• Clinical and Experimental Pediatrics
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• v.53 no.2
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• pp.195-202
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• 2010

Purpose : Despite recent advances in pulmonary hypertension management and surgery, appropriate guidelines remain to be developed for operability in congenital heart disease with pulmonary artery hypertension (PAH). Our aim was to evaluate clinical outcomes of patients with severe PAH who underwent surgical closure of left-to-right shunt lesions (LRSL) on the basis of pulmonary reactivity. Methods : We retrospectively reviewed 21 patients who underwent surgical closure of LRSL with severe PAH (${\geq}8$ Wood unit) from January 1995 to April 2009. The median age at operation was 26 years. Atrial septal defect, ventricular septal defect (VSD), VSD and patent ductus arteriosus (PDA), and PDA was present in 11, 4, 4, and 2 patients, respectively. Results : Operability was based on vasoreactivity of PAH. Of the 21 patients, 5 showed response to pulmonary vasodilator therapy and 8 showed vasoreactivity after balloon occlusion of defects. The remaining 8 patients were considered operable because of significant left-to-right shunt (Qp/Qs ${\geq}1.5$). Five patients underwent total closure of defects and 16 were left with small residual shunts. The median follow-up duration was 32 months. There was no significant postoperative mortality or morbidity. Systolic pulmonary artery pressure (PAP) decreased in all but 2 patients. All patients except 1 showed improvement of New York Heart Association functional class. Conclusion : Closure of LRSL in patients with severe PAH on the basis of pulmonary vasoreactivity seems reasonable. PAP and clinical symptoms improved in most patients. Further research is needed for the evaluation of long-term results.

• Clinical and Experimental Pediatrics
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• v.45 no.12
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• pp.1528-1533
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• 2002

Purpose : Isolated noncompaction of the ventricular myocardium(INVM) is one of the unclassified cardiomyopathies that is characterized by numerous, excessively prominent trabeculations, and deep intertrabecular recesses. We performed this study to evaluate the clinical features of INVM in children. Methods : The medical records of 10 patients with INVM were reviewed. We analyzed the clinical manifestations, hemodynamics, pattern of inheritance, and long-term prognosis of INVM in children. Results : Age at diagnosis was $45{\pm}53months$(1 day-14 years) with follow-up lasting as long as 78 months. Most INVM was asymptomatic on diagnosis. Associated cardiac anomalies were noted in six patients(ventricualr or atrial septal defect, patent ductus arteriosus with mitral valve prolapse, or mitral valve cleft). Depressed or flat changes of T wave in lead II, III and aVF were observed on electrocardiography. Various arrhythmia including WPW syndrome with paroxysmal supraventricular tachycardia, third-degree atrioventricular block, and familial sick sinus node dysfuction were observed. The degree of trabeculation in INVM was significantly prominent from level of mitral valve to apex compared to age-matched control. Familial recurrences were noted in two patients. The systolic function of the left ventricle was decreased in 20% of patients during the follow-up period, but systemic embolism or ventricular tachycardia was not observed. Conclusion : INVM is not a rare disorder. The cardiac function may be deteriorated in children as well as adults during long-term follow up. Thus early diagnosis and long-term follow-up must be done. So, the nation-wide multicenter clinical study would be mandatory to evaluate the incidence, long-term prognosis, and establishment of objective diagnostic criteria of INVM.

• Clinical and Experimental Pediatrics
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• v.45 no.12
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• pp.1512-1518
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• 2002

Purpose : Twins have a higher mortality and morbidity than singletons. Co-twin with one fetal death is particularly at risk. We investigated the neonatal outcome of live co-twins when one fetus had died after the 20th gestational week, and associated risk factors. Methods : A retrospective study was performed in fifteen cases of twin pregnancy with single intrauterine fetal deaths after the 20th gestational week during the period from January 1996 to December 2000 at Chonnam University Hospital. Results : Gestational age was $33.7{\pm}3.2weeks$, birth weight was $1,992{\pm}592g$. Interval between one fetal death being detected and the delivery of a live co-twin was $32.4{\pm}29.5days$. There were 11 cases(73.3%) of premature babies less than 37 gestational weeks. Main causes of preterm delivery were preterm labor and premature rupture of membranes. Hematologic findings suggesting disseminated intravascular coagulopathy(DIC) were not found in all mothers before delivery, and was not associated with DIC and encephalomalacia of the live co-twin. Perinatal outcome of fifteen live co-twins was as follows : six were normal(40%), three were DIC(20.0%), three were encephalomalacia(20.0%), one suffered intrauterine growth retardation, there was one case of twin to twin transfusion syndrome, and one of congenital heart disease(atrial septal defect with pulmonary stenosis). The occurrence of DIC and encephalomalacia in live co-twins was not related to placental chorionicity, birth weight, gestational week, and the interval between the detection one fetal death and the delivery of a live co-twin. Conclusion : We could not find any maternal hematologic problems in twin pregnancies complicated by one fetal death. Twenty percent of live co-twins showed DIC and encephalomalacia. However, its associated risk factors were not found. We need to investigate more closely the cases of live co-twins with one intrauterine fetal death.

• Journal of Chest Surgery
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• v.36 no.8
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• pp.559-565
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• 2003

By improving the flow pattern in Fontan circuit, total cavopulmonary connection (TCPC) could result in a better outcome than atriopulmonary connection Fontan operation. For the patients with impaired hemodynamics after atriopulmonary Fontan connection, conversion to TCPC can be expected to bring hemodynamic and functional improvement. We studied the results of the revision of the previous Fontan connection to TCPC in patients with failed Fontan circulation. Material and method: From October1979 to June 2002, eight patients who had failed Fontan circulation, underwent revision of previous Fontan operation to TCPC at Yonsei University Hospital. Intracardiac anomalies of the patients were tricuspid atresia (n=4) and other functional single ventricles (n=4). Mean age at TCPC conversion was 14.0$\pm$7.0 years (range, 4.6~26.2 years) and median interval between initial Fontan operation and TCPC was 7.5 years (range, 2.4~14.3 years). All patients had various degree of symptoms and signs of right heart failure. NYHA functional class was 111 or IV in six patients. Paroxysmal atrial fibrillation (n:f), cyanosis (n=2), intraatrial thrombi (n=2), and protein losing enteropathy (PLE) (n=3) were also combined. The previous Fontan operation was revised to extracardiac conduit placement (n=7) and intraatrial lateral tunnel (n=1). Result: There was no operative death. Major morbidities included deep sternal infection (n=1), prolonged pleural effusion over two weeks (n=1), and temporary junctional lachyarrhythrnia (n=1). Postoperative central venous Pressure was lower than the preoperative value (17.9$\pm$3.5 vs. 14.9$\pm$1.0, p=0.049). Follow-up was complete in all patients and extended to 50,1 months (mean, 30.3$\pm$ 12.8 months). There was no late death. All patients were in NYHA class 1 or 11. Paroxysmal supraventricular tachycardia developed in a patient who underwent conversion to intraatrial lateral tunnel procedure, PLE was recurred in two patients among three patients who had had PLE before the convertsion. There was no newly developed PLE. Conclusion: Hemodynamic and functional improvement could be expected for the patients with Fontan circulatory failure after atriopulmonary connection by revision of their previous circulation to TCPC. The conversion could be performed with low risk of morbidity and mortality.

• Journal of Chest Surgery
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• v.36 no.8
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• pp.566-575
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• 2003

We reviewed our 18-year surgical experience of biventricular repair for double-outlet right ventricle. Material and Method: One hundred twelve consecutive patients (80 males and 32 females) who underwent biventricular repair for double-outlet right ventricle between May 1986 and September 2002 were included. We assessed risk factors for early mortality and reoperation. Reoperation-free survival rate and actual survival rate were analysed. Result: Most common type of ventricular septal defect was subaortic (n=58, 52%) and non-committed type was second most common (n=32, 29%). Four different surgical methods were used: intraventricular baffle repair (n=71 , 63%): right ventricle to pulmonary ariery conduit interposition or REV with left ventricle to aorta baffle repair (n=24, 21 .4%): arierial switch operation with left ventricle to pulmonary artery baffle (n=14, 12.5%): Senning atrial switch operation with left ventricle to pulmonary artery baffle (n=3, 2.7%). Thirty four patients(30%) underwent palliative procedures before definite repair. Twenty three patients (21%) required reoperations. There were 12 (10.7%) early deaths and 4 late deaths. Age younger than 3 months at repair (p=0.003), cardiopulmonary bypass and aortic cross clamp time (p=0.015, p=0.067), type of operation (arterial switch operation) (p <0.001) and type of ventricular septal defect (subpulmonic type) (p=0.002) were revealed as risk factors for early death in univariate analysis, while age under 3 months was the only significant risk factor in multivariate analysis. Patients younger than 1 year of age (p=0.02), pulmonary artery angioplasty at definitive repair (p=0.024), type of ventricular septal defect (non-committed) (p=0.001), type of operation (right ventricle to pulmonary artery conduit interposition and REV operation) (p=0.028, p=0.017) were risk factors for reoperation in univariate analysis but there was no significant risk factor in multivariate analysis. Follow-up was available on 91 survivals with a mean duration of 110.8$\pm$56.4 (2~201) months. 5, 10 and 15 year survival rates were 86.5%, 85% and 85% and reoperation free survival were 85%, 71.5%, 70%. Conclusion: Age under 3 months at repair, subpulmonic ventricular septal defect and arterial switch operation were significant risk factors for early mortality. Patients with non-committed ventricular septal defect and who underwent conduit interposition or REV operation were risk factors for reoperation. With careful attention to chose best timing and surgical approach depending on morphologic characteristics, biventricular repair for double outlet right ventricle can be achieved with good long-term outcome.

• Journal of Chest Surgery
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• v.34 no.8
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• pp.597-603
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• 2001

Background: The purpose of writing this article is to get better clinical results and further clinical improvement based on subject to 110 cases of cardiac surgery which were performed and clinically analyzed. Material and Method: Since January 1995, the patent ductus arteriosus surgery had started in our hospital. In February 1999, an open heart surgery had started and up to September 2000, total of 110 cases were performed as of double ligation of patent ductus arteriosus(10 cases) and open heart surgery(100 cases). Result: Among the patients, Korean-Chines was 74(67.3%) and Han-Chinese was 35(31.8%). Congenital heart disease was 95 cases and acquired valvular heart disease was 15 cases. 83 cases of acyanotic congenital heart disease consisted of ventricular septal defect(VSD) with associated anomaly(45 cases), atrial septal defect(ASD) with associated anomaly(20 cases), patent ductus arteriosus(PDA) with associated anomaly(11 cases), cogenital aortic stenosis(5 cases), double chamber right ventrical(1 case) and Ebstein's anomaly(1 case). Among the 12 cases of cyanotic congenital heart disease, 11 cases of tetralogy of Fallot underwent total correction. Among the 15 cases of acquired valvular heart disease, valvular replacement(7 cases), double valve replacement (3 cases), mitral valve replacement(3 cases) and aortic valve replacement(1 case) were performed. And 8 cases of valvuloplasty were performed by using of commissurotomy, chordal plasty, plasty of papillary muscle, ring type annuloplasty, repair of leaflet. Conclusion: on cogenital heart disease, short term results of surgery for acyanotic cogenital heart disease was good. Among the cyanotic congenital heart disease, tetralogy of Fallot showed a little difference of recovery according to the surgery method so that further follow up observation was needed for long term result. On acquired valvular heart disease, especially, in terms of short term result of valvuloplasty, was relatively good, but further follow up observation was also needed for long term result. There wasn's any operative mortality.

• Journal of Chest Surgery
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• v.29 no.5
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• pp.542-547
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• 1996

All patients who underwent video-assisted thoracic surgery (VATS) for diagnostic purposes from Jan. 1992 to Aug. 1995 were reviewed. The total number of patients were 111 with 57 male and 54 female, and the mean age was 49 years (range 1 to 74). Multiple biopsies from more than one location were performed in 17 patients , pleural biopsies were performed In 49 patients, lung biopsies in 43 patients, mediastinal mass or Iymph node biopsies in 33 patients, and two pericardium biopsies and one dia- phragm biopsy, for a total of 128 biopsies. Seventeen pleural biopsy cases and one lung biopsy case underwent operation under local anesthesia , the rest were performed under general anesthesia. In patients who underwent lung biopsy, the mean age was 49.1 ye rs (range 22~ 73). The operating time was 40 to 170 minutes (mean 97), intravenous or intramuscular injection for pain control was required 0 to 22 times(mean 4.7), and chest tube was inserted from 1 to 26 days(mean 7). In all patients except two, a diagnosis was obtained from the biopsy and complication was encountered in one patient in whom intraoperative paroxysmal atrial tachycardia was detected. In 7 patients, a thorn- cotomy had to be done due to pleural adhesion or intraoperative bleeding, and 7 patients had postoperative complications associated with the chest tube. In the pleural biopsy group, the mean age was 49 years (range 17~ 74). The operating time was 25 to 80 minutes (mean 49), intravenous or intramuscular injection for pain control was needed 0 to 20 times (mean 3.6), and the chest tube was i.nserted for 0 to 67 days(mean 9.8). In all the patients, a diagnosis was possible. The chest tube was inserted for longer than 7 days in 11 patients. In the Iymph node biopsy roup, the mean age was 44.2 years (range 1 ~ 68). The operating time was )0 to 3)5 minutes(mean 105), pain control was required 0 to 15 times(mean 3.2), and a chest tube was kept in place for 1 to 36 days(mean 6.1). In one patient, a diagnosis was not possible and a chest tube was kept in place for longer than 7 days in 7 patients. In the multiple biopsy group, the mean age was 53.1 years(range 20~ 71). The operating time was 15 to 165 minutes(mean 85), and pain control was done from 0 to 17 times(mean 3.1). The chest tube was kept in place for 1 to 16 days (mean 7.9).

• The Korean Journal of Nuclear Medicine
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• v.29 no.4
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• pp.484-491
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• 1995

It is well known that hepatobiliary agent are taken up by metastatic hepatocellular carcinoma(HCC) as well as primary HCC. But the reported cases of the extrahepatic metastasis of HCC diagnosed by hepatobiliary scintigraphy are for the most part hematogenous ones. The relation of the uptake pattern of hepatobiliary agent in the primary and metastatic HCC is also still remains unknown. So we undertook this study to evaluate the relation of the hepatobiliary scintigraphic patterns of primary and metastatic HCC with different metastatic routes. Nine patients with primary HCC and twelve cases of metastatic HCC including four lung metastases, one bone metastasis, one right atrial metastasis, one peritoneal wall metastasis, and five lymph node metastases were studied with $^{99m}Tc-DISIDA$ scintigraphy. The images were taken on 10, 30 minutes, 1, 2, 4-6 hours. The overall detection rates of hematogenous metastases(lung and bone) is 60%(3 of 5), direct metastasis(right atrium and peritoneal wall), 100%(2 of 2) and lymphatic metastases, 0%(0 of 5). In four of five metastatic cases demonstrated with hepatobiliary scintigraphy, biliary agent is also taken up by primary HCC lesions. And the appearing time of the radioactivity in the direct metastatic HCC lesioin is same as that of primary HCC and in the cases of hematogenous metastasis, earlier than that of primary HCC. Hepatobiliary scintigraphy is more useful in the diagnosis of the metastatic HCC than primary HCC, in the cases of hematogenous and direct metastasis.

• Journal of Chest Surgery
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• v.40 no.10
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• pp.659-666
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• 2007

Background: Preserving the subvalvular apparatus after mitral valve replacement (MVR) results in better ventricular function and a better outcome. In conjunction, mitral valve repair (MVr) is associated with a better outcome, yet little is known about the hemodynamics and outcomes between patients undergoing MVr and MVR with chordal preservation. We prospectively evaluated the hemodynamic changes and outcomes of patients undergoing MVr and MVR with chordal preservation. Material and Method: Fifty-four patients with mitral regurgitation (MR) who under-went MVR with chordal preservation (n=21) or MVr (n=33) were studied. The patients' characteristics, the intra-and postoperative hemodynamics and the use of cardiac medications, the postoperative outcome and the complications were recorded during the hospital stay. All the patients were followed up for at least 6 months post-operatively for determining their morbidity and mortality. Result: The patients' characteristics were similar between the groups, except for the presence of atrial fibrillation and congestive heart failure which was more frequent in the MVR group. Also, the preoperative left ventricular ejection fraction was lower in the MVR group than in the MVr group ($64{\pm}9%$ versus $69{\pm}5%$, respectively, p=0.043). There were no significant differences of the hemodynamics between the groups. The use of inotropic drugs and pacemakers during the 12hrs postoperatively was more common in the MVR group than in the MVr group (48% versus 24%, p=0.025 and 52% versus 24%, p=0.035, respectively). The other postoperative outcomes were similar in both groups for at least the 6 months follow-up period. Conclusion: MVR with chordal preservation was comparable with regard to the hemodynamics and clinical outcomes, supporting the beneficial effect of preserving the subvalvular apparatus after MVR.

• Clinical and Experimental Pediatrics
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• v.45 no.5
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• pp.615-621
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• 2002

Purpose : The neonatal cardiac catheterization and angiogram for transcatheter therapy are still essential methods in congenital heart disease, so we reviewed our experience with neonatal cardiac catheterization over 10 years at a single institution. Methods : A retrospective review of all 139 neonatal catheterizations from January 1991 to December 2000 at Sejong Heart Institution was performed. The purpose of the catheterizations, ages, body weights and the complications by the exam was surveyed. Results : The mean age of our 139 patients was 14.9 days and the mean body weight was 3.3 kg. As for the diagnosis : TGA in 49 patients, PAIVS in 26 patients, DORV in 14 patients, PS in 14 patients, PAVSD in 11 patients. For the cardiac catheterization, general anesthesia was performed in 65% of total and fluoroscopy time was $20.1{\pm}14.5$ minutes during cardiac catheterization. In the interventional cardiac catheterization which was 75% of the total, the age and body weight were statistically the same but the irradiation time was longer than the diagnostic catheterization( P=0.001). There were 48 cases of atrial septostomy, 16 cases of balloon pulmonary valvuloplasty and 25 cases of transcatheter pulmonary valvotomy. Complications of cardiac catheterization were found in 16.3%, but there was no difference between interventional catheterization and diagnostic catheterization. Conclusion : Therapeutic cardiac catheterization in neonates is a relatively safe and effective method in congenital heart disease.