bluuebunny/crossref_metadata_2025_split huggingface dataset:
Persistence of the left superior caval vein: can it potentiate obstructive lesions of the left ventricle?
Authors: Agnoletti Gabriella , Annecchino Francesco , Preda Laura , Borghi Adele 
Published: August 2008

AbstractRecent evidence has suggested that persistence of the left superior caval vein is associated with a high incidence of obstructive lesions of the left heart. To shed more light on this issue 1085 patients with congenital heart disease were studied retrospectively, with the aim of estimating the prevalence of a persistent left superior caval vein and its associated anomalies, focusing attention on obstructive lesions in the left and right ventricles. Patients with isomerism of the atrial appendages, or hypoplastic left heart syndrome, were excluded. A persisting left superior caval vein was present in 57 patients (5.2%). The overall incidence of obstructive lesions of the left heart was higher in patients with than in those without a persistent left superior caval vein (31.6 versus 7.8%,p< 0.001). Relative hypoplasia of the left ventricle was also higher in patients with persistent left superior caval vein (14 versus 0.8%,p< 0.001). The obstructive lesions found in the left heart, compared with the number in those without a left caval vein, were: mitral stenosis, 5.2 versus 0.7%; subaortic stenosis, 5.3 versus 0.9%; aortic coarctation, 17.5 versus 5.8% (p< 0.01); all of these in association, 3.5 versus 0.4%. In contrast, the incidence of obstructive lesions of the right heart was similar in the two groups of patients. It is concluded that persistence of the left superior caval vein can perturb the normal development of the left ventricle, being strongly associated with obstructions to left ventricular inflow and outflow.
The Italian Multicentric Study on Epidemiology of Congenital Heart Disease: first step of the analysis
Authors: Bosi Giuliano , Scorrano Marco , Tosato Giovanna , Forini Elena , Chakrokh Roxana ,   and the Working Party of the Italian Society of Pediatric Cardiology
Published: August 2008

AbstractWe present the aims, methodology and initial results from the Italian Multicentric Study for the registration and follow-up of congenital heart disease. The general aims are to measure the prevalence of congenital heart disease in different geographic areas of Italy, and to assess the survival and outcome of affected babies. During the years 1992 and 1993, eighteen centers for Pediatric Cardiology spread all over the Country enrolled 1445 new babies with congenital cardiac malformations from a population of 341, 647 surveyed livebirths. The new cases were registered using the same methodologic criterions of the EUROCAT study in order to evaluate differences and/or similarities between the studies. The prevalence varied between 1.8‰ and 8.1‰; the average being 4.6%. The large range in prevalence is presumed to be related to different customs and hierarchies in flow and referral of patients. We provide total prevalence of individual lesions, and distribution of sentinel cardiac anomalies, in the Italian study and compare them with EUROCAT. Isolated ventricular septal defect is the most common lesion (39%); followed by atrial septal defect (7.5%); pulmonary valvar stenosis (7.3%); atrioventricular septal defects (5.4%); patency of the arterial duct (3.8%); complete transposition (3.7%); tetralogy of Fallot (3.3%); aortic coarctation (2.4%); aortic valvar stenosis (2.2%); and left heart hypoplasia (1.8%). The echographic stratification of ventricular and atrial septal defects, by location and size, was in keeping with the findings of the EUROCAT study. Because of the recent widespread availability of color-Doppler tecniques, the stratification of aortic and pulmonary valvar stenosis was an innovative approach in our study. Among the complex cardiovascular anomalies, double inlet ventricle and pulmonary atresia had a proportion of about 2% each; with double outlet right ventricle, common arterial trunk, Ebstein's malformation, tricuspid atresia, interrupted aortic arch and totally anomalous pulmonary venous connection having a proportion ranging from 0.5 to 0.8%. We discuss clinical features, such as frequency of extracardiac anomalies and familial aggregation of congenital heart disease, in comparison with the EUROCAT data.
Asymmetrically short tendinous cords causing congenital tricuspid regurgitation: improved understanding of tricuspid valvar dysplasia in the era of color flow echocardiography
Authors: McElhinney Doff B. , Silverman Norman H. , Brook Michael M. , Hanley Frank L. , Stanger Paul 
Published: August 2008

AbstractBackgroundTricuspid regurgitation as a manifestation of an isolated congenital anomaly of the tricuspid valve is rare. Cross-sectional and color Doppler echocardiography allow improved evaluation of tricuspid valvar function. As a result, the heterogeneous category of congenital tricuspid valvar dysplasia may be better understood from a functional point of view. We are reporting a distinct entity in which tricuspid valvar regurgitation results from failure of coaptation due to short tendinous cords tethering the septal leaflet.Patients and ResultsThree children with significant primary tricuspid regurgitation were evaluated, treated, and followed. On echocardiographic evaluation, a central regurgitant jet of moderate or severe degree was directed toward the atrial septum through poorly coapting tricuspid valvar leaflets, which did not approximate due to tethering of the septal leaflet by abnormally short cords. In one patient, the tricuspid valve was otherwise normal; in the other two the leaflets and cords were also thickened. Two patients underwent surgery at 9 and 11 years of age. The cords tethering the septal leaflet were augmented by interposing appropriate lengths of expanded polytetrafluoroethylene suture and performing commissural annuloplasty. Both patients are asymptomatic 33 and 42 months postoperatively, with mild residual tricuspid regurgitation that has not changed since surgery. The other patient, an 8 month-old infant, has not yet undergone surgery.ConclusionsAsymmetric tendinous cords of the tricuspid valve causing tethering of the septal leaflet is a distinct cause of tricuspid regurgitation that can be recognized with echocardiography. Although rare, the importance of recognizing this lesion lies in its being readily amenable to surgical repair.
Echocardiographic diagnosis of totally anomalous pulmonary venous connection to the azygos vein
Authors: Musolino Anna Maria , Santoro Giuseppe , Marino Bruno , Formigari Roberto , Guccione Paolo , Pasquini Luciano 
Published: August 2008

AbstractTotally anomalous pulmonary venous connection to the azygos vein is a rare congenital heart malformation in which all the pulmonary venous blood returns anomalously to the azygos vein. Among 111 consecutive patients with totally anomalous pulmonary venous connection undergoing surgical correction at our institution between June 1982 and September 1997, this malformation was present in seven cases. By echocardiography, using a subxyphoid short-axis view at the atrial level and a modified suprasternal sagittal view, the malformation was diagnosed when the pulmonary venous confluence was traced posteriorly and superiorly relative to the right pulmonary artery and right bronchus, finally reaching reach the superior caval vein. Totally anomalous pulmonary venous connection to the azygos vein was misdiagnosed in the first two patients, both by echocardiography and angiocardiography. In the subsequent five patients, a precise diagnosis was obtained by echocardiography. Echocardiography, therefore, can be considered an accurate diagnostic tool permitting recognition of totally anomalous pulmonary venous connection to the azygos vein, and permitting corrective surgery without recourse to catheterization and angiography.
Isolated connection of the right superior caval vein to the left atrium: non-invasive neonatal diagnosis
Authors: Kakadekar Ashok P. , McKay Roxane , Tyrrell Michael J. 
Published: August 2008

AbstractIsolated anomalous drainage of the right superior caval vein to the left atrium is a very rare cause of cyanosis in the newborn. Herein, the cross-sectional echocardiography and colour Doppler findings of this malformation are described.
Double-switch operation for congenitally corrected transposition and Ebstein's malformation
Authors: Nikoloudakis Nikolaos , Lindinger Angelika , Schäfers Hans-Joachim 
Published: August 2008

AbstractAn infant is described with congenitally corrected transposition and Ebstein's malformation. Banding of the pulmonary trunk had been previously performed because of a muscular ventricular septal defect. The patient underwent the double-switch procedure with the intention of unloading the morphologically right ventricle and the malformed tricuspid valve. This resulted in prompt postoperative functional and haemodynamic improvement.
An unusual type of combined aortic coarctation due to fibromuscular dysplasia
Authors: Suarez William A. , Kurczynski Thaddeus W. , Bove Edward L. 
Published: August 2008

AbstractA 3-year-old patient is described with an unusual form of co-arctation due to hypoplasia of the transverse arch and fibromuscular dysplasia involving a long segment of the thoracic aorta. Surgical repair required resection of the aorta from the distal transverse arch to the mid-descending thoracic aorta, and replacement with a 16-mm Dacron interposition graft. This case demonstrates the importance of preoperative evaluation of the entire aorta in the presence of co-arctation due to fibromuscular dysplasia.
Lobar emphysema due to anomalous aortic origin of the left pulmonary artery
Authors: Absher Dale R. , Kriss Vesna Martich , Cottrill Carol M. 
Published: August 2008

AbstractThe unusual case of an infant with aortic origin of the left pulmonary artery is presented. The patient developed a rare complication of lobar emphysema due to bronchial compression from the enlarged right pulmonary artery. Operative anastomosis of the left pulmonary artery to the pulmonary trunk was successful, with subsequent resolution of the lobar emphysema.
Hypoplastic left heart syndrome with right aortic arch, bilateral arterial ducts and origin of the left subclavian artery from the left pulmonary artery
Authors: Patel Chandrakant R. , Specter Michael L. , Zahka Kenneth G. 
Published: August 2008

AbstractThe rare association, in a left-sided heart with hypoplastic left heart syndrome, of right aortic arch, bilateral patent arterial ducts and origin of the left subclavian artery from the left pulmonary artery are described. Cardiac catheterization was performed because of the abnormal anatomy of the arch noted at echocardiographic examination. This abnormality is of surgical importance when planning the Norwood operation.
Ascites and weight loss in a child: due to congenital division of the right atrium
Authors: Alkhulaifi Abdul M. , Serraf Alain , Planché Claude 
Published: August 2008

AbstractCongenital division of an atrial chamber is a very rare congenital malformation that more commonly affects the left atrium but which may, in rare circumstances, involve the right atrium. Such a divided right atrium may present with symptoms consistent with increased portal venous pressure. Reported is a case with unusual clinical presentation. The patient underwent resection of the dividing shelf with good postoperative results.
A strange complex of diffuse congenital cardiovascular disease and cardiomyopathy, with localised myocardial calcification
Authors: Gerlis L. M. , Somerville J. 
Published: August 2008

AbstractThis unique case is of a man, followed clinically since infancy, who had a ventricular septal defect which closed spontaneously, a small arterial duct, and a minor degree of aortic coarctation, all without obvious symptoms. He later developed progressive cardiac failure which was attributed to some obscure form of congenital cardiomyopathy. He died at the age of 45 years. Necropsy showed a grossly abnormal arrangement of ventricular myocardial fascicles and bands, with absence of the papillary muscles causing tricuspid and mitral regurgitation. The various malformations are considered to be a gross example of a diffuse congenital cardiovascular disease complex, to the best of our knowledge previously undescribed.
Morphology of the elastic pulmonary arteries in pulmonary hypertension: a quantitative study
Authors: McLeod Karen A. , Gerlis Leon M. , Williams Gordon J. 
Published: August 2008

AbstractRecent studies of intravascular ultrasound of the pulmonary arteries suggest that the technique can detect intimal and medial thickening in patients with pulmonary hypertension, potentially providing a method of assessing severity of pulmonary vascular disease in life. A major drawback of the technique is that only the elastic pulmonary arteries are accessible to current ultrasound catheters. The aim of this study was to determine whether morphological changes in vessels accessible to intravascular imaging reflect severity of pulmonary vascular disease and are of a sufficient degree to be detectable by current ultrasound catheters. Morphometric studies of the elastic pulmonary arteries were performed in specimens of lungs from 24 patients who had died with pulmonary hypertension (aged 3 weeks–9 years) and compared with measurements from infants who had died from sudden infant death syndrome. Morphological changes evident in the elastic pulmonary arteries in pulmonary hypertension included luminal dilation and medial thickening but these changes were too variable to be predictive of the severity of peripheral pulmonary vascular disease. Intimal thickening and atherosclerosis were present only in those with advanced pulmonary hypertensive disease. The changes of medial thickening and luminal dilation, nonetheless, are too variable to be predictive of the severity of peripheral pulmonary vascular disease. Thus, imaging by intravascular ultrasound may help confirm advanced pulmonary vascular disease, but is unlikely to differentiate less severe pulmonary vascular disease when the patient may still potentially be operable.
Transcatheter embolization of congenital coronary arterial fistulas in adults
Authors: Kambara Antônio M. , Pedra Carlos A. C. , Esteves C,sar A. , Cano Manoel N. , Braga Sérgio L. N. , Souza Amanda G. M. R. , Souza J. Eduardo M. R. , Fontes Valmir F. 
Published: August 2008

AbstractIn this report, we describe our experience with transcatheter occlusion of congenital coronary arterial fistulas in adults. From November 1992 to November 1996, 5 symptomatic patients, aged from 47 to 70 years, underwent transcatheter occlusion of fistulas using a retrograde arterial approach. All had chest pain or dyspnea on exertion. Detachable balloons were used in 4 patients, and Gianturco coils in 1. Detachable balloons were implanted through a Debrun system, while the coils were implanted through a 5 French right coronary Judkins catheter. Both were passed through an 8 French guiding catheter (Amplatz II). Each patient had a single fistula. The fistulas originated from the right coronary artery in 3 patients, and from the circumflex artery in 2. They drained into the pulmonary trunk in 3 patients, into the right atrium in 1, andinto a bronchial artery in the other. All fistulas were occluded completely in the catheterization laboratory, and the procedures were uncomplicated. At follow up, 3 patients underwent coronary angiography, and there was no evidence of recanalization. Transcatheter embolization in adults of single congenital coronary fistulas with detachable balloons and coils is safe and effective and can be regarded as an acceptable alternative to surgery.
Effect of radiofrequency catheter ablation of accessory pathways on autonomic tone in children
Authors: Sehra Ruchir , Hubbard Joyce E. , Straka Susan P. , Fineberg Naomi S. , Zipes Douglas P. , Engelstein Erica D. 
Published: August 2008

AbstractBackgroundRadiofrequency catheter ablation is standard treatment for children with re-entrant supraventricular tachycardias. Autonomic changes have been noted after such ablation for atrioventricular nodal re-entry tachycardia, but not as well documented with atrioventricular re-entry over an accessory pathway.Methods and resultsIn 10 normal paediatric volunteers and 12 children referred for electrophysiologic testing and radiofrequency ablation of supraventricular tachycardia, non-invasive autonomic function tests and tilt-table testing were performed, and the variability in 24-h heart rate was analysed. Patients with supraventricular tachycardia underwent these tests both 24–72 h before and 24 h after ablation. Patients with tachycardia underwent additional autonomic testing to assess the sensitivity of baroreceptors and the intrinsic heart rate with autonomic blockade immediately before and after ablation. One non-invasive autonomic function test, namely handgrip, demonstrated significant differences (p < 0.05) in diastolic blood pressure before and after ablation, though these values did not differ from controls. Significant decreases were noted in two indexes of the variability of heart rate before and after ablation (p < 0.05). Certain tilt test variables also demonstrated significant differences between controls and those with tachycardia subsequent to ablation. Intracardiac testing demonstrated changes (p < 0.05) in sinus cycle lengths, effective refractory periods and/or blood pressures at baseline and during testing of the sensitivity of baroreceptors before and after ablation. These changes were consistent with increased sympathetic or decreased parasympathetic tone. With autonomic blockade, these differences were abolished.ConclusionsCatheter ablation of accessory pathways in children was associated with changes consistent with increased sympathetic or decreased parasympathetic tone. These autonomic changes persisted 24 h after the ablation procedure.
Right ventricular diastolic function after repair of tetralogy of Fallot: its relationship to the insertion of a ‘transannular’ patch
Authors: Eročlu Ayşe Güler , Sarioşlu Ayse , Sariočlu Tayyar 
Published: August 2008

AbstractExamined was the effect of surgical technique, particularly the insertion of a transannular patch, on right ventricular diastolic function, and the relationship of forward flow in the pulmonary arteries during late diastole to right ventricular diastolic function in patients with tetralogy of Fallot. Transtricuspid, superior caval venous and pulmonary arterial Doppler spectrals were obtained and compared between 44 patients who had been repaired with a transannular patch; 14 patients who had been repaired with muscular resection and/or pulmonary valvotomy; six who had been repaired with an infundubular patch; and 32 normal children. The velocities of forward flow during late diastole in the pulmonary arteries of normal children ranged from 19.8 to 29.4 cm s−1(mean 24.9 ± 2.8 cm s−1) throughout the respiratory cycle. Restrictive right ventricular physiology, defined on the basis of increased forward flow in the pulmonary arteries during late diastole (> 30 cm s−1) was present in 25 (57°) of 44 patients with tetralogy of Fallot repaired using a transannular patch. Right ventricular volume was 50.1 ± 23.7 cm3in patients with a restrictive right ventricle and 64.9 ± 21.4 cm3in patients in whom the ventricle was non-restrictive (p< 0.03). QRS duration was 140 ± 18 and 156 ± 24 ms in patients with restrictive and non-restrictive right ventricular physiology respectively (p< 0.003). Restrictive physiology was not encountered in patients with tetralogy in whom the pulmonary valve had been preserved. It is concluded that right ventricular restriction is present in many patients with tetralogy of Fallot at mid-term follow-up subsequent to repair using a ‘transannular’ patch. Restriction is associated with smaller right ventricular size and less prolongation of the QRS complex.
Difficulties generated by the small, persistently patent, arterial duct
Authors: Raaijmaakers Brigitte , Nijveld Aagje , van Oort Anton , Tanke Ronald , Daniëls Otto 
Published: August 2008

AbstractOver recent years, echo-Doppler cardiography has shown that a small, sometimes silent, arterial duct exists in more patients than previously recognized. To know the incidence of an arterial duct subsequent to therapy, we studied retrospectively our patients undergoing open-heart surgery and surgical or catheter closure. Three groups of patients were studied: those with patency of the duct subsequent to open heart surgery without any sign of patency before or during surgery, those with persistent duct after surgical ligation and those with persistent patency after attempted catheter occlusion with the Rashkind device. In the first group (of 431 children) four (0.9%) had persistence of this duct, of which three were silent. In the second group, patency persisted in four of 100 patients (4%), three being silent. In the last group there were five persisting shunts, three producing no murmur, in 30 patients (17%). We compared our results with those reported in the literature and conclude that echo-Doppler cardiography is needed to detect persistent shunting across a duct after therapy, since most of the residual ducts in this study were silent. This means that clinical findings alone cannot be relied upon, and careful echo-Doppler cardiography is essential. Also, the process of closure of a persistent duct by surgical ligation or transcatheter intervention is no guarantee of success. The risk of infective endocarditis is important in such persistent ducts and, at present, it is unknown either for a small, silent duct or in a persistent duct that remains open after attempted transcatheter closure, but now is in association with a foreign body.
Seasonality in live births with congenital heart disease in Malta
Authors: Grech Victor 
Published: August 2008

AbstractThis study was carried out to analyse seasonal variations in live births with congenital heart disease in the overall population of Malta. Included were all patients diagnosed as having congenital heart disease by echocardiography, cardiac catheterization, surgery or post mortem, by 1 year of age, and who were born between in Malta between 1990 and 1994. Results were analysed by χ2 and by Edwards' cyclic method. There were 231 cases of live born congenital heart disease, which were divided into 114 cases not requiring intervention and 117 cases requiring intervention. Although the overall prevalence of congenital heart disease did not demonstrate any seasonal variation, the lesions requiring intervention showed a significant peak in September by Edwards' method (p = 0.03), which was enhanced by seasonal analysis (p = 0.003). These results were confirmed by conventional χ2 and χ2 for trend. The lesions not requiring intervention, which were comprised almost entirely (96%) of mild pulmonary stenosis and small ventricular septal defects, failed to show any seasonal trend. The literature regarding such seasonal trends in these malformations and statistical analysis of seasonality of congenital heart disease are reviewed. An environmental factor such as a maternal viral infection or treatment of such infections during the first trimester of pregnancy from November to January may precipitate congenital heart disease in predisposed Maltese foetuses.