Search

Background: In the recent case of R v. Taj, the Court of Appeal of England and Wales upheld the conviction of a defendant who, in a psychotic delusional state, mistook his non‐threatening victim to be a terrorist, violently attacking him. The law typically allows honest mistakes (even if unreasonable) as a basis for self‐defence (in this case the defence of others). However, because Taj's delusions were found by the court to have been caused by voluntary alcohol consumption, special legal (prior‐fault) intoxication rules were applied to block his defence; Taj was convicted and sentenced to 19 years imprisonment for attempted murder. Argument We focus here on the simple question—what does it mean to be intoxicated? On the facts, Taj did not have drugs active in his system at the time of the attack, but the court nonetheless insisted that Taj's delusional mistake was 'attributable to intoxication'; namely, to drink‐ and drug‐taking in the previous days and weeks. This extended conception of intoxication was questionably distinguished from psychosis induced by withdrawal. Furthermore, the court was unreceptive to evidence of a long‐standing, underlying mental health disorder. We argue that the court's expanded view of intoxication is problematic, in that intoxication‐induced psychosis cannot be sharply distinguished from other causes such as mental disorders; and even if it could be distinguished, it should not give rise to blame and punishment in the same way as does conduct induced by chemically active intoxicants ('drug‐on‐board'). Conclusion: The courts' expansion of the definition of intoxication is both legally and forensically problematic, introducing legal vagaries where the clinical science is already vague; and with intoxication frequently interlocking with historic intoxication and secondary or comorbid mental health conditions, the decision risks inappropriately and/or over‐criminalizing defendants. [ABSTRACT FROM AUTHOR]

The authors briefly respond to various commentaries about an article they published in the journal regarding how courts administer justice in cases involving criminal defendants who were intoxicated during the commission of their crimes, particularly focusing upon the British case R v Taj. The article discusses legal concepts such as mens rea and self-defence.

The principal aim of this article is to introduce a new criminal law reform initiative: The Criminal Law Reform Now Network (CLRN Network). The article begins in Part 1 by setting the scene for law reform in this jurisdiction, exposing and discussing four major challenges that await any would-be reformer or network: 1) The Political Red Line, 2) The Political Preference for Simple Headlines, 3) The Political Indifference to Principles of Criminalisation, and 4) The Division Between Academics and Practitioners.From here, in Part 2, we introduce the ambitions and processes envisaged for the new CLRN Network. Launched in 2017, the mission of the CLRN Network is to facilitate collaboration between academics and other legal experts to gather and disseminate comprehensible proposals for criminal law reform to the wider community. The aim is to include members of the public and mainstream media as well as legal professionals, police, policymakers and politicians. Proposals from the CLRN Network might require legislation, but will not be restricted to such projects. Reforms which public bodies such as the Home Office, Police or CPS could bring about by internal policies may be included, as well as reforms which require the support of some of the judiciary, bearing in mind the proper judicial constraints on law making. The CLRN Network will be ready to consult with and make suggestions to anyone who has the power to bring about reform. [ABSTRACT FROM AUTHOR]

Left ventricular outflow obstructions ( LVOTO) encompass a series of stenotic lesions beginning within the anatomic left ventricular outflow tract and ending in the descending thoracic aorta, occurring alone or in series. The degree of stenosis and hemodynamic consequences may vary from mild and well tolerated to severe and functionally limiting. The majority of LVOTO (with the exception of calcific and rheumatic aortic stenosis) are congenital in etiology. Echocardiography has become an integral tool in the assessment of patients with congenital heart disease. This article is a contemporary review of the clinical features of LVOTO and the role of echocardiography in this patient population. [ABSTRACT FROM AUTHOR]

Social capital may be defined as social relationships that confer actual or potential benefits. It can therefore be understood as a particular type of resource. Recent research has drawn attention to how connections and relationships (networking) both at home and abroad can be crucially important for small and medium-sized enterprises (SMEs) seeking to export or invest abroad. However, relatively little is known about how SMEs initiate, develop and maintain network relationships. This paper reports a study of 32 British SMEs exporting, or attempting to export, to Brazil and of domestic institutional agencies whose role was to facilitate business conducted between British and Brazilian SMEs. The study explored both the functions of social capital for the SMEs and the process whereby it was developed. Its findings confirm the value of social capital in international entrepreneurship. It can provide information, interpretation, market opportunities, and some degree of protection against the risks associated with foreignness, newness and smallness. The study also confirms the vital importance of personal trust in sustaining social capital between small firms. [ABSTRACT FROM AUTHOR]

Transcatheter closure for atrial septal defect (ASD) and patent foramen ovale (PFO) is a promising alternative to surgical closure or anticoagulant therapy. A potential complication is thrombus formation on the device after implantation. From February 2001 to June 2003, 66 patients with atrial communication were treated successfully with the Amplatzer device (16 septal and 20 PFO occluders) or the CardioSEAL device (30). Patients were discharged on antiplatelet medication (aspirin and clopidogrel) and/or anticoagulation. Fifty patients (76%) had transesophageal echocardiography (TEE) 1 month after device implantation (28 ± 10 days). No patient experienced a thromboembolic episode during follow-up. TEE revealed that thrombus formation occurred more frequently on the CardioSEAL device (5 of 23 patients; 22%) than on the Amplatzer device (0 of 27 patients; 0%) (p = 0.02). Although thrombus disappeared or markedly diminished after additional anticoagulation therapy in 3 patients, 1 patient had surgical explantation of the device due to progressive increase in the size of thrombus with hypermobility despite intensive anticoagulation therapy. There was no variable associated with the presence of thrombus formation on the occluder other than the use of the CardioSEAL device. One month after insertion, the CardioSEAL device is more likely to have thrombus present than the Amplatzer device. [Copyright &y& Elsevier]

Focuses on a study which examined differences in the resource profile and experience of Sino-foreign joint ventures in the non-consumer electronics and fast moving consumer goods sector in China. Concept and profile of value chain activities in the country; Methods and results of the study; Implications for joint ventures.

There tends to be more extensive occupational specialization outside the core production area in British manufacturing companies than in German ones. This appears to be associated with the emphasis in Britain on professionalism in industry, a concept which is largely absent in Germany. The British approach is seen to have unfavourable consequences for the status of production and of manufacturing industry in general, with quite likely depressing effects on its performance. The origins of the contrast between Britain and Germany are explored by reference to the development of post-school vocational education in the two countries, and this leads to the conclusion that strategic choices about contemporary work organization were in effect made at a societal level a century ago. The analysis indicates that industrial policy cannot be confined to changes within the industrial sector atone. [ABSTRACT FROM AUTHOR]

This paper examines the class position of 156 Birmingham first-line supervisors, and how the supervisors perceive that position. ft concludes that they occupy a class position which is only marginally superior to that of manual workers. In their perceptions, however, they range from ascribing a middle class position for themselves to a working class one. These perceptions are assessed in terms of six indicators. The less that supervisors see their role and standing in the firm falling short of what they believe to be appropriate, and the closer they identify themselves with senior management, the higher tends to be the class position they see themselves occupying. It would be an oversimplification, the paper suggests, to conclude that the supervisors studied belong in a straightforward manner either to a proletarianized middle class or to a working class group which has adopted middle class characteristics. [ABSTRACT FROM AUTHOR]

This paper develops modes of analysis for three major issues in the study of trade unions as organizations. These are, first, their distinctiveness as a discrete type of organization, secondly, the nature of their membership attachment; and thirdly, their twin rationales of representation and administration. The integration of these analyses within a new framework is then pursued. This framework serves to suggest propositions requiring empirical investigation and reference is made to some results from a preliminary study. [ABSTRACT FROM AUTHOR]

A study of 82 business organizations provides data on the disputed question of whether organization technology or organization size is more closely associated with the elements of organization structure. Technological variables are found to be associated with structure in certain definable respects, but, on the whole, size enjoys a much higher relationship. Partial correlational analysis serves to clarify these patterns of association. Measures of technology used in previous studies are seen to be problematic for two reasons. They can obscure the differential elements involved in technology. Secondly, they may to an important degree be acting as surrogates for inter-industry differences which include the influence of industrial managerial ideologies and environments. [ABSTRACT FROM AUTHOR]

This paper critically examines available theoretical models which have been derived front statistically established patterns of association between contextual and organizational variables. These models offer an interpretation of organizational structure as a product of organizational structure as a product of primarily economic constraints which contextual variable are impose. It is argued that available models in fact attempt to explain organization at one remove by ignoring the essentially political process, whereby power-holders within organizations decide upon courses of strategic action. This `strategic choice' typically includes not only the establishment of structural forms but also the manipulation of environmental features and the choice of relevant performance standards. A theoretical re-orientation this kind away from functional imperatives and towards' a recognition of political action is developed and illustrated in the man body of the paper. [ABSTRACT FROM AUTHOR]

Signal-averaged electrocardiography is a sensitive and specific technique for detecting the slow conduction electrophysiologic substrates of reentrant monomorphic ventricular tachycardia. Although well established, the method has not been used for electrophysiologic assessment after right ventriculotomy for the intracardiac repair of congenital heart disease. This 8-year prospective study provided this assessment. Recordings were obtained from 242 post-ventriculotomy patients with congenital heart disease aged 16 to 72 years (139 males, 103 females). Because ventricular volume or pressure overload can prolong the QRS duration, 40 unoperated patients with hemodynamic overload served as controls. Orthogonal X, Y, and Z body surface electrodes were used to detect ventricular late potentials by permitting the examination of portions of the electrocardiogram otherwise obscured by noise and artifacts. Positive signal-averaged electrocardiographic (SAECG) results were based on established criteria derived from 3 time-domain variables calculated by an automated algorithm. Late potentials were detected in 151 of 242 patients (62%) and were significantly higher than controls (p = 0.0001). Radiofrequency ablation of an inducible slow conduction substrate rendered 20 of 23 positive post-ventriculotomy SAECG results negative, and surgical revision of the ventriculotomy scar rendered 19 of 19 positive SAECG results negative. In conclusion, negative SAECG results connote the absence of a reentrant substrate, and therefore, the absence of risk for reentrant monomorphic ventricular tachycardia, whereas positive SAECG results connote the presence of a slow conduction substrate and the potential risk for monomorphic ventricular tachycardia. [Copyright &y& Elsevier]

Although a significant minority of patients with cyanotic congenital heart disease (CCHD) are thrombocytopenic, the pathogenesis and prevalence have not been established. This study was designed to address these 2 issues. We included 105 patients with CCHD (60 men and 45 women; aged 21 to 54 years). Systemic arterial oxygen saturations were 69% to 78%. Hematocrits were 62% to 74% with normal iron indexes. In 26 of 105 patients (25%), platelet counts were <100 × 109/L. The diagnosis was Eisenmenger syndrome in all 26 patients with thrombocytopenia. Platelet production was determined by flow cytometric reticulated platelet counts. Megakaryocyte mass was determined indirectly by thrombopoietin levels. Disseminated intravascular coagulation was based on prothrombin time, activated partial thromboplastin time, and d-dimers. Platelet activation was determined by levels of platelet factor 4 and β thromboglobulin. Reference ranges were derived from 20 normal acyanotic controls. A reduction in absolute reticulated platelet counts implied decreased platelet production (p <0.001). Normal thrombopoietin levels implied normal megakaryocyte mass. Normal prothrombin time, activated partial thromboplastin time, and d-dimers excluded disseminated intravascular coagulation. Normal platelet factor 4 and β thromboglobulin indicated absent or minimal platelet activation. Twenty-five percent of the patients with CCHD were thrombocytopenic because platelet production was decreased despite normal megakaryocyte mass. We hypothesized that right-to-left shunts deliver whole megakaryocytes into the system arterial circulation, bypassing the lungs where megakaryocytic cytoplasm is fragmented into platelets, thus reducing platelet production. In conclusion, platelet counts in CCHD appear to represent a continuum beginning with low normal counts and ending with thrombocytopenia. [Copyright &y& Elsevier]

This article presents a reply by sociologist John Child to the comments of Tyler about his article on organizational sociology, previously published in the journal "Sociology." One of the barriers to progress in sociology is the difficulty some of us have in appreciating that an argument for theoretical development does not necessarily imply a total rejection of previous work. Tyler's comments, published in the 1973 issue of the journal "Sociology," seem to reflect this difficulty. He has represented my argument on "Strategic Choice," published in the 1972 issue of the journal, as so extreme a critique of recent organization sociology as to be unrecognizable. Tyler states that the core of my critique appears to be the substantial irrelevance of objective constraints, of any kind, to the prediction of administrative behaviour. If this were correct, then I would indeed have been citing the caprice of dominant coalitions as the major independent variable in a theory of organizations. In contrast my concern was, to quote from the article that with the role of strategic choice as a necessary element in any adequate theory of organizational structure, and I have suggested that many available explanations over-emphasize constraints upon that choice.

This article presents information about the book "Sociology and Modern Systems Theory," by Walter Buckley. Buckley's purpose is to examine the modern systems perspective as the basis for a more adequate model of the sociocultural system than those prominent in current social science. Buckley joins with the many critics of models relying on concepts such as equilibrium, homeostasis, or functional imperatives, in arguing that these fail to deal satisfactorily with salient phenomena such as conflict, deviance, coercive power, and structural elaboration. Systems theory, which Buckley presents at some length, focuses upon interaction in terms of information transmission. As such its greatest potential is as a means to further understanding of social process. At the individual or small group level, where goals, action, information feedback, and corrective action can be fairly readily identified, the systems model goes further than others in current favour towards reconciling psychological and sociological behavioural considerations.

Hematologic management of adults with cyanotic congenital heart disease has received little recent attention. The lack of practical therapeutic guidelines prompted us to consolidate our observations on 124 cyanotic adults for general physicians, cardiologists, and hematologists who care for these patients. Specific attention focused on regulation of erythrocyte mass and concepts of compensated and decompensated erythrocytosis, symptoms of deficient tissue oxygen transport, hyperviscosity and iron deficiency, the potential relation between elevated hematocrit levels and brain injury, hemostasis, urate metabolism, and renal function. Cerebral infarction was not seen in any patient. Phlebotomy is best reserved for treatment of symptomatic hyperviscosity. Iron therapy is indicated for symptomatic iron deficient erythropoiesis. Abnormal hemostatic mechanisms are the rule. Antithrombotic medications have little or no role in treatment. Hyperuricemia is the result of abnormal renal uric acid excretion not urate overproduction, and serves as a marker of abnormal renal function. Drugs that promote urate excretion are the preferred maintenance treatment in symptomatic hyperuricemic patients. [ABSTRACT FROM AUTHOR]

The Y-balance test (YBT) is one of the few field expedient tests that have shown predictive validity for injury risk in an athletic population. However, analysis of the YBT in a heterogeneous population of active adults (e.g., military, specific occupations) involving multiple raters with limited experience in a mass screening setting is lacking. The primary purpose of this study was to determine interrater test-retest reliability of the YBT in a military setting using multiple raters. Sixty-four service members (53 males, 11 females) actively conducting military training volunteered to participate. Interrater test-retest reliability of the maximal reach had intraclass correlation coefficients (2,1) of 0.80 to 0.85 with a standard error of measurement ranging from 3.1 to 4.2 cm for the 3 reach directions (anterior, posteromedial, and posterolateral). Interrater test-retest reliability of the average reach of 3 trails had an intraclass correlation coefficients (2,3) range of 0.85 to 0.93 with an associated standard error of measurement ranging from 2.0 to 3.5cm. The YBT showed good interrater test-retest reliability with an acceptable level of measurement error among multiple raters screening active duty service members. In addition, 31.3% (n = 20 of 64) of participants exhibited an anterior reach asymmetry of >4cm, suggesting impaired balance symmetry and potentially increased risk for injury. [ABSTRACT FROM AUTHOR]

Circumferential dissection of the ascending aorta is a rare subtype of Type A aortic dissection which involves the entire intimal layer of the aorta which can cause a variety of devastating sequelae, given its propensity to involve the aortic valve and coronary arteries. Echocardiography is essential in making the diagnosis and visualize its involvement of the cardiovascular system, as invasive aortography can be limited in visualization of this unusual variant of aortic dissection. We present a patient who suffered a circumferential dissection of the ascending aorta which also caused severe aortic regurgitation and ischemia from left main coronary artery obstruction. [ABSTRACT FROM AUTHOR]

The aim of this study was to evaluate the prognostic value of brain natriuretic peptide (BNP) in outpatients with the Eisenmenger syndrome (ES). BNP is often elevated in patients with cyanotic congenital heart disease. The clinical utility of BNP in patients with cyanotic congenital heart disease and the ES has not been clearly delineated. Records of adults with ES who had undergone serum BNP measurement were reviewed. The primary end point was death or heart failure admission. Fifty-three patients were included, with 15 patients (28%) meeting the primary end point (death in 7, heart failure hospitalization in 8). Mean and median baseline BNP in patients meeting the primary end point were 322 ± 346 and 179 pg/ml, compared to 100 ± 157 and 41 pg/ml in those not meeting the primary end point (p = 0.0029). A Cox proportional-hazards model using baseline BNP between the 2 groups yielded a hazard ratio of 1.84 (95% confidence interval [CI] 1.19 to 2.85, p = 0.006). The relative risk for baseline BNP level >140 pg/ml was 4.62 (95% CI 1.80 to 11.3, p = 0.008). Patients who met the primary end point increased their BNP levels by 42.5 pg/ml per year (95% CI 12.09 to 72.95, p = 0.006) compared to 7.2 pg/ml per year (95% CI 2.01 to 12.47, p = 0.007) in patients who did not meet the primary end point. In conclusion, elevated BNP levels are predictive of death or heart failure admission in patients with the ES. A serum BNP level >140 pg/ml is a useful tool in identifying high-risk patients. [ABSTRACT FROM AUTHOR]

Single-ventricle congenital heart disease (SVCHD) requires multiple palliative surgical procedures that leave visible surgical scars and physical deficits, which can alter body-image and self-esteem. This study aimed to compare sex and age differences in body-image, self-esteem, and body mass index (BMI) in adolescents and adults with SVCHD after surgical palliation with those of a healthy control group. Using a comparative, cross-sectional design, 54 adolescent and adult (26 male and 28 female) patients, age 15-50 years, with SVCHD were compared with 66 age-matched healthy controls. Body-image and self-esteem were measured using the Multidimensional Body-Self Relations Questionnaire-Appearance Scale and Rosenberg Self-Esteem Scale. Height and weight were collected from retrospective chart review, and BMI was calculated. Female adolescents and adult patients with SVCHD reported lower body image compared with males patients with SVCHD and healthy controls ( p = 0.003). Specific areas of concern were face ( p = 0.002), upper torso or chest (p = 0.002), and muscle tone ( p = 0.001). Patients with SVCHD who were <21 years of age had lower body image compared with healthy controls ( p = 0.006). Self-esteem was comparable for both patients with SVCHD and healthy peers. There were no sex differences in BMI; BMI was higher in subjects >21 years of age ( p = 0.01). Despite the similarities observed in self-esteem between the two groups, female patients with SVCHD <21 years of age reported lower perceived body-image. Our findings support the need to recognize poor psychological adjustment related to low self-esteem in patients with SVCHD; female patients warrant increased scrutiny. Strategies to help patients with SVCHD cope with nonmodifiable aspects of body-image during the difficult adolescent-to-young adult years may potentially enhance self-esteem and decrease psychological distress. [ABSTRACT FROM AUTHOR]

This study used a retrospective analysis of adults with single-ventricle physiology to ascertain the predictive power of cardiopulmonary stress-testing parameters in determining patients at increased risk of suffering from adverse clinical outcomes. We found that the specific parameters of percent of maximum predicted heart rate achieved and maximum oxygen consumption were significantly correlated with adverse clinical outcomes in patients with single-ventricle congenital heart disease. [ABSTRACT FROM AUTHOR]

The prevalence of migraine headaches (MH) is 12% in the general population and increases to 40% in patients with patent foramen ovale. This study evaluated the prevalence of MH in patients with congenital heart disease (CHD). Of 466 patients contacted from the UCLA Adult Congenital Heart Disease Center, 395 (85%) completed a questionnaire to determine the prevalence of MH. Patients were stratified by diagnosis of right-to-left, left-to-right, or no shunt. A group of 252 sex-matched patients with acquired cardiovascular disease served as controls. The prevalence of MH was 45% in adults with CHD compared to 11% in the controls (p <0.001). Of the 179 patients with MH, 143 (80%) had migraines with aura and 36 (20%) had migraines without aura versus 36% and 64% observed in the controls (p <0.001). The frequency of MH was 52% in the right-to-left shunt group, 44% in the left-to-right, and 38% in the no shunt group (p = NS). In patients with a right-to-left shunt who underwent surgical repair, 47% had complete resolution of MH, whereas 76% experienced >50% reduction in headache days per month. In conclusion, the prevalence of MH in all groups of adults with CHD is 3 to 4 times more than a sex-matched control population, with increasing prevalence of MH in patients with no shunt, left-to-right, and right-to-left shunt. The higher than expected frequency of MH in patients with CHD without an intracardiac shunt, suggests additional mechanisms to explain the significant association with MH. [Copyright &y& Elsevier]

Myocardial performance index (MPI) is an echocardiographic Doppler-derived measure of ventricular function previously validated in patients with congenital heart disease. It may be preferred over conventional noninvasive measures of ventricular function in patients with complex anatomy because it is dependent on neither geometric shape nor heart rate. Brain natriuretic peptide (BNP) is a predictor of systolic and diastolic dysfunction in anatomically correct hearts. The correlation of BNP to MPI in patients with congenital heart disease was determined. Fifty-four adults with congenital heart disease were evaluated. BNP was measured using standardized assays. Doppler echocardiography was performed within 6 months of BNP assay. There were no changes in clinical status during this interval. An experienced observer was blinded and evaluated all echocardiographic images, and MPI and ejection fraction (EF) were determined. Left ventricular (LV) or univentricular MPI was calculated in 34 patients and right ventricular (RV) MPI was calculated in 23 patients. Pearson’s correlation coefficient test showed that BNP significantly correlated with LV/univentricular MPI (r = 0.461, p = 0.006) and RV MPI (r = 0.748, p <0.0001), whereas LV/univentricular EF and RVEF had no significant correlation with BNP (r = −0.189, p = 0.172; r = 0.066, p = 0.729, respectively). In patients with congenital heart disease, BNP correlated significantly with MPI, but not with LV, RV, or univentricular EF. This is particularly true in patients with geometrically variable right ventricles in which EF may be more difficult to assess. In conclusion, these findings emphasize the unique ability of both BNP and MPI to assess global ventricular function in geometrically complex hearts. [Copyright &y& Elsevier]

The Working Group on research in adult congenital heart disease (ACHD) was convened in September 2004 under the sponsorship of National Heart, Lung, and Blood Institute (NHLBI) and the Office of Rare Diseases, National Institutes of Health, Department of Health and Human Services, to make recommendations on research needs. The purpose of the Working Group was to advise the NHLBI on the current state of the science in ACHD and barriers to optimal clinical care, and to make specific recommendations for overcoming those barriers. The members of the Working Group were chosen to provide expert input on a broad range of research issues from both scientific and lay perspectives. The Working Group reviewed data on the epidemiology of ACHD, long-term outcomes of complex cardiovascular malformations, issues in assessing morphology and function with current imaging techniques, surgical and catheter-based interventions, management of related conditions including pregnancy and arrhythmias, quality of life, and informatics. After research and training barriers were discussed, the Working Group recommended outreach and educational programs for adults with congenital heart disease, a network of specialized adult congenital heart disease regional centers, technology development to support advances in imaging and modeling of abnormal structure and function, and a consensus on appropriate training for physicians to provide care for adults with congenital heart disease. [Copyright &y& Elsevier]

In patients with cyanotic congenital heart disease (CCHD), a right-to-left shunt results in systemic hypoxemia. Systemic hypoxemia incites a compensatory erythrocytosis, which increases whole blood viscosity. We considered that these changes might adversely influence myocardial perfusion in CCHD patients. Basal and hyperemic (intravenous dipyridamole) perfusion measurements were obtained with [13N]ammonia positron emission tomographic imaging in left (LV) and right (RV) ventricular and septal myocardium in 14 adults with CCHD [age: 34.1 yr (SD 6.5)]; hematocrit: 62.2% (SD 4.8)] and 10 healthy controls [age: 34.1 yr (SD 6.5)]. In patients, basal perfusion measurements were higher in LV [0.77 (SD 0.24) vs. 0.55 ml·min-1·g-1 (SD 0.09), P < 0.02], septum [0.71 (SD 0.16) vs. 0.49 ml·min-1·g--1(SD 0.09), P < 0.001], and RV [0.77 (SD 0.30) vs. 0.38 ml·min-1·g-1 (SD 0.09), P < 0.001]. However, basal measurements normalized for the rate-pressure product were similar to those of controls. Calculated oxygen delivery relative to rate-pressure product was higher in the patients [2.2 (SD 0.8) vs. 1.6 (SD 0.4) × 10-5 ml O2·min-1·g tissue-1·(beats· mmHg)-1 in the LV, P < 0.05, and 2.0 (SD 0.7) vs. 1.4 (SD 0.3) × 10-5 ml O2·min-1·g tissue-1·(beats·mmHg· in the septum, P < 0.01]. Hyperemic perfusion measurements in CCHD patients did not differ from controls [LV, 1.67 (SD 0.60) vs. 1.95 ml·min-1·g-1 (SD 0.46); septum, 1.44 (SD 0.56) vs. 1.98 ml·min-1·g-1 (SD 0.69); RV, 1.56 (SD 0.56) vs. 1.65 ml·min-1·g-1 (SD 0.64), P = not significant], and coronary vascular resistances were comparable [LV, 55 (SD 25) vs. 48 mmHg·ml-1·g·min (SD 16); septum, 67 (SD 35) vs. 50 mmHg· ml-1·g·min (SD 21); RV, 59 (SD 26) vs. 61 mmHg·ml-1·g·min (SD 27), P = not significant]. These findings suggest that adult CCHD patients have remodeling of the coronary circulation to compensate for the rheologic changes attending chronic hypoxemia. [ABSTRACT FROM AUTHOR]

Objectives: The purpose of this study was to evaluate adult congenital heart disease (CHD) training among U.S. cardiology fellowship programs. Background: Although training recommendations for caring for adults with CHD exist, the educational patterns and numbers of specialists remain unknown. Methods: We surveyed U.S. directors of 170 adult cardiology and 45 pediatric cardiology (PC) fellowship programs. Adult program surveys contained 1 single-response and 10 multiple-choice questions; pediatric program surveys contained 1 single-response and 13 multiple-choice questions. Results: Ninety-four adult cardiology fellowship directors (55%) and 34 PC directors (76%) responded. Of adult programs, 70% were in university hospitals and 40% were associated with PC groups. Those with PC-affiliation had more adult CHD clinics (p &#60; 0.02) and more adult CHD inpatient (p &#60; 0.02) and outpatient (p &#60; 0.002) visits than those without PC affiliation. Most PC programs were in children’s hospitals (38%) or children’s hospitals within adult hospitals (50%). Eighty-two percent had associated adult cardiology programs. Pediatric programs followed adult CHD patients in various care settings. Over one-third of adult and pediatric programs had ≤3 lectures annually regarding adult CHD. Nine adult and 2 pediatric programs offered adult CHD fellowships, and only 31 adult and 11 pediatric fellows pursued advanced CHD training in the last 10 years. Conclusions: Adult CHD didactic and clinical experiences for cardiology fellows vary widely. Few programs offer advanced CHD training, and the number of specially trained physicians is unlikely to meet projected workforce requirements. Adult cardiology programs with PC affiliation have increased CHD experience and might provide good educational models. [Copyright &y& Elsevier]

Hypoxemic erythrocytotic residents of high altitudes lack coronary atherosclerosis and have low cholesterol levels. It was postulated that hypoxemic erythrocytotic adults with cyanotic congenital heart disease (CCHD) might be analogous. The incidence of coronary atherosclerosis in this patient population has not been established, and hypocholesterolemia has not previously been recognized. Accordingly, 279 patients were divided into 4 groups: group A: 143 cyanotic patients not operated on (54 men and 89 women, aged 18 to 69 years); group B: 47 cyanotic patients (28 men and 19 women rendered acyanotic by operation at age 22 to 69 years); group C: 41 acyanotic patients not operated on (22 men and 19 women, aged 22 to 75 years); and group D: 48 patients acyanotic before and after operation (24 men and 24 women, aged 21 to 70 years). Coronary arteries were studied angiographically in 59 patients and at necropsy in 5 subjects aged 37 to 56 years. Total cholesterol was &#60;160 mg/dl in 58% of group A, 52% of group B, 10% of group C, and 12% of group D (p &#60;0.000001, chi-square analysis). Angiograms disclosed dilated coronary arteries without obstruction. Necropsy disclosed ectatic coronary arteries with structural abnormalities of the media. In conclusion, this study provides the first quantitative and qualitative data on antiatherogenic changes in lipoproteins in adults with CCHD. The coronary arteries are atheroma free because hypocholesterolemia acts in concert with the antiatherogenic properties of upregulated nitric oxide, hyperbilirubinemia, hypoxemia, and low platelet counts. The persistence of hypocholesterolemia after the surgical elimination of cyanosis suggests a genetic determinant. [Copyright &y& Elsevier]

We sought to establish pathogenetic links between electrophysiology, histopathology, and ventricular tachyarrhythmias in patients with Ebstein''s anomaly. The atrialized right ventricle (ARV) is the site of mechanically inducible ventricular tachyarrhythmias, but relations between the arrhythmogenic substrate, the type of tachyarrhythmias, and the trigger(s) have not been established. This study comprised 23 patients (10 men and 13 women; aged 18 to 58 years; mean 32 ± 3) who did not undergo surgery and 6 pre- and postoperative patients with Ebstein''s anomaly, diagnosed by transthoracic and transesophageal echocardiography. Twenty-one patients had classic Ebstein''s anomaly and 2 had mild forms. Signal-averaged electrocardiograms (SAECGs) identified slow conduction by using 3 time-domain variables calculated by an automated algorithm and inspected visually. Two variables were required to establish the presence of late potentials. SAECGs were repeated in 6 patients after surgical exclusion of the ARV. Five surgical specimens of the ARV and the true right atrium were examined histologically. Mathematic simulations were used to illustrate anchored and unanchored spiral/scroll waves. SAECGs were positive in 21 patients with classic Ebstein''s anomaly and were negative postoperatively in the 6 so studied. The ARV was characterized histologically by clusters of cardiomyocytes isolated within a fibrous matrix. We hypothesize that SAECGs identify slow conduction residing in the ARV, and that excitation of this arrhythmogenic substrate provokes spiral/scroll waves that cannot anchor because clusters of cardiomyocytes are isolated within a fibrous matrix. The waves meander erratically as polymorphic ventricular tachycardia or break up into ventricular fibrillation. [Copyright &y& Elsevier]

Extrapulmonary and intrapulmonary radiologic features of Eisenmenger syndrome and primary pulmonary hypertension (PPH) and the clinical expressions that coincide with the radiologic features of these 2 forms of pulmonary hypertension have not previously been characterized. Computed tomography (CT) and pulmonary CT angiography (CTA) utilized GE High Speed Advantage or Siemens Evolution electron beam tomographic (EBT) scanners in 31 patients with cyanotic pulmonary vascular disease (PVD) (group A: 12 men and 19 women; mean age 38 ± 4 years) and in 13 women with PPH-acyanotic PVD (group B: mean age 30 ± 3 years). Ten group A and 3 group B patients underwent imaging twice, yielding 57 studies. Group A patients’ extrapulmonary radiologic features included proximal pulmonary arteries that were consistently enlarged, and were aneurysmal in 13%, causing bronchial compression and atelectasis. Thromboses were uniformly present and mild in 71% and moderate to massive in 29% of patients. Massive proximal thromboses caused asphyxic death and augmented right-to-left shunts by increasing flow resistance. Mild to extensive mural calcific deposits occurred in 26% of patients. Intrapulmonary radiologic features included intrapulmonary embolic infarction, intrapulmonary hemorrhage, bronchial/systemic arterial collaterals, and neovascularity. Group B patients’ extrapulmonary radiologic features included proximal pulmonary arteries that were consistently enlarged but not aneurysmal and devoid of thromboses. Mild to extensive mural calcific deposits occurred in 23% of these patients. Intrapulmonary radiologic features were confined to mosaic attenuation and bronchial/systemic collaterals. [Copyright &y& Elsevier]

Most patients with advanced congestive heart failure have altered thyroid hormone metabolism. A low triiodothyronine level is associated with impaired hemodynamics and is an independent predictor of poor survival. This study sought to evaluate safety and hemodynamic effects of short-term intravenous administration of triiodothyronine in patients with advanced heart failure. An intravenous bolus dose of triiodothyronine, with or without a 6- to 12-hour infusion (cumulative dose 0. 1 5 to 2.7 microg/kg), was administered to 23 patients with advanced heart failure (mean left ventricular ejection fraction 0.22 +/- 0.01). Cardiac rhythm and hemodynamic status were monitored for 12 hours, and basal metabolic rate by indirect calorimetry, echocardiographic parameters of systolic function and valvular regurgitation, thyroid hormone, and catecholamine levels were measured at baseline and at 4 to 6 hours. Triiodothyronine was well tolerated without episodes of ischemia or clinical arrhythmia. There was no significant change in heart rate or metabolic rate and there was minimal increase in core temperature. Cardiac output increased with a reduction in systemic vascular resistance in patients receiving the largest dose, consistent with a peripheral vasodilatory effect. Acute intravenous administration of triiodothyronine is well tolerated in patients with advanced heart failure, establishing the basis for further investigation into the safety and potential hemodynamic benefits of longer infusions, combined infusion with inotropic agents, oral triiodothyronine replacement therapy, and new triiodothyronine analogs. [ABSTRACT FROM AUTHOR]

Dilatation and tortuosity of extramural coronary arteries are prevalent in cyanotic congenital heart disease. Two pathogenetic variables are operative, namely endothelial vasodilator substances and medial structural abnormalities. [Copyright &y& Elsevier]