Congenital Partial Pericardial Defect Presenting as Chest Pain / 대한흉부외과학회지

Patients with congenital pericardial defect display few symptoms and this is an unusual disease, so it is generally found incidentally in most of the cases. In some cases, symptoms such as chest pain are found due to partial cardiac herniation. We report here on a 14-year-old girl with dyspnea and chest pain that started during physical activity 3 months before the hospital visit. She was diagnosed with herniation of the left atrial appendage with a partial pericardium defect and she was treated with an operation.

A Case of Renovascular Hypertension Controlled by Renal Artery Embolization / 소아과

Renal artery stenosis is a major cause of renovascular hypertension and the most common cause of treatable secondary hypertension. There are several methods to treat renal artery stenosis, including surgery, percutaneous transluminal renal angioplasty(PTRA), and renal artery stenting(RAS). But, renal artery embolization can be tried in atherosclerotic stenosis, multiple stenosis, microaneurysm, and stenosis difficult to try PTRA or RAS. We report a case of renovascular hypertension in a 14-year-old female who had multiple segmental renal artery stenosis. Hypertension was controlled by renal ablation therapy with renal artery embolization.

Assessment of Myocardial Ischemia using Myocardial Perfusion Scan in Kawasaki Disease

PURPOSE: Myocardial scintigraphy has been reported to be useful in adults, but its usefulness in children is limited. This study was done to determine the feasibility and accuracy of 99mTc-MIBI SPECT(sestamibi , methoxy-isobutyl-isonitrarite myocardial single emirs sion computed tomography, Dp-SPECT) after dipyridamole infusion to detect coronary obstructive lesions in Kawasaki disease(KD). METHODS: Dp-SPECT was performed in 21 control(group 1), 8 with coronary aneurysm(group 2) and 16 with coronary obstructive lesion(group 3) in children with KD. The spatial distribution of Dp-SPECT was determined and analyzed visually. Group 2 and 3 underwent coronary angiogram within 3 months of Dp-SPECT. RESULTS: Chest pain occurred more frequently in group 2. No subject required supplemental oxygen or were complicated by myocardial ischemia. The specificity of Dp-SPECT for control subject was 95% & 62% if using a cut-off criterion of abnormality, utilizing moderate or mild perfusion defect, respectively. The false positive rate was high in anterior, apical and anteroseptal segments but not related to age. The abnormalities found on Dp-SPECT was observed in 25% of aneurysmal coronary arteries but not related to its size. The sensitivity, specificity, and accuracy of Dp-SPECT was 100%, 84.5% and 79% in coronary stenosis greater than 75%, respectively. CONCLUSION: The interpretation of Dp-SPECT should be careful in KD because of discrepancies between Dp-SPECT & angiography, But DP-SPECT is noninvasive and easily applicable. It monitors the occurrence and progression of coronary stenosis due to KD. Besides Dp-SPECT may be applicable after arterial switch operation or to myocardial sinusoid.

The Effect of Long-term Steroid Therapy on Cardiac Function in Nephrotic Syndrome

PURPOSE: The aim of this study is to assess the cardiac effect of long-term steroid therapy in nephrotic syndrome and the validity of LV functional parameters as an early predictor of subclinical cardiac dysfunction. METHODS: The study group was composed of 21 patients diagnosed as minimal change nephrotic syndrome(NS), being managed with prednisone over 6 months or within 6 weeks after stopping medication. The control group was composed of 23 healthy children without cardiopulmonary dysfunction. The functional parameters of the left ventricle, including systolic and diastolic indices were measured using 2D-doppler echocardiography. RESULTS: There was a significant increase of left ventricular mass index in the study group(P value<0.05). Isovolumetric relaxation time of the left ventricle was prolonged significantly in the study group(P value<0.05). The systolic phase area and the ratio of systolic and diastolic phase area of pulmonary vein flow profile were increased significantly in the study group(P value<0.05). CONCLUSION: In NS patients managed with long-term steroid therapy, left ventricular diastolic functional abnormality may be present even though functional limitation is not yet evident. In the future, the longitudinal study will be needed conderning the cardiac effect of long-term steroid treatment.

The Effect of Long-term Steroid Therapy on Cardiac Function in Nephrotic Syndrome

PURPOSE: The aim of this study is to assess the cardiac effect of long-term steroid therapy in nephrotic syndrome and the validity of LV functional parameters as an early predictor of subclinical cardiac dysfunction. METHODS: The study group was composed of 21 patients diagnosed as minimal change nephrotic syndrome(NS), being managed with prednisone over 6 months or within 6 weeks after stopping medication. The control group was composed of 23 healthy children without cardiopulmonary dysfunction. The functional parameters of the left ventricle, including systolic and diastolic indices were measured using 2D-doppler echocardiography. RESULTS: There was a significant increase of left ventricular mass index in the study group(P value<0.05). Isovolumetric relaxation time of the left ventricle was prolonged significantly in the study group(P value<0.05). The systolic phase area and the ratio of systolic and diastolic phase area of pulmonary vein flow profile were increased significantly in the study group(P value<0.05). CONCLUSION: In NS patients managed with long-term steroid therapy, left ventricular diastolic functional abnormality may be present even though functional limitation is not yet evident. In the future, the longitudinal study will be needed conderning the cardiac effect of long-term steroid treatment.

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.

Aortic insufficiency after Arterial Switch Operation in Transposion of the Great Arteries

BACKGROUND: The arterial switch operation (ASO) has become a procedure of choice for transposition of the great arteries (TGA) with or without ventricular septal defect (VSD). In spite of current low surgical mortality in immediate surgical period, there are potential problems of late morbidity and mortality. In this study, we evaluate the prevalence and severity of aortic insufficiency (AI) and associated factors with AI. METHOD: We retrospectively reviewed medical records and echocardiogram of 125 patients (M89, F36) underwent ASO for TGA at Seoul National University Children's Hospital from July, 1987 to June, 1997. To evaluate for associated factors, we divided patients of TGA to four hemodynamic groups (Group I, simple TGA (n-53); Group II, associated with ventricular septal defect (VSD) (n-50); Group III, associated with VSD and PS (n-18); Group IV, associated with PS (n-4)). Grade of AI was assessed by color Doppler analysis (Perry's et al). RESULTS: Total prevalence rate of AI was 35.2% (31/88) and grade of AI were mild in 61%, moderate in 39%. The incidence of AI in each group was: Group I 25.0% (10/40), Group II 26.0% (9/36), Group III 92.0% (12/13). According to this study, pulmonary stenosis was the only significant factor to AI. During follow-up (39+/-24 Months), in spite of the normal left ventricular function in most patients with AI, a few patients (3 cases) suffered from progressive AI and some moderate AI patients (2 cases) showed slightly decreased LV function. Two moderate and progressive AI lossed. CONCLUSION: In this study, AI (after ASO) was not uncommon and pulmonary stenosis was an important factor to AI. In some cases, AI became worse and developed decreased LV function. This suggests that meticulous follow-up after ASO is necessary.