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A patient with borderline left ventricle successfully underwent biventricular repair following a staged surgical approach to promote left ventricular growth. Despite initial concerns about left ventricle size, apex formation and adequate size of atrioventricular valve indicated potential for future growth. The patient demonstrated significant left ventricular growth, resulting in stable biventricular circulation and a favourable outcome over a three-year postoperative follow-up period.Clinical Registration Number: Institutional Review Board of Osaka City General Hospital, number 1902139.

Introduction: Coronary artery anomalies are rare congenital variations of the arteries that supply the heart. Examples of these anomalies include the development of a single coronary artery (SCA) to supply the entire myocardium, two left anterior descending arteries (LAD) occupying the anterior interventricular sulcus, and three posterior descending arteries (PDA) occupying the posterior interventricular sulcus. Reports of rare, simultaneously occurring coronary artery anomalies serve as valuable references for medical education and clinical practice but are scarce in the literature. The present study describes a human cadaveric heart where a SCA gives rise to distinct dual LADs and three PDAs. The study aims to characterize the anomalies and investigate the anatomy, histology, and clinical considerations of this unique coronary anatomy. Methods: The SCA, dual LAD, and triple PDA were discovered during routine cadaveric dissection in a medical education curriculum. The heart was eviscerated, and the anomalies were cleaned of extraneous tissue and photographed. Color replacement was performed on the images to aid in structure identification. The SCA was characterized using Lipton's classifications, and the dual LAD was classified according to the thirteen variations (“Types”) of dual LAD described in the literature. Length, luminal area, and plaque buildup were recorded for each of the relevant arteries. A constriction on the great cardiac vein and an unknown tissue mass associated with the left main coronary artery (LMCA) were examined via histological staining and light microscopy. Courses of the coronary artery anomalies were superimposed on volume rendered computerized tomographic angiography images depicting typical coronary anatomy for comparison. Results: The SCA arose from a solitary coronary ostium in the right coronary sinus and traveled 0.57 cm from the aortic trunk before branching the right coronary artery (RCA), long LAD, LMCA, and descending septal artery. The RCA maintained typical anatomic course before terminating as a right-dominant triple PDA. The long LAD coursed pre-pulmonic along the anterior interventricular sulcus for 13.89 cm before terminating near the apex of the heart. The LMCA tracked retro-aortic, between the aorta and the atrial wall, for 5.67 cm before bifurcating into the left circumflex artery (LCX) and 10.8 cm short LAD. Multiple anastomoses were noted involving the long LAD, short LAD, second PDA, third PDA, diagonal branches of the short LAD, and left marginal artery. The constriction on the great cardiac vein, caused by an overlaying left diagonal artery, decreased the venous wall thickness and altered its tissue composition. The unknown tissue adhered to LMCA appeared mucinous in origin. Conclusions: The heart presented in this study was supplied by a unique (Lipton's) RIII-C SCA with Type IV dual LAD and right-dominant triple PDA. Unexpected complications can arise during cardiac procedures for patients with coexisting coronary artery anomalies such as these. This report may serve as an important reference for cardiothoracic surgeons, interventional cardiologists, coronary angiographers, and medical educators when presented with a similar case and determining therapeutic design.

When participants respond to a task-relevant stimulus attribute by pressing a left or right key with the respective index finger, reaction time is shorter if task-irrelevant left-right stimulus location corresponds to that of the response key than if it does not. For right-handers, this Simon effect is larger for right-located than left-located stimuli; for left-handers this Simon-effect asymmetry is reversed. A similar asymmetry has been found for right-footers pressing pedals with their feet. For analyses that separate stimulus- and response-location factors, these asymmetries appear as a main effect of response location, with responses being faster with the dominant effector. If the Simon-effect asymmetry is strictly a function of effector dominance, it should reverse for left-footers responding with their feet. In Experiment 1, left-dominant persons showed faster responses with the left than right hand but with the right than left foot, a finding consistent with prior research on tapping actions. Right-dominant persons also showed the right-foot asymmetry but, unexpectedly, not the typical asymmetry with hand responses. To evaluate whether hand-presses yield results distinct from finger-presses, in Experiment 2 participants performed the Simon task with finger-presses and hand-presses. The opposing asymmetries for right- and left-dominant persons were evident for both response modes. Our results are consistent with the view that the Simon effect asymmetry is primarily due to differences in effector efficiency, usually but not always favoring the dominant effector.

Advanced endovascular technology and techniques allow interventional radiologists to utilize novel ways of basilar artery recanalization in the setting of acute ischemic stroke, especially when routine approaches are not eligible. Several authors described nonstandard revascularization techniques in acute ischemic strokes due to basilar and middle cerebral arteries occlusions with full technical and clinical success. In this report, we present retrograde right vertebral artery recanalization using left posterior communicating artery for subsequent anterograde balloon angioplasty and stenting of a right vertebral artery ostium followed by full vertebrobasilar blood flow restoration. The case underscores the complexity of arterial thrombotic events, the beneficial role of endovascular intervention in vertebral occlusions and the necessity of prospective studies that identify optimal methods of treating vertebrobasilar stroke due to large vessel occlusions and their effectiveness and safety. Keywords: Ischemic, Stroke, Vertebral, Basilar, Occlusion, Recanalization

Unbalance in atrioventricular septal defect can be found in more than one anatomic level and in different degrees at each level. The definition of “unbalance” has historically been focused in comparing the dimensions of main cardiac structures, such as the atrioventricular valve and the ventricles. However, the hemodynamic aspects of unbalance need to be considered as having, at least, similar relevance. New concepts and already described parameters must be combined and understood as a whole to help the surgical decision-making process.

Arrhythmogenic cardiomyopathy with right dominant form (ACR) is a rare heritable cardiac cardiomyopathy disorder associated with sudden cardiac death. Pathogenic variants (PVs) in desmosomal genes have been causally related to ACR in 40% of cases. Other genes encoding nondesmosomal proteins have been described in ACR, but their contribution in this pathology is still debated. A panel of 71 genes associated with inherited cardiopathies was screened in an ACR population of 172 probands and 856 individuals from the general population. PVs and uncertain significance variants (VUS) have been identified in 36% and 18.6% of patients, respectively. Among the cardiopathy-associated genes, burden tests show a significant enrichment in PV and VUS only for desmosomal genes PKP2 (plakophilin-2), DSP (desmoplakin), DSC2 (desmocollin-2), and DSG2 (desmoglein-2). Importantly, VUS may account for 15% of ACR cases and should then be considered for molecular diagnosis. Among the other genes, no evidence of enrichment was detected, suggesting an extreme caution in the interpretation of these genetic variations without associated functional or segregation data. Genotype-phenotype correlation points to (1) a more severe and earlier onset of the disease in PV and VUS carriers, underlying the importance to carry out presymptomatic diagnosis in relatives and (2) to a more prevalent left ventricular dysfunction in DSP variant carriers., (© 2022 The Authors. Human Mutation published by Wiley Periodicals LLC.)

Background: Right dominant heart (RDH) in fetuses can occur with a number of cardiac as well as noncardiac anomalies. Analysis of the enlargement of the right cardiac chamber in the fetus remains a major challenge for sonographers and echocardiographers. The aim of this study was to report the experience with prenatal diagnosis of RDH in the fetuses over a 7-year period. Methods: Fetuses with prenatal diagnosis of RDH from July 2009 to July 2016 were evaluated in two different categories: according to the gestational age, Group I (n = 154, second trimester) and Group II (n = 298, third trimester); and according to the fetal echocardiography diagnosis, Group A (n = 452, abnormal cardiac structure) and Group B (n = 90, normal cardiac structure). Differences in categorical variables were assessed by Chi-square exact test and continuous variables were evaluated by independent Student's t-test or Mann–Whitney U-test depending on parametric or nonparametric nature of the data. Results: Over a 7-year period, 452 fetuses were referred for the assessment of suspected RDH. Left-sided obstructive lesions were observed most frequently in the fetuses with RDH. When comparing Group I with Group II and Group A with Group B, the latter groups exhibited significant differences in the right/left ventricle (RV/LV) ratio (1.435 vs. 1.236, P = 0.002; 1.309 vs. 1.168, P = 0.047), RV width Z-score (1.626 vs. 1.104, P < 0.001; 1.553 vs. 0.814, P = 0.014), and above +2 cutoff percentages (14.3% vs. 22.5%; P = 0.038; 21.5% vs. 12.2%, P = 0.046). Multivariable logistic regression revealed no variables associated with perinatal survival. Conclusions: The study demonstrates that RDH warrants careful attention to the possible presence of a structural cardiac anomaly, especially left-sided obstructive lesions. A diagnosis of RDH is best supported by a combination of the RV Z-score and RV/LV ratio. Most of the fetuses with RDH and structurally normal hearts had favorable outcomes.

Background: Primary progressive aphasia (PPA) is a degenerative disease that presents as progressive decline of language ability with preservation of other cognitive functions in the early stages. Three subtypes of PPA are known: progressive nonfluent aphasia, semantic dementia, and logopenic aphasia (LPA). Patients and Methods: We report the case of a 77-year-old patient with PPA whose clinical findings did not correspond to the three subtypes but mainly fit LPA. Unlike other LPA patients, however, this patient showed a right hemisphere predominant glucose hypometabolism and tau accumulation and a left hemisphere predominant amyloid deposition. The right-handed patient presented with comprehension difficulty followed by problems naming familiar objects. This isolated language problem had deteriorated rapidly for 2 years, followed by memory difficulties and impairment of daily activities. Using a Korean version of the Western Aphasia Battery, aphasia was consistent with a severe form of Wernicke's aphasia. According to the brain magnetic resonance imaging and 18F-fludeoxyglucose positron emission tomography results, right hemisphere atrophy and hypometabolism, more predominant on the right hemisphere than the left, were apparent despite the fact that Edinburgh Handedness Questionnaire scores indicated strong right-handedness. On Pittsburgh compound B-PET, amyloid accumulation was asymmetrical with the left hemisphere being more predominant than the right, whereas 18F-T807-PET showed a right dominant tau accumulation. Conclusions: This is the first report of atypical PPA, in which the patient exhibited crossed aphasia and asymmetrical amyloid accumulation.

The central nervous system (CNS) develops motor strategies that minimize various hidden criteria, such as end-point variance or effort. A large body of literature suggests that the dominant (D) arm is specialized for such open-loop optimization-like processes, whereas the nondominant (ND) arm is specialized for closed-loop postural control. Building on recent results suggesting that the brain plans arm movements that take advantage of gravity effects to minimize muscle effort, the present study tests the hypothesized superiority of the dominant arm motor system for effort minimization. Thirty participants (22.5 ± 2.1 yr old; all right-handed) performed vertical arm movements between two targets (40° amplitude), in two directions (upward and downward) with their two arms (dominant and nondominant). We recorded the arm kinematics and electromyographic activities of the anterior and posterior deltoid to compare two motor signatures of the gravity-related optimization process; i.e., directional asymmetries and negative epochs on phasic muscular activity. We found that these motor signatures were still present during movements performed with the nondominant arm, indicating that the effort-minimization process also occurs for the nondominant motor system. However, these markers were reduced compared with movements performed with the dominant arm. This difference was especially prominent during downward movements, where the optimization of gravity effects occurs early in the movement. Assuming that the dominant arm is optimal to minimize muscle effort, as demonstrated by previous studies, the present results support the hypothesized superiority of the dominant arm motor system for effort minimization. NEW & NOTEWORTHY The hypothesis of hemispheric specialization for feedforward and feedback control has been developed and is still debated. Here, taking advantage of recent knowledge on optimal planning and control of arm movements in the gravity field, we recorded kinematics and electromyographic activities during vertical arm movements performed with the dominant and nondominant arms. Our results reveal that the nondominant arm takes advantage of gravity effects to minimize muscle effort, but less than the dominant arm.

Acute myocardial infarction is a condition that classically presents with chest pain and corresponding biomarkers and changes on electrocardiogram. Although most causes of acute coronary syndrome are due to acute plaque rupture resulting in coronary thrombosis, an increasingly prevalent condition known as spontaneous coronary artery dissection (SCAD) is becoming more commonly diagnosed. SCAD is characterized by a tear in the tunica media resulting in an intramural hematoma. Depending on the size of the hematoma, progressive extension can ultimately lead to coronary occlusion. Our team presents a 52-year-old female patient that presented with substernal chest pain and positive cardiac enzymes. Urgent coronary catheterization revealed bilateral SCAD involving the left anterior descending and posterior descending arteries in a right coronary dominant circuit. Our patient was treated with medical therapy alone and was safely discharged to home after close monitoring in the coronary care unit. Our team hopes to contribute to a growing body of evidence that bilateral SCAD can occur and can be successfully treated without percutaneous coronary intervention., Competing Interests: None to declare., (Copyright 2021, Mararenko et al.)

Introduction: Semantic dementia (SD) is characterized by fluent speech, anomia, and loss of word and object knowledge with varying degrees of right and left anterior-medial temporal lobe hypometabolism on [18F] fluorodeoxyglucose (FDG)-PET. We assessed neurobehavioral features in SD patients across 3 FDG-PET-defined metabolic patterns and investigated progression over time. Methods: Thirty-four patients with SD who completed FDG-PET were classified into a left- and right-dominant group based on the degree of hypometabolism in each temporal lobe. The left-dominant group was further subdivided depending on whether hypometabolism in the right temporal lobe was more or less than 2 standard deviations from controls (left+ group). Neurobehavioral characteristics determined using the Neuropsychiatric Inventory Questionnaire (NPI-Q) were compared across groups. Progression of NPI-Q scores and FDG-PET hypometabolism was assessed in 14 patients with longitudinal follow-up. Results: The right-dominant group performed worse on the NPI-Q and had a greater frequency of abnormal behaviors and more severe disinhibition compared to the left-dominant group. Performance on the NPI-Q and severity of disinhibition correlated with right medial and lateral, but not left, temporal lobe hypometabolism. Severity of abnormal behaviors worsened over time in most left-dominant and left+ patients but appeared to improve in the 2 right-dominant patients with longitudinal follow-up. All groups showed progressive worsening of metabolism in both temporal lobes over time, with hypometabolism spreading from anteromedial to posterior temporal regions. However, the degree of temporal lobe asymmetry remained relatively constant over time. Conclusion: In SD, neurobehavioral features, especially disinhibition, are associated with right medial and lateral temporal lobe hypometabolism and commonly develop over time even in patients that present with left-dominant patterns of hypometabolism. [ABSTRACT FROM AUTHOR]