bluuebunny/crossref_metadata_2025_split huggingface dataset:
Univentricular repair for complex double right ventricle and transposed great arteries
Authors: Serraf Alain , Jonas Richard A , Burke Redmond P , Castaneda Aldo R , Mayer John E 
Published: August 2008

AbstractThe Fontan operation was initially designed to provide a “physiological correction” of tricuspid atresia, but its indications have been extended to more complex forms of congenitally malformed hearts. In selected patients with two adequately sized ventricles, but highly complex intra cardiac anatomy which makes a biventricular repair more hazardous and which would require multiple reoperations, a univentricular repair using Fontan's principle has been proposed. Between 1978 and 1992, a modified Fontan operation was carried out in 34 such patients. Of the patients. 13 had double outlet right ventricle and 21 had transposed great arteries. Anomalies of ventricular loop were present in 14 patients. A non-committed ventricular septal defect was found in 26 whilst multiple defects were present in 10. All patients had anomalies of the atrioventricular valves, with straddling of the tricuspid valve,11and/or of the mitral valve11, and abnormal tricuspid5, or mitral4cordal attachments. Two patients had mild tricuspid hypoplasia, 1 had mitral hypoplasia and 3 had a cleft mitral valve. Fourteen patients had anomalies of both atrioventricular valve but only 6 had mild atrioventricular valvar incompetence. Pulmonary stenosis or atresia was seem in 26. All the patients had at least one or more (mean 2.35) intracardiac anomalies that preculded a biventricular repair. The Fontan operation was performed at a median age of 4.75 years. There were 4 early (11.4%, 70% CL: 5.1–19.8) and 2 late deaths, with a 4 year actuarial survival rate of 79.6±8.1%. Univariate analysis did not reveal any risk factor for early of late death. Follow-up at a mean interval of 3.6 ±2.6 years was available in all but 1 survivor. There were 6 early and 2 late reoperations, and 3 patients required implantation of a pacemaker. In conclusion, the modified Fontan operation can provide satisfactory early and mid term results in patients with two ventricles but with complex intracardiac anatomy which precludes a biventricular repair.
Intracardiac or extracardiac conduit modification of the Fontan procedure in hearts with univentricular atrioventricular connection and left superior caval vein draining to coronary sinus
Authors: van Son Jacques A.M. , Falk Volkmar , Mohr Friedrich W. 
Published: August 2008

AbstractIn 3 patients with isomeric morphologically left atrial appendages, univentricular atrioventricular connection, concordant ventriculoarterial connections, bilateral superior caval veins, with the left one draining via the coronary sinus, together with absence of any communicating vein, interruption of inferior caval vein with drainage via a right-sided (n=2) or left-sided (n=l) azygos vein, the hepatic venous blood was rerouted via the large coronary sinus into the pulmonary arterial circulation. In a fourth patient with similar pathology, having interruption of the left-sided inferior caval vein with drainage to the left-sided superior caval vein via a left-sided azygos vein and a large communicating vein, the pathway from the left superior caval vein to the coronary sinus was correspondingly small. An extracardiac conduit was therefore constructed between the hepatic veins and the left pulmonary artery so as to reroute the hepatic venous blood into the pulmonary arterial circulation. At a mean follow-up of 8.5 months, all patients are clinically well and none of them have developed pulmonary arteriovenous malformations. To avoid the latter complication in Fontan physiology, especially in the setting of an interrupted inferior caval vein with drainage via the azygos vein, we believe that it is preferable to reroute the hepatic venous blood into the pulmonary circulation.
Catheter-based closure of atrial septal defects with a newly developed nitinol double disc: an experimental study
Authors: Bjørnstad Per G. , Smevik Bjarne , Fiane Arnt E. , Tønnessen Tor Inge , Fosse Erik 
Published: August 2008

AbstractWe report experimental use of a new device for transcatheter closure of atrial septal defects. The device was implanted in surgically created atrial septal defects in two pigs. The devices were easy to implant, and the procedures were short. Both devices were implanted correctly in the desired position, and closed the defect completely. No procedural complications nor problems were encountered. The technique is certainly worth trying in the clinical setting. The device appears to be particularly favourable for use in children because it can be implanted through a 7 French sheath.
Primary involvement of the mitral and aortic valves in Takayasu's arteritis
Authors: Bernstein Harold S. , Ursell Philip C. , Teitel David F. 
Published: August 2008

AbstractA 3-year-old boy developed acute pulmonary edema following 6 months of recurrent fevers and thrombocytosis. Echocardiography demonstrated moderate-to-severe insufficiency of both mitral and aortic valves. Angiography and intravascular ultrasound were used to diagnose this unusual presentation of Takayasu's arteritis. To the best of our knowledge, this is the first histological demonstration of acute and chronic inflammatory changes in the mitral valve in these circumstances.
Supravalvar aortic stenosis due to organizing thrombus in an asymptomatic child
Authors: Elkins Louis W. , Muehrcke Derek D. , Jonas Richard A. 
Published: August 2008

AbstractA large mass arising from the aortic valve caused supravalvar aortic stenosis in a 4-year-old girl. The differential diagnosis included an organized thrombus or myxoma. Following surgical excision, there was resolution of choreioform movements and tongue thrusting, which had been present for 1 week preoperatively.
Left atrial mass due to cancica endomyocarditis in a newborn
Authors: Şenocak Filiz , Akçayöz Ayşegül , Özkutlu Süheyla 
Published: August 2008

AbstractWe describe a case of Candida endomyocarditis in a neonate not diagnosed until autopsy. Echocardiography had demonstrated a left atrial mass. Since cardiac candidiasis seems to be occuring with greater frequency in neonatal intensive care units, echocardiography and blood cultures should be done to exclude this previously rare event.
Fatal outcome of pregnancy in the Eisenmenger syndrome
Authors: Brach-Prever Silvia , Sheppard Mary , Somerville Jane 
Published: August 2008

AbstractA mildly symptomatic 28-year-old women with Eisenmenger syndrome became pregnant against advice, and presented at 17 weeks insisting on continuing the pregnancy. She underwent an emergency Caesarean section for pre-eclampsia at 26 weeks, delivering a live 620 g boy, who survived to leave the hospital. In the post-partum period, she presented increasing hypoxemia, despite treatment with noradrenaline, nitric oxide and prostacyclin. She died 2 weeks after delivery from pulmonary haemorrhage. Necropsy showed histological signs of severe pulmonary vascular disease, with evidence of diffuse fibrinoid necrosis, vasculitis and haemorrhage.
The diagnosis of innocent murmurs in childhood
Authors: Advani Najib , Menahem Samuel , Wilkinson James L. 
Published: August 2008

AbstractInnocent murmurs are common in childhood. They require accurate diagnosis to avoid unnecessary anxiety and/or restrictions. With this in mind, we studied prospectively children diagnosed by a paediatric cardiologist as having innocent murmurs to review the necessity of investigations in excluding organic abnormalities. We included 63 children in the study. The majority had the so-called Still's, or musical/vibratory murmur. None had abnormal investigations, suggesting that such investigations are largely superfluous for the purpose of diagnosis in those patients with innocent murmurs seen by a paediatric cardiologist. They are often done, nonetheless, as part of the assessment and/or expectations of the parent or the referring physician.
Clinical and morphologic features of perimembranous ventricular septal defect with overriding of the aorta – the so-called Eisenmenger ventricular septal defect. A study making comparisons with tetralogy of Fallot and perimembranous ventricular defect without aortic overriding
Authors: Fukuda Toyoki , Suzuki Takaaki , Ito Tsutomu 
Published: August 2008

AbstractThe aim of our study was to elucidate the clinical and morphologic features of those perimembranous ventricular septal defects which extend between the ventricular outlets, particularly when found in association with anterior deviation of the muscular outlet septum and overriding of the aorta – the so called Eisenmenger ventricular septal defect. From 1990 through 1998, we have undertaken surgical correction in 203 patients with perimembranous ventricular septal defect. Of these, 15 patients had the Eisenmenger ventricular septal defect. We conducted retrospective analyses of the clinical records, catheterization data, and angiocardiographic and echocardiographic finding of these patients. Comparative studies were then made with the patients having tetralogy of Fallot, and those with simple perimembranous ventricular septal defects without overriding of the aorta. In the patients with the Eisenmenger ventricular septal defect, the extent of anterior deviation of the outlet septum was comparable with that seen in tetralogy of Fallot, but there was less rightward displacement of the aortic valvar orifice. In contrast to earlier investigators, however, we found evidence of progressive narrowing of the subpulmonary infundibulum in those with the Eisenmenger defect. These morphological features were reflected in the clinical features, since all patients showed evidence of increased pulmonary flow and congestive heart failure in early infancy, but with twothirds of them subsequently developing right-to-left shunting. We conclude, therefore, that the Eisenmenger ventricular septal defect is a discrete cardiac abnormality in which the morphologic substrate of anterior deviation of the outlet septum gives rise to a potential for progressive narrowing of the subpulmonary infundibulum. Surgical management, therefore, needs to take account of such narrowing as an additional cardinal morphologic feature.
Abnormal responses for blood pressure in children and adults with surgically corrected aortic coarctation
Authors: Hauser Michael , Kuehn Andreas , Wilson Neil 
Published: August 2008

AbstractBackgroundDespite successful surgical repair of aortic coarctation, life expectancy is reduced, and up to one-third of patients remain or become hypertensive. So as to characterize the responses for blood pressure, we have studied 55 patients with surgically repaired coarctation. Their mean age was 11.3 ± 5.97 years. We documented maximal uptake of oxygen, anaerobic threshold, plasma renin activity and blood pressures during a Bruce protocol treadmill test. The velocity across the site of repair as imaged by crosssectional echocardiography was measured before and after exercise. We measured the changes in heart rate and blood pressure subsequent to an infusion of 1 ug per kg of isoprenalin, monitoring blood pressure over 24 hours in all patients.ResultsWhen compared with 40 healthy age-matched controls, the patients with coarctation had a normal exercise capacity. Resting systolic blood pressures above the 95th percentile were present in 45% of the patients. Exercise-induced hypertension, and an elevation in the average systolic 24 hour blood pressures, were observed, but less frequently than elevated baseline values, suggesting that socalled white-coat” hypertension may be present in this population. Abnormal reactions and elevation of plasma renin activity were related to a history of paradoxical hypertension at the time of surgery. Attenuation of the circadian rhythm for blood pressure was a frequent finding, and may have implications in the development of long-term damage to end-organs. A high correlation was found between mean systolic blood pressure measured by 24 hour monitoring and left ventricular hypertrophy (r=0.65, p<0.05).ConclusionsAbnormalities in blood pressure occurred independently of significant mechanical obstruction. Despite successful surgical repair, abnormalities in the shape of the aortic arch, reduced sensitivity of baroreceptor reflexes, and neurohumoral factors may all contribute to the development of hypertension.
Outcomes for children with cardiomyopathy awaiting transplantation
Authors: Nield Lynne E. , McCrindle Brian W. , Bohn Desmond J. , West Lori J. , Coles Jhon G. , Freedom Robert M. , Benson Lee N. 
Published: August 2008

AbstractObjectiveTo determine factors associated with outcomes after listing for transplantation in children with cardiomyopathies.BackgroundChildhood cardiomyopathies form a heterogeneous group of diseases, and in many, the prognosis is poor, irrespective of the etiology. When profound heart failure develops, cardiac transplantation can be the only viable option for survival.MethodsWe included all children with cardiomyopathy listed for transplantation between 12/89 and 4/98 in this historical cohort study.ResultsWe listed 31 patients, 15 male and 16 female, 27 with dilated and 4 with restrictive cardiomyopathy, for transplantation. The median age at listing was 5.7 years, with a range from fetal life to 17.8 years. Transplantation was achieved in 23 (74%), with a median interval from listing of 54 days, and a range from zero to 11.4 years. Of the patients, 14 were transplanted within 30 days of listing. Five patients (16%) died before transplantation. Within the Canadian algorithm, one of these was in the third state, and four in the fourth state. One patient was removed from the list after 12 days, having recovered from myocarditis, and two remain waiting transplantation after intervals of 121 and 476 days, respectively. Patients who died were more likely to be female (5/5 vs. 11/26; p=0.04) and to have been in the third or fourth states at listing (5/5 vs. 15/26; p=0.04). The use of mechanical ventricular assistance, in 10 patients, was not a predictor of an adverse outcome. While not statistically significant, survival to transplantation was associated with treatment using inhibitors of angiotensin converting enzyme, less mitral regurgitation, a higher mean ejection fraction and cardiac index, and lower right ventricular systolic pressure.ConclusionsChildren with cardiomyopathy awaiting transplantation have a mortality of 16% related to their clinical state at the time of listing.
Clinical and electrophysiologic evolution of the Wolff-Parkinson-White syndrome in children: impact on approaches to management
Authors: Vignati Gabriele , Balla Eva , Mauri Luigi , Lunati Maurizio , Figini Alberto 
Published: August 2008

AbstractThere is less certainty about the recommendations for radio-frequency ablation as a therapeutic option for Wolff-Parkinson-White syndrome in children as opposed to adults because of the different natural history and the age-related risks of ablation. To help decision-making, we evaluated the long term clinical evolution and electrophysiologic characteristics of pre-excitation in our series of children and young adults. We reviewed the clinical course of 109 patients below the age of 18 years who had been followed up over a period of 9+4 years, with a range from one to 27 years. This corresponded to 986 patient-years. We examined also the electrophysiologic data from 98 of those patients who underwent a transesophageal study.At the discovery of pre-excitation, 59% of patients were asymptomatic, while 29% developed supraventricular tachycardia during follow-up. The peak incidence of the onset of supraventricular tachycardia occurred during infancy. These patients had the highest incidence of subsequent spontaneous disappearance of the tachycardia (53%), such a favourable evolution being encountered in only 12% of patients in whom the abnormal rhythm first appeared after 12 years of age. Chronic medical treatment was required in 47% of patients, and proved completely effective in 45% of cases. The potential to induce atrial fibrillation, and the incidence pre-excitation considered to be high risk, with the shortest pre-excited RR interval equal to or less than 220msec, was lowest in the group of patients aged less than 6 years of age, and highest in those older than 12 years of age (p <0.001). Pathways producing arrhythmia with high risk were more common in symptomatic (29%) than in asymptomatic patients (7%) (p<0.001). No mortality occurred. On the basis of our findings, we suggest that ablation should be avoided before the age of 5 or 6 years. Thereafter, the procedure should become the first line of treatment for symptomatic patients older than 12 years of age.
Radiofrequency catheter ablation for the treatment of supraventricular tachycardias in children and adolescents
Authors: Iturralde Pedro , Colín Luís , Kershenovich Sergio , Guevara Milton E. , Medeiros Argelia , Buendia Alfonso , Attie Fause 
Published: August 2008

AbstractWe report our experience in radiofrequency catheter ablation between April, 1992 and December, 1998, in which we treated 287 parients less than 18 years of age (mean 14.3±3.1 years) with supraventricular tachycardia. Accessory pathways were the arrhythmic substrate in 252 of the patients (87.8%), the patients having a total of 265 accessory pathways. Atrioventricular nodal re-entry was the cause of tachycardia in 26 patients (9.0%), while atrial flutter was detected in the remaining 9 patients (3.1%). We were able successfully to eliminate the accessory pathway in 236 patients (89%), but 25 patients had recurrent arrhythmias. Ablation proved successful in all cases of atrioventricular node re-entry tachycardia, the slow pathway being ablated in 25 patients, and the fast pathway in only one case. Recurrence of the arrhythmia occurred in three patients (11.5%). We performed a second ablation in these children, all then proving successful. The ablation was successful in all cases of atrial flutter, with one recurrence (11.1%). Overall, therefore, ablation was immediately successful in 271 patients (94.4%), with a recurrence of the arrhythmia in 29 cases (10.7%). The incidence of serious complications was 2.09% There was one late death due to infective endocarditis, 3 patients suffered complete heart block, 1 had mild mitral regurgitation, and 1 patient developed an haematoma in the groin. We conclude that radiofrequency catheter ablation can now be considered a standard option for the management of paroxysmal supraventricular tachycardias in children and young adults.
Successful palliation of Ebstein's malformation on the first day of life following fetal diagnosis
Authors: Deleon Mary Ann M. , Gidding Samuel S. , Gotteiner Nina , Backer Carl L. , Mavroudis Constantine 
Published: August 2008

AbstractAbstract Severe Ebstein's malformation has a poor prognosis when diagnosed during the neonatal period. Surgical options that have proved successful in older patients have been associated with high mortality and morbidity in neonates. We report here our success with a policy of induction at term and immediate surgical intervention when Ebstein's malformation was diagnosed prenatally.
Abnormal pattern of flow in the superior caval vein in children after open heart surgery
Authors: Ichihashi Ko , Shiraishi Hirohiko , Momoi Mariko 
Published: August 2008

AbstractAbstract To investigate the pattern of flow in the superior caval vein in children after open heart surgery, we examined the flow velocity in 37 partients who had undergone surgical correction of either ventricular or atrial septal defects. The control group consisted of 64 age-matched children. We used pulsed Doppler examinations to record the velocity curves. We measured the hightest and lowest peak flow velocities during systole and diastole, and calculated ratios for the various measurements.We found that the velocity of the wave in systole is lower than that of the wave in diastole in the postoperative patients, which is contrary to the normal pattern. The velocity in systole increases more than that in diastole during inspiration, which is also diffetent from the normal pattern. The change in pattern of flow velocity cure during respiration was smaller in patients after surgery than in normal subjects, which might indicate that there is less reserve ability in the right atrium in postoperative patients.
Arrangement of the systemic and pulmonary venous components of the atrial chambers in hearts with isomeric atrial appendages
Authors: Min Jee-Yeon , Kim Chi-Yon , Oh Mee Hye , Chun Yi Kyeong , Suh Yeon-Lim , Kang I-Seok , Lee Heung-Jae , Seo Jeong-Wook 
Published: August 2008

AbstractBackgroundThe morphological definition of atrial chambers, and the determination of atrial laterality, are based on analysis of the structure of the atrial appendages. The systemic and pulmonary venous connections to the heart, nonetheless, are important in the management of patients having isomeric appendages. In this study, therefore, we analysed the morphology of the postero-superior walls of the atrial chambers so as to provide evidence concerning the morphogenetic background of those hearts, and to improve operative management.MethodsWe reviewed 15 autopsied specimens with isomeric right appendages, and 10 with isomeric left appendages, paying particular attention to the morphology of the systemic and pulmonary venous connections. The postero-superior walls of the atrial chambers can be made up of the atrial body, the systemic venous components, or the pulmonary venous component. We analysed the contributions made by each of these components.ResultsThe postero-superior walls of the atrial chambers were markedly variable, but could be grouped into five patterns. Bilaterally well-developed systemic venous components and absence of the pulmonary venous component within the hypoplastic atrial body were present in 9 hearts with extracardiac pulmonary venous connections in the setting of right isomerism. Bilaterally well-developed systemic venous components, and a hypoplastic pulmonary venous component within the hypoplastic atrial body, were present in 5 hearts with intracardiac pulmonary venous connections in right isomerism. Bilaterally well-developed systemic venous components, and a hypoplastic pulmonary venous component within the sizable atrial body, were present in 1 heart with an intracardiac pulmonary venous connection in right isomerism. A well-developed pulmonary venous component within the atrial body, and hypoplasia of one systemic venous component, were present in 7 hearts with left isomerism. A well-developed pulmonary venous component within the atrial body, and hypoplasia of bilateral systemic venous components, were present in 3 hearts with left isomerism.ConclusionsThe postero-superior walls of the atrial chambers in hearts with isomeric atrial appendages can be analysed on the basis of a compound structure made of bilateral systemic venous components, a central pulmonary venous component, and the body of the atrium. Hearts with isomeric right appendages have absence or hypoplasia of the pulmonary venous component, while hearts with isomeric left appendages have hypoplastic systemic venous components.
Fetal echocardiographic features of twisted atrioventricular connections
Authors: Abdullah Mustafa , Yoo Shi-Joon , Hornberger Lisa 
Published: August 2008

AbstractWe present an example of corrected transposition with twisted discordant atrioventricular connections in which both fetal and postanatal echocardiograms were obtained. We correlate the fetal echocardiograms with the postanatal echocardiograms, placing emphasis on the echocardiographic clues to the diagnosis which were present in fetal life. The diagnosis should be suspected when the cardiac chambers and greatarteries show an unexpected spatial relationship for the given segmental connections, and when the axes of opening of the atrioventricular valves are not parallel.