bluuebunny/crossref_metadata_2025 huggingface dataset:
Impact of the characteristics of patients and their clinical management on outcomes in children with homozygous familial hypercholesterolemia
Authors: Al-Shaikh Adnan M. , Abdullah Mustafa H. , Barclay Andrew , Cullen-Dean Geraldine , McCrindle Brian W.
Published: August 2006

Objective: To relate clinical factors to the development of cardiovascular atherosclerosis for patients with homozygous familial hypercholesterolemia. Background: Homozygous familial hypercholesterolemia is associated with extreme elevations in levels of cholesterol causing aggressive atherosclerosis. Methods: We reviewed 10 children, 8 of whom were male, assessed at a single institution. We found that individual characteristics, levels of lipid, cardiovascular investigations, and management were related to the activity of low density lipoprotein receptors. Results: Activity of low density lipoprotein receptors was defined as absent, being less than 2% of normal, in 4 patients who presented at the ages of 0.3, 1.4, 1.8, and 4.5 years, respectively. The activity was minimal, representing 5%–30% of normal, in another 4 patients presenting at the ages of 6.1, 9.6, 9.9, and 12 years, and was undetermined in 2 patients who presented at the ages of 3.5, and 12.1 years. Levels of low density lipoprotein cholesterol at presentation ranged from 12.2 to 24 millimoles per litre. Plasmapheresis was performed bi-weekly in 9 patients. Patients with absence of receptor activity were less likely to have a serial decrease in the levels of low density lipoprotein cholesterol prior to plasmapheresis, and one of these patients was increased to weekly plasmapheresis. In addition, they had more aggressive cardiovascular involvement of the coronary arteries, aortic valve and aorta, requiring surgical intervention at the age of 8 and 12 years in 2 patients, with sudden death at the age of 3.1 years in one patient. In contrast, 1 patient with minimal receptor activity had surgical intervention at the age of 16.6 years and another patient died suddenly at the age of 33.6 years. Conclusion: Complete cardiac assessment is recommended at presentation. The frequency of plasmapheresis should be adjusted according to the activity of low density lipoprotein receptors and the individual response of the patient.
Late neointimal proliferation following implantation of stents for relief of pulmonary arterial stenosis
Authors: Tomita Hideshi , Yazaki Satoshi , Kimura Kohji , Ono Yasuo , Yagihara Toshikatsu , Echigo Shigeyuki
Published: August 2006

On the assumption that the diameter of the reference vessel might determine the thickness of neointimal coverage of stents placed in the pulmonary arteries, we analyzed the angiograms of 28 lesions in 17 patients who underwent follow-up cardiac catheterization. Excluding 2 lesions where late stenosis was determined mainly by recoil of greater than 30%, we investigated the neointimal thickness of 26 lesions in 16 patients. Several factors that might contribute to late re-stenosis were also analyzed. Age and body weight at implantation ranged from 0.8 to 20 years, with a median of 6 years, and from 6.8 to 77.5 kg, with a median of 17.6 kg. Follow-up interval was from 6 to 15 months, with a median of 6 months.

There was a significant increase in diameter, as well as a reduction in pressure gradient, immediately after the implantation of stents. Although there was no significant difference between the achieved diameter and the diameter of the stent at follow-up, the diameter of the lesion at follow-up was significantly smaller than the diameter achieved by stenting. The increase in the pressure gradient at follow-up was slightly greater in 4 lesions where the late reduction in diameter was greater than 30% than in the 18 lesions where this was less than 30% (p = 0.05). The diameter of the reference vessel, and the diameter by stenting correlated with the late reduction in diameter. All lesions with diameter reduced greater than 30% had a reference diameter of less than 6.1 mm, and a diameter achieved by stenting of less than 6.5 mm. Late loss in luminal diameter directly correlated with the thickness of the neointimal coverage. In conclusion, close observation should be mandatory following implantation of stents in small pulmonary arteries.
Soluble endothelial adhesion molecules during paediatric cardiovascular surgery with or without cardiopulmonary bypass
Authors: Osmancik Pavel , Hambsch Jörg , Schneider Peter , Bellinghausen Wilfried , Tarnok Attila
Published: August 2006

Background: Paediatric cardiovascular surgery with or without cardiopulmonary bypass induces a complex pattern of pro- and anti-inflammatory responses. It is suspected that they may contribute to changes on the vascular endothelium. The endothelial response to cardiosurgical trauma and cardiopulmonary bypass, especially in children, has yet to be well established. Patients and methods: We studied 29 children undergoing cardiovascular surgery with cardiopulmonary bypass, comparing them with 21 not undergoing bypass. The groups did not differ significantly with respect to age, sex, weight and preoperative parameters. Blood samples were drawn 24 h before surgery, after onset of anaesthesia, after onset of cardiopulmonary bypass and after rewarming in those undergoing bypass, or immediately after surgery in the control group, 4 h and 2 days after surgery, at discharge, and months after surgery during out-patient follow-up. Serum levels of soluble E-selectin, P-selectin and P-selectin glycoprotein ligand-1 were measured by enzyme-linked immunoassay. Results: Paediatric cardiovascular surgery leads perioperatively to the significant decreases of the serum levels of soluble P- and E-selectin, as well as of soluble P-selectin glycoprotein ligand-1 (all p < 0.05). The time course, and all concentrations, of these molecules were not significantly different with and without bypass. The decreases, however, were more pronounced with cardiopulmonary bypass. Preoperative baseline values were reached months after surgery. Conclusion: Endothelial activation of release of adhesion molecules is reduced during paediatric cardiovascular surgery. Endothelial activity is more perturbed with cardiopulmonary bypass and for a long time after surgery.
Anomalous subaortic course of the left brachiocephalic (innominate) vein: echocardiographic diagnosis and report of an unusual association
Authors: Leal Samira Morhy Borges , Andrade Jose L. , de Souza Mirna , Soares Andressa Mussi , Tavares Glaucia M. Penha , Afiune Jorge Yussef , Guerra Vitor Coimbra , Demarchi Lea M. Ferreira , Mathias Wilson
Published: August 2006

Subaortic left brachiocephalic, or innominate, vein is an uncommon finding in congenital heart disease, usually associated with obstruction of the right ventricular outflow tract. We describe our experience with 14 patients in whom the lesion was identified echocardiographically, 12 of them with right ventricular obstruction, one with totally anomalous pulmonary venous connection in the absence of obstruction to the right ventricular outflow tract, and the final one with a normal heart. A precise diagnosis of this venous anomaly is of great importance, since it needs to be differentiated from a central pulmonary artery, a pulmonary venous confluence, or an ascending vertical vein in totally anomalous pulmonary venous connection. In patients referred for surgery without catheterization, an incorrect echocardiographic diagnosis could lead to disastrous surgical results.
Transcatheter creation of an atriopulmonary communication in the Hemi-Fontan or Glenn circulation
Authors: McMahon Colin J. , El Said Howaida G. , Mullins Charles E.
Published: August 2006

There have been several modifications of the Fontan procedure, which has been used for palliation of the functionally univentricular circulation since its conception in the early 1970s. The development of intrahepatic veno-venous fistulas, pulmonary arteriovenous fistulas, and communications from the hepatic vein to the pulmonary venous atrium, are some of the complications encountered by patients with cavo-pulmonary communications. We describe transcatheter creation of an atriopulmonary communication, with simultaneous implantation of a stent, as a method of reducing or preventing pulmonary arteriovenous fistulas and associated right-to-left shunting in this setting. The intervention has been undertaken in two patients following a Hemi-Fontan procedure, itself created subsequent to a Glenn anastomosis. The combination of these procedures creates communicating atriopulmonary and cavopulmonary circulations.
Supplementation of thyroid hormone in children undergoing cardiac surgery
Authors: Mainwaring Richard D. , Nelson Jerald C.
Published: August 2006

Triiodothyronine has been introduced as a promising new supplement for patients undergoing openheart surgery. This manuscript will review the existing literature regarding supplementation with triiodothyronine in children.

Infants and children undergoing cardiac surgery experience a profound decrease in levels of free and total triiodothyronine. These decreases are of sufficient magnitude to effect cardiac function. Supplementation with triiodothyronine has been shown to enhance myocardial performance, improve recovery of circulating levels of triiodothyronine, and to result in an increase in mixed venous oxygen saturation. As encouraging as these results are, there is no definitive study which demonstrates that triiodothyronine improves clinical outcome in children. Thus, while triiodothyronine appears to have promise as a supplement for children undergoing openheart surgery, more studies are needed to establish the efficacy of such therapy scientifically.
Dissolution of tungsten coils does not produce systemic toxicity, but leads to elevated levels of tungsten in the serum and recanalization of the previously occluded vessel
Authors: Peuster Matthias , Fink Christoph , von Schnakenburg Christian , Hausdorf Gerd
Published: August 2006

Aim: To evaluate the failure of mechanically detachable spirals produced from tungsten (MDS, Balt, Montmorency, France) and the toxicity of elevated levels of tungsten in the serum subsequent to their implantation. Methods: We reviewed findings in 21 patients in whom tungsten coils had been used to occlude pathologic vessels, aneurysms and fistulas between 1996 and 1999. We achieved clinical follow-up, and measured renal and hepatic function, in 14 of the 21 patients. Results: Decreased radiopacity of the coils was observed in 9 of 13 patients who had follow-up fluoroscopy during repeat cardiac catheterization. Repeat angiography of the vessel occluded by the coil was performed in 7 patients, 5 of whom showed recanalization. Levels of tungsten in the serum were analyzed 6 to 35 months after implantation of coils in 8 patients. The mean concentration was 6.43 µg/l, with a range from 2 to 14.4 µg/l, normal values being less than 0.2 µg/l. Conclusion: Tungsten coils may dissolve over time and lead to markedly elevated levels of tungsten in the serum, with recanalization of previously occluded vessels. Despite lack of clinical and laboratory data in patients with elevated levels of tungsten in the serum, our study suggests that the clinical use of mechanically detachable coils produced from tungsten should no longer be recommended.
End-systolic wall stress is a major determinant of postoperative left ventricular dysfunction in patients with congenital mitral regurgitation
Authors: Murakami Tomoaki , Nakazawa Makoto , Nakanishi Toshio , Momma Kazuo
Published: August 2006

To clarify the contribution of afterload to left ventricular performance after repair of mitral regurgitation, we evaluated echocardiographically 8 children who had undergone surgical repair for isolated congenital mitral regurgitation. We examined the relationship between left ventricular systolic function and preload, afterload, and contractility. The left ventricular systolic function was strongly correlated with the afterload after the surgical repair. In the postoperative state, reducing afterload by vasodilators could be a useful means of treating cardiac failure, in addition to using catecholamines to increase the contractility.
Preoperative identification of coronary arterial anatomy in complete transposition, and outcome after the arterial switch operation
Authors: McMahon Colin J. , El Said Howaida G. , Feltes Timothy F. , Watrin Carmen H. , Hess Beth A. , Fraser Charles D.
Published: August 2006

Background: Perceived correlation between the coronary arterial anatomy in patients with complete transposition, and the outcome of the arterial switch procedure, has made preoperative identification of their patterns standard practice. Purpose: Our purpose was to assess the accuracy of preoperative echocardiographic identification of coronary arterial patterns, to evaluate the necessity of preoperative imaging by angiography, and to determine the impact of the coronary arterial anatomy on outcome. Methods: We reviewed the medical records of all patients referred for an arterial switch operation between August 1995 and January 2000. The anatomy as described at the time of the operation using the Leiden convention was compared to the preoperative echocardiographic and angiographic findings. Results: The procedure had been performed in 67 patients, at a mean age of 9 days, with a range from 3 days to 15 months. In 42 patients, the ventricular septum was intact, while 21 patients had a ventricular septal defect, and the other four had double outlet right ventricle with the aorta anterior and rightward. In 52 patients, the left coronary artery arose from sinus #1, and the right from sinus #2. In 8 patients, the interventricular branch of the left coronary artery arose from sinus #1, with the circumflex coronary artery arising together with the right coronary artery from sinus #2. In three patients, all three coronary arteries arose from sinus #1, while in the remaining individual patients, a large conal branch arose with the left coronary artery from sinus #1, the right coronary and left anterior descending arteries arose from sinus #1, all three coronary arteries took origin from sinus #2, and the left anterior descending and right coronary artery arose from sinus #1 with no circumflex coronary artery identifiable, respectively. In two patients (4%), we identified an intramural coronary arterial course. Echocardiography and angiography were comparable (81% versus 86%) in delineating the coronary arterial anatomy. Patients with a single arterial orifice, or an atypical coronary arterial anatomy, had a slightly longer stay on the intensive care unit, and in the hospital, but showed no difference in mortality. In fact, there was no early mortality (70% confidence limits; 0–2.9%), while two patients died late (2.9%). Conclusion: We conclude that complex coronary arterial anatomy does not preclude a successful arterial switch procedure, although patients with a single coronary artery, or other arterial patterns, had a slightly longer hospital course. Preoperative echocardiographic evaluation is comparable to non-selective coronary angiography. Irrespective of complexity, nonetheless, the coronary arteries can successfully be translocated, obviating the need for preoperative coronary angiography.
Epidemiology of cardiomyopathies in children and adolescents: a retrospective study over the last 10 years
Authors: Malčić Ivan , Jelušić Marija , Kniewald Hrvoje , Barišić Nina , Jelašić Dražen , Božikov Jadranka
Published: August 2006

We conducted a retrospective study at the Department of Paediatric Cardiology of the University Hospital Centre Rebro, Zagreb, over the period from 1988 to 1998, so as to assess the epidemiology of childhood cardiomyopathies. The patients were categorized according to the guidelines of the Task Force on Cardiomyopathies of the World Health Organization and the International Society and Federation of Cardiology. We identified 121 infants, children and adolescents as having cardiomyopathy, giving an average occurrence for all cardiomyopathies of 38.81 for each 10,000 patients examined in our outpatient clinics for paediatric cardiology. Of the patients, 50 were female (41.3%) and 71 were male (58.7%). The cardiomyopathy was of the dilated variant in 52 patients (42.9%), with 43 patients (35.5%) having hypertrophic cardiomyopathy, and 6 patients (4.8%) identified with restrictive cardiomyopathy. We encountered no patients with arrhythmogenic right ventricular cardiomyopathy. In nine patients (7.4%), it proved impossible to classify the cardiomyopathy. We placed 11 patients (9.0%) in the group of specific cardiomyopathies. Most of those with dilated cardiomyopathy had been diagnosed prior to the age of 3 years (RR 1.9, 95% CI 1.4–2.47). There were no statistically significant differences in the incidences of dilated as compared to hypertrophic cardiomyopathy (Z 0.923, p = 0.1779), but we encountered a significantly lower occurrence of restrictive cardiomyopathy (Z 6.044, p < 0.001). Of those with hypertrophic cardiomyopathy, 15 patients (34.8%) had the asymmetric variant, while 28 patients (65.2%) exhibited the concentric form. During the period of follow-up, 10 patients died, 4 with dilated cardiomyopathy, 4 with hypertrophic cardiomyopathy, 1 with restrictive cardiomyopathy, and 1 with a specific cardiomyopathy. We encountered 12 (9.9%) patients who, besides cardiomyopathies, also suffered from neuromuscular disorders. Most of these had dilated cardiomyopathy. Mitochondrial disorders, in contrast, were more frequently found in patients with hypertrophic cardiomyopathy.
Echocardiographic evaluation, management and outcomes of bilateral arterial ducts and complex congenital heart disease: 16 years' experience
Authors: Peirone Alejandro , Abdullah Mustafa M. , Dicke Frank , Freedom Robert M. , Smallhorn Jeffrey
Published: August 2006

Bilateral arterial ducts represent an uncommon form of pulmonary or systemic blood supply associated with complex congenital cardiac disease. We sought to determine the role of high-resolution cross-sectional echocardiography and color flow Doppler ultrasonography in assessing this condition, as well as to describe the management and outcome in a group of patients. A retrospective review was conducted of 11 newborns identified over a 16-year period as having bilateral arterial ducts. Pulmonary atresia associated with non-confluent pulmonary arteries was the dominant lesion, with the heterotaxy syndrome also frequently being recognized. Echocardiography best identified the source of blood supply to either the pulmonary or systemic circulations, allowing differentiation from collateral vessels. Stenosis of the right or left pulmonary artery at the initial site of ductal insertion needs careful evaluation on follow-up. Management of patients with this condition remains a challenge, as indicated by the poor outcomes observed in our series.
Isolated non-compaction of the myocardium diagnosed in the fetus: two sporadic and two familial cases
Authors: Moura Claudia , Hillion Yvette , Daikha-Dahmane Farida , Eydoux Patrice , Fallet Catherine , Oury Jean François , Azancot Annabelle
Published: August 2006

Isolated non-compaction of the ventricular myocardium is characterized by numerous and prominent trabeculations and deep intertrabecular recesses. This rare disease is due to an arrest of myocardial morphogenesis. Most cases, when seen in children, are associated with obstructive malformations. Isolated non-compaction is even rarer in childhood, and affects predominantly the myocardium of the left ventricle. Morbidity and mortality resulting from cardiovascular complications is high. In most cases, transplantation is the final option. To our knowledge, this rare cardiac malformation has yet to be diagnosed in the fetus. We report here two sporadic cases, one male and one female, and 2 familial cases, both male, which were diagnosed prenatally and followed by fetal echocardiography. Our study indicates that isolated non-compaction is a primary disorder of early fetal development. Our cross-sectional echocardiographic examinations revealed a fetal cardiomyopathy, with prominent and numerous trabeculations and deep intertrabecular recesses of the myocardium at the apex of the ventricles. In contrast with postnatal experience, we found isolated non-compaction mostly in the right ventricle. Systolic dysfunction was found in all cases. The diagnosis was confirmed by histology in 3 fetuses dying with cardiac failure, and by postnatal cross-sectional echocardiography in the fetus who survived. Two male fetuses belonged to a family in which 3 individuals were subsequently found to be affected. We discuss the issues of prenatal diagnosis, natural history, and myocardial histology.
Familial primary hypertrophic osteoarthropathy in association with congenital cardiac disease
Authors: Levin Solomon E. , Harrisberg Jeffrey R. , Govendrageloo Kenny
Published: August 2006

It is rare to find congenital cardiac disease in association with familial primary hypertrophic osteoarthropathy. We have now encountered three siblings, two of whom had digital clubbing, patent arterial ducts and delayed closure of the cranial fontanels. The third infant was unusual in that there was no clubbing, or cranial abnormality, despite a small ventricular septal defect. To the best of our knowledge, this association has not previously been observed.
Intrahepatic right-to-left shunting after the Fontan operation
Authors: Giamberti Alessandro , Anderson Robert H. , de Leval Marc R.
Published: August 2006

We describe two patients with right isomerism, corrected with a fenestrated Fontan operation, who suffered severe progressive cyanosis. Cardiac catheterisation in both revealed a massive right-to-left shunt from the inferior caval vein, through the liver, to a hepatic vein draining directly to the left side of the intra-atrial baffle. The anomalous vein was successfully ligated in both patients.
Refinements in the implantation of pulmonary arterial stents: impact on morbidity and mortality of the procedure over the last two decades
Authors: McMahon Colin J. , El Said Howaida G. , Vincent Julie A. , Grifka Ronald G. , Nihill Michael R. , Ing Frank F. , Fraley J. Kennard , Mullins Charles E.
Published: August 2006

Introduction: There is limited data on medium to long-term outcome, and the morbidity and mortality associated with the implantation, of pulmonary arterial stents. Purpose: To assess changes in morbidity and mortality over the last two decades. Methods: Retrospective analysis of all patients stented between September, 1989 and July, 2001. Results: We implanted 664 Palmaz stents in 338 patients. The overall number included 229 patients who had undergone repair of tetralogy of Fallot, in whom 468 stents were implanted, 61 patients with congenital stenosis of the branches of the pulmonary trunk, in whom we placed 115 stents, 16 patients after an arterial switch operation who had 38 stents, and 32 patients after the Fontan operation who had 43 stents implanted. The mean age was 12.2 years, and the mean weight was 38 kg. The mean systolic pressure gradient decreased from 41 to 8.7 mmHg, the mean diameter of the stented vessel increased from 5.4 to 11.2 mm, and the ratio of right ventricular to femoral arterial pressure decreased from 0.66 to 0.45, each of these being significant at the level of p being less than 0.01. At a mean follow-up of 5.6 years, the mean gradient was 20 mmHg, the mean ratio of pressure between right ventricle and femoral artery was 0.5, and mean luminal diameter was 9.3 mm. Complications included migration of the stent in 8 patients, and pulmonary edema, hemoptysis and death in 5 patients each. There has been no mortality or morbidity since July of 1997. Technical changes include conservative serial dilations in congenital pulmonary arterial stenosis, avoidance of over-dilation, and simultaneous implantation of stents in the right and left pulmonary arteries in those with systemic pulmonary arterial pressure. Technological advances included shorter stents, improved balloon profiles, and central inflation of the stents. Conclusions: Modification of stenting practices, and increased experience of the operators over the last two decades, has virtually abolished any morbidity or mortality associated with the implantation of stents for congenital or postoperative pulmonary arterial stenoses.
Mortality and risk factors for late deaths in tetralogy of Fallot: the Japanese Nationwide Multicentric Survey
Authors: Niwa Koichiro , Hamada Hiromichi , Nakazawa Makoto , Terai Masaru , Tateno Shigeru , Sugimoto Satoru , Watanabe Hiroshi , Murakami Arata , Ohta Mayumi , Ishizawa Akira , Katoki Toshiyuki , Mori Katuhiko , Yasui Seiyo , Kawahira Youichi , Akagi Teiji , Haraguchi Naho , Gatzoulis Michael A
Published: August 2006

Objectives: We have compared mortality and risk factors for late deaths in patients with tetralogy of Fallot undergoing surgical repair in 1972 and 1982 in a Japanese multicentric study, examining in particular the impact of time of repair. Background: There is limited information on the effect that time of repair, and our constantly changing approach to it, has on late outcome in repaired tetralogy of Fallot. Methods: We analysed the Japanese registry of deaths occurring after surgical repair of tetralogy of Fallot. We studied two postoperative 1-year cohorts of survivors of surgery performed in 12 centers. Of the patients, 122, aged 29 ± 12 years, had undergone repair in 1972, their age at repair being 9.6 years. An additional 186 patients, aged 23 ± 8.7 years, had been repaired in 1982 at the age of 7.7 years. Results: Annual mortality, as judged per 100,000 population of patients with tetralogy of Fallot, declined from 0.387 in 1972 to 0.196 in 1982. Significant differences were deaths following surgery (27% vs. 13%, p < 0.001), patching of the subpulmonary outflow tract (48% vs. 89%, p < 0.001), and transjunctional patching (13% vs. 63%, p < 0.001). Late death was observed in 6 vs. 3 patients (9/308, 2.9%). The actuarial rate of survival calculated over 14 years was 97% vs. 98%. Reoperation was performed in 5 vs. 9 patients (14/308, 4.5%). Risk factors for late death were age at repair (p = 0.01), and history of reoperation (p < 0.001). Transjunctional patching (p = 0.01) proved to be associated with late mortality only in patients repaired in 1972. Conclusions: Late survival was excellent, with a low incidence of reoperations in both groups of patients. The era of repair has a big influence on total and operative mortality, but has only a small impact on late mortality. Ongoing analysis of follow-up will possibly reveal subsequent changes with time.
The need for cardiac follow-up in adults with mild congenital cardiac disease
Authors: Kamphuis Mascha , Vliegen Hubert W. , Vogels Ton , Zwinderman Koos H. , van der Wall Ernst E. , Verloove-Vanhorick S. Pauline , Ottenkamp Jaap
Published: August 2006

Objective: To discuss the need for continuing cardiac surveillance in adults with hemodynamically insignificant congenital cardiac disease. Methods: In 82 patients with mild congenital cardiac malformations, aged from 17 to 32 years, we investigated the subjective health status, the health-related quality of life, any difficulties encountered in daily life, the regularity of follow-up, current diagnosis, and antibiotic prophylaxis. Results: The subjective health status, and the health-related quality of life, did not differ from those of the general population. Nevertheless, patients experienced unnecessary difficulties with choice of sport, obtaining insurance cover, and education. After clinical re-evaluation, diagnosis and antibiotic regimes had to be changed in 9 patients (11%). Conclusions: Patients with a mild congenital cardiac malformation consider themselves to be in good health. So as to fine tune the diagnosis, and update the information supplied to the patients, we suggest that at least a cardiological assessment be carried out at the age of 16 to 18 years. In this way, patients might be protected from unnecessary difficulties, such as restrictions for sport or the charging of unjustifiably high rates for insurance.
Pulmonary arterial and intracranial calcification in the recipient of a twin–twin transfusion
Authors: Kei Olivia C. Y. , Duncan Walter J. , Human Derek G.
Published: August 2006

Pulmonary arterial and intracranial calcifications are rarely found in children. A female infant, the recipient of a twin–twin transfusion syndrome was found, by ultrasound and computed tomography, to have both pulmonary arterial and intracerebral calcification. A rare condition, termed idiopathic arterial calcification of infancy, is the likely cause. This condition carries a poor prognosis and is usually fatal.
Low prevalence of femoral venous thrombosis after cardiac catheterizations in children: a prospective study
Authors: Ruud Ellen , Natvig Solveig , Holmstrøm Henrik , Wesenberg Finn
Published: August 2006

Objective: Cardiac catheterization is an important and frequent diagnostic intervention in children, but few systematic studies have explored the associated venous thrombotic complications. We have prospectively evaluated the prevalence of venous thrombosis, diagnosed by ultrasonography, in children catheterized at our department. Materials and methods: We examined 50 children with weight greater than seven kilograms for thrombosis of the femoral vessels. Prophylactic anticoagulation was given to half of the children who were to undergo left-sided catheterization. The enrolled children had ultrasonography of the site of puncture the day before, and the day after, the cardiac catheterization. During catheterization, blood samples were taken from 33 of the children for analysis of thrombophilic agents. Results: The children, with a median age of 34 months, had been catheterized 103 times, comprising the 50 current and 53 previous procedures. Over the course of the current procedure, interventions of various types were performed in 18 children (36%). We diagnosed thrombophilic predispositions in four patients. Despite the thrombophilic tendencies, and a high frequency of interventions, we did not encounter a single case of femoral venous thrombosis. Nor did we find any evidence of arterial thrombosis. Conclusion: Our study indicates that catheterization procedures in children are currently associated with a low prevalence of femoral venous thrombosis. Continuous assessment of vascular complications, nonetheless, is still required to prevent late effects, and to ensure the best quality of cardiac catheterisations in children.
Quality of life after repair of tetralogy of Fallot
Authors: Walker Woolf T. , Temple I. Karen , Gnanapragasam James P. , Goddard Jonathan R. , Brown Elspeth M.
Published: August 2006

Objective: To determine the quality of life in individuals with corrected tetralogy of Fallot. Methods and subjects: Questionnaires concerning quality of life were sent to all 87 surviving patients aged between 16 and 40 years who had undergone intracardiac repair of tetralogy of Fallot and follow-up in the Wessex Cardiothoracic Unit, and to 87 age and sex matched controls, with medically treated haemodynamically insignificant ventricular septal defects. Results: The only significant difference found between the cases and controls was in requirements for schooling, where those with tetralogy of Fallot were more likely to require additional educational help at school (p = 0.044). For all other aspects of quality of life examined by the questionnaire, including social and genetic history, exercise ability, and health related quality of life, no significant differences were found. Different operative techniques, such as transjunctional patching, right ventriculotomy, and previous palliative shunting, did not affect the quality of life of our population with Tetralogy of Fallot, on average twenty years after their surgery, although the range of operative techniques was limited. Neither age at surgery, nor time since surgery, was correlated with measurements of quality of life. Conclusions: Those who have undergone surgical correction of tetralogy of Fallot have a normal quality of life, with few differences compared to controls.
Pulmonary hypertension due to spontaneous premature ductal constriction in fetal life: association with right bundle branch block
Authors: Benettoni Alessandra , Rustico Maria Angela
Published: August 2006

We describe a case of fetal pulmonary hypertension and tricuspid regurgitation due to non pharmacologically induced ductal constriction observed at 36 weeks' gestational age. The hypertension resolved spontaneously soon after birth, with no functional consequences. Right bundle branch block is the only permanent anomaly, still being seen on the electrocardiogram at the age of 34 months.
Giant fibroma of the right ventricle
Authors: Zidere Vita , Lubaua Inguna , Lacis Aris
Published: August 2006

Cardiac fibromas are rare benign tumours of connective tissue that occur most frequently in children within the left ventricle. Spontaneous regression has not been observed, and surgical intervention is usually required. We have successfully treated a 1-year old girl with a giant fibroma of the right ventricle using the principles of the Batista procedure. The diagnosis was primarily made using transthoracic echocardiography.
Innocent murmurs: the perception of the parents versus that of the child
Authors: Advani Najib , Menahem Samuel , Wilkinson James L.
Published: August 2006

Innocent murmurs in childhood are common, and often they do not reflect cardiac disease. We have performed a prospective review, by means of a questionnaire, to clarify the parental perception of the innocent murmur identified in their child. Whenever possible, depending on age, we also sought the input of the child.

Of a cohort of 63 infants and children with innocent murmurs, a response was obtained from 40 parents and 8 children to an initial questionnaire, with 26 parents replying to a second questionnaire a year later. No parent reported any symptoms in their children, or had imposed any restrictions. Of the parents, however three sets considered regular review was necessary. We conclude that the diagnosis of an innocent murmur by a paediatric cardiologist, aided by investigations including cross-sectional echocardiography, and an opportunity for explanation and reassurance, led to the child being treated normally.
Successful transluminal angioplasty of renal arterial stenosis using the transcarotid approach
Authors: Takigiku Kiyohiro , Satomi Gengi , Yasukochi Satoshi
Published: August 2006

We successfully performed percutaneous transluminal angioplasty to treat severe renovascular hypertension with left ventricular failure in a 5-month-old infant. Using the transcarotid approach, we dilated the stenotic left renal artery without any difficulties, using progressively larger balloons designed for dilation of coronary arteries.
Report from the Task Force of the European Society of Cardiology for the interpretation of the neonatal electrocardiogram
Authors: Schwartz Peter J. , Garson Arthur , Paul Thomas , Stramba-Badiale Marco , Vetter Victoria L. , Villain Elisabeth , Wren Christopher
Published: August 2006

The prevention of unexpected cardiac events in the young remains elusive. Among them, some are lethal. Early identification of life-threatening arrhythmogenic disorders which often manifest in infancy, childhood or even later, may allow initiation of effective preventive therapies. A large prospective study has indicated that some infants with prolongation of the QT interval in the first week of life died suddenly, and would have previously been labelled as victims of the sudden infant death syndrome. Furthermore, in infants with this diagnosis, post-mortem molecular screening has revealed the presence of the long QT syndrome. As an evolution of this background, some European countries have begun to consider the possibility of introducing in their National Health Services the performance of an electrocardiogram during the first month of life in all newborns, as part of a programme for cardiovascular screening. Most adult cardiologists, however, have no or minimal experience with electrocardiograms recorded in infants. Accordingly, the European Society of Cardiology has instituted a Task Force with the objective of creating guidelines for the interpretation of the neonatal electrocardiogram, focusing on the most clinically relevant abnormalities and on the ensuing options for management and referral. The main objective of the present document is to provide adult cardiologists with a practical approach to neonatal electrocardiography, and paediatricians and neonatologists with a tool that should facilitate medical interaction on cardiologic issues. There are important differences between neonatal and adult electrocardiograms. When a cardiologist examines the electrocardiogram of an apparently normal and healthy infant, the focus has to be on distinguishing between patterns that should cause no alarm, and those that require action or additional investigations. To provide clues for this distinction has been the main objective of the members of this Task Force. Whenever possible or appropriate, we have also suggested steps in management.
The Shadow of Opera: Dramatic Narrative and Musical Discourse in Gawain
Authors: BEARD DAVID
Published: August 2006

The opera Gawain (1991; revised 1994 and 1999) brought together Harrison Birtwistle and the poet David Harsent in a reworking of the late fourteenth-century narrative poem Sir Gawain and the Green Knight. That Birtwistle asked Harsent to make a large number of alterations to his original libretto has already been documented. The present article, which draws on the sketching processes of both librettist and composer, reveals the nature and the ramifications of those changes. The discussion is particularly concerned with the contradictions and multiple narrative layers that resulted from the Harsent–Birtwistle collaboration, and with the composer’s suggestion that there is both a secret drama in the orchestra, and instruments that function like unheard voices. A consideration of Birtwistle’s sketches also reveals a preoccupation with line, in particular with finding different ways of shadowing vocal and instrumental parts, and ambiguously shrouding originary lines in layers of varied reflection. These musical devices represent Birtwistle’s response to Harsent’s interest in divided subjectivity, and to the idea that Gawain should develop a sense of his own identity.
Supremacy, Debasement, and Symbolic Investiture in Lawrence Kramer's Modern ‘Opera’: a Review of Lawrence Kramer, Opera and Modern Culture: Wagner and Strauss (Berkeley and London: University of California Press, 2004), ISBN 0 520 24173 8 (hb)
Authors: Downes Stephen
Published: August 2006

Lawrence Kramer’s latest book offers a reading of ‘Opera’ and modernity in terms of a crisis of ‘symbolic investiture’. This latter concept, developed out of the work of Pierre Bourdieu and its application by Eric Santer, is defined by Kramer as ‘the process by which social institutions grasp the inner being of the individual in its essence, and in so doing both define and confer that essence […] Although often in need of later supplements to sustain its power, this process is dramatic and discontinuous, enacted in defining moments that resonate through the rest of the person’s life and “fate”’. This symbolic investiture ‘can come in moments of rapture as well as terror, enfranchisement as well as confinement, even if each of these terms always harbors traces (if not more) of its opposite’ (5). Kramer tackles his project through interpreting examples of revolt against the concept of the norm (which, as Foucault famously argued, is based on disciplinary regulations specific to modernity) in figures of variance, deviance, and difference. He is especially interested in how ‘normative’ subjectivity can be revitalized by borrowing from the ‘abnorm’; how the supreme, as a hypertrophied form of the norm, has a secret kinship with the debased. This is a topic powerfully expressed in the exceptional states of supremacy and debasement characteristic of ‘Opera’. It is tied to Opera’s emergence at the historical moment of the ascendancy of bourgeois subjectivity and its norms, regulations, and desires.
Michael Brocken, The British Folk Revival, 1944–2002 (Aldershot: Ashgate, 2003), ISBN 0 7546 3281 4 (hb), 0 7546 3282 2 (pb)
Authors: Russell Ian
Published: August 2006

For those of us who have lived with and through the second British folk music revival, it seems most apt that this important chapter of our lives should merit a serious academic history. We watched and were part of a radical youth movement that grew phenomenally in the late 1950s, burgeoned in the 60s, stumbled and stagnated through the 70s, but recovered its composure to mature in the late 80s; and now, half a century on, the fruits of this movement have become an established and significant part of the nation’s soundscape, as much a part of British culture as brass bands or choral singing. Much work has been and is being done to document every twist and turn of this revival, meticulously noting the key players, the setting up of clubs and festivals, broadcasting and recording developments, and so on; and, understandably, there is a welter of data on which to draw, including key witnesses to consult. One could argue that such histories are primarily of interest to the insider. But Brocken’s study, by contrast, attempts to take the view of the outsider, analysing the political agenda of the ‘revival’s’ architects and assessing its impact in terms of cultural studies as an aspect of popular music – in itself a most laudable enterprise.
Dietary Vitamin K Variability Affects International Normalized Ratio (INR) Coagulation Indices
Authors: Couris Rebecca , Tataronis Gary , McCloskey William , Oertel Lynn , Dallal Gerard , Dwyer Johanna , Blumberg Jeffrey B.
Published: August 2006

Background: Changes in daily vitamin K intake may contribute to marked variations in the International Normalized Ratio (INR) coagulation index in patients receiving oral warfarin anticoagulant therapy, with potentially serious adverse outcomes. Thus, patients receiving warfarin therapy are routinely counseled regarding this drug-nutrient interaction and are instructed to maintain consistent vitamin K intakes, though little quantitative information about this relationship is available. Objective: To determine the quantitative impact of variability in dietary vitamin K1 (phylloquinone) intake, assessed by a validated patient self-monitoring instrument, on weekly INR in patients receiving warfarin anticoagulant therapy. Methods: A prospective dietary assessment study was conducted at the Massachusetts General Hospital in Boston. Sixty outpatients (37 males and 23 females) were selected with a mean age 60.3 ± 16.8 years, who began oral warfarin anticoagulant therapy within 14 days prior to their first clinic visit to an outpatient anticoagulation therapy unit. Exclusion criteria included more than 2 drinks of alcohol per day, inability to speak English, and concurrent disease states affecting warfarin therapy such as liver disease and terminal illness. Over the five-week study period, participants recorded daily intakes in specified amounts of all food items appearing on a validated dietary self-assessment tool. Concomitant use of prescription and/or non-prescription medications was also obtained. Concurrent daily warfarin dose and adherence to the drug regimen, concomitant use of prescription and/or non-prescription medications known to interact with warfarin, and weekly INR were obtained. Week-to-week changes in vitamin K intake, warfarin dose, and INR were determined and cross-correlated. Results: Forty-three patients (28 males and 15 females) completed the study and 17 dropped out. Pearson’s correlation coefficient revealed the variability in INR and changes in vitamin K intake were inversely correlated (r = –0.600, p < 0.01). Multiple regression analysis (r = 0.848) indicated that a weekly change of 714 mug dietary vitamin K significantly altered weekly INR by 1 unit (p < 0.01) and a weekly change of 14.5 mg warfarin significantly altered weekly INR by 1 unit (p < 0.01) after adjustment for age, sex, weight, height, and concomitant use of medications known to interact with warfarin. Conclusions: Patients taking warfarin and consuming markedly changing amounts of vitamin K may have a variable weekly INR with potentially unstable anticoagulant outcomes.
Effects of Dietary Diacylglycerol on the Energy Metabolism
Authors: Kimura Shuichi , Tsuchiya Hitomi , Inage Hiroko , Meguro Shinichi , Matsuo Noboru , Tokimitsu Ichiro
Published: August 2006

In the present study, we characterized diacylglycerol (DAG) oil in terms of energy metabolism by comparing oxygen consumption and respiratory quotient (RQ), as well as blood parameters, in rats after administration of emulsions containing either DAG or triacylglycerol (TAG) with similar fatty acid composition. Twelve male Wistar rats (250–280 g) were acclimatized, and then catheterized into the stomach, and held individually in Bollman restraining cages. After 24 hours fasting, each emulsion containing either DAG or TAG (10 g/kg body weight) was infused. Oxygen consumption and fat oxidation in the DAG group gradually increased after administration of the DAG emulsion and became significantly higher than those of the TAG group. RQ in both groups dropped after administration of emulsion, but the values of the DAG group were significantly lower than that of the TAG group. The postprandial serum triglyceride level was significantly increased from 60 minutes after administration in the TAG group, and 240 minutes in the DAG group, compared to the preprandial level. The serum triglyceride level tended to be lower in the DAG group than in the TAG group at 60 minutes and thereafter, and the statistical difference between the TAG and DAG groups was significant at 300 minutes after administration. These results suggest that DAG oil infusion might lead to higher energy expenditure and lipids oxidation compared to TAG oil with a similar fatty acid composition.
Das primäre Adenokarzinom der Appendix als Differentialdiagnose des fortgeschrittenen Ovarialkarzinoms
Authors: Kunz  , Makek
Published: August 2006

Maligne Tumoren der Appendix sind selten. Meist handelt es sich um Karzinoide, die abzugrenzen sind gegenüber den extrem seltenen Adenokarzinomen. Metastatische muzinöse Adenokarzinome der Appendix werden nur als Kasuistiken mitgeteilt. Klinisch manifestieren sich die Tumoren meist als akute Appendizitis, als ruptierte Appendizitis, als Tumor im rechten unteren Abdominalquadranten oder als pelviner Tumor, welche bei gleichem sonographischen Bild in der Regel als Ovarialtumor verkannt werden. Intraoperativ sind fortgeschrittene primäre Adenokarzinome der Appendix mit Ovarialmetastasen von einem Ovarialkarzinom FIGO III nicht zu unterscheiden. Die definitive Diagnose wird vom Pathologen gestellt. Diese Merkmale treffen auch für den eigenen vorgestellten Fall zu. Die operative Therapie des isolierten primären Appendixkarzinoms besteht in der Hemikolektomie, in günstigen Fällen in der Appendektomie, beim metastasierenden Karzinom nach den Richtlinien des fortgeschrittenen Ovarial- oder Kolonkarzinoms. Die Wirkung der Chemotherapie ist ungenügend dokumentiert.
Uterus myomatosus als Ursache für Erythrozytose
Authors: Kunz  , Bleisch
Published: August 2006

Die myominduzierte Erythrozytose ist ein seltenes und wahrscheinlich weitgehend verkanntes Krankheitsbild. Betroffen sind prä- und postmenopausale Frauen. Charakterisiert ist das Syndrom durch erhöhte Hämoglobin- und Hämatokritwerte sowie Erythrozytose bei normalem Plasmavolumen und die Tatsache, dass sich diese Werte nach Myomektomie oder Hysterektomie prompt und anhaltend korrigieren. Normalen hämatologischen Parametern bei Patientinnen mit myombedingter Hypermenorrhoe und Menorrhagie kann ein MES (myomatous erythrocytosis syndrome) zugrunde liegen.