Current Characteristics of Infective Endocarditis with Congenital Heart Disease: A Retrospective Survey of 121 Cases between 1985 and 2006

BACKGROUND AND OBJECTIVES: The relative proportion of infective endocarditis (IE) with congenital heart disease (CHD) has increased because of improved survival with CHD. This may affect the current profile of IE with CHD. The aim of this study is to assess the changing profiles of IE with CHD. SUBJECTS AND METHODS: All cases diagnosed from January 1985 to May 2006 were retrospectively reviewed and were analyzed according to the diagnosed period (period I-prior to 1995, period II-after 1996). Duke criteria were used for the definition of diagnosis. RESULTS: As a whole, 121 episodes occurred (63 episodes in period I and 58 episodes in period II). Although the mean age was similar for patients diagnosed in the two periods, the proportion of younger age patients was larger during period II. During period II, the number of non-surgical ventricular septal defect (VSD) cases decreased and the number of Rastelli procedure cases increased. Negative blood cultures were similar in patients for both periods. S. viridans was the most common causative organism in patients during both periods. Post-surgical cases and IE after a dental procedure increased during period II. The in-hospital mortality rate, total complication rate, and proportion of IE requiring early surgery were not different for patients in either period. However, the interval to early surgery was shorter for patients during period II (period I 35+/-21.1 days, period II 22+/-16.5 days, p=0.041). CONCLUSION: Our study indicates that IE in CHD is still a major problem with similar clinical features exhibited during the two different periods. The presence of non-surgical VSD as a major underlying defect indicates that one needs to pay more attention to the education of the population at risk.

A Case of Left Isomerism Presented with Pulmonary Hypertension Caused by Congenital Absence of the Portal Vein

Left isomerism is characterized by bilateral left-sidedness and multiple associated cardiac and visceral anomalies. The clinical manifestation of left isomerism mainly depends upon the cardiac lesions. Occasionally an individual will have a normal heart and be presented with the extracardiac anomalies. A 3-year-old girl with a diagnosis of left isomerism was presented with pulmonary hypertension and intermittent hypoglycemia. Computerized tomography of the abdomen revealed absence of the portal vein and portosystemic shunt. The superior mesenteric and splenic veins joined as a common trunk, bypassed the liver and drained the left renal vein and hemiazygos vein. Her pulmonary hypertension was considered as a consequence of the portosystemic shunt. We report a case of left isomerism in association with absence of the portal vein and a review of literatures.

Thirteen-year Experience of Permanent Epicardial Pacing in Children / 대한흉부외과학회지

BACKGROUND: We investigated the longevity, thresholds of epicardial pacemaker and causes of reoperation in the pediatric patients who underwent epicardial pacemaker implantation performed during the last 13 years MATERIAL AND METHOD: 121 operations were performed in 83 patients from January 1989 to July 2002. We analyzed the stimulation threshold, resistance, R-wave and P-wave, and sensitivity of pacemaker lead at initial implantation. Longevity and causes of reoperations were investigated. RESULT: At implantation, epicardial ventricular mean stimulation threshold was 1.2+/-0.1 (0.1~5) mV, mean resistance was 519.1+/-18.1 (319~778) Ohm, and mean R-wave sensitivity was 8.9+/-0.7 (4~20) mV, and mean P wave sensivity was 2.5+/-0.7 (0.4~12) mV. The mean longevity of pacemaker generator was 64.7+/-3.7 (2~196) months. The reoperation free rate was 94.6% for 1 year, 93.6% for 2 years, 80.8% for 5years, 63.7% for 7 years, and 45.5% for 10 years. The causes of reoperation were battery waste in 26 cases and lead malfunction in 9 cases. There was no postoperative death related to pacemaker malfunction. CONCLUSION: In the childrens, average longevity of epicardial pacemaker was within acceptable range. 19.1% of the patients required pacemaker related reoperation. However, recent developments, including steroid eluting lead, 6.7% of the patients required pacemaker related reoperation, look promising in expansion of pacemaker life span.

Long-term Results of Surgical Correction for Partial Atrioventricular Septal Defects: Seventeen-year Experience / 대한흉부외과학회지

BACKGROUND: In this study, we analyzed the long-term surgical outcome of partial atrioventricular septal defects during the past 17 years at Seoul National University Hospital. MATERIAL AND METHOD: A retrospective analysis on mortality, survival, and reoperation and their risk factors was done in 93 patients who underwent surgical correction of partial atrioventricular septal defects between April 1986 and December 2002. 32 patients were male and 61 were female with a median age of 68 months (3~818 months) and a mean follow-up period of 108 months (1~200 months). RESULT: There were 4 operative deaths (4.3%) and one mortality during the follow-up period. 3, 5, 10, and 15 year actuarial survival rates were 95.7%, 94.3%, 94.3%, and 94.3%, respectively. After the surgical correction, left atrioventricular valve incompetence was improved in 61patients (67.7%), remained same as the preoperative status in 14 patients (15.1%), and was aggravated in 12 patients (12.9%). Reoperation was performed in 8 patients (9.0%) after a mean interval of 38.6 months (3~136 months). Freedom from reoperation rates at 3, 5, 10, and 15 years after surgical correction were 94.0%, 91.4%, 91.4%, and 88.2%, respectively. Reasons for reoperation were 7 left atrioventricular valve incompetence, 2 left ventricular outflow tract obstruction, a residual atrial septal defect, a left atrioventricular valve stenosis, and a right ventricular failure. Left ventricular outflow tract obstruction was the only statistically significant factor. In ten patients, significant arrhythmia was developed and three of them were supraventricular arrhythmia. Complete atrioventricular block occurred in 7 patients and permanent pacemakers were implanted in six of them. CONCLUSION: Surgical corrections of partial atrioventricular septal defects were performed with low operative mortality. Sicnce left atrioventricular valve incompetence was the most common cause of reoperation and left ventricular outflow tract obstruction was the only risk factor for reoperation, a precise estimation of the left atrioventricular valve morphology and the structure of left ventricular outflow tract are needed. Although left ventricular outflow tract obstruction rarely developed, reoperation was frequently required and resection of subaortic tissue could be performed but the possibility of recurrence was high, so modified Konno operation could be performed with satisfactory results. Complete atrioventricular block developed frequently in early periods, but was overcome with a precise anatomical understanding of conduction system and experience.

Long-term Clinical Study of Total Anomalous Pulmonary Venous Return

PURPOSE: Total anomalous pulmonary venous return is a rare congenital heart disease, which is fatal if untreated, especially, if the obstruction in pulmonary venous return is obstructed. With the technical development of echocardiogram, we are now able to do an early diagnosis if TAPVR and perform surgery. Accordingly, this report was aimed to evaluate patients with TAPVR as well as evaluate the problematic factors in order to contribute to future treatment. METHODS: From Sept. 1st, 1985 to June 30, 1999, we retrospectively evaluated 107 patients who were diagnosed TAPVR by echocardiogram and catheterization and angiogram. TAPVR in complex heart diseases were excluded. RESULTS: The patients were composed of 64 boys and 43 girls with the mean age of 206 days. The subtype of TAPVR is as follows : supracardiac type 45%, cardiac type 33%, infracardiac type 9% and mixed type was 13%. In 39 patients(36%), pulmonary venous obstruction was detected by echocardiogram and all the patients with infracardiac type TAPVR, except one patient, revealed obstructive pulmonary venous return. Surgery was performed in 96 patients and the mean age at operation was 270 days old. The operative mortality was not different statistically among subtypes(P=0.212) and also was not different between obstructive type and nonobstructive type(P=0.370). The mean age at operation from 1994 to 1999 was younger than that of up to 1994. Pulmonary vein stenoses after operation were detected in 14 patients(14.6%) and only in 3 patients developed, pulmonary vein stenoses at the same site of previous ones. Thus, pulmonary vein stenoses after operation could be mainly considered as secondary due to operation. CONCLUSION: The results from our study reveal that operative results for TAPVR were much improved with early diagnosis and operation and those results were not dependent on the subtypes of TAPVR. Postoperative pulmonary vein stenosis was a poor prognostic factor and for early detection, catheterization and angiogram could be recommended if there is suspicion.

Assessment of Myocardial Ischemia using Myocardial Perfusion Scan in Kawasaki Disease

PURPOSE: Myocardial scintigraphy has been reported to be useful in adults, but its usefulness in children is limited. This study was done to determine the feasibility and accuracy of 99mTc-MIBI SPECT(sestamibi , methoxy-isobutyl-isonitrarite myocardial single emirs sion computed tomography, Dp-SPECT) after dipyridamole infusion to detect coronary obstructive lesions in Kawasaki disease(KD). METHODS: Dp-SPECT was performed in 21 control(group 1), 8 with coronary aneurysm(group 2) and 16 with coronary obstructive lesion(group 3) in children with KD. The spatial distribution of Dp-SPECT was determined and analyzed visually. Group 2 and 3 underwent coronary angiogram within 3 months of Dp-SPECT. RESULTS: Chest pain occurred more frequently in group 2. No subject required supplemental oxygen or were complicated by myocardial ischemia. The specificity of Dp-SPECT for control subject was 95% & 62% if using a cut-off criterion of abnormality, utilizing moderate or mild perfusion defect, respectively. The false positive rate was high in anterior, apical and anteroseptal segments but not related to age. The abnormalities found on Dp-SPECT was observed in 25% of aneurysmal coronary arteries but not related to its size. The sensitivity, specificity, and accuracy of Dp-SPECT was 100%, 84.5% and 79% in coronary stenosis greater than 75%, respectively. CONCLUSION: The interpretation of Dp-SPECT should be careful in KD because of discrepancies between Dp-SPECT & angiography, But DP-SPECT is noninvasive and easily applicable. It monitors the occurrence and progression of coronary stenosis due to KD. Besides Dp-SPECT may be applicable after arterial switch operation or to myocardial sinusoid.

Congenital Heart Disease and Associated Extracardiac Anomalies in Autopsies

PURPOSE: In order to find out how frequently and what kind of extra-cardiac anomalies (CAs) is associated with congenital heart disease (CHD) in general and in each CAs, autopsies with congenital cardiovascular malformations were studied. METHODS: Autopsy files of Seoul National University Children's Hospital from 1991 to 1995 were reviewed to find cases of CHDs. Other extra-CAs were tabulated from the records of autopsy files. The study population was comprised of 156 cases (86 still birth or product of terminated pregnancy; 66 livebirth; 4 unknowns-whether still birth or livebirth). RESULTS: The sex ratio was 1.14:1 males to females. In still birth cases, gestational age ranged from 16 to 42 weeeks and in live birth cases, age ranged from 1 day to 1 year 7 months (mostly neonates). The ventricular septal defect (VSD) was the most common CA, followed by coarctation of aorta, tetralogy of Follot in this order of frequency. No extra-CA was found in 55 cases, 1 extra-CA in 54 cases, 2 extra-CAs in 35 cases, 3 or more extra-CAs in 12 cases. VSD was commonly associated with Edward syndrome and holoprosencephaly, tetralogy of Fallot with holoprosencephaly, and coarctation with cystic hygroma. The most commonly involved organ system was the central nervous system followed by the digestive system, facial anomaly, and so on. Chromosomal anomaly associated with CHDs was 24 cases. Common individual anomalies were cystic hygroma, Edward syndrome, holoprosencephaly, and so on. CONCLUSION: As the fetus and infants with CHD have high incidence of associated extra-CAs, collaborative works involving dysmorphologist, geneticist, pathologist, surgeons, and pediatric cardiologist are needed. For further studies of CA and extra-CAs, a systematic database andprecise medical records and interdepartmental collaboration are necessary.

Influence of Anatomy, Associated Anomalies, Age, and Surgical Methods on the Surgical Results of Aortic Coarctation / 대한흉부외과학회지

One hundred forty-four patients underwent operation for coarctation of the aorta at Seoul National University Children's Hospital between June 1986 and Decembsr 1995. Age ranged 0.1 to 191 months. Of these 78.5%(113) were infants. We classified the patients in terms of the anatomic location of coarctatiln and the associatCd anomalies(I[40]= primary coarctation, II[74]=isthmic hypoplasia, lII[30]=tubular hypoplasia involving transverse arch, A[63]=with ventricular septal defect, B[28]=with other major cardiac defects). Subcalvian flap coarctoplasty(60), resection and anastomosis(44), extended aortoplasty(26), and onlay patch(14) were used as surgical methods. Overall operative mortality was 16.0(23/144)%. The hospital mortality was significantly higher in patheints with type III, subtype B, younger age(under 3 months), extended aortoplasty(p<0.01). However, one-stage total repair in patients with subtype A or B were not found to be a predictor of hospital death. Restenosis had occured in 18 patients among 121 survivals(14.9%). The mean follow-up period was 29.1+/-28.8(0~129.2) months. Preoperative, immediate postoperative(within 3 months after operation) and postoperative(later than 6 months after operation) echocardiographic data on the dimensions of ascending aorta(AA), transverse arch(TA), an4 aortic isthmus(Al) were available in 77 patients(I=20, II=42, III= 15). Preoperative and postoperative aortic isthmus(All) and tra sverse arch indices(TAI), defined as TA/AA and AI/AA respectively, were compared. Immediate postoperative All in type I, II and TAI in type III were significantly smaller in stenotic than non-stenotic group suggesting incomplete relieves of stenotic segment Younger age, subclavian coarctoplasty in patient under 3 months of age were round to be the risk factors for restenosis in this series. In conclusion, We found that aortic arch index and transverse arch index can be a useful tool to figure out the anatomic and clinical characteristics of the patients with aortic coarctation, and that anatomy, associated anomalies, age, and surgical methods may influence the surgical outcome of the coarctation repair.

Radiological evaluation of congenital pulmonary vein obstruction

Congenital obstuction of pulmonary vein without anomalous drainage can cause long-standing pulmonary congestion and pulmonary arterial hypertension, and it may include stenosis of individual pulmonary veinsor total pulmonary vein atresia. We reviewed seven cases of pulmonary vein obstruction, five of which accompanied other cardiac anomalies. Right pulmonary veins were involved in all seven cases including one bilateral case. Pulmonary veins were occluded totally in five and partially in three lungs. Pumonary catheterization and angiography were done for diagnosis. Chest radiographs of total occlusion cases showed decreased lung volume, features of pulmonary edema, interstitial lesions, and pleural thickening, which were quite specific, whereas pulmonry venous dilatation was dominant findings in partial obstruction cases. Pulmonary perfusion scans (n=3) showed total perfusion defects in the cases of total occlusion of veins. MR imaging (n=2) demonstrated total occlusion of pulmonary veins in the venoatrial junction in two, and membranous focal obstruction in one lung. Two patients had pneumonectomy and histological confirmation, Although catheterization and angiography are essential for the diagnosis, MR imaging is thought to be useful for the diagnosis of pulmonary vein obstruction.

An experimental study on balloon catheter pulmonary valvuloplasty: Comparison between single and double balloon catheters

Balloon pulnounary valvuloplasty is an established method in the treatment of congenital valvular pulmonary stenosis. Balloon valvuloplasty was performed by using one or two balloons in a total of 19 mongrel dogs. Cross sectional area ratio ranging 145-406% was used to compare the relative sizes of the pulmonary annulus the balloon Hemodynamic changes and cardiac damages in single and double balloon groups were analyzed. Survival time of double balloon group was longer than that of single balloon group(P0.05). Major microscopic findings were sloughing of the endotheliu, fibrin deposition, hemorrhage, localized myocardial infarction and so on. In conclusion, double balloon pulmonary valvuloplasty is superior to single balloon technique in survival time and hemodynamics. Therefore, double, balloon technique is recommended for the pulmonary valvuloplasty.

An experimental study on balloon catheter pulmonary valvuloplasty: Comparison between single and double balloon catheters

Balloon pulnounary valvuloplasty is an established method in the treatment of congenital valvular pulmonary stenosis. Balloon valvuloplasty was performed by using one or two balloons in a total of 19 mongrel dogs. Cross sectional area ratio ranging 145-406% was used to compare the relative sizes of the pulmonary annulus the balloon Hemodynamic changes and cardiac damages in single and double balloon groups were analyzed. Survival time of double balloon group was longer than that of single balloon group(P0.05). Major microscopic findings were sloughing of the endotheliu, fibrin deposition, hemorrhage, localized myocardial infarction and so on. In conclusion, double balloon pulmonary valvuloplasty is superior to single balloon technique in survival time and hemodynamics. Therefore, double, balloon technique is recommended for the pulmonary valvuloplasty.

The muscular ventricular septal defect and multiple ventricular septal defects in some congenital heart diseases

No abstract available.

Clinical Consideration of Coarctation of the Aorta

No abstract available.

E-Type Prostaglandin Therapy for Neonatal Cyanotic CHD

No abstract available.

Clinical Characteristics of Ventricular Tachycardia on 24 hour ECG in Children

No abstract available.

Clinical Observation on Amiodarone Treatment for Critical Arrhythmia in Children

No abstract available.

Mitral Atresia Associated with Corrected Transposition of Great Arteries

A case of congenital absence of right atriovntricular (A-V) connection of heart is reported. Patient was 81 day old male infant with chief complaints of dyspnea and irritability. Clinical evaluation revealed increased pulmonary vascularity on chest x-ray, absence of right A-V connection, regurgitation of left A-V valve, right-sided ventricular hypoplasia and L-TGA. Clinical assessment of the case was tricupsid atresia with LTGA and mitral regurgitation. Pulmonary artery banlding and artial septectomy was performed. Parenteral alimentation through the femoral veins resulted in obstruction of IVC and subsequent multiorgan failure. The autopsy findings of the heart were corrected transposition of great arteries with associated anomallies of right-sided mitral atresia, Ebstein's malformation of left-sided tricuspid valve, ventricular septal defect, muscular hypertrophy of right-sided left ventricle with luminal obliteration. Long segment of IVC was completely occluded due to occlusive thrombi with fungal infection. Bilateral kidneys were infarcted and azygos vein was markedly engorged. Microscopic examination revealed candidal colonization in the inferior vena cava and septic embolzation in brain.

Mitral Atresia Associated with Corrected Transposition of Great Arteries

A case of congenital absence of right atriovntricular (A-V) connection of heart is reported. Patient was 81 day old male infant with chief complaints of dyspnea and irritability. Clinical evaluation revealed increased pulmonary vascularity on chest x-ray, absence of right A-V connection, regurgitation of left A-V valve, right-sided ventricular hypoplasia and L-TGA. Clinical assessment of the case was tricupsid atresia with LTGA and mitral regurgitation. Pulmonary artery banlding and artial septectomy was performed. Parenteral alimentation through the femoral veins resulted in obstruction of IVC and subsequent multiorgan failure. The autopsy findings of the heart were corrected transposition of great arteries with associated anomallies of right-sided mitral atresia, Ebstein's malformation of left-sided tricuspid valve, ventricular septal defect, muscular hypertrophy of right-sided left ventricle with luminal obliteration. Long segment of IVC was completely occluded due to occlusive thrombi with fungal infection. Bilateral kidneys were infarcted and azygos vein was markedly engorged. Microscopic examination revealed candidal colonization in the inferior vena cava and septic embolzation in brain.

Estimation of Pressure Difference Between Right and Left Ventricle in Ventricular Septal Defect with Doppler Echocardiography

No abstract available.

Anomalous origin of the left coronary artery from the pulmonary artery

Anomalous orignin of the left Coronary artery from the pulmonary artery is very rare, and being alleviatedsurgically, recognition of the lesion and associated hemodynamics is important. Authors had experenced a case ofthe anomalous orgin of left coronary artery from the pulmonary artery.