bluuebunny/crossref_metadata_2025 huggingface dataset:
A light‐sensitive mechanism differently regulates transcription and transcript stability of ω3 fatty‐acid desaturases (FAD3, FAD7 and FAD8) in soybean photosynthetic cell suspensions
Authors: Collados Raquel , Andreu Vanesa , Picorel Rafael , Alfonso Miguel
Published: August 2006

The ω3 fatty‐acid desaturases: FAD7 and FAD8 (plastid) and FAD3 (reticular) are responsible for trienoic fatty‐acid (TA) production in plants. The expression of these enzymes seemed to be regulated differently in response to light. Darkness leads to a decrease in total TA level. Under such conditions, FAD3 and FAD8 transcript levels were undetectable but increased after re‐illumination concomitant with TA levels, indicating a transcriptional control. On the contrary, FAD7 transcript levels were similar to illuminated control cells, suggesting the presence of a post‐transcriptional control mechanism. Furthermore, FAD7 mRNA stability increased dramatically in darkness. Analysis of FAD7 protein accumulation using specific antibodies revealed that FAD7 was very stable whatever the light or darkness conditions. These results indicate that FAD7 enzyme availability is not limiting for 18:3 production in darkness. Our data point to an additional post‐translational regulatory mechanism that controls the activity of FAD7 in response to light.
XenopusElrB, but not ElrA, binds RNA as an oligomer: Possible role of the linker
Authors: Devaux Agathe , Colegrove-Otero Lucy J. , Standart Nancy
Published: August 2006

We have shown that ElrA and ElrB,XenopusELAV homologues, bind the Vg1 mRNA 3′UTR translation element inXenopusoocytes and implicated ElrB in mediating translational repression during oogenesis. Here we report that, while ElrA and ElrB are 69% identical and both exhibit RNA binding in the nM range, recombinant ElrB, but not ElrA, is able to oligomerise. This oligomerisation is also seen with the endogenous protein. Both RNA binding and oligomerisation require the linker region flanked with two RNA recognition motifs. Our data demonstrate a novel and unique property of ElrB which may be important for its function as a translational regulator.
Malaria in pregnancy: Access to effective interventions in Africa
Authors: Yartey J.E.
Published: August 2006

Malaria infection during pregnancy (MIP) poses substantial risks to the mother, her fetus and the newborn. Consequences of MIP include severe anemia, placental parasitemia and intrauterine growth retardation, which contribute to low birth weight, a principal cause of infant mortality in the African region. Effective interventions for the prevention and control of MIP include Intermittent preventive treatment (IPT), Insecticide treated nets (ITNs), and case management, and are being deployed by countries. The global political and fiscal environment is favorable with increasing resources to support the scale‐up of interventions. What is needed at country level is strong collaboration among malaria and reproductive health programs and partners, to maximize the use of available resources for scaling‐up to achieve the Millennium Development Goals. Equally important is the need for continuous advocacy at all levels to keep malaria high on the global agenda and maintain the current global commitment and momentum.
Women's right to health and the Millennium Development Goals: Promoting partnerships to improve access
Authors: Shaw D.
Published: August 2006

The Millennium Development Goals (MGD) represent a commitment of 189 member states that adopted them during the Millennium Summit in September 2000. This UN General Assembly recognized that gender equality and women's empowerment are both central to achieving sustainable development by means of combating poverty, hunger and disease. Neither reproductive nor sexual health was explicitly articulated in the original MDGs and indicators—a critical omission, as globally women are more disadvantaged than men. However, a clear link exists between all of the MDGs and the reproductive and sexual health of women, who cannot contribute to sustainable development, unless their right to health is met through improved access. The FIGO 2006 World Report on Women's Health addresses many issues critical to the success of the MDGs, with a focus on how partnerships have become a crucial vehicle to improve access to health for women.
Giving Social Ties, Reciprocity in Modern Society
Authors: Adloff Frank , Mau Steffen
Published: August 2006

The purpose of this paper is to discuss the meaning of giftgiving and reciprocity in modern society and thereby following the pointers left by Marcel Mauss. A critique will be made of the dichotomy of self-interest and normatively orientated action that forms the basis of sociology. For this conceptual dichotomization has caused forms of social interaction that cannot be localized either on the side of self-interest or on that of morality. It is the logic of the gift and the reciprocity thus evoked that in our view accompanies and structures all forms of interaction, from the social micro to the macrolevel. It is shown that in modern societies gifts and reciprocities form their own orders of interaction, and not only on a microsocial level. The principle of reciprocity even accompanies as a rule transfers owing to (state) compulsion as well as economic, selective exchange. As a basic principle of processes of sociation it is, fundamentally, present everywhere and in some areas it is explicitly and openly in effect (for example in welfare state transfers). Sociology has for too long overlooked the fact that this principle cannot be traced back either to normativist or to utilitarian explanations and nevertheless represents a principle of construction of modern societies.
Timing of co-phenylcaine administration before rigid nasendoscopy: a randomized, controlled trial
Authors: Pothier D D , Hall C E J , Gillett S , Nankivell P
Published: August 2006

Rigid nasendoscopy is a commonly used method of examining the nasal cavity and postnasal space. Co-phenylcaine is useful for its vasoconstrictive and anaesthetic properties, but the length of time allowed for it to take effect is variable. We performed a single-blind, randomized, controlled trial to determine whether it was better to allow one or 10 minutes for co-phenylcaine to take effect. Fifty patients were randomized into two groups, 25 in each. Patients in the 10 minute group experienced less discomfort (p=0.02) and less pain (p=0.018) than those in the one minute group. Ease of examination was also greater in the 10 minute group, as was the quality of the image obtained (p<0.001).
Role of information leaflets in improving the practice of informed consent for patients undergoing septoplasty
Authors: Winterton R I S , Alaani A , Loke D , Bem C
Published: August 2006

Aim: To evaluate the effectiveness of an information leaflet in improving patient understanding of the procedure and complications of septoplasty.

Design: The baseline knowledge of a group of patients who had attended a pre-assessment clinic prior to septoplasty was assessed. The procedure and its complications were then verbally explained. The patients' knowledge was then re-assessed on the morning of surgery and any improvements noted. In the second arm of the study, an information leaflet was introduced at the time of verbal instruction and any differences in improvement in knowledge were assessed.

Results: Data from the two groups were analysed using an analysis of covariance with differences in baseline (pre-instruction) knowledge controlled. Additional improvements in mean recall score following leaflet distribution were highly statistically significant when compared with mean recall in the control group (p<0.001).

Conclusion: The use of information leaflets increases patients' knowledge about a surgical procedure and its potential complications.
Etanercept, a tumour necrosis factor α receptor antagonist, and methotrexate in acute sensorineural hearing loss
Authors: Street I , Jobanputra P , Proops D W
Published: August 2006

Patients with autoimmune inner-ear disease (AIED) are treated with high doses of steroids in the short term when suffering an acute hearing loss. As a consequence, substances such as methotrexate have been employed in the role of steroid-sparing agents. Additionally, it is known that tumour necrosis factor α (TNFα) is an important mediator of the inflammatory process, inhibition of which may be of benefit in AIED. This case report illustrates the use of a TNFα inhibitor in combination with methotrexate, which is known to be an effective combination in rheumatoid arthritis but has yet to be described for sensorineural hearing loss. We conclude that progressive AIED may respond well to TNFα inhibition, whilst more difficult cases, such as this example, could benefit from combining such therapy with methotrexate.
Tragal cartilage harvesting and ‘in the ear’ earphones: a pilot study
Authors: Pothier D D , Charaklias N
Published: August 2006

‘In the ear’ headphones are held in position by the cartilaginous skeleton of the pinna. The tragus is an important part of this skeleton and plays a significant role in holding these devices in place. We designed a retrospective case–control study to determine whether the harvesting of tragal cartilage had any effect on the ability to wear ‘in the ear’ earphones. Fifty patients who had undergone ear surgery requiring harvesting of tragal cartilage, along with 50 age-matched controls who had undergone similar procedures without tragal cartilage harvesting, were sent a questionnaire about their experiences with ‘in the ear’ earphones both pre- and post-operatively. Ten of the 12 patients in the tragal harvesting group who had used earphones pre- and post-operatively had had problems with earphones post-operatively, which in eight cases were due to pain or the devices falling out. Of the 12 respondents in the non-tragus group, only one had similar problems (p<0.05). Tragal harvesting may affect the ability of patients to wear certain types of headphones post-operatively and this should be discussed pre-operatively with the patient.
Annual prevalence of diagnosed schizophrenia in the USA: a claims data analysis approach
Authors: WU ERIC Q. , SHI LIZHENG , BIRNBAUM HOWARD , HUDSON TERESA , KESSLER RONALD
Published: August 2006

Background. Schizophrenia is a debilitating chronic mental illness. However, the annual prevalence of schizophrenia is not well understood because of under-representation of schizophrenia patients in epidemiological surveys. This study used multiple administrative claims databases to estimate the annual prevalence of diagnosed schizophrenia in the USA.

Method. The annual prevalence of diagnosed schizophrenia in the USA was estimated for different health insurance coverage groups. The prevalence for privately insured individuals was calculated from an administrative claims database of approximately 3 million privately insured beneficiaries covering the period 1999–2003. The prevalence for Medicaid enrollees was calculated from California Medicaid claims covering the period 2000–2002. The prevalence for Medicare and Medicaid/Medicare dual eligibles was estimated using a combination of both databases. Published statistics were used to estimate the prevalence of schizophrenia in the uninsured and veteran populations and to weight the prevalence rates obtained to the population of the USA.

Results. The 12-month prevalence of diagnosed schizophrenia in the USA in 2002 was estimated at 5·1 per 1000 lives. The Medicaid population was identified with the highest prevalence rate among the populations studied. Sensitivity analyses taking into consideration the Veterans Affairs population only changed the estimate slightly to 5·3 per 1000 lives.

Conclusion. Analyses of administrative claims data contribute to the understanding of the prevalence of diagnosed schizophrenia.
The feasibility and need for dimensional psychiatric diagnoses
Authors: HELZER JOHN E. , KRAEMER HELENA C. , KRUEGER ROBERT F.
Published: August 2006

Background. It is our contention that both categorical and dimensional approaches to diagnosis are important for clinical work and research alike, and that each approach has its drawbacks and advantages. As the processes toward developing DSM-V and ICD-11 progress, we suggest that another exclusively categorical revision of psychiatric taxonomies will no longer be sufficient and that adding a dimensional component is a necessary step if these taxonomies are to continue serving the future clinical and research needs of psychiatry as they have so effectively done in the past.

Method. We begin the paper with a review of terminology related to categories and dimensions and briefly review literature on advantages and disadvantages of both approaches.

Results. A review of relevant literature supports both the need for and feasibility of augmenting traditional categorical diagnoses with dimensional information.

Conclusion. We conclude with a proposal for preserving traditional categorical diagnostic definitions, but adding a dimensional component that would be reflective of and directly referable back to the categorical definitions. We also offer a specific proposal for adding a dimensional component to official taxonomies such as the DSM and the ICD in a way that fully preserves the traditional categorical approach.
Perioperative administration of angiotensin converting enzyme inhibitors decreases the severity and duration of pleural effusions following bidirectional cavopulmonary anastomosis
Authors: Thompson LeNardo D. , McElhinney Doff B. , Culbertson Casey B. , Hardy Christian E. , Brook Michael M. , Reddy V. Mohan , Hanley Frank L.
Published: August 2006

Background: Pleural effusions after bidirectional cavopulmonary anastomosis remain a significant cause of morbidity. Prolonged effusions in such patients have been associated with persistent elevations in plasma renin and angiotensin II. Methods: We conducted a controlled study in 36 patients (median age 8 months) undergoing bidirectional cavopulmonary anastomosis. Enalapril (5 mcg/kg) was administered intravenously within 1 hour of surgery and every 12 hours thereafter in 18 patients; when these patients were tolerating feeds, enalapril was switched to enteral captopril (3 mg/kg/day) every 8 hours. The other 18 patients did not receive perioperative angiotensin converting enzyme inhibitors. Using standardized criteria for discontinuation of chest tubes (< 2 mL/kg/day), volume and duration of pleural drainage were compared between groups. Results. There were no differences between groups in demographic, diagnostic, or hemodynamic factors. There was no difference in cardiopulmonary bypass time between groups and no difference in postoperative pulmonary arterial pressures. The duration of pleural drainage was shorter (2.2 ± 1.4 vs 5.9 ± 1.4 days, p< 0.001) and the volume less during the first 24 hours (4.7 ± 1.2 vs 7.7 ± 2.1 mL/kg, p< 0.001) and overall (10.6 ± 2.4 vs 19.6 ± 4.5 mL/kg, p< 0.001) in patients who received angiotensin converting enzyme inhibitors than those who did not. Readmission for persistent effusions was required in 3 patients who did not receive angiotensin converting enzyme inhibitors and none who did (p= 0.11). Conclusions. Perioperative administration of angiotensin converting enzyme inhibitors is associated with decreased severity and duration of pleural effusions following bidirectional cavopulmonary anastomosis.
The value of transesophageal echocardiography in transcatheter closure of atrial septal defects in the oval fossa using the Amplatzer septal occluder
Authors: Latiff Haifa A. , Samion Hasri , Kandhavel Geetha , Aziz Bilkis A. , Alwi Mazeni
Published: August 2006

Background: From January, 1997, as part of an international multicentric trial, we have been closing small-to-moderate atrial septal defects within the oval fossa using the Amplatzer Septal Occluder (ASO, AGA Medical). Methods: All patients with defects within the oval fossa deemed potentially suitable for transcatheter closure were investigated by transesophageal echocardiography with the aim of gaining extra information that might alter the decision to use the device to close the defect. Views were obtained in transverse and longitudinal planes, permitting measurements of the diameter of the defect, and its distance from the atrioventricular valves, coronary sinus, and pulmonary veins. Additionally, we sought to identify multiple defects, and to exclude sinus venosus defects. Results: Of 56 patients with left-to-right shunts, 41 (73.2%) were deemed suitable for closure with the Amplatzer Septal Occluder. All underwent the procedure successfully, with no complications. This includes 5 patients with multiple small defects that were sufficiently close to the main defect to be closed with a single device. Only two of these had been detected on the transthoracic study. In the remaining 15 of 56 patients, transcatheter closure was deemed unsuitable. In 9 patients, this was due to the limitation of the size of the device available during the period of study, this representing a relative contraindication. In the remaining 6 (10.7%), transcatheter closure was not performed because multiple defects were too far apart to be closed with a single device in 3 patients, two patients were noted to have a sinus venosus defect, and another was noted to have anomalous connection of the right upper pulmonary vein to the right atrium. Excluding patients contraindicated due to the size of the defect alone, transesophageal echocardiography provided extra information in one-tenth of our patients, which altered the decision regarding management. Conclusion: Transesophageal echocardiography is indispensable in the evaluation of patients undergoing transcatheter closure of atrial septal defect.
High-altitude precipitation and exacerbation of protein-losing enteropathy after a Fontan operation.
Authors: McMahon Colin J. , Hicks John M. , Dreyer William J.
Published: August 2006

We describe the development and exacerbation of protein-losing enteropathy after relocating to an environment at an altitude of 3695 feet in El Paso, Texas, in a patient who had undergone a Fontan operation. This report should heighten awareness to the possibility of such patients developing protein-losing enteropathy at high-altitude, with hypoxemia-induced pulmonary vasoconstriction, and subsequent elevation of central venous pressure, the most likely underlying mechanism.
Rapid onset of intrapulmonary arteriovenous shunting after surgical repair of tetralogy of Fallot with pulmonary atresia
Authors: Ofoe Victor D. , Pratap Usha , Slavik Zdenek
Published: August 2006

We describe a 2-year-old girl with tetralogy of Fallot and pulmonary atresia, palliated as a neonate with a right modified Blalock Taussig shunt, who developed severe cyanosis following total correction in the absence of corresponding evidence of parenchymal lung disease on the chest X-ray. Selective pulmonary angiography showed new intrapulmonary shunting involving only the right middle and lower lobes only. The cyanosis resolved rapidly subsequent to inhalation of nitric oxide. To our knowledge, this is the first documented case of rapid onset of localised intrapulmonary right-to-left shunting, involving only two lung lobes, following biventricular repair for complex congenital heart disease.
Noncompaction of the myocardium associated with Roifman syndrome
Authors: Mandel Karen , Grunebaum Eyal , Benson Lee
Published: August 2006

Noncompaction of the ventricular myocardium, sometimes referred to as “spongy myocardium”, appears as excessive and prominent trabeculations and deep intratrabecular recesses within the ventricular wall, usually involving the left ventricle, although the right ventricle and interventricular septum can also be affected. It may occur with or without additional heart malformations. Roifman syndrome is a constellation of antibody deficiency, spondyloepiphyseal dysplasia, facial dysmorphism, growth retardation, and retinal dystrophy. We report a patient with Roifman syndrome who presented with noncompaction of the left ventricular myocardium. Our findings expand the spectrum of diseases associated with noncompaction. The recognition of noncompaction among patients with Roifman syndrome is important, as the immune deficiencies may be subtle and undiagnosed until adulthood. Thus, some patients may first present with cardiac failure.
Can subclinical valvitis detected by echocardiography be accepted as evidence of carditis in the diagnosis of acute rheumatic fever?
Authors: Özkutlu Süheyla , Ayabakan Canan , Saraçlar Muhsin
Published: August 2006

Aim: Subclinical valvar insufficiency, or valvitis, has recently been identified using Doppler echocardiography in cases of acute rheumatic fever with isolated arthritis or chorea. The prognosis of such patients with acute rheumatic fever and subclinical valvitis is critical when determining the duration of antibiotic prophylaxis. We aimed, therefore, prospectively to investigate the association of silent valvitis in patients having rheumatic fever in the absence of clinical evidence of cardiac involvement, and to evaluate its prognosis. Methods and Results: Between November 1998 and September 1999, we identified 26 consecutive patients with silent valvitis in presence of rheumatic fever but in the absence of clinical signs of carditis. The patients, eight female and 18 male, were aged from 6 to 16 years, with a mean of 9.9± 2.7 years. Major findings were arthritis in 16, chorea in 7, and arthritis and erythema marginatum in 1 patient. Two cases had arthralgia with equivocal arthritic signs and Doppler echocardiographic findings of pathologic mitral regurgitation. Silent pathologic mitral regurgitation was found in 12 cases, and aortic regurgitation in 2 cases. All patients with arthritic findings were treated with acetylsalicylic acid with one exception, this patient receiving both prednisone and acetylsalicylic acid. No antiinflammatory treatment was given to patients with chorea. After a mean follow-up of 4.52 months, valvar regurgitation disappeared in 4 patients, including the one with migratory arthralgia and no other major criterions. All six patients with chorea and silent carditis still have mitral insufficiency. Conclusion: Acute rheumatic fever without clinical carditis is not a benign entity. Doppler echocardiographic findings of subclinical valvar insufficiency, therefore, should be considered as carditis when seeking to establish the diagnosis of acute rheumatic fever.
A comparison of tissue doppler imaging and velocities of transmitral flow in children with elevated left ventricular preload
Authors: Harada Kenji , Tamura Masamichi , Yasuoka Kenji , Toyono Manatomo
Published: August 2006

Compared with transmitral velocities of flow, myocardial wall velocities obtained by tissue Doppler imaging are less influenced by left atrial pressure. The data supporting this assumption, however, are limited in patients with congenital cardiac disease. The aim of this study was to compare the effects of left ventricular preload on transmitral inflow and velocities assessed by tissue Doppler imaging. Tissue Doppler imaging, and conventional Doppler echocardiography with simultaneous invasive hemodynamic studies, were performed in 33 patients with a simple ventricular septal defect or patency of the arterial duct. Transmitral velocities (E, A) and mitral annular velocities (Ea, Aa) were measured, permitting calculation of the ratio of E to Ea. The ratio of pulmonary to systemic flows, and mean left atrial pressure, were also measured. In 10 of 33 patients, echocardiographic and hemodynamic studies were performed 4 to 5 months after surgery.

The E and A values in the patients were greater than those in the controls (p < 0.01). In contrast, neither Ea nor Aa differed between the two group. The ratio of E to Ea in the patients increased significantly compared with that in the controls (8.9 ± 2.1 vs 7.3 ± 1.3, p < 0.01). The E value was directly related to mean left atrial pressure and the ratio of pulmonary to systemic flows. The velocities measured by Tissue Doppler imaging, however, had no significant relationship to either of these measurements. The ratio of E to Ea correlated well with mean left atrial pressure (r = 0.75, p < 0.01). In 10 post-operative patients, the values for E and A decreased from 119 ± 14 to 89 ± 10 cm/sec (p < 0.01) and from 91 ± 22 to 61 ± 9 cm/sec (p < 0.01), respectively. No significant changes were noted in the values of Ea and Aa. The ratio of E to Ea, and mean left atrial pressure, decreased from 10.3 ± 1.9 to 8.2 ± 1.5 (p < 0.01) and from 11.0 ± 1.8 to 7.4 ± 1.0 mmHg (p < 0.01), respectively. The percentage change in left atrial pressure correlated with the percent change in the ratio of E to Ea (r = 0.64, p < 0.05).

The present study showed that the velocities obtained with tissue Doppler imaging are less dependent on mean left atrial pressure in children with elevated left ventricular preload caused by a left-to-right shunt.
Self-management of oral anticoagulation in children with congenital heart disease
Authors: Christensen Thomas D. , Attermann Jørn , Hjortdal Vibeke E. , Maegaard Marianne , Hasenkam J. Michael
Published: August 2006

Objective: The concept of self – management of oral anticoagulation has been shown to entail better quality of treatment than conventional management when assessed in selected adults. We have extended the concept of self – management to include children with congenital cardiac disease, hypothesizing self-management of oral anticoagulation is also possible in this subset of patients. Our aim was to assess the quality of self-management. Methods: We trained 14 children aged from 2.2 to 15.6 years, with a mean age of 9.7 years, and their parents, in domiciliary analysis of the International Normalized Ratio and necessary adjustment of dosage of coumarin. The curriculum for training lasted for 27 weeks, and the patients and their parents were followed for a period of up to 31 months by weekly measurement of the values obtained for the International Normalized Ratio. Results: The patients were observed over a mean of 547 days, with a range from 214 to 953 days. The patients were within the therapeutic targetted range of the International Normalized Ratio for a median of 65.5% of the time, with a range from 17.6 % to 90.4 %. None of the patients experienced thromboembolic or bleeding complications requiring doctoral intervention. All the patients and their parents expressed full satisfaction with the treatment. Conclusion: Selfmanagement of oral anticoagulation provides a good quality of treatment, which is feasible and safe in selected children with congenital cardiac disease.
Psychosocial functioning in children after transplantation of the heart, and heart and lungs
Authors: Spurkland Ingrid , Bjørbaek Tone , Hagemo Petter
Published: August 2006

We studied 10 boys, and 15 girls, all below the age of 16, who had been referred to the National Hospital in Norway for evaluation for transplantation of either the heart, or the heart and lungs. These represent the complete cohort of patients being considered for transplantation between 1990 and 1997. Of the 25 children and their families, 24 sets underwent a comprehensive psychosocial assessment, including interviews with both parents and their children. The parents completed the Child Behavior Checklist and the General Health Questionnaire. We had accepted 15 children for transplantation and placed them on the waiting list. The others were rejected for medical reasons, and 3 died whilst waiting for an organ. One was reconsidered for conventional surgery and removed from the list. Transplantation was performed in 11 children, whilst one of the patients we had rejected underwent transplantation abroad, and was included in the study. This left 12 patients in the final sample, with a mean age of 8 years, and with a range from 11 months to 13.9 years. We reassessed their psychosocial and physical functioning two years after transplantation. Of those undergoing transplantation of the heart and lungs, two were severely affected by progressive obliterative bronchiolitis. The others were in good general physical condition. At the assessment prior to transplantation, three already fulfilled the criterions for diagnosis of an overanxious disorder. Two others had symptoms of anxiety and depression, but without fulfilling the accepted criterions. At follow- up, two patients retained this psychiatric diagnosis. Increased levels of stress were uncovered in the parents prior to surgery, but these had normalised at follow-up. The study shows that, in general, physical and psychological conditions improve in children undergoing transplantation, but they and their parents live in a stressful environment, and are in need of psychosocial support both before and after transplantation.
Incidence and predictors for the development of significant supradiaphragmatic decompressing venous collateral channels following creation of Fontan physiology
Authors: Weber Howard S.
Published: August 2006

The occurrence of supradiaphragmatic decompressing venous collateral channels following construction of a bidirectional cavopulmonary connection or completion of the Fontan operation resulting in abnormal systemic hypoxemia has been infrequently described. In addition, the incidence and predictors of these channels have not been well delineated, especially in those patients without formation of such structures preoperatively. I evaluated, retrospectively, 40 patients who had undergone either construction of a bidirectional cavopulmonary shunt or completion of the Fontan operation, and who had complete pre and postoperative hemodynamic and angiographic data. Of the patients, 17 (43%) had developed a total of 21 decompressing venous collateral channels, of which 7 (18%) were considered to be hemodynamically significant requiring transcatheter coil occlusion. Of all variables examined, seven patients with significant decompressing collaterals had a greater transpulmonary gradient at follow-up catheterization (8 + /− 2 vs 5 + /− 2 mmHg, p= .01) and lower systemic saturations at routine clinical follow-up visits (82 + /− 5 vs 89 + /− 5 mmHg, p= .007) in comparison to the 33 others. When not evident preoperatively, decompressing venous collateral channels develop in a significant number of patients following conversion to Fontan physiology. If sufficiently large, they may produce lower than expected systemic saturations for the observed cardiac physiology. The larger decompressing channels are more likely to occur when a greater transpulmonary gradient exists postoperatively, which may require cardiac catheterization and transcatheter coil occlusion.
Reduction in levels of triidothyronine following the first stage of the Norwood reconstruction for hypoplastic left heart syndrome
Authors: Mainwaring Richard D. , Healy Regina M. , Meier Frederick A. , Nelson Jerald C. , Norwood William I.
Published: August 2006

Objective: Thyroid hormone has important effects on cardiovascular performance. This study was performed to evaluate the changes in levels of triiodothyronine following the first stage of reconstruction for hypoplastic left heart syndrome. Methods: We enrolled 14 newborns with hypoplastic left heart syndrome scheduled for first stage reconstruction. Blood samples were obtained pre-, intra-, and post-operatively. Levels of free and total triiodothyronine were determined by radioimmunoassay. Statistical comparison was performed using Wilcoxon's signed rank test. Results: The levels of free triiodothyronine decreased from a baseline of 355 ± 31 pg/dl to 205 ± 21 pg/dl upon the institution of bypass, and declined to a level of 135 ± 9 pg/dl at 24 hours postoperatively. Similarly, levels of total triiodothyronine decreased from 101 ± 15 ng/dl to 65 ± 4 ng/dl upon the institution of bypass, and continued to decline during the first 24 hours postoperatively. Levels of free and total triiodothyronine had returned to baseline by the fifth postoperative day. Conclusions: The data demonstrate significant decreases in levels of free and total triiodothyronine during the early postoperative period. These changes in levels of thyroid hormone may have adverse effects on cardiac function during this phase of recovery.
A pilot study of expenditures on, and utilization of resources in, health care in adults with congenital heart disease
Authors: Moons Philip , Siebens Kaat , Geest Sabina De , Abraham Ivo , Budts Werner , Gewillig Marc
Published: August 2006

Background: Congenital cardiac disease may be a chronic condition, necessitating life-long followup for a substantial proportion of the patients. Such patients, therefore, are often presumed to be high users of resources for health care. Information on utilization of resources in adults with congenital heart disease, however, is scarce. Methods: This retrospective pilot study, performed in Belgium, investigated 192 adults with congenital heart disease to measure the annual expenditures and utilization of health care and compared the findings with data from the general population. We also sought to explore demographic and clinical parameters as predictors for the expenditures. Results: Hospitalization was documented in 20.3% of the patients, with a median length of stay of 5 days. The overall payment by health insurance associations in 1997 was 1794.5 ECU per patient, while patients paid on average 189.5 ECU out-of-pocket. For medication, the average reimbursement and out-of-pocket expenses were estimated at 78 ECU and 20 ECU, respectively. Expenditures for patients with congenital heart disease were considerably higher than the age and gender-corrected expenditures for the general population (411.7 ECU), though this difference was accounted for by only one-eighth of the cohort of those with congenital heart disease. In general, higher expenditures were associated with abnormal left ventricular end-diastolic diameter, female gender, functional impairment and higher age, although the explained variance was limited. Conclusion: Our study has provided pilot data on the economic outcomes for patients with congenital heart diseases. We have identified parameters that could predict expenditure, but which will have to be examined in future research. This is needed to develop guidelines for health insurance for those with congenital heart diseases.
Pulmonary atresia with intact ventricular septum, right-sided aortic arch and ventriculocoronary connection – prenatal echocardiographic diagnosis
Authors: Patel Chandrakant R. , Dahms Beverly B. , Sallee Denver
Published: August 2006

The authors describe the prenatal echocardiographic diagnosis of a rare case of pulmonary atresia with intact ventricular septum, right-sided aortic arch, and ventriculocoronary connection in a fetus at 21 weeks gestation. The diagnosis was confirmed at autopsy.
Biventricular repair in a patient having double outlet right ventricle with unusual straddling of the mitral valve
Authors: Fukushima Satsuki , Yagihara Toshikatsu , Uemura Hideki
Published: August 2006

The authors achieved biventricular repair in a 1-year-old boy having double outlet right ventricle and unusual straddling of the mitral valve. There was a substantial abnormal muscular bar present within the right ventricle, and some of the tendinous cords of the mitral valve were attached to this structure. Preoperative echocardiography was particularly informative in enabling the surgeon to arrange the optional operative procedures. The postoperative course was smooth and uneventful.
Are cyanosed adults with congenital cardiac malformations depressed?
Authors: Popelová Jana , Slavík Zdeněk , škovránek Jan
Published: August 2006

Objective: To assess the incidence of depression, and the ability to interact socially, in adult patients with chronic cyanosis and congenital cardiac malformations. Design: Prospective study of consecutive patients. Setting: Single institution, tertiary referral centre. Patients: Between 1993 and 2000, we assessed 76 patients with congenital cardiac malformations and persistent cyanosis, having a median age of 36.5 years, with a range from 19 to 64 years, at the time of referral. Female patients accounted for just under half (48.6%) of the sample. Just under two-fifths of the cohort (39.5%) had functionally univentricular cardiac anatomy, while 14.8% had tetralogy of Fallot with pulmonary atresia and aorto-pulmonary collateral arteries, and 17% had the Eisenmenger syndrome. During the period of follow-up, 17 (22.4%) of the patients died. Assessment: We used clinical interviews and non-invasive assessment, employing Zung's questionnaire which provides a scale for the self-rating of depression. On this scale, a score above 50 points is indicative of depression. Results: Of the survivors, 32 (54%) completed the self-rating questionnaires. Of these, 20 responders (63%) considered that they lead full lives, including sexual activities, while 26 (81%) had never harboured suicidal thoughts. Depression was diagnosed in 11 responders (34%), with a mean score of 66.9, standard deviation of 8.7, and a range from 53 to 89. The remaining 21 patients (66%) were without signs of depression, scoring a mean of 41.5, with standard deviation of 5.5, and a range from 35 to 46. Depression was associated with older age (40.5 years versus 33.5 years, p =0.01), worse functional state in the classification of the New York Heart Association (2.95 versus 2.48, p =0.03), and unemployment (p < 0.0001), but independent from the severity of cyanosis, the level of the haematocrit, the saturation of oxygen, or previous surgical treatment. Conclusions: To our knowledge, this is the first evidence suggesting a relatively high incidence of depression in adults with congenital cardiac malformations and persistent cyanosis. Larger, multi-centric studies will be needed to confirm or refute these findings.
Cardiopulmonary exercise performance in adult survivors of the Mustard procedure
Authors: Hechter S. J. , Webb G. , Fredriksen P. M. , Benson L. , Merchant N. , Freeman M. , Veldtman G. , Warsi M. A. , Siu S. , Liu P.
Published: August 2006

Most patients with the Mustard procedure are now adults. To date, however, there have been few reports on resting and exercise hemodynamics in a large population of adults with this circulation. The aim of this study is to describe such parameters in one of the largest and oldest populations of adults with the Mustard procedure. The database of the University of Toronto Congenital Cardiac Centre for Adults was examined to identify 84 adults with the Mustard procedure who have undergone cardiopulmonary exercise tests. Magnetic resonance imaging and echocardiography studies were obtained in order to assess right ventricular size, function and baseline hemodynamics. Patients achieved lower maximum uptake of oxygen, maximal heart rate, forced vital capacity, forced expiratory volume in 1 second, and oxygen saturations at maximal exercise compared to a healthy population. Magnetic resonance imaging showed significantly different right ventricular ejection fractions between patients and controls. There were no effects of operative variables or preoperative hemodynamics on current exercise capacity. Patients after the Mustard procedure have subnormal exercise capacities. Factors such as chronotropic incompetence, peripheral deconditioning, and impaired lung function may be responsible for these results.
Clinical features of isolated noncompaction of the myocardium in children
Authors: Neudorf Ulrich E. , Hussein Ahmad , Trowitzsch Eckart , Schmaltz Achim A.
Published: August 2006

Isolated noncompaction of the myocardium, also known as “spongy myocardium”, is a rare disease in children and adults. It is suggested that, during early development of the heart, the primary spongy structure persists due to an arrest of compaction. No other cardiac malformations are found, but there are familial occurences, relations to genetic disorders or syndromes such as Melnick-needles-syndrome or Xq28-linked cardiomyopathy, and reports of conduction disorders.

We have now diagnosed isolated noncompaction in seven children aged between five weeks and 5.5 years. Three are doing well with anticongestive therapy, while transplantation of the heart was performed in one. Three of the children have died, but in only one case due to cardiac failure.

Our experience emphasises the need rapidly to establish the diagnosis, to search for associated extracardiac abnormalities, and to consider transplantation at an early stage.
Congenital absence of aortic valvar leaflets: a rare variant of the hypoplastic left heart syndrome
Authors: Rouillard Kenneth P. , Moore Phillip , Silverman Norman H.
Published: August 2006

Congenital absence of aortic valvar leaflets is a rare and fatal variant of the hypoplastic left heart syndrome. We describe a recent patient seen at our institution with this lesion, illustrating a combined echocardiographic and angiographic approach that delineates both anatomy and physiology. The early mortality experienced in previous reports, as well as unsuccessful surgical palliation in our case, should promote further discussion regarding the optimal treatment.
Spongy cardiomyopathy in a neonate
Authors: Halbertsma Feico J. J. , van't Hek Louis G. F. M. , Daniëls Otto
Published: August 2006

Isolated noncompaction of ventricular myocardium is a rare cardiomyopathy, presumed to originate from a developmental abnormality in the evolution of the heart, and resulting in sponge-like myocardium. Isolated ventricular noncompaction can present with a variety of symptoms, but usually includes heart failure. The diagnosis is often made by echocardiography, which reveals a very distinct image of the myocardium, with many deep, confluent recesses and dense trabeculations. We encountered such findings in a moribund neonate presenting with cardiogenic shock with extremely low shortening fractions. After treatment with intravenous and oral cardiotonics, coupled with afterload reduction, we were able to optimize the balance between ventricular filling and myocardial contractility, resulting in markedly improved cardiac function as judged clinically, and as measured by echocardiography. As far as we know, this is one of the youngest patients yet reported to have a good recovery.
Rapid formation of collateral arteries in a neonate with interruption of the aortic arch
Authors: Starreveld J. Sander , van Rossum Albert C. , Hruda Jaroslav
Published: August 2006

In a neonate born prior to term with a weight of 1825 grams, and diagnosed prenatally as having atrioventricular septal defect and Down's syndrome, we found the aortic arch to be interrupted between the left carotid artery and the left subclavian artery, with the arterial duct being the only route of distal perfusion. Three days later, however, echocardiographic interrogation revealed marked collateral connections between the aortic arch and the descending aorta, the picture then mimicking coarctation rather than interruption of the aortic arch. The rapid development of the collateral arteries was confirmed by magnetic resonance imaging and during cardiac surgery.
Balloon angioplasty of critical coarctation in a 970-gram premature infant
Authors: McMahon Colin J. , Alromani Ahmed , Nihill Michael R.
Published: August 2006

We describe a 970-gram infant born prematurely at 28 weeks gestation. On the third day of life, she presented in heart failure with Shone's syndrome and severe coarctation of the aorta. Despite initiation of intravenous prostaglandins, she developed progressive severe biventricular dysfunction and acidosis. Balloon angioplasty of the coarctation was successfully performed on the fifth day of life, with a dramatic improvement in ventricular function. At seven weeks of age, after improvement of ventricular function, she underwent surgical resection of the coarctation and arch advancement. At six months of age she is thriving and developing normally.
A national network for the tele-education of canadian residents in pediatric cardiology
Authors: Finley J. P. , Beland M. J. , Boutin C. , Duncan W. J. , Dyck J. D. , Hosking M. C. K. , Nykanen D.
Published: August 2006

A trial of 11 video-conferenced teaching sessions for residents in pediatric cardiology was performed by the 7 training programs in Canada in order to share expertise in specialized areas, to expose trainees to educational telemedicine, and to acquaint residents with other programs and personnel. Topics included cardiac pathology, arrhythmias, magnetic resonance imaging, fetal physiology, pulmonary hypertension, and cardiomyopathy. The sessions were evaluated by 93 residents by questionnaire for content and technology. Session content was highly rated. Videoconference picture quality was highly rated, but sound quality and visual aids were rated as neutral or unsatisfactory by a significant minority,related to problems with several early sessions, subsequently corrected. 60% of respondents rated the videoconferences as good as live presentations. Presenters were generally satisfied although they required some adjustments to videoconferencing. The average cost per session was $700 Canadian. Videoconferencing of resident educational sessions was generally well accepted by most presenters and residents, and the trial has formed the basis for a national network. Adequate organizational time, and careful attention to audiovisual needs, are most important. Videoconference guidelines are suggested for presenters based on this experience.
Pseudoachondroplasia in a child with prolapse of the mitral valve
Authors: Karakurt Cemşit , Karademir Selmin , Üner Çiǧdem
Published: August 2006

Pseudoachondroplasia is an autosomal dominant variant of osteochondroplasia that results in mild to severe short-limb dwarfism and early-onset of osteoarthrosis. It has been linked to results from mutations in the gene for cartilage oligomeric matrix protein. We describe a 4-year-old boy with pseudoachondroplasia who also had prolapse of the mitral valve. To the best of our knowledge, this association has not previously been reported.
Living morphogenesis of the ventricles and congenital pathology of their component parts
Authors: de la Cruz María V. , Markwald Roger R. , Krug Edward L. , Rumenoff Lila , Gómez Concepción Sánchez , Sadowinski Stanislaw , de Jesús Galicia Teresa , Gómez Fernando , García Marcela Salazar , Guzmán Laura Villavicencio , Angeles Leticia Reyes , Moreno-Rodriguez Ricardo A.
Published: August 2006

Living morphogenetic studies show that each definitive ventricle is constructed from different primitive cardiac segments, and each has its specific anatomical features. These ventricular segments are the atrioventricular junction; the primitive inlet segment, part of the primary heart tube, which initially provides the inlets of each ventricle; the primitive outlet segment, which gives rise to both ventricular outlets; and the apical trabeculated regions of the right and left ventricles which grow from the primary heart tube, respectively. In this review, we describe regional pathology based on the relationship of these primitive ventricular components. We propose that the abnormal morphogenesis of one of these segments gives origin to regional ventricular pathology. For example, abnormal embryogenesis of the atrioventricular canal produces malformations of the atrioventricular junctions, such as double inlet ventricle, absence of one atrioventricular connection, and straddling and overriding atrioventricular valves. Similarly, abnormal morphogenesis of the primitive outlet segment gives rise to malformations of the subarterial region of each ventricle, along with the valves guarding these vessels. The principal anatomical features of these malformations of the ventricular inlets and outlets are described, and their possible morphogenesis is discussed. Due to the fact that the apical trabeculated region of each ventricle arises from a separate primitive segment, each ventricle can be identified according to the pattern of its apical trabeculations. This feature is crucial in the elucidation of complex congenital pathology, such as discordant atrioventricular connections.