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.

The Assessment of Pulmonary Parenchymal Damage in Congenital Heart Disease using Lung Perfusion Scan

PURPOSE: In acyanotic congenital heart disease of children with left to right shunt, 99mTc- Macroaggregate albumin (MAA) was employed to determine the scintigraphic severity of pulmonary parenchymal damage. METHODS: These data of lung perfusion scan using 99mTc-MAA were compared with hemodynamic values obtained from cardiac catheterization. RESULTS: The mean Pulmonary arterial pressure (> or = 30mmHg), total pulmonary resistance (> or = 2 Wood unit) & ratio of pulmonary vascular resistance/systemic resistance (Rp/Rs > or = 0.2) were proportionally related to higher perfusion ratio of upper and lower lung field. The diagnostic values of perfusion ratio of upper & lower field of both lung (cut off value > or = 0.8) for pulmonary hypertension were as follows : 60-65% of sensitivity, 75-90% of specificity, 72-86% of positive predictive value & 68-69% of negative predictive value. The mottled perfusion defect was frequently found in patients with pulmonary hypertension (mean pulmonary arterial pressure > or = 30mmHg, Rp > or = 2 Wood unit). The degree of perfusion defect as indicator of severe pulmonary parenchymal damage was utilized clinically as determinator of operability and morbidity for acyanotic shunt lesions perioperatively. CONCLUSION: The scintigraphic severity determined by 99mTc-MAA lung perfusion scan could be valid for evaluating the extent of pulmonary parenchymal damage in acyanotic congenital heart disease complicated by pulmonary hypertension.

Follow-up Study after Atrial Switch Operation for Complete Transposition of the Great Arteries

BACKGROUND: Since the introduction of atrial switch operation, the outlook for patients with complete transposition of the great arteries (TGA) has improved dramatically. However, many survivors are afflicted with postoperative complications and continue to demand medical attention. We therefore performed the study for the evaluation of the clinical course in the patients with complete TGA after atrial switch operation. METHODS: We analyzed the data of 51 patients who underwent the atrial switch operation for complete TGA at Seoul National University Hospital between January 1981 and June 1993 retrospectively. RESULTS: The patients were composed of 36 males and 15 females (mean age at operation, 15+/-30 months, range 2 months-18 years). Senning operation was undertaken in 43 and Mustard operation in 8. Among these, 27 had simple and 24 complex complete TGA. The early mortality rate within 1 month postoperatively was 39% and the late mortality rate 16%. The actuarial survival rate after 10 years was 78%. Of these, 17 cases have been followed up so far. Although the significant tricuspid regurgitation was noted in 9, right ventricular systolic function was maintained in all of them. Preoperative sinus node dysfunction was noted in 1 case. Postoperatively, premature ventricular contractions were found in 8 cases, atrial flutter in 4, and sinus node dysfunction in 7. Subclinical pulmonary venous obstruction was found in 2 and systemic venous obstruction in 1. At present, one takes digoxin for prevention of atrial flutter and 7 take digoxin, furosemide, and/or angiotensin-converting enzyme inhibitors for prevention of ventricular functional deterioration. Twelve cases were in New York Heart Association functional class I at the latest follow-up and 5 were in class II. CONCLUSION: This study revealed that morbidity and mortality were not low after atrial switch operation. However, considering the good long-term functional status in the survivors, atrial switch operation could be an alternative to arterial switch operation in complicated cases of TGA.

Follow-up Study after Atrial Switch Operation for Complete Transposition of the Great Arteries

BACKGROUND: Since the introduction of atrial switch operation, the outlook for patients with complete transposition of the great arteries (TGA) has improved dramatically. However, many survivors are afflicted with postoperative complications and continue to demand medical attention. We therefore performed the study for the evaluation of the clinical course in the patients with complete TGA after atrial switch operation. METHODS: We analyzed the data of 51 patients who underwent the atrial switch operation for complete TGA at Seoul National University Hospital between January 1981 and June 1993 retrospectively. RESULTS: The patients were composed of 36 males and 15 females (mean age at operation, 15+/-30 months, range 2 months-18 years). Senning operation was undertaken in 43 and Mustard operation in 8. Among these, 27 had simple and 24 complex complete TGA. The early mortality rate within 1 month postoperatively was 39% and the late mortality rate 16%. The actuarial survival rate after 10 years was 78%. Of these, 17 cases have been followed up so far. Although the significant tricuspid regurgitation was noted in 9, right ventricular systolic function was maintained in all of them. Preoperative sinus node dysfunction was noted in 1 case. Postoperatively, premature ventricular contractions were found in 8 cases, atrial flutter in 4, and sinus node dysfunction in 7. Subclinical pulmonary venous obstruction was found in 2 and systemic venous obstruction in 1. At present, one takes digoxin for prevention of atrial flutter and 7 take digoxin, furosemide, and/or angiotensin-converting enzyme inhibitors for prevention of ventricular functional deterioration. Twelve cases were in New York Heart Association functional class I at the latest follow-up and 5 were in class II. CONCLUSION: This study revealed that morbidity and mortality were not low after atrial switch operation. However, considering the good long-term functional status in the survivors, atrial switch operation could be an alternative to arterial switch operation in complicated cases of TGA.

Intermediate Term Follow-up Results of Hypertrophic Cardiomyopathy in Children

PURPOSE: Hypertrophic cardiomyopathy (HCMP) is a primary cardiac muscular disease with high risk of sudden cardiac death. This study is performed to understand the clinical features of children with HCMP. METHODS: Retrospective analysis of the medical records of the patients diagnosed at Seoul National University Children's Hospital between October 1985 and June 1996 was done. Excluded the patients who had been lost to followed up within 12 months after diagnosis. RESULTS: During follow-up (mean 56 months, range 1 month to 13 years), 3 of the 24 patients (male 15 and female 9, mean age at diagnosis 5.9 years, range 2 months-15 years) died suddenly and 1 died of persistent heart failure after cardiac resuscitation. Cumulative death rate for 56 months was 22.3%. Eighteen were treated with -blocker including 1 with additional amiodarone. Normal quality of life except competetive exercise were maintained in the 95% of the survivors. In this study, moderate to severe dyspnea on exertion (New York Heart Association III/IV) was the only significant risk factors of death (p<0.05). On the surface electrocardiogram, QTc interval was significantly longer in the study group comparing with the age and sex matched control group. Right ventricular outflow obstruction was associated in the 3 (12%) and was common in infants (33%). CONCLUSIONS: Overall mortality is high in children with HCMP. In our study. cumulative death rate for 56 months is 22.3%. Overt dyspnea on exertion was the only identifiable factor of mortality.

A Case of Chronic Granulomatous Disease Which is Improved by Interferon- Therapy

Chronic granulomatous disease is a group of rare disorders characterized by a disease of oxidative metabolism of phagocytes with deficit of antimicrobial activity. Also, it has clinical features of chronic pyogenic infection, granuloma formation, hypergammaglobulinemia, anemia and leukocytosis.We experienced a case of chronic granulomatous disease with chronic recurrent infection, hypergammaglobulinemia and liver abscess. It was confirmed by modified NBT test. The result of patient's NBT test was less than 11%. The patient received subcutaneous injection of interferon-gamma, 200 million unit (0.1mg/m2 of body surface area per dose), on three consecutive days per week for 3 weeks and her symptom was improved. Then, we report a case of chronic granulomatous disease which is improved by interferon- therapy with brief review of literature.

The relationship between neonatal hypoglycemia and newborn iron status in hypoglycemic large-for-gestational age infants

No abstract available.