Evaluation of Prognostic Factors in Corrected Transposition of the Great Arteries at Mid-term Follow-up

PURPOSE: The prognosis of patients with corrected transposition of the great arteries(C-TGA) is variably affected by associated intracardiac defects, systemic right ventricular function, tricuspid valve competence, and conduction disturbances. This study aims to evaluate the importance of those factors at mid-term follow-up. METHODS: Medical records of 94 patients(males 58, females 36; mean age at last follow-up, 12+/-9 years; mean follow-up duration, 9+/-6.4 years) diagnosed between January 1980 and May 2002 at Seoul National University Children's Hospital were studied retrospectively. RESULTS: Among 94 patients, operations were performed in 72 patients(classic operations in 55; double switch operations in 17). Among prognostic factors including associated intracardiac anomalies(at least moderately severe tricuspid insufficiency(TI), ventricular septal defect, pulmonary stenosis and pulmonary atresia), intracardiac operation and complete atrioventricular block, TI was the only significant factor for death(P=0.001), and in turn, Ebstein anomaly and high grade atrioventricular block predicted TI. 20-year survival without TI was 77%, but only 35% with TI(P=0.0002); excluding perioperative death, the 20-year survival rates with and without TI were 48% and 87% respectively(P= 0.008). There was no statistical difference in 20-year survival rate or association with TI between classic and double switch operation. CONCLUSION: TI was the major prognostic factor for C-TGA and was associated with Ebstein anomaly and high grade atrioventricular block at mid-term follow-up. Long-term follow-up is required to evaluate other factors, including double switch operations and associated intracardiac defects more exactly.

Follow-up Study of Children with Anthracycline Cardiotoxicity

PURPOSE: We studied the relationship between anthracycline cumulative dose and anthracycline cardiotoxicity in childhood cancer and followed up 40 children with anthracycline cardiotoxicity. METHODS: A retrospective study was performed in 154 children who received anthracycline chemotherapy between January 1995 to December 2000. Cardiotoxicity was defined when the left ventricular fractional shortening(FS) was below 26%; it was divided into two groups, mild and severe cardiotoxicity, according to the FS. We followed up survivors with cardiotoxicity, and checked their present cardiac function by physical activity, echocardiography, electrocardiography(EKG) and chest X-ray. RESULTS: Of the 154 children treated with anthracyclines, forty(26.0%) were diagnosed as cardiotoxicity. The incidence of cardiotoxicity increased in exponential fashion with increases in the cumulative dose of anthracyclines. There was minimal increase of incidence until a dose of 300 mg/m2 after which the incidence increased rapidly. After mean 3.8+/-1.8 year follow-up of 23 survivors with cardiotoxicity, FS increased significantly. EKG and chest X-rays were not helpful for the diagnosis of cardiotoxicity because of their low sensitivity and specificity. CONCLUSION: Although convenient, non-invasive and inexpensive, EKG and chest X-rays were not helpful for the follow-up of anthracycline cardiotoxicity. Almost all survivors with anthracycline cardiotoxicity have improved in both physical activity and echocardiographic findings after discontinuation of anthracyclines.

Evaluation of Resting Blood Pressures, Left Ventricular Function and Mass in Young Children with Successful Coarctoplasty in Infancy

BACKGROUND AND OBJECTIVES: Cardiovascular morbidities and mortalities due to persistent hypertension, left ventricular (LV) dysfunction and increased LV mass have been reported in adolescents and adults with successful coarctoplasty. We evaluated progressive properties by measuring resting blood pressures (BP), LV functions and the masses in young children with successful coarctoplasty in infancy. SUBJECTS AND METHODS: BP in the right arm and the left ankle, LV function and mass, by the use of M-mode and pulsed wave Doppler ultrasound, were measured in 25 patients, with a mean age of 6.4+/-3 years; and a mean age at the time of repair of 0.22+/-0.24 years, and in 22 control subjects, with a mean age of 5.8+/-2.4 years. All subjects were divided into two groups based on age (more than 5 years of age and less than 5 years of age) and were analyzed independently. RESULTS: BP in the right arm and left ankle, LV function and mass showed no consistent differences between the two groups. However, as age increased, there was a tendency of wide pulse pressure (PP) in the right arm of patients contrary to that in the control subjects. The relative increase in the PP was mainly due to a relative decrease in the diastolic BP. There was a significant increase in the LV mass index of patients with increasing age (p<0.01). The LV mass index showed significant positive correlations with the systolic BP (p<0.05) and the pulse pressure (p<0.05) of the right arm of the patients. CONCLUSION: We recommend careful long-term follow-up through out adulthood to reduce morbidity and mortality, even for children who received successful coarctoplasty in early infancy.