• Clinical and Experimental Pediatrics
• /
• v.46 no.7
• /
• pp.661-667
• /
• 2003

Purpose : This study was performed to find the chief clinical problems associated with the ages of adult patients of tetralogy of Fallot(TOF) who had undergone total correction. Methods : Of the 30 patients who were registered at the Grown-Up Congenital Heart Disease (GUCH) Clinic of Samsung Medical Center for TOF, a retrospective investigation was carried out on 28 patients who underwent total correction. Results : Mean age at retrospective study was 30.8(range : 16-53) years old. Age at total correction was 15.8(range : 2-49) years old. Problems after corrective surgery were assessed. They were arrhythmia, pulmonary valve regurgitation, left pulmonary artery stenosis, residual ventricular septal defect, mitral valve regurgitation, tricuspid valve regurgitation, right ventricle outflow tract obstruction, aortic valve regurgitation, infective endocarditis and protein losing enteropathy. After repair of TOF, such arrhythmias as atrial arrhythmia and AV conduction disturbances were observed in some patients. Cardiomegaly was found significantly in the subjects with arrhythmia(P<0.05), and arrhythmia was less observed in patients who underwent surgery at a young age. Eight patients required a reoperation; the main indications were residual ventricular septal defect, right ventricle outflow tract obstruction and peripheral pulmonary artery stenosis. Conclusion : The majority of the patients seemed to live normal lives after Tetralogy of Fallot repair. However, as residual anatomic and functional abnormalities exist postoperatively, continued careful follow-up is needed to detect and correct structural and functional abnormalities.

• Journal of Chest Surgery
• /
• v.39 no.8 s.265
• /
• pp.579-587
• /
• 2006

Background: Preoperative risk analysis for Fontan candidates is still less than optimal in that patients with apparently low risks may have poor surgical outcome; prolonged pleural drainage, protein losing enteropathy, pulmonary thromboembolism and death. We hypothesized that low pulmonary vascular compliance (PVC) is a risk factor for prolonged pleural effusion drainage after the Fontan operation. Material and Method: A retrospective review of 96 consecutive patients who underwent the Extracardiac Fontan procedures (median age: 3.9 years) was performed. Fontan risk score (FRS) was calculated from 12 categorized preoperative anatomic and physiologic variables. PVC $(mm^2/m^2{\cdot}mmHg)$ was defined as pulmonary artery index $(mm^2/m^2)$ divided by total pulmonary resistance $(W.U{\cdot}/m^2)$ and pulmonary blood flow $(L/min/m^2)$ based on the electrical circuit analogue of the pulmonary circulation. Chest tube indwelling time was log-transformed (log indwelling time, LIT) to fit normal distribution, and the relationship between preoperative predictors and LIT was analyzed by multiple linear regression. Result: Preoperative PVC, chest tube indwelling time and LIT ranged from 6 to 94.8 $mm^2/mmHg/m^2$ (median: 24.8), 3 to 268 days (median: 20 days), and 1.1 to 5.6 (mean: 2.9, standard deviation: 0.8), respectively. FRS, PVC, cardiopulmonary bypass time (CPB) and central venous pressure at postoperative 12 hours were correlated with LIT by univariable analyses. By multiple linear regression, PVC (p=0.0018) and CPB (p=0.0024) independently predicted LIT, explaining 21.7% of the variation. The regression equation was LIT=2.74-0.0158 PVC+0.00658 CPB. Conclusion: Low pulmonary vascular compliance is an important risk factor for prolonged pleural effusion drainage after the extracardiac Fontan procedure.

• Clinical and Experimental Pediatrics
• /
• v.48 no.9
• /
• pp.991-997
• /
• 2005

Purpose : Cow's milk protein-induced enterocolitis(CMPIE) is a symptom complex of vomiting and/or diarrhea caused by delayed hypersensitivity and may result in serious complications. This study was undertaken to identify high risk factors to facilitate the early recognition of CMPIE. Methods : We reviewed the data of 101 patients, aged 15 to 45 days, admitted due to vomiting and/or diarrhea between 2003 and 2004. After excluding 13 patients absolutely breast-fed and 2 patients transferred from other hospitals with the impression of CMPIE, the 86 study subjects were divided into three groups based on the underlying etiologies; CMPIE, infectious and non-infectious group. Results : CMPIE was diagnosed in 11 patients(12.8%). On admission, failure to gain weight(P=0.003), hypoalbuminemia(P=0.003), peripheral leukocytosis(P=0.015), and metabolic acidosis(P=0.014) were more significant in the CMPIE group than in the others. Multiple logistic regression analysis showed that the independent predictors of high risks for CMPIE were failure to gain weight <10 g/day(OR, 10.25[95% CI, 1.62-65.06]) and serum hypoalbuminemia <3.5 g/dL(OR, 9.18[95% CI, 1.69-49.74]). Cow's milk challenges were performed in the 11 CMPIE patients; vomiting(81.8%), abnormal stool test(80.0%), peripheral leukocyte count and absolute neutrophil count(ANC) increase(100.0%) (P<0.05), and enteropathy(100.0%). Conclusion : CMPIE is not a rare clinical disease in early infancy. The high risk factors of CMPIE were identified as follow : failure to gain weight below 10 g/day, hypoalbuminemia on admission and a rapid decrease during admission. Cow's milk challenge test with endoscopic duodenal biopsy was helpful to confirm CMPIE.

• Journal of Chest Surgery
• /
• v.36 no.8
• /
• pp.559-565
• /
• 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
• /
• v.35 no.3
• /
• pp.177-181
• /
• 2002

Background: Follow-up studies have shown that although outcomes have improved substantially over time, results of the Fontan operation and its modifications remain suboptimal. In this study, we reviewed our experience with the extracardiac conduit Fontan operation, with a focus early and midterm change of internal diameter of PTFE conduit. Material and Method: Between April 1997 and July 2000 were reviewed. Twelve patients (M:6, F:6, mean age 42.04 $\pm$ 12.43months, mean body weight 13.80$\pm$ 1.94kg) underwent extracardiac conduit Fontan operation with expanded PTFE graft. Mean cardiopulmonary bypass time was 109.7$\pm$26.99minute and mean operation time was 455$\pm$89.51minute. Intraoperative fenestration was performed in 10 patients. The aortic cross clamping was not performed in all patients. Result: There was no early deaths and no postoperative dysrhythmia. Postoperative protein losing enteropathy and prolonged pleural effusion occurred in 1(8.3%) and 4 patients(33.3%). Conduit patency was evaluated by magnetic resonance imaging studies. A 9.84$\pm$3.84% mean reduction in conduit internal diameter and there was no statistical correlation between the change of internal diameter of conduit and the postoperative duration after partial correlation analysis(r=0.019, p=0.955). Conclusion: These results demonstrate that the extracardiac conduit Fontan operation provies good early and midterm results and may reduce the prevalence of late arrhythmia. And there is no correlation between the change of internal diameter of conduit and the postoperative duration after extracardiac conduit Fontan operation with the expanded PTFE graft conduit.