bluuebunny/crossref_metadata_2025_split huggingface dataset:
Long-term cardiac follow-up of children with perinatally acquired human immunodeficiency virus-type 1 infection
Authors: De Simone Luciano , de Martino Maurizio , Galli Luisa , Manetti Adriano , Favilli Silvia , Pollini Iva , Calabri Giuseppe , Mannelli Francesco , Vierucci Alberto 
Published: August 2008

AbstractSince 1985 a physical and instrumental cardiac study was carried out in 41 children with perinatal HIV-1 infection in order to assess the prevalence and natural history of cardiac involvement. Children were followed for 66 (range 8-109) months; eight of 41 died of HIV-1 related diseases. The cumulative proportion of children with cardiac manifestations at age of seven years was 14.9% (95% CI: 4.9-31.5%). Cardiac abnormalities were detected in eight of 41 patients (19.5%) and were more significantly (p=0.003) recorded in children who died of HIV-1 related diseases (five of eight, 62.5%). Left ventricular systolic function was normal in most patients during the follow-up and did not depend on zidovudine treatment. Two patients had a severe dilated cardiomyopathy and underwent subsequently a stable regression as far as the clinical and instrumental frame was concerned. Only one patient died because of cardiac disease. We conclude that in our group ofperinatally HIV- 1 infected children, cardiac involvement is relatively rare, represents a late complication compared to other related HIV-1 diseases and occurs in advanced stages of illness. Cardiac disease is therefore a hallmark of poor prognosis, but is rarely the ultimate cause of death.
Unguarded tricuspid orifice with Uhl's malformation
Authors: Agarwala Brojendra , Waldman J. Deane , Carbone Michele 
Published: August 2008

SummaryA case is reported where unguarding of the tricuspid orifice was associated with Uhl's malformation. Echocardiographic features of the condition permitted the diagnosis during life. A therapeutic plan is proposed which involves surgical closure of the unguarded tricuspid orifice.
Muscular ventricular septal defect in a human embryo of 11 mm crown-rump length (six weeks)—no case of developmental arrest
Authors: Wenink Arnold C. G. 
Published: August 2008

AbstractA human embryo of 11 mm crown-rump length (six weeks, stage 15) is described, in which a muscular defect is present in the ventricular septum, in addition to the interventricular communication normal for this stage of development. The defect consists of large confluent spaces between very loose trabeculations. Its morphology is identical to that of the central muscular ventricular septal defect well recognized in the mature heart. It is suggested that the defect was caused by widening of the sinusoidal spaces which are temporarily present in the normal developing septum.
Hemodynamic and angiographic findings following arterial switch repair for complete transposition
Authors: Srinivas Arun , Ranjit Madathil , Wilkinson James L. , Goh Tiow , Edis Brian , Menahem Samuel , Fong Lance , Weintraub Robert 
Published: August 2008

AbstractThe arterial switch operation is the treatment of choice for complete transposition, and for the Taussig-Bing anomaly, with good early and mid-term results. This retrospective study examined the findings obtained at routine follow-up cardiac catheterization after primary arterial switch repair. We catheterized 111 patients after a mean of 16.9 months after surgery. These included 67 patients with an intact ventricular septum, 33 with a ventricular septal defect, and 11 with the Taussig-Bing anomaly. Right ventricular pressures were mildly elevated (mean 33.9±10.2 mm Hg) in the overall group with a mean pressure ratio between right and left ventricles of 0.34±0.1. Of the patients 74% had gradients across the right ventricular outflow tract of under 20 mm Hg, while three (2.7%) had gradients over 30 mm Hg. A significant gradient across the left ventricular outflow tract occurred in one patient (0.9%), while significant neo-aortic stenosis was not seen. The neo-aortic root remained dilated, with mild aortic valvar incompetence being seen in 12%, with none having higher grades of regurgitation. Left ventricular ejection fraction was normal in all patients, while left ventricular end-diastolic pressure was elevated in 38%. Coronary arterial stenosis was not seen, but one patient (0.9%) had left ventricular apical dyskinesia. Overall, therefore, we conclude that cardiac hemodynamics and ventricular systolic function after primary arterial switch are good. Minor gradients to the pulmonary arteries, and mild neo-aortic valvar incompetence were commonly noted. Left ventricular end-diastolic pressure was elevated in over a third of the patients. These subclinical and subtle abnormalities warrant ongoing follow-up to determine their long-term significance.
Early definitive repair of tetralogy of Fallot: a review of 74 cases
Authors: Abramov Dan , Barak Jacob , Raanani Ehud , Birk Einat , Vidne Bernardo A. 
Published: August 2008

AbstractDefinitive repair of tetralogy of Fallot was performed on 74 patients under two years of age, including 40 under the age of one year and 13 less than 6 months of age. In six patients, there had been previous construction of a systemic-to-pulmonary arterial shunt. Patching across the ventriculo-pulmonary junction was required in 46 patients, placement of conduits in six, and procedures producing unifocaliza-tion in three. Three patients died during the 60-day postoperative period of observation. Of the 71 survivors, 64 recovered without complications. One patient had neurologic sequels, one required prolonged mechanical ventilation, two reoperation, and one balloon dilation of residual left pulmonary arterial stenosis. Morbidity and mortality were not significantly higher in those patients undergoing surgery at earlier than 6 months of age, although transjunctional patching was more common in that age group. Previous construction of a shunt was associated with a higher prevalence of distal stenosis and distortion of the pulmonary arteries, which needed surgical repair in two out of six patients (33%). Since the outcome of definitive repair is favourable in patients with tetralogy of Fallot younger than six months of age, we now advocate definitive surgery for all young symptomatic patients by the age of six months.
Echocardiographic delineation of anomalous origin of the right subclavian artery from the right pulmonary artery
Authors: Law Yuk , Smallhorn Jeff , Adatia Ian 
Published: August 2008

AbstractWe describe a case of anomalous origin of the right subclavian artery from the right pulmonary artery detected by cross-sectional echocardiography in an infant with type B interruption of the aortic arch. Preoperative recognition and surgical reimplantation of the right subclavian artery are important to prevent subsequent subclavian steal syndrome.
Double inlet left ventricle associated with a subpulmonary tissue tag, dissection of the pulmonary trunk and pheochromocytoma
Authors: Medlicott Shaun A.C. , Harder Joyce R. , Denmark Lloyd N. 
Published: August 2008

AbstractPrevious studies have demonstrated the individual association of subpulmonary tissue tags, dissection of the pulmonary trunk and primitive neuroectodermal tumors with congenital heart disease. We are reporting a case of double inlet left ventricle with all these associations as it is exceptionally rare for all features to be manifest in one individual.
Coexistence of atrioventricular block and Wolff-Parkinson-White syndrome in children
Authors: Sigfússon Gunnlaugur , Webber Steven A. , Beerman Lee B. 
Published: August 2008

AbstractCoexistence of atrioventricular block and Wolff-Parkinson-White syndrome is uncommon, but may occur in children, particularly with certain congenital heart defects. Our study describes 5 children who had pre-excitation and underlying disturbance of atrioventricular conduction. All of these patients had structural heart defects, consisting of discordant atrioventricular connections in three, double inlet left ventricle in one, and a ventricular septal defect associated with straddling of the tricuspid valve in the other. In 4 of the patients, the presence of atrioventricular block was initially masked by the pre-excitation. When the conduction through an accessory pathway is lost, the child may be left with atrioventricular block, which is poorly tolerated in the setting of complex cardiac anatomy. Awareness of this association of Wolff-Parkinson-White syndrome and atrioventricular block is important in understanding the natural history and the management of children with congenital heart defects that are associated with tenuous atrioventricular conduction.
Successful one stage biventricular correction of aortic atresia with a ventricular septal defect and discordant ventriculo-arterial connections
Authors: Ebels Tjark , Dapper Friedhelm , Bauer Jurgen , Bohle Rainer M. , Hagel Karl J. , Nikkels Peter G.J. , Hehrlein Friedrich W. 
Published: August 2008

SummaryAortic atresia is rare in the setting of a normally developed left ventricle with a ventricular septal defect. In this combination, as far as we know, it has been described only with concordant ventriculo-arterial connections, for which seven one-stage biventricular repairs have now been described. We describe here the case of a 3½-month-old male infant with a similar combination but with discordant ventriculo-arterial connections and severe pulmonary hypertension. It was the right ventricle which achieved normal size in this arrangement, presumably because of the ventricular septal defect. One-stage biventricular correction was accomplished, employing a single pulmonary allograft, aided by massive doses of Prostaglandin El. As far as we know this is the first report of the combination of aortic atresia, discordant ventriculo-arterial connections, a ventricular septal defect and balanced ventricles. We complement our surgical account, nonetheless, with a description of a comparable specimen from our anatomic museum.
Use of balloon expandable stents in the palliative relief of obstructed right ventricular conduits
Authors: Hayes Alison M. , Nykanen David G. , McCrindle Brian W. , Smallhorn Jeffrey F. , Freedom Robert M. , Benson Lee N. 
Published: August 2008

AbstractObjectivesThe purpose of this study was to determine the efficacy of balloon expandable stents in the relief of obstructed conduits in the right heart, and to review both the immediate success and longevity of this palliation.Methods and resultsEndovascular stents where placed in 17 children, median age 4.7 yrs (range 0.46–16.2 yrs), at a median of 1.6 yrs (range 0.33–6.7 yrs) after placement of conduits at surgery. A second stent was required in two children, either because of migration of the initial stent or residual proximal stenosis. Mean right ventricular to systemic pressure ratios were reduced from 0.77 ±0.24 to 0.50±0.13, and the minimum area of the pulmonary outflow tract increased from 24±12% to 69±26% of that expected for body surface area. Further surgery was needed in 8 patients following placement of the stent, in 5 within 10 months of implantation, due to inadequate relief of obstruction. Nonetheless, 3 of these 8 children achieved satisfac­tory palliation 18, 21 and 24 months prior to requiring insertion of a new conduit. Actuarial freedom from reoperation was 44% at 24 months. The remaining 9 children continue to benefit from the relief in obstruction provided by the stent at a mean interval from implantation of 24±8 months. In this group, the mean Doppler gradient across the right ventricular outflow tract was 35 ±13 mmHg (n=6).ConclusionsThese early data indicate that endovascular stents are useful in the palliation of obstructed right ventricular conduits. In the short-term, the relief of obstruction achieved is well maintained, and has either delayed or obviated the need for insertion of new conduits.
Misleading echocardiographic diagnosis of anomalous origin of the right coronary artery from the pulmonary artery
Authors: Michielon G. , Stellin G. , Milanesi O. 
Published: August 2008

AbstractWe report on a case of a newborn with echocardiographic diagnosis of anomalous origin of the right coronary artery from the main pulmonary artery. Intraoperative inspection demonstrated a proximal anomalous course of the right coronary artery but a normal origin from the right sinus of Vansalva of the Aorta. We suggest a complete investigation, including coronary angiography, when isolated and rare congenitaj malformations of the coronary arteries are suspected.
Surgical correction of a patient with discordant atrioventricular and concordant ventriculo-arterial connections
Authors: Morita Kiyozo , Kurosawa Hiromi , Miyamoto Hisaki 
Published: August 2008

AbstractThe combination of discordant atrioventricular and concordant ventriculoarterial connections is an extremely rare entity, with only 8 cases of successful surgical repair being previously reported. Although from the clinical standpoint this anomaly is similar to complete transposition, this unusual combination is clearly suited by its morpho-physiological features to an atrial rather than an arterial switch procedure. A 17-year-old boy with this malformation, associated also with severe pulmonary hypertension, underwent a palliative Senning procedure, leaving the ventricular septal defect open and banding the pulmonary trunk. Postoperatively, hypoxemia was markedly improved, and was associated with a reduction of the level of pulmonary hypertension, contributing to marked symptomatic improvement.
The echocardiographic anatomy of ventricular septal defects
Authors: Gatzoulis Michael A. , Li Jia , Ho Siew Yen 
Published: August 2008

AbstractMany of the controversies surrounding the description of ventricular septal defects arise from differences in the perspectives from which they are viewed. In this review, we analyse these defects as seen in cross-sectional echocardiographic images, correlating them with morphologic specimens. The classification we advocate, which now has a suitable pedigree, is a simple descriptive method distinguishing between perimembranous, muscular, and doubly committed types of defects. The approach is to categorise the defects as seen from the right ventricle, the usual port of access for surgeons. The term ‘perimembranous’ highlights the proximity of the atrioventricular conduction axis to the margin of the defects in which the remnant of the membranous septum forms a direct border. This system is applicable to all interventricular communications, no matter how malformed the heart may be in which they are enclosed.
Effects of positive pressure ventilation and inspired oxygen on pulmonary vascular resistance and tissue oxygen delivery in neonatal pigs
Authors: Ransbæk Flemming , Hansen Soren B , Austin Erle H. , Santamore William P. 
Published: August 2008

AbstractManagement of pulmonary vascular resistance in neonates with congenital heart disease is important for stabilization before and after surgical interventions. Thus, we determined which combina tion of positive end-expiratory pressure ventilation and fraction of oxygen in the inspired air increases pulmonary vascular resistance without compromising delivery of oxygen to the tissue. Eight piglets were anesthetized, intubated and ventilated. Pulmonary flow and pulmonary arterial and left atrial pressures were monitored continuously. At all levels of inspired oxygen (1.00, 0.21 and 0.15), ventilation at a pressure of 15 cm of water increased pulmonary vascular resistance. At all levels of positive pressure ventilation, a fraction of 0.15 of inspired oxygen increased pulmonary vascular resistance. The combination of a ventilatory pressure of 15 cm of water and inspired oxygen of 1.00, or ventilatory pressure at 5 cm of water and oxygen delivery of 0.15, produced similar changes in pulmonary vascular resistance (19.1 ± 2.8 vs. 20.0 ± 3.8 mmHg/(L/min)) and cardiac output (0.78 ± 0.07 vs. 0.93 ± 0.10 L/min) but, the higher level of positive pressure plus 1.00 inspired oxygen gave a significantly higher arterial oxygen saturation (0.99 ± 0.03 vs. 0.72 ± 0.19%) and delivery of oxygen to the tissues (13.7 ± 2.9 vs. 7.4 ± 1.5 ml 02/min, p<0.05). Thus, both high positive pressure ventilation and hypoxia increase pulmonary vascular resistance. Only high pressure ventilation plus high concentrations of inspired oxygen, however, increased pulmonary vascular resistance without compromising delivery of oxygen, suggesting that this combination is a superior means of increasing pulmonary vascular resistance.
Palliative arterial switch for complete transposition with ventricular septal defect
Authors: Elizari Amalia , Somerville Jane 
Published: August 2008

AbstractA 28-year-old female patient with complete transposition, ventricular septal defect and persistence of the arterial duct underwent a palliative arterial switch procedure in 1976 at 7 years of age. Therefore, she has survived for 22 years and lives a near normal life. She is married, has been counselled against pregnancy and has increasing cyanosis with the typical features of the Eisenmenger syndrome.
‘You didn't see them, but now you do!': use of balloon occlusion angiography in the identification of systemic venous anomalies before and after cavopulmonary procedures
Authors: Ovaert Caroline , Filippini Luc H. P. M , Benson Lee M. , Freedom Robert M. 
Published: August 2008

AbstractWe describe the importance of angiographic identification of collateral venous channels by balloon occlusion venography after bidirectional cavopulmonary connections. Use of the balloon occlusion technique is essential for identification of these vessels, as they can easily be missed by standard venous angiography, with possible clinical consequences for postoperative management.
Successful management of a pregnancy at high risk because of Eisenmenger reaction
Authors: Daliento Luciano , Menti Liana , Lenardo Laura Di 
Published: August 2008

AbstractInformed medical care, appropriate maternal and fetal monitoring, and cooperation among experienced obstetricians, anesthesists cardiologists, particularly during delivery and the early puerperium associated with psychological preparation of the mother, were the bases of the successful management of a pregnant 28-year-old woman at high risk because of the Eisenmenger reaction.
Atrial thrombus after the Fontan-operation: predisposing factors, treatment and prophylaxis
Authors: Kaulitz R. , Ziemer G. , Bergmann F. , Luhmer I. , Kallfelz H. C. 
Published: August 2008

AbstractThrombosis of the right side of the heart has previously been a rarely reported early or late postoperetive complication after a modified Fontan-procedure.In 4(5.5%) of 72 surviving patients who underwent a total cavopulmonary connection for various forms of univentricular atrioventricular connection or other complex lesion a thrombus within the right atrium was diagnosed by transthoracic echocardiogaphy either early (in 3 cases) or late (in one cases) postoperatively. Predisposing haemodynamic abnormalities could be identified in all four patients, but no patient was found to have severe abnormalities of coagulation. Two patients presented with acute clinical symptoms on the third and twelfth day after initial surgery. They underwent emergency reoperation with thrombectomy followed by prophylactic treatment with coumadin or aspirin after initial heparinization. In the other 2 patients, routine postoperative echocardiography revealed a right atrial thrombus without specific clinical symptoms 25 days or 3 months postoperatively. Thrombolytic therapy with an initial bolus of tissue plasminogen activator (0.1 mg/kg) followed by continuous infusion (1 mg/kg/day) combined with heparinization yielded complete resolution of the thrombus after 27 and 40 hours, respectively, as demonstrated on follow-up echocardiograms. Our own experience, coupled with a review of the literature, indicate that prophylactic anticoagulation therapy with coumadin should continue for at least 4 to 6 months for patients with an increased risk for postoperative thrombosis. For infants and young children with a low risk for postoperative thrombosis, aspirin should be given routinely for 6 months postoperatively.
Morphometric study of the great arterial trunks and their branches in the human fetal heart with perimembranous ventricular septal defects
Authors: Alvarez Luis , Aránega Antonia , Vélez Celia , Melguizo Consolación , González Amanda R. , Saucedo Robert 
Published: August 2008

AbstractMorphometric methods were used to study the great arterial trunks in a total of 22 human fetuses and newborn subjects weighing from 1 to 3.8 kg (mean 2.22 ± 0.85 kg). All specimens displayed concordant atrioventricular and ventriculoarterial connections, and all had perimembranous ventricular septal defects. Thirteen different parameters were measured in each specimen, comprising the orifices of the great arterial trunks, ascending aorta, vessels branching from the aortic arch, the aortic isthmus, descending aorta, pulmonary trunk, orifices of the pulmonary arteries, and arterial duct. The values were Compared with patterns of normality established in an earlier study using hearts from 496 human fetuses and neonates weighing form 60 to 5000 g. Minimum square regression analyses were used to study the relationships between fetal and neonatal log body weight in kilograms, and the various cardiometric parameters. We believe that these morphometric data have immediate clinical and surgical applications in the treatment of fetal and neonatal cardiovascular disorders.
The feasibility of high-dose dobutamine stress echocardiography in children
Authors: Pahl Elfriede , Chrystof Dale , Webb Catherine L. , Shulman Stanford T. , Chaudhry Farooq A. 
Published: August 2008

AbstractWe recently reported our experience with exercise stress echocardiography in children with prior history of Kawasaki disease and coronary arterial involvement. Dobutamine stress echocardiography has been used in adults (doses up to 50 μg/kg/min) for diagnosis and risk stratification of coronary arterial disease. To date, no studies have assessed the safety and utility of high-dose dobutamine for the detection of abnormalities of wall motion in children, although low dose dobutamine (5 μg/kg/min) has been used to assess left ventricular function after use of anthracycline in children. Our study group consisted of 10 children known to have Kawasaki disease, and 16 children who had undergone heart transplantation. After baseline transthoracic echocardiographic imaging, dobutamine was infused in an incremental fashion, 10–40 μg/kg/min intravenously, to attain peak heart rate. Heart rate and blood pressure were monitored continuously, and 4 standard echocardiographic images were acquired at each stage of dobutamine infusion and at recovery. Images were digitized, and the left ventricular segments were reviewed to assess wall motion. No patients suffered serious side effects such as arrhythmias or hypotension. Studies were complete in all 26 patients. One study proved positive, with a new abnormality of wall motion identified corresponding to a critical right coronary arterial stenosis. Peak heart rate and blood pressure were similar for the 2 groups. High dose dobutamine stress echocardiography, therefore, is feasible and safe in children, and may detect flow-limiting coronary arterial stenoses noninvasively. The technique may safely be applied to other subsets of children where assessment of ischemia and contractile reserve is warranted.
Post-Operative cardiac strangulation
Authors: Waldman J.Deane , Karp Robert B. , Ferdinand Francis 
Published: August 2008

AbstractA patient is reported in whom cardiac strangulation occurred through a lateral pericardial defect surgically created for repair of congenital heart disease. To avoid this potentially fatal complication, the inferior rim of an iatrogenic lateral pericardial defect should be tacked down to preclude cardiac herniation.