Staged Fontan procedure for mitral atresia associated with severe tricuspid regurgitation, pulmonary hypertension, and pulmonary artery distortion

Optimal initial palliation and a subsequent staged approach is mandatory for high-risk Fontan candidates. We describe the case of mitral atresia with severe tricuspid regurgitation and pulmonary hypertension successfully managed by repeated palliation from the neonatal period and 2-stage Fontan surgery. A 1-month-old boy diagnosed with mitral atresia and double-outlet right ventricle underwent pulmonary artery banding at 1 month of age, followed by repeated pulmonary artery banding accompanied by tricuspid annuloplasty and atrial septal defect enlargement at 6 months. Because of the presence of pulmonary artery distortion, right ventricular dysfunction, and borderline pulmonary vascular resistance, a hemi-Fontan procedure was conducted with extended pulmonary artery plasty when the boy was 3 years and 8 months old. Cardiac catheterization done 3 months after showed improvement in risk factors, and the final Fontan operation (total cavopulmonary connection) was successfully done in conjunction with repeated tricuspid annuloplasty when the boy was 4 years and 5 months old. The patient remains in excellent clinical condition at the last follow-up 5 years after the final Fontan procedure with sinus rhythm and good ventricular function.     

Cerebral type of Lewy body disease

A cerebral type of Lewy body disease (LBD) is proposed. Lewy body disease was split formerly into three types: brainstem type, transitional type and diffuse type. The diffuse type is now called diffuse Lewy body disease (DLBD). These three types are characterized pathologically by the presence of a large number of Lewy bodies in the CNS. In the brainstem type, Lewy bodies are numerous in the brainstem and diencephalon nuclei, and in DLBD, a vast number are present not only in these nuclei but also in the cerebral cortex and amygdala. In the cerebral type of LBD, as many Lewy bodies are found in the cerebral cortex and in the amygdala as there are in DLBD, but only rarely are they present in the brainstem and diencephalon nuclei. Thus, this type of LBD is different from other types in that it has no parkinson pathology. Therefore, parkinsonism fails to occur throughout the whole clinical course of this disease. The existence of a cerebral type of LBD suggests that Lewy bodies occur in the cerebral cortex earlier than in the brainstem nuclei and that cortical Lewy bodies appear even when the mesocortical dopaminergic system is intact. In addition, this might explain why dementia frequently precedes parkinsonism in DLBD.     

A human friendly reporting and database system for brain PET analysis

We have developed a human friendly reporting and database system for clinical brain PET (Positron Emission Tomography) scans, which enables statistical data analysis on qualitative information obtained from image interpretation. Our system consists of a Brain PET Data (Input) Tool and Report Writing Tool. In the Brain PET Data Tool, findings and interpretations are input by selecting menu icons in a window panel instead of writing a free text. This method of input enables on-line data entry into and update of the database by means of pre-defined consistent words, which facilitates statistical data analysis. The Report Writing Tool generates a one page report of natural English sentences semi-automatically by using the above input information and the patient information obtained from our PET center’s main database. It also has a keyword selection function from the report text so that we can save a set of keywords on the database for further analysis. By means of this system, we can store the data related to patient information and visual interpretation of the PET examination while writing clinical reports in daily work. The database files in our system can be accessed by means of commercially available databases. We have used the 4th Dimension database that runs on a Macintosh computer and analyzed 95 cases of^I8F-FDG brain PET studies. The results showed high specificity of parietal hypometabolism for Alzheimer’s patients.     

Effects of sevoflurane on autonomic nerve activities controlling cardiovascular functions in rats

Effects of different inspiratory concentrations of sevoflurane (fluorometyl-1,1,1,3,3,3,-hexafluoro-2-propylether) on blood pressure, heart rate and efferent activities of cardiac sympathetic, cardiac parasympathetic and renal sympathetic nerves were examined using rats either under the resting condition or during noxious mechanical stimulation of a hindpaw. Under the resting condition, an increase in the inspiratory concentration of sevoflurane from 2.1% to 4.2% gradually caused a decrease in blood pressure and heart rate. With the increase in the sevoflurane concentration, cardiac sympathetic nerve activity decreased, whereas renal sympathetic nerve and cardiac parasympathetic nerve activities did not change significantly. When noxious mechanical stimulation was applied to a hind-paw by pinching, blood pressure and heart rate, renal sympathetic and cardiac sympathetic nerve activities all increased at the 2.1% concentration of sevoflurane. The responses of these parameters were attenuated at the 3.1% concentration of sevoflurane and almost disappeared at the 4.2% concentration. Cardiac parasympathetic nerve activity did not change significantly during the pinching stimulation throughout the 2.1–4.2% concentration increase.(Kurosawa M, Meguro K, Nagayama T et al.: Effects of sevoflurane on autonomic nerve activities controlling cardiovascular functions in rats. J Anesth 3: 109–117, 1989)     

Alleviation of gallbladder complications by treatment of hepatic arterial embolization with caerulein

Transcatheter arterial embolization (TAE) with the concurrent use of caerulein was assessed for the purpose of preventing gallbladder complications often seen after TAE of hepatic carcinoma. Ninety-six cases with primary hepatic carcinoma, who had undergone TAE in the right hepatic arterial region over the past 4 years, were divided into three groups: 22 cases for which embolization was possible on a selective basis by passing the catheter to the peripheral side beyond the bifurcated region of the cystic artery; 40 cases who had undergone TAE in which caerulein was not administered, from the central side of the bifurcated region of the cystic artery; and 34 cases given 20 g caerulein 15–30 min before TAE. A comparison was made using the abdominal pain, pyrexia, rate of leukocytosis and the US findings of the gallbladder as the indices of the gallbladder complications. As a result, it become evident that it was possible to prevent or alleviate gallbladder complications if caerulein were administered before TAE in cases where the embolizing substances were infused in the right hepatic artery from the central side of the bifurcated region of the cystic artery. It was conclusively shown that the gallbladder blood flow decreases if the organ is contracted by caerulein, which in turn causes a decrease in the inflow of the embolizing substances whereby complications are alleviated.     

Neural correlates of cross-modal binding

Little is known about how the brain binds together signals from multiple sensory modalities to produce unified percepts of objects and events in the external world. Using event-related functional magnetic resonance imaging (fMRI) in humans, we measured transient brain responses to auditory/visual binding, as evidenced by a sound-induced change in visual motion perception. Identical auditory and visual stimuli were presented in all trials, but in some trials they were perceived to be bound together and in others they were perceived as unbound unimodal events. Cross-modal binding was associated with higher activity in multimodal areas, but lower activity in predominantly unimodal areas. This activation pattern suggests that a reciprocal and 'competitive' interaction between multimodal and unimodal areas underlies the perceptual interpretation of simultaneous signals from multiple sensory modalities.     

Parental death and bipolar disorder: a robust association was found in early maternal suicide

BACKGROUND: Previous studies have suggested that early parental death may be associated with the emergence of bipolar disorder in later life. However, it remains unknown whether this association applies specifically to parental death due to suicide or only to early parental death. The present study aimed to explore whether suicide as well as the non-suicidal death of father, mother, or siblings are associated with an increased risk for bipolar disorder, and whether the possible association is modified by the age at which the subject experiences such a death in the family. METHODS: The subjects were born in 1960 or later and were first admitted to or had first contact with Danish psychiatric facilities between 1981 and 1998 with a diagnosis of bipolar disorder, and fifty age-matched controls per case were extracted. The effects of the deaths of relatives were estimated by means of a conditional logistic regression analysis. RESULTS: Among 947 subjects with bipolar disorder and 47,350 controls, those having experienced the parental suicide were significantly associated with an increased risk for BPD (incidence rate ratios: 1.83 [95% confidence interval: 1.07 to 3.12] for paternal suicide, 3.44 [1.97 to 6.00] for maternal suicide), whereas the non-suicidal death of parents showed no such association. Those having experienced maternal suicide at some point before reaching 10 years of age were seven times as likely to develop bipolar disorder. LIMITATIONS: The cohort members were followed until, but not exceeding, the age of 38. CONCLUSION: Early parental, particularly maternal, suicide increases the risk for bipolar disorder in the offspring. Possible explanations include a family history of mental disorders as well as psychosocial factors.     

Association of the insertion/deletion polymorphism of the angiotensin I-converting enzyme gene in patients of migraine with aura

Recently, several angiotensin I-converting enzyme (ACE) inhibitors and an angiotensin II receptor blocker were demonstrated to have a clinically important prophylactic effect in migraine. ACE is one of the key enzymes in the rennin-angiotensin-aldosterone system, which modulates vascular tension and blood pressure. In humans, serum ACE levels are strongly genetically determined. Individuals who were homozygous for the deletion (D) allele showed increased ACE activity levels. To investigate the role of ACE polymorphism in headache, we analyzed the ACE insertion (I)/deletion (D) genotypes of 54 patients suffering from migraine with aura (MwA), 122 from migraine without aura, 78 from tension-type headache (TH), and 248 non-headache healthy controls. The ACE D allele were significantly more frequent in the MwA than controls (p<0.01). The incidence of the D/D genotype in MwA (25.9%) was significantly higher than that in controls (12.5%; p<0.01; odds ratio=5.26, 95% confidence interval: 1.69-16.34, adjusted for age and gender). No differences in the remaining groups were found. Our results support the conclusion that the D allele and the D/D genotype in the ACE gene is a genetic risk factor for Japanese MwA. There seems to be a possible relationship between ACE activity and the pathogenesis of migraine.