Long term prognosis of patients who had a Fontan operation / 소아과

PURPOSE: This study assessed the long term survival rate and long term complications of patients who had a modified Fontan operation for functionally univentricular cardiac anomaly. METHODS: Between June 1986 and December 2000, 302 patients with a functional single ventricle underwent surgical interventions and were followed up until February 2006. The mean follow-up period was 8.3+/-5.3 years (range 3.5-18 years). Their median age was 2.4 years at the Fontan operation. The survival rate, the incidence and the risk factor of late complications were evaluated retrospectively. RESULTS: The verall survival rate was 91 percent at 5 years and 87 percent at 10 years. In multivariate analysis, early calendar year of operation and significant regurgitation were risk factors of death. The surviving patients showed NYHA functional class I in 82 percent, class II in 15 percent, and class III in 3 percent. Redo Fontan operations were necessary in 8.8 percent of patients at average 12.8+/-3.6 years after initial Fontan operation. The most common cause of Fontan conversion was atrial arrhythmia. The incidence of thromboembolic events was 9.3% and these complications were associated with the occurrence of atrial tachyarrhythmia. Supraventricular tachycardia including atrial flutter or fibrillation were reported on the follow-up examination by 11.2 percent of survivors after 8.4+/-5.6 years. Atriopulmonary connection showed higher rates of late tachycardia than lateral tunnel operation. CONCLUSIONS: This study revealed that the recent survival rate of Fontan type operation was satisfactory, but the occurrence of late complications after a Fontan type operation increased with the longer survival. There is a need for strict follow up and early treatment of late complications in patients who had a Fontan operation.

Acute Myocarditis in Children: Clinical Aspects and Outcomes

PURPOSE: Myocarditis is an insidious inflammatory disorder of the myocardium. We investigated clinical characteristics, laboratory data, prognosis, and outcomes in patients with acute myocarditis. METHODS: We retrospectively analyzed the medical records of 52 myocarditis patients who were admitted to the SNUCH from 1985 to 2005. We compared progressed group (included mortality and dilated cardiomyopathy) with recovery group. RESULTS: The median age was 4.12 years. The median follow-up duration was 2.84 years. Sixteen patients(30.8%) recovered myocardial function. Twelve patients(23.1%) died; ten of them died because of fulminant myocarditis. Sixteen patients(30.8%) progressed to severe dilated cardiomyopathy. Twenty-one patients were treated with intravenous immunoglobulin(IVIG), and six patients took oral prednisone. Oral prednisone and IVIG showed no significant treatment effects(P=0.284, P=0.695). Six patients underwent temporary pacemaker due to complete atrioventricular block. Three patients underwent extracorporeal membrane oxygenation(ECMO), and 1 patient survived. Recently, survival rate for children with myocarditis was increased due to early intensive care and non-pharmacologic therapy(ECMO, ventricular assist device). CONCLUSIONS: Steroid and IVIG were ineffective treatments in acute myocarditis. Patients with myocarditis needed an early intensive care. We expect that early aggressive treatments could improve patients' outcomes.

Causes of Syncope in Children

PURPOSE: Syncope is not rare and is caused by various conditions ranging from common physiologic derangements to life-threatening conditions. However, there are limited reports regarding syncope in children. We retrospectively analyzed our experiences of the syncope in children. METHODS: Retrospective analysis of the medical records of patients with syncopal episodes between October 1985 and June 1996. RESULTS: Sixty-eight patients (male; 36, female; 32, mean onset age 10.9 +/- 3.7, range 3-18 years) were evaluated. Causes were identified in 67.6% (46/68): neurocardiogenic syncope in 25 (36.8%), cardiac syncope in 21 (30.8%). Among the children with cardiac syncope, rhythm disturbances were major and occurred in 19[complete heart block 1, sinus node dysfunction 3, atrial flutter 2, atrial fibrillation 1, paroxysmal supraventricular tachycardia 2, ventricular tachycardia (VT) 10]. Syncope occurred in two patients with structural defects, one with diffuse coronary arteriopathy and the other with double outlet right ventricle with pulmonary hypertension, although the causes remained uncertain. Among those with VT, associated abnormalities were: TU complex abnormalities in 5, cardiac tumor in 2, cardiomyopathy in 1, unidentified in 2. Various precipitating factors were described in 39 (57.4%); eleven (52.4%) of 21 with cardiac syncope, syncope was exercise related. Among the selected 12 with complicated neurocardiogenic syncope (exercise related, associated with ventricular arrhythmia, or with postoperative congenital heart disease), head-up tilt test with or without isoproterenol infusion reproduced syncope in 10 patients (83.3%). Sudden cardiac death was found in two children; 1 with exercise related polymorphic VT, 1 with VT and cardiac tumor. CONCLUSION: Various causes can be identified after scrupulous evaluation. Cardiac causes should be considered especially in case of exercise related syncope. Head-up tilt test is an effective diagnostic method in neurocardiogenic syncope.

Influence of Phototherapy on Incidence of Patent Ductus Arteriosus in Very Low Birth Weight Infants

PURPOSE: Patent ductus arteriosus (PDA) is a common disease in very low birth weight infants (VLBWI). Hemodynamically significant PDA increases the morbidity and mortality of premature infants. Based on experimental model, light inhibits the constriction of immature piglet's ductal rings. No specific mechanism adequately explains the effect of light on the relaxation of PDA. Several hypotheses, including activation of photosensitive metabolites, alterations in receptors, or alterations in prostaglandin metabolism, have been postulated. The purpose of this study was to evaluate the influence of phototherapy on incidence of PDA in VLBWI. Mehtods : Sixty-three infants with birth weights less than 1,500 gm from March 1994 to February 1996 who were admitted in NICU of Seoul National University Children's Hospital were included. Thirty-four infants from March 1995 to February 1996 were shielded with aluminium foils on left chest during phototherapy (Shield group) and twenty-nine infants from March 1994 to February 1995 were not shielded (No shield group : control group). We investigated the incidence and the perinatal risk factors of PDA. RESULTS: 1) The incidence of PDA was 18% in shield group and 41% in control group. There was statistically significant between the two groups (P<0.05). 2) There was not statistically significant between two groups with gestational age, birth weight, sex, delivery mode, etc. 3) The perinatal risk factors which were statistically significant were group and presence of respiratory distress syndrome (RDS), and use of artificial surfactant. With linear logistic regression analysis, only group (OR=8.3, 95% CI=1.17-58.69) and presence of RDS (OR=21.3, 95% CI=1.39-329.81) were proved to be related to the occurrence of PDA. CONCLUSIONS: We conclude that chest shielding during phototherapy is a simple and inexpensive method to decrease the incidence of PDA.