bluuebunny/crossref_metadata_2025_split huggingface dataset:
Infective endocarditis on an occluder closing an atrial septal defect
Authors: Bullock A. M. , Menahem S. , Wilkinson J. L. 
Published: August 2008

AbstractClosure of atrial septal defects be means of intravenous catheterisation has been undertaken using a variety of devices as an alternative to surgical closure.1–5We describe the first case, to the best of our knowledge, of infective endocarditis complicating a successful transcatheter closure. This highlights the potential risk of this procedure, and emphasises the need for appropriate antibiotic prophylaxis until complete endothelialization of the device has occurred.
Divided left atrium in association with tricuspid atresia and discordant ventriculo-arterial connections
Authors: Dindar Aygun , Brawn William J. , De Giovanni Joseph V. 
Published: August 2008

AbstractDivision of the morphologically left atrium is a rare abnormality, constituting around 0.1% of congenital heart disease. It may coexist with other cardiac abnormalities, and this association may obscure its recognition. We report an uncommon association with tricuspid atresia and discordant ventriculo-arterial connections, the latter dominating the clinical picture. Accurate diagnosis was made by transthoracic cross-sectional echocardiography, and the patient was referred to surgery without need for cardiac catheterisation.
Aneurysm of the pulmonary trunk with patent arterial duct
Authors: Alva Carlos , Argüero Ruben , Valero Guillermo 
Published: August 2008

AbstractWe present a 14-year-old female with a massive aneurysm of the pulmonary trunk occurring secondary to bacterial endocarditis of the patent arterial duct. Echo Doppler imaging, magnetic resonance and angiography confirmed the diagnosis. On intermediate follow-up after surgical correction, the echo studies and cardiac catheterization disclosed no residual lesions. The patient is now asymptomatic.
Atrioventricular septal defect with separate right and left atrioventricular valvar orifices in a patient with foetal hydantoin syndrome
Authors: Grech Victor , Vella Cecil 
Published: August 2008

AbstractThe teratogenic properties of phenytoin, including cardiac malformations, have been previously documented. We report one patient with foetal hydantoin syndrome and atrioventricular septal defect with common atrioventricular junction but separate right and left atrioventricular valves, an association that has not been described, to the best of our knowledge.
Tetralogy of Fallot with non-confluent pulmonary arteries and aortopulmonary septal defect
Authors: Dipchand Anne I. , Giuffre Michael , Freedom Robert M. 
Published: August 2008

AbstractAortopulmonary septal defect and tetralogy of Fallot is a rare combination. We report a case of tetralogy of Fallot, non-confluent pulmonary arteries with the left arising from the arterial duct, and a large aortopulmonary septal defect diagnosed by echocardiogram and confirmed by cardiac catheterization.
Declining mortality from congenital heart disease related to innovations in diagnosis and treatment: a population-based study
Authors: Grech Victor , Savorja-Ventura Charles 
Published: August 2008

AbstractThe majority of live births with severe congenital cardiac malformations that do not undergo intervention of some form die prematurely. The population of Malta provides an ideal closed cohort from which to monitor trends. We have observed a significant declining specific mortality from congenital heart disease, and circumstantial evidence relates this decline to the introduction of new techniques used in the diagnosis and treatment of these malformations.
Cross-sectional echocardiographic demonstration of duct-related intimal tissue plaques within the pulmonary artery
Authors: Gennery A. R. , Hunter A. S. 
Published: August 2008

AbstractThe cross-sectional and colour flow mapping echocardiographic appearances of extension of ductal tissue within the pulmonary artery in three infants referred for echocardiographic assessment of a cardiac murmur are reported. The ductal plaques described may be common but are not necessarily of clinical significance.
An unusual tethering of the bridging leaflets in atrioventricular septal defect producing a communication from left atrium to right ventricle
Authors: Delgado C , onnin O , Barturen F 
Published: August 2008

AbstractWe describe a 39-year-old woman who was diagnosed as having an unusual atrioventricular septal defect with a communication from left atrium to right ventricle. A common atrioventricular junction, with partially separated right and left atrioventricular orifices, was found at transoesophageal ultrasonic examination. Both bridging leaflets were attached to the underside of the atrial septum, which was grossly malaligned relative to the ventricular septum. The shunt was exclusively from left atrium to right ventricle because of the overriding of the left atrioventricular valve, with the left component of the inferior bridging leaflet firmly fused to the ventricular septal crest.
Outcome of pregnancy in women with congenital heart disease
Authors: Schmaltz A. A. , Neudorf U. , Winkler U. H. 
Published: August 2008

AbstractImprovements in diagnosis and surgical technique for correction have led to an increasing number of women with congenital heart disease reaching the child-bearing age. Pregnancy places considerable strain on the heart and circulation and necessitates marked cardiorespiratory adaptation. Today, with the exception of the Eisenmenger syndrome, there is no increased mortality associated with pregnancy in congenital heart disease. In contrast, there is still considerable morbidity, due to congestive heart failure, thromboembolic complications and disturbances of rhythm.Fetal outcome is complicated by a high rate of spontaneous abortions (20–25%), retardation of fetal growth, and premature delivery (almost 100% in cyanotic mothers). Based on an extensive review of the literature, we discuss the specific risks in pregnancy depending on the hemodynamic situations produced by different heart defects. We also discuss the risks and advantages of different regimens for anticoagulation.Counselling concerning contraception is frequently inadequate. The most important problems are thromboembolic complications with the use of hormonal contraception, and hyper- and dysmenorrhea in those using intrauterine devices. Finally, the genetic risks must be considered, differentiating between single gene defects and the sex of the parents suffering from congenital heart diseases.
Sudden death in an adult with a small ventricular septal defect and an aneurysmal membranous septum
Authors: Sarubbi B , Gerlis L M , Ho S Y , Somerville J 
Published: August 2008

AbstractAn apparently healthy man of 26 years of age suddenly died. He was known to have had a small ventricular septal defect and complete right bundle branch block from early childhood. At post-mortem examination the small ventricular septal defect was found associated with an aneurysm of the membranous septum. Histological examination showed a normal atrioventricular node and bundle, adjacent to the aneurysm. There was fibrous interruption at the commencement of the right bundle branch, which was considered the basis of the bundle branch block. It was also considered that the right bundle branch block was of the hereditary type and that this, rather than the aneurysm of the membranous septum, was responsible for the unexpected death.
Helen Brooke Taussig MD May 24, 1898 to May 21, 1986
Authors: Neill Catherine A. , Clark Edward B. 
Published: August 2008

AbstractThe life and accomplishments of Helen Taussig are reviewed at the centennial of her birth in 1898. Now, a little over 50 years since the first Blalock Taussig shunt in 1944, her legacy remains a model of brilliant scientific innovation and loving patient care.“Faithful indeed is the spirit that remembers” (Emily Bronte, Remembrance)
Induction of cardiac anomalies with all-trans retinoic acid in the chick embryo
Authors: Broekhuizen Monique L. A. , Wladimiroff Juriy W. , Tibboel Dick , Poelmann Robert E. , Wenink Arnold C. G. , Groot Adriana C. Gittenberger-de 
Published: August 2008

SummaryTo study the interaction between hemodynamics and morphology in abnormal development of the heart, it is essential to have an animal model with specific and reproducible cardiac malformations. A standardized method was developed for inducing such an anomaly in the chick embryo. For this purpose, all-trans retinoic acid, dissolved in 2% dimethylsulphoxide, was applied on the vitelline membrane of the embryo in concentrations of 1.0 μgm and 0.3 μgm. A total of 207 embryos, staged according to Hamburger and Hamilton, were treated at stages 10 (33 hours of incubation) and 15 (50–55 hours of incubation). Three control groups consisted of embryos solely treated with dimethylsulphoxide, sham operated chicks, and untreated embryos, respectively. The embryos were sacrificed on the 9th, 10th and 11th days of incubation and hearts were examined by microdissection. Additional histologic studies were undertaken in 110 cases. Embryos treated with retinoic acid at both stages of development showed a high percentage of lesions falling within the general spectrum of double outlet right ventricle. The abnormality varied from merely a rightward shift of the aorta to cases with an additional subaortic ventricular septal defect, which, in combination with the rightward positioned aorta, was classified as a double outlet right ventricle. Histologic examination showed the presence of muscular tissue between the leaflets of the aortic and mitral valves. No other types of cardiac malformations were seen. Our results, with respect to timing, resemble data from studies made of hearts after surgical ablation of neural crest at comparable stages. Both method and timing of treatment with retinoic acid are important factors for the induction of specific and reproducible cardiac anomalies. This chick model allows for further detailed study of development of double outlet right ventricle, as well as the assessment of early detectable hemodynamic changes.
Expression of myosin heavy chain in neonatal human hearts
Authors: Wessels Andy , Mijnders Tom A. , de Gier-de Vries C. , Vermeulen Jacqueline L. M. , Vir´gh Szabolcs , Lamers Wouter H. , Moorman Antoon F. M. 
Published: August 2008

AbstractWe have investigated the spatial distribution of the α and β isoforms of myosin heavy chain in the force-producing ventricular myocardium, the ventricular conduction system and the atrioventricular junctional region of five neonatal human hearts–three born prior to and two at term. The results can be summarized as follows: 1)the force-producing myocardium:in each heart, the β isoform was found to be the predominating isoform, β-negative cells never being observed. Considerable amounts of the α isoform were observed only in those hearts that were obtained from patients dying within the first two weeks of postnatal life. This transient expression is probably correlated with the postnatal increase in levels of circulating thyroid hormone. 2)the ventricular conduction system:in addition to the expression of the β isoform, a strong expression of the α isoform was detected in all the components of the ventricular conduction system in the two full-term hearts. In the preterm hearts, however, this strong expression was mainly restricted to the atrioventricular node and the penetrating atrioventricular bundle. The level of expression of the α isoform in the conduction system of the neonatal hearts, therefore, might reflect the state of maturity of this system at the time of birth. 3)the atrioventricular junctional region:in each heart examined, structures were detected that resembled immunohistochemically and morphologically the atrioventricular node. Although the expression of the isoforms of myosin heavy chain in the conduction system, and in these comparable structures, cannot, as yet, be interpreted with regard to function, these resemblances, coupled with the same developmental background for these structures (they all develop from the so-called “primary ring”) suggest functional affinities during this stage of cardiac development.
An anatomical study of 110 cases with deficiency of the aorticopulmonary septum with emphasis on the role of the arterial duct
Authors: Gerlis Leon M. , MacGregor Candida C. d'A. , Ho Siew Yen 
Published: August 2008

AbstractTo assess the prevalence and role of the arterial duct in hearts with incomplete development of the aorticopulmonary septum, 110 autopsy specimens, comprising 100 examples of common arterial trunk and 10 cases with aorticopulmonary window, were studied. In addition to intracardiac malformations, these specimens were analyzed to determine the side of the aortic arch, the presence and location of any interruption of the arch, the presence of any anomaly of origin and course of the subclavian arteries, and the status of the arterial duct. The arterial duct was present in 33 cases, absent in 63 cases and undetermined in four cases with common arterial trunk. The duct provided an essential pathway for flow in the 20 cases with interrupted aortic arch, and in two cases with interruption of the proximal portion of the left pulmonary artery. The presence of the duct in 11 cases, in functional terms, was not essential. In the 10 hearts with aorticopulmonary window, the duct was present in eight. It was an obligatory part of the circulatory pathways in three cases where the aortic arch was interrupted. The prevalence of non-obligatory ducts was 71% in hearts with aorticopulmonary window compared to 15% in hearts with common arterial trunk. The prevalence of the duct in cases with aorticopulmonary window suggests this lesion is a later developmental defect. The functional role of an arterial duct in these hearts should be properly assessed in the clinical situation.
Use in children of an additional umbrella for transcatheter occlusion of residual patency of the arterial duct following initial insertion of an umbrella device
Authors: Yousef Saad Al , Khan Ali , Mullins Charles E. , Sawyer William 
Published: August 2008

SummaryAfter an initial attempt to close arterial ducts by the transcatheter insertion of an umbrella device had left significant residual patency, further attempts at closure were made in 14 patients by the insertion of an additional umbrella device. The ages of the patients ranged from four to 11 years. Overall, 17 attempts were made to insert additional devices at intervals of between six to 26 months after the insertion of the first umbrella. Successful insertion of a device was achieved in two patients only at a third attempt. One patient required the insertion of a third umbrella to achieve complete occlusion of the patent duct. Complete closure of the residual patency was achieved in 13 of the 14 patients. The remaining patient has been scheduled for insertion of a third umbrella.
Anomalous mitral arcade leading to congenital mitral insufficiency with an associated anomaly of the tricuspid valve
Authors: Mandke Jyoti V. , Dharnidharka Vikas R. , Sanzgiri Vasundhara P. 
Published: August 2008

SummaryA 21-day-old girl presented with congestive heart failure of acute onset and Doppler evidence of mitral regurgitation, which subsequently proved fatal. Necropsy revealed an anomalous arcade lesion of the mitral valve. The tendinous cords of the tricuspid valve were also abnormal, a finding, to the best of our knowledge, not reported before.
Extracorporeal membrane oxygenation and cyanotic congenital heart disease
Authors: Pearson Gale A. , Firmin Richard K. , Leanage Ranjit 
Published: August 2008

AbstractWorldwide figures suggest that two percent of appropriate referrals for neonatal extracorporeal membrane oxygenation turn out to have previously covert congenital heart disease. This is despite the fact that expert cardiological evaluation is routine prior to cannulation. The experience in the United Kingdom includes such a case which is reported here. The implications for the role of pediatric cardiologists in such a service are considered.
Morphologic and functional analysis of myocardial status in pulmonary atresia with intact ventricular septum—an angiographic, histologic and morphometric study
Authors: Daliento Luciano , Scognamiglio Roldano , Thiene Gaetano , Hegerty Anita , Ho Siew Yen , Caneve Francesca , Anderson Robert H. 
Published: August 2008

SummaryWe studied 11 patients with pulmonary atresia and intact ventricular septum who died at ages ranging from one day to three years. All but two neonates died after surgery. Pre-surgical catheterization with left and right cineventriculography was performed in nine patients, and quantitative parameters were calculated. By histologic examination of transverse transmural sections, the incidence and extent of myocardial ischemia or infarction were determined, as well as the percentage area per field occupied by vessels, myocytes orientated longitudinally or transversely, interstitial space, and disarrayed fibers. Comparable sections were obtained from six normal neonates to constitute a control group. Myocardial ischemia or infarction were frequent complications. Morphometric analysis demonstrated a significant difference between the control group and the patients with regard to the percentage of myocytes (longitudinal and transverse), the percentage of area occupied by myocardial disarray, and the presence of fibrosis. The highest values for the proportion of disarray were seen in those patients with small right ventricles. The percentage of myocytes varied, but the highest degree of disarray always correlated with the lowest percentage of myocytes. The highest percentage value of left ventricular disarray was associated with the lowest ratio of left ventricular mass to end diastolic volume (inadequate hypertrophy). The lowest indices of contractility and left ventricular pump function were associated with myocytes less than 55% of normal. A linear correlation was found between the percentage area of fibrosis and the ratio of systolic pressure to end systolic volume in the left ventricle, arid between fibrosis and ejection fraction. Values for fibrosis exceeding 15% were associated with decreased functional indices. Disarray, therefore, seems to express a primary inability of myocardium to respond to anomalous overload with adequate hypertrophy and/or hyperplasia.
Pulmonary atresia with intact ventricular septum in the fetus
Authors: Allan Lindsey D. , Cook Andrew 
Published: August 2008

AbstractThe objective of this study was to review the echocardiographic findings and autopsy correlates of pulmonary atresia with an intact ventricular septum when diagnosed during fetal life. All cases with this lesion (nearly 60) seen in the Perinatal Cardiology unit of Guy's Hospital since 1983 are part of this study. More than half of the cases were detected within the last two years, and all bar three were referred because of suspicion of a cardiac malformation detected on routine obstetric scanning. The cases were divided into two distinct groups. On the one hand were those with dilatation of the chambers of the right heart. These had a uniformly poor prognosis. Severe regurgitation of the tricuspid valve was a characteristic feature. On the other hand were the cases with cavitary hypoplasia due to mural overgrowth. Direct measurements of pressure in one such case revealed suprasystemic values in the right ventricle. Although contemplated, fetal intervention proved impossible. Autopsy revealed cases with either valvar or infundibular atresia. In most cases diagnosed prior to 24 weeks of gestation, mothers opted for termination of pregnancy. All cases with dilatation of the right ventricle died. Of pregnancies which continued, only 30% of children are still alive, all but one being less than two years of age at present. The use of cross-sectional and Doppler echocardiography permits the accurate diagnosis of pulmonary atresia with an intact ventricular septum during fetal life. Autopsied cases showed strong correlation with the echocardiographic findings. The poor prognosis in most cases warrants consideration in the future of intervention during fetal life.
Determinants for outcome of hypoplastic right ventricle with duct-dependent pulmonary blood flow presenting in the neonatal period
Authors: Rigby Michael L. , Salgado Micelia , Silva Celia 
Published: August 2008

SummaryA retrospective study was undertaken of patients with hypoplastic right ventricles, either with pulmonary atresia and intact ventricular septum or critical pulmonary valvar stenosis, and duct-dependent pulmonary blood flow who were investigated at the Royal Brompton Hospital between January 1976 and December 1990. The diagnosis was made on the basis of at least one diagnostic method (cross-sectional echocardiography, cardiac catheterization and angiography, or autopsy). Of the patients, 56 (82%) were found to have an imperforate pulmonary valve or infundibulum (pulmonary atresia), while 12(18%) had critical pulmonary valvar stenosis. The ratio of the diameters of the tricuspid and mitral valvar orifices was measured angiographically during diastole, and the right ventricle was analyzed according to the presence or overgrowth of the inlet, apical trabecular and outlet components. A correlation was made between the severity of the disease and the outcome. The overall mortality was 53% when those not undergoing any surgery were excluded, and significant differences were found between the group dying and those who survived. The incremental risk factors for death were a ratio between the diameter of the tricuspid and mitral valves of less than 0.6; the presence of fistulous communications with the coronary arteries; and obliteration of the apical trabecular component of the right ventricle. There was no significant difference between the group with pulmonary valvar atresia and the group with critical stenosis of the pulmonary valve.
Antegrade diastolic pulmonary arterial flow as a marker of right ventricular restriction after complete repair of pulmonary atresia with intact septum and critical pulmonary valvar stenosis
Authors: Redington Andrew N. , Penny Daniel , Rigby Michael L. , Hayes Alison 
Published: August 2008

AbstractIn order to examine right ventricular diastolic function after complete correction of pulmonary atresia with intact ventricular septum and critical pulmonary valvar stenosis, we undertook a prospective analysis of seven patients who had previously undergone successful repair. Comparison was with age and sex-matched controls. The relationship between antegrade pulmonary arterial diastolic flow and other indices of right ventricular dysfunction were compared. The ratio of the velocity of early rapid filling to that of atrial systolic flow was no different from normal, but the deceleration of early rapid filling showed significant shortening with inspiration in patients, but not in control subjects. All patients, but none of the controls, demonstrated significant antegrade diastolic pulmonary arterial flow during late diastole. Despite “normal” ratios ofvelocity of early rapid filling to systolic atrial flow, patients after complete correction of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis have abnormal right ventricular diastolic function typical of a restrictive physiology. This presumably reflects incomplete adaptation due to the presence of endomyocardial fibrosis.
Laser or radiofrequency pulmonary valvotomy in neonates with pulmonary atresia and intact ventricular septum—description of a new method avoiding arterial catheterization
Authors: Redington Andrew N. , Cullen Seamus , Rigby Michael L. 
Published: August 2008

SummaryWe describe a new method of transvenous laser or radiofrequency perforation of the pulmonary valve with subsequent balloon pulmonary valvoplasty. The technique obviates the need for arterial catheterisation and considerably shortens the time needed for the procedure. Uncomplicated and successful relief of pulmonary valvar atresia was achieved in both patients attempted, although one ultimately required a Blalock-Taussig shunt because of increasing muscular obstruction of the right ventricular outflow tract.