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An 18-year-old girl with atrial septal defect and Andersen syndrome is reported. Andersen syndrome was described as a hereditary disease characterized by periodic paralysis, prolongation of the QT interval with ventricular arrhythmia and characteristic physical features including low set ear and micrognathia. We successfully performed cardiac operation for this rare associated malformation.

Between November 1985 and April 2003, surgical treatment for ventricular septal perforation (VSP) after acute myocardial infarction was performed in 16 patients. Patients were divided into 2 groups by method of operation. One group is infarct exclusion technique (n = 10). The other group is trans right ventricular (RV) approach (n = 6). No significant differences were observed between 2 groups in preoperative states. Operative death rate was high in both groups. Five patients (50%) were died in infarct exclusion group, 4 patients (67%) in trans RV group. Infarct exclusion technique needed longer extracorporeal circulation time (201 +/- 33 min) than trans RV approach (170 +/- 32 min). Although trans RV approach is attractive for its simplicity, 3 cases died of rupture of remained left ventricle infarction area. On the other hand, there were no mortality cases caused by left ventricle rupture in infarct exclusion technique group. As this result, we select infarct exclusion technique as a surgical correction for VSP.

We investigated the alterations in regional cerebral blood flow (rCBF) in mitochondrial encephalomyopathy (MEM), using [123I]N-isopropyl-p-iodoamphetamine (IMP) or 99mTc-hexamethyl propyleneamine oxime SPECT in 10 MEM patients.Four of the patients had MEM with lactic acidosis and strokelike episodes (MELAS), 2 had Kearns-Sayre syndrome (KSS), 1 had myoclonic epilepsy with ragged red fibers (MERRF) and 3 had cytochrome C oxidase deficiency (CCOD). Cerebral perfusion reserve was obtained from 6 patients (3 MELAS, 1 MERRF, 1 KSS, 1 CCOD) for a comparative analysis using the split-dose 123I-IMP SPECT method before and after the injection of acetazolamide.All patients with MELAS showed focal hypoperfusion in the parietal and/or occipital lobes. Follow-up studies (3 MELAS patients) revealed extension or improvement in the abnormal perfusion. The hypoperfused lesions were correlated with abnormal CT/MRI findings. Perfusion was normal in 1 MERRF, 2 KSS and 3 CCOD patients, whereas CT/MRI findings in 1 MERRF, 1 KSS and 1 CCOD patient were abnormal. The cerebral perfusion reserve in 3 MELAS patients was decreased significantly compared with that in patients with other types of MEM (MELAS 7.4%, other MEM 33.8%; p0.05).The rCBF was altered specifically in patients with MELAS, suggesting that brain perfusion SPECT will be useful in diagnosing and assessing such patients. The decreased cerebral perfusion reserve in patients with MELAS may represent an important feature of the pathogenesis of the strokelike episodes. The SPECT findings of patients with other types of MEM (MERRF, KSS and CCOD) were normal.

The mechanism whereby watershed (WS) infarcts develop remains controversial, although a hemodynamic cause is usually assumed. The aim of this study was to investigate the relationship between the site of WS infarcts and the hemodynamic status of the cerebral circulation.From among 96 consecutive patients with angiographically confirmed unilateral major cerebral artery obstruction (occlusion or70% stenosis), we investigated 29 patients with supratentorial WS infarcts on magnetic resonance imaging. The regional cerebral blood flow and perfusion reserve were quantified using the split-dose [123I]iodoamphetamine SPECT method, coupled with intravenous injection of 1 g of acetazolamide. Seven patients had a cortical WS infarct between the superficial branches of the anterior and middle cerebral arteries (MCAs) or between the middle and posterior cerebral arteries (Group C), and 22 had a deep WS infarct between the superficial branches and deep penetrating arteries of the MCA (Group D). Moreover, the patients in Group D were classified into two subgroups, i.e., Type A (n = 12), with lesions lying in the centrum semiovale above the level of the lateral ventricles, and Type B (n = 10), with lesions lying in the corona radiata adjacent to the lateral ventricles.Comparison of the Type of WS infarct with the clinical course of onset showed that sudden onset was more frequent in Group C than in Group D (p0.05). The perfusion reserve in the affected MCA territory in Group D (20.1% +/- 15.6%) was significantly lower than that in Group C (43.8% +/- 10.8%; p0.01) and that in 20 hemispheres (10 control subjects) without a major arterial lesion (54.7% +/- 16.4%; p0.01). Among the Group D patients, the patients with Type A infarcts showed a significantly lower perfusion reserve compared with those with Type B infarcts (p0.05).Patients with deep WS infarcts, especially Type A infarcts, showed severe hemodynamic impairment, whereas patients with cortical WS infarcts showed preserved perfusion reserve which appeared to be secondary to the embolism. The mechanism of development of WS infarcts is multifactorial, and distinguishing among these WS infarcts and from other types of infarct is important, because different pathogenic mechanisms require different therapeutic strategies.

Regional distributions of 99mTc-hexamethyl propyleneamine oxime (99mTc-HMPAO) and 99mTc-ethyl cysteinate dimer (99mTc-ECD) were compared in the normal brain.Six paid, healthy volunteers (mean age 26 yr) had high-resolution neuroperfusion SPECT using both 99mTc-HMPAO and 99mTc-ECD on separate days.Regional distribution of the two tracers differed. Technetium-99m-HMPAO accumulated more in the thalamus, frontal lobe, temporal lobe and cerebellum than 99mTc-ECD, which accumulated more in the occipital and parietal lobes. There was a considerable difference in the accumulation of the two tracers in the medial temporal lobe. The percent accumulations of 99mTc-HMPAO and 99mTc-ECD in the medial temporal lobe compared with the mean global cerebral cortical accumulation were 93.9% +/- 2.4% and 83.1% +/- 4.1% (mean +/- s.d.), respectively.The results suggest that 99mTc-HMPAO and 99mTc-ECD require specific and separate criteria for diagnosing temporal lobe pathologies, such as dementia and temporal lobe epilepsy.

Clinical evaluation of 123I-iomazenil, a new imaging agent for central-type benzodiazepine receptors with SPECT, was performed in patients with ischemic cerebrovascular disease. We investigated 15 patients with angiographically-proven severe occlusive lesions (occlusion or70% stenosis) in the unilateral carotid system. 123I-iomazenil SPECT images were compared with cerebral blood flow (CBF) images and the cerebral perfusion reserve, which were measured using the "split dose 123I-IMP SPECT method" before and after the intravenous injection of 1 g of acetazolamide. For the detection of ischemic lesions, CBF images were superior to 123I-iomazenil images based on visual analysis. Regarding the count ratio of the affected MCA territory to the non-affected (L/N), 123I-IMP was lower than 123I-iomazenil in most of the cases. In five patients showing "crossed cerebellar diaschisis" by 123I-IMP, asymmetry of the cerebellar accumulation was observed in only one patient with 123I-iomazenil, which was less prominent than with 123I-IMP. There was no significant correlation between the L/N ratio with 123I-iomazenil and the cerebral perfusion reserve in the affected MCA territory. However, in some cases showing a decreased L/N ratio (90%) with 123I-iomazenil, a decreased CBF with normal perfusion reserve and cerebral hemi-atrophy were observed with 123I-IMP and MRI, which suggested the influence of neuronal loss due to chronic ischemia. These results indicate that 123I-iomazenil SPECT, which provides new information regarding neuronal loss due to ischemic damage to the brain, is useful for the evaluation of ischemic cerebrovascular disease.

To better understand cortical perfusion, we developed a method for a three-dimensional display technique with 99mTc-hexamethylpropyleneamine oxime (HMPAO) SPECT.Twelve patients with higher cortical dysfunction due to Alzheimer's disease and 18 age-matched controls were examined. Data acquisition was performed after intravenous injection of 740 MBq of 99mTc-HMPAO. After reconstructing the transaxial images, the three-dimensional images were obtained by modified volume rendering, where the surfaces were displayed in the corresponding colors as the maximum cortical value within a depth of 2 cm.In the control studies, almost all surface cortices were over 60% of the maximum cerebellar value. In Alzheimer's disease patients, areas of perfusion below 60% were detected in the temporo-parietal lesions and frontal lobe lesions in 6 of 12. These findings correlated with the neurological dysfunction.This method provides realistic three-dimensional information about surface cortical perfusion, which was found to be useful in clinical investigations of higher cortical dysfunction due to degenerative or cerebrovascular diseases.

In the management of major cerebral artery obstruction, cerebral perfusion reserve is key to introducing cerebral revascularization surgery. The purpose of this study was to evaluate the feasibility of assessing cerebral perfusion reserve by consecutive 99mTc-hexamethyl-propyleneamine oxime (99mTc-HMPAO) SPECT with 5% carbon dioxide (CO2) inhalation.The CO2 inhalation and consecutive 99mTc-HMPAO SPECT study was performed on 30 chronic ischemic cerebrovascular disease patients with unilateral major cerebral artery obstruction and on 27 patients without. CO2 reactivity was expressed as the percent increase of 99mTc-HMPAO accumulation from the baseline (%Change) and as a constant k' that was the ratio of 99mTc-HMPAO accumulation per 1 mmHg change of end-tidal CO2 tension by exponential curve fitting.The mean %Change and k' in the middle cerebral artery (MCA) territory on the side without an obstructive lesion or in the cerebellum ranged from 10.0% to 11.1% and from 0.98% to 1.13% per mmHg, respectively. In the MCA territory, an obstructive lesion was noted in 5.9% versus 0.54% per mmHg in the contralateral MCA territory (p0.01). Eleven of 30 patients with major cerebral artery obstruction revealed significant asymmetry in the k' value between bilateral MCA territories.The results showed compromised cerebral perfusion reserve in the obstructed major cerebral artery territory. The present method was proven clinically useful for evaluating cerebral perfusion reserve in patients with unilateral major cerebral artery obstruction.