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.

Congenital Heart Disease and Associated Extracardiac Anomalies in Autopsies

PURPOSE: In order to find out how frequently and what kind of extra-cardiac anomalies (CAs) is associated with congenital heart disease (CHD) in general and in each CAs, autopsies with congenital cardiovascular malformations were studied. METHODS: Autopsy files of Seoul National University Children's Hospital from 1991 to 1995 were reviewed to find cases of CHDs. Other extra-CAs were tabulated from the records of autopsy files. The study population was comprised of 156 cases (86 still birth or product of terminated pregnancy; 66 livebirth; 4 unknowns-whether still birth or livebirth). RESULTS: The sex ratio was 1.14:1 males to females. In still birth cases, gestational age ranged from 16 to 42 weeeks and in live birth cases, age ranged from 1 day to 1 year 7 months (mostly neonates). The ventricular septal defect (VSD) was the most common CA, followed by coarctation of aorta, tetralogy of Follot in this order of frequency. No extra-CA was found in 55 cases, 1 extra-CA in 54 cases, 2 extra-CAs in 35 cases, 3 or more extra-CAs in 12 cases. VSD was commonly associated with Edward syndrome and holoprosencephaly, tetralogy of Fallot with holoprosencephaly, and coarctation with cystic hygroma. The most commonly involved organ system was the central nervous system followed by the digestive system, facial anomaly, and so on. Chromosomal anomaly associated with CHDs was 24 cases. Common individual anomalies were cystic hygroma, Edward syndrome, holoprosencephaly, and so on. CONCLUSION: As the fetus and infants with CHD have high incidence of associated extra-CAs, collaborative works involving dysmorphologist, geneticist, pathologist, surgeons, and pediatric cardiologist are needed. For further studies of CA and extra-CAs, a systematic database andprecise medical records and interdepartmental collaboration are necessary.