardia has improved after combined treatment with amidarone and atenonl. One case of chaotic atrial rhythm has improved spontaneously and other 2 cases of chaotic arial rthythm have improved after treatment with digoxin or after combined therapy with amidarone and digoxin. Junctional ectopic tachycardia is partially controlled with mexiletine. 3) The shortening fractions of left ventricle at diagnosis were 14-21% and improved to over 30% in all after 2 to 9 months of follow-up. CONCLUSIONS: We have shown that control of persistent tachycardia resolved the left ventricular dysfunction. We suggest that patients with dilated cardiomyopathy be carefully screened for tachyarrhythmia as a curable cause of ventricular dysfunction and that left ventricular function be assessed in the asymptomatic patients with persistent tachycardia.
Animals ; Arrhythmias, Cardiac ; Cardiomyopathies ; Cardiomyopathy, Dilated ; Child ; Child, Preschool ; Chungcheongnam-do ; Diagnosis ; Digoxin ; Electrocardiography ; Follow-Up Studies ; Heart Failure ; Heart Ventricles ; Hospitals, General ; Humans ; Medical Records ; Mexiletine ; Referral and Consultation ; Tachycardia* ; Tachycardia, Ectopic Atrial ; Tachycardia, Ectopic Junctional ; Ventricular Dysfunction ; Ventricular Dysfunction, Left ; Ventricular Function, Left

PURPOSE: Although the data of radiofrequency catheter ablation (RFCA) of supraventricular tachycardia in adults has been accumulated in several centers in Korea, few data are available on its efficacy and safety in pediatric ages. We reviewed the data in young patients who underwent this procedure in a pediatric cardiology center, to evaluate the indications, early results, complications, and short-term follow-up data. METHODS: We retrospectively reviewed the medical records and RFCA procedure reports of 17 children who underwent this procedure in pediatric cardiology center, Asan Medical Center, Seoul, Korea from January 1992 to July 1996. The mean follow-up periods was 17.8 months. RESULTS: 1) The median age of the patients was 12.3 years and all of them except two patients had structurally normal hearts. The indications of RFCA were preoperative open heart surgery in a patient, drug refractory tachycardia in 4 patients and patient's choice in other 12 patients. 2) The mechanisms of supraventricular tachycardia were 9 atrioventricular reentrant tachycardia, 6 atrioventricular nodal reentrant tachycardia (AVNRT), 1 atrial flutter and 1 atrial ectopic tachycardia (AET). Among accessory pathways, preexcitation was in 6 patients and accessory pathway located in the right side in 7 patients. In all patient with AVNRT except one, slow pathway was ablated. In the case of AET, ectopic focus located in the left atrium near the orifice of right lower pulmonary vein. 3) 16 of 18 procedures (88.9%) were successful and in 2 occasions with right sided accessory pathway and AET, the procedure failed. Ectopic focus was successfully ablated at the second attempt. The mean total procedure time was 2.7 hours (n=16) and the mean fluoroscopic time was 45.3 minutes (n=12). The average number of RF application was 11 times. There was no significant complications related with procedures. 4) There were 5 recurrences (29.4%), in 2 patients with accessory pathway and 2 patients with AVNRT. Most recurrences occured in 6 weeks after procedure. CONCLUSIONS: RFCA is a good alternative for the treatment of supraventricular tachycardia also in young patients as in adults. Early results of RFCA shows resonably good success rate but somewhat higher rate of recurrence in our center. We expect the results of RFCA in children will be improved in near future after some period of learning curve.
Adult ; Atrial Flutter ; beta-Aminoethyl Isothiourea ; Cardiology ; Catheter Ablation* ; Child* ; Chungcheongnam-do ; Follow-Up Studies ; Heart ; Heart Atria ; Humans ; Korea ; Learning Curve ; Medical Records ; Pulmonary Veins ; Recurrence ; Retrospective Studies ; Seoul ; Tachycardia ; Tachycardia, Atrioventricular Nodal Reentry ; Tachycardia, Ectopic Atrial ; Tachycardia, Supraventricular* ; Thoracic Surgery

Atrial flutter is a potentially life threatening complication of repair or palliation of congenital heart disease and is frequently drug resistant. Recent studies in human have demonstrated that common type atrial flutter is due to macro-reentry in the right atrium and that the reentrant circuit may involve slow conduction in the low posteroseptal area of the right atrium. Catheter ablation of atrial flutter has been successful using radiofrequency energy. Application of radiofrequency energy at the isthmus between the inferior vena cava and tricuspid ring was effective in preventing recurrence of atrial flutter in a 6 year old boy after open heart surgery of common atrium. Despite concerns regarding ablation scar in growing children and persistence of underlying structural abnormalities in postoperative patients, radiofrequency ablation of atrial flutter appears to be another useful therapeutic option in management of this not so rare but potentially malignant and often resistant arrhythmia.
Arrhythmias, Cardiac ; Atrial Flutter* ; Catheter Ablation* ; Catheters* ; Child* ; Cicatrix ; Heart Atria ; Heart Defects, Congenital ; Humans ; Male ; Recurrence ; Thoracic Surgery ; Vena Cava, Inferior

PURPOSE: Histologic studies have revealed ectopic location and hypoplasia of sinus nodal tissue in left atrial isomerism. The purpose of this study is to characterize the atrial rhythms corresponding to these histologic abnormalities in left atrial isomerism. Electrocardiograms in left atrial isomerism are characterized by multiple atrial rhythms with abnormal P-wave axes, but the complex nature of atrial rhythms in left atrial isomerism remains to be clarified. METHODS: Surface electrocardiograms and Holter recordings of 16 patients with left atrial isomerism were retrospectively reviewed. For comparison, atrial rhythms in 8 patients with right atrial isomerism were studied with the same study protocol. RESULTS: In left atrial isomerism, atrial rhythm with abnormal superior P-wave axis was the most frequent in 69% of patients and multiple atrial rhythms were observed in 37% in surface electrocardiograms. In Holter recordings, transient or persistent slow atrial rhythms with or without visible P waves associated with junctional escape were documented in 9 out of 16(56%) patients. In comparison, multiple atrial rhythms were as frequent as in left atrial isomerism, but slow atrial rhythm with junctional escape was not present in 8 patients with right atrial isomerism. CONCLUSION: It is concluded that transient or persistent slow atrial rhythms associated with junctional escape, suggestive of atrial nodal dysfunction, are characteristic and helpful in the diagnosis of left atrial isomerism.
Axis, Cervical Vertebra ; Diagnosis ; Electrocardiography ; Heterotaxy Syndrome* ; Humans ; Retrospective Studies ; United Nations

The objective of this study was to determine how the neonatal mortality rate has changed since surfactant (S) therapy was introduced in our Neonatal Intensive Care Unit (NICU), and to evaluate the efficacy of surfactant therapy in respiratory distress syndrome (RDS) patients. Incidences of risk babies such as outborns, prematurity, low birth weight infants and RDS, and neonatal mortality rates were compared between 'pre' (control, 1988 to 1991, n=4,861) and 'post' S period (study, 1993 to 1996, n=5,430). In RDS patients of 'post' S period, neonatal mortality rate was compared between S-treated and non-treated patients, and chest X-ray and ventilatory parameters were compared between pre- and post-72 hr of surfactant treatment. Surfactant therapy showed short term effects, judging by the decrease of early neonatal deaths and improvement of chest X-ray and ventilatory parameters in RDS patients. The overall neonatal mortality rate had a tendency to decrease in spite of increased incidences of risk babies in 'post' S period but it was less than expected. The reasons were thought to be that we had a high proportion of risk babies, and there was some bias in patient selection for surfactant therapy and its use. In conclusion, with the active prevention of risk baby delivery and appropriate use of surfactant, better results could be expected.
Female ; Human ; Incidence ; Infant Mortality ; Infant, Newborn ; Male ; Pulmonary Surfactants/therapeutic use* ; Respiratory Distress Syndrome/mortality* ; Respiratory Distress Syndrome/epidemiology ; Respiratory Distress Syndrome/drug therapy* ; Risk Factors