bluuebunny/crossref_metadata_2025_split huggingface dataset:
Long-term results after valvotomy for congenital aortic valvar stenosis in children
Authors: Lambert Virginie , Obreja Daniela , Losay Jean , Touchot-Koné Anita , Piot Jean-Dominique , Serraf Alain , Lacour-Gayet François , Planche Claude 
Published: August 2008

AbstractAs interest increase in the Ross procedure performed as a therapeutic option for children with congenital aortic valvar stenosis, it becomes increasinly important to know the late results of aortic valvotomy in this population. We have therefore examined retrospectively the medical records of 121 consecutives survivors undergoing aortic valvotomy before 10 years of age between 1974 and 1992. The mean age at the first valvotomy was 29 months, with a range from 3 days to 10 years. The mean duration of follow up was 9.4 years, with a range from 1.6 to 22 years. Fifteen patients (12.3%; 70% CL: 10–16) died: 9 following reoperation, and 6 late after surgery. Death was related to the hearts in 86% of cases. The actuarial survival rate was 79% (70% CL: 72/84) at 10 years. Young age at the first valvotomy, and the number of procedures, emerged as risk factors of secondary mortality. Reoperations on the aortic valve, 73 in all, were required in 56 patients. The second procedure was done after a mean interval of 6 years, with a rangefrom 1 day to 18 years. This was for restenosis in three-quarters of the cases. The aortic valve was replaced in 30 patients, at a mean of 9 years, and with a range from 9 months to 18 years, after the first procedure. The survival without replacement at 20 years was 29% (70% CL: 15–49). No factor was identified with a relationship either to reoperation or valvar replacement. Long term results after aortic valvotomy, therefore, show a high late mortality, frequent reinterventions, and an almost inescapable eventual need for valvar replacement. The ongoing use of the Ross operation is justified, even if longterm studies in children are still needed to validate its use.
The middle aortic syndrome: an important feature of Williams' Syndrome
Authors: Radford Dorothy J. , Pohlner Peter G. 
Published: August 2008

AbstractThe middle aortic syndrome, with diffuse narrowing of the thoracic and abdominal aorta, was present in 10 of 18 patients with Williams' syndrome (55%). There were 3 thoracic coarctations, and 2 abdominal coarctations, with gradients greater than 20 mmHg across the zone of narrowing. Seven patients had mild renal arterial stenosis, and 6 had visceral arterial stenoses. Ten were hypertensive. Measured dimensions of the aortic lumen failed to increase with age in 3 males who had serial angiographic studies. One developed mesenteric arterial stenosis, with mild bilateral renal arterial stenoses, between the ages of 9 and 19 years. Aortic intravascular ultrasound performed in 2 patients confirmed abnormally thick vessel walls with small lumens. Diffusely narrowed and thick-walled stiff arteries, lacking elastin, are a feature of Williams' syndrome. The arteriopathy tends to progress with age, and systemic hypertension is common in teenagers and beyond. The middle aortic syndrome was present in more than half our patients, and does not necessarily reflect a bias because of cardiologic referral. Aortography with measurement of aortic diameters and delineation of the visceral branches is an important requirement for complete evaluation of patients with Williams' syndrome.
Transthoracic three-dimensional echocardiography for the assessment of straddling tricuspid or mitral valves
Authors: Vogel Michael , Ho Siew Yen , Lincoln Christopher , Anderson Robert H. 
Published: August 2008

AbstractBackgroundThe advent of 3D echocardiography has provided a technique which, potentially, could afford significant additional information over conventional cross-sectional echocardiography in the assessment of patients with straddling atrioventricular valves prior to surgical correction.MethodsEight patients, aged from 1 month to 9˙2 years, were examined with 3D echocardiography. All but three had discordant ventriculoarterial connections or double outlet right ventricle. Data suitable for reconstruction was acquired with transthoracic scanning. Right and left ventricular volumes were calculated in the 3D dataset.Results3D echocardiography proved capable of defining the exact degree of straddling by imaging theproportion of tension apparatus attached to either side of the ventricular septum. It was able also to display the atrioventricular junction “en face”, thus permitting identification of the precise site of insertion of the muscular ventricular septum relative to the atrioventricular junction. This made it possiblefirst, to calculate the degree of valvar override, and second, to predict the location of the penetrating atrioventricular bundle. End-diastolic volume of the right ventricle in those with straddling tricuspid valves was 73 (61–83)% of normal, and, of the left ventricle in those with mitral valvar straddling 71 (40‐97)% of normal.Conclusions3D echocardiography can aid in planning the optimal surgical procedure in patients with straddling or overrriding atrioventricular valves, as it provides diagnostic information superiorto standard crosssectional techniques. It also allows for exact measurement of the volumes of the respective ventricles.
Myocardial high-energy phosphate metabolism is altered after treatment with anthracycline in childhood
Authors: Eidenschink Andrea B. , Schröter Gerrit , Müller-Weihrich Stefan , Stern Heiko 
Published: August 2008

AbstractObjectivesWe aimed to investigate whether changes in high-energy phosphate metabolism after treatment of children and young adults with anthracycline can be demonstrated non-invasively by31P magnetic resonance spectroscopy.BackgroundAbnormal myocardial energy metabolism has been suggested as a mechanism for anthracycline-induced cardiotoxicity. Deterioration in such has been shown in animal studies by resonance spectroscopy.MethodsWe studied 62 patients, with a mean age of 13.5 ±5 years,3.7±4.3 years after a cumulative anthracycline dose of 270±137 mg/m2. Normal echocardiographic findings had been elicited in 54 patients. The control group consisted of 28 healthy subjects aged 20±7 years. Resonance spectrums of the anterior left ventricular myocardium were obtained at 1.5 Tesla using an image-selected in vivo spectroscopy localization technique.ResultsThe ratio of phosphocreatine to adenosine triphosphate after blood correction was 1.09±0.43 for the patients, and 1.36±0.36 (mean±SD)for controls (p = 0.005), with a significantly reducedmean ratio even in the subgroup of patients with normal echocardiographic results ( l.11 ± 0. 44 versus1.36±0.36, p=0.01). The ratio did not correlate with the cumulative dose of anthracycline. The ratio of phosphodiester to adenosine triphosphate was similar in patients and controls (0.90±0.56 versus 0.88±0.62).ConclusionsIn patients treated with anthracyclines in childhood, myocardial high-energy phosphate metabolism may be impaired even in the absence of cardiomyopathy. Our data support the concept that anthracycline-induced cardiotoxicity is not clearly dose dependent.
Familial absent pulmonary valve syndrome without deletions of chromosome 22q11
Authors: McElhinney Doff B. , Hanley Frank L. , Stanger Paul 
Published: August 2008

AbstractDeletions of chromosome 22q11 are common in patients with tetralogy of Fallot, and in those with absent pulmonary valve syndrome. In this report, we describe a pair of siblings with absent pulmonary valve syndrome, neither of whom had deletions of chromosome 22q11. The finding of familial absent pulmonary valve syndrome without deletion of 22q11 in our patients suggests an alternative genetic basis for this rare condition.
Fetal diagnosis of lethal dysfunction of the right heart in three siblings
Authors: Hornung Tim S. , Heads Alison , Wright Chris , Hunter Stewart 
Published: August 2008

AbstractA woman, having already delivered one child, underwent fetal echocardiography during thre subsequent pregnancies. All three showed enlargement and poor function of the right-sided chambers. The first was still-born, the second died as a neonate, while the third pregnancy was terminated. Pathological examination revealed the same findings in each fetus, possibly representing a variation of Uhl's anomaly, or alternatively a hitherto unrecognised cardiomyopathic process.
Early extubation after surgical repair of tetralogy of Fallot
Authors: Shekerdemian Lara S. , Penny Daniel J. , Novick William 
Published: August 2008

AbstractIn recent years, post-operative intensive care of the child with congenital cardiac disease has placed an emphasis on earlier weaning from mechanical ventilation. We describe our experience of postoperative fast-tracking of children undergoing cardiac surgery during a charitable mission in Venezuela, where resources and equipment were severely limited. During our stay, 11 children, with a median age of 2 years, underwent total correction of tetralogy of Fallot. The median duration of ventilation was 2.5 hours, and all patients were extubated within 12 hours of surgery. Effective analgesia was achieved without the need for continuous intravenous infusions of opiates. This experience shows that early extubation can safely be carried out in well-selected patients after surgery to correct congenital cardiac malformations. This allows faster throughput of patients, and helps provide an efficient and cost-effective service.
Recanalization of an occluded modified Blalock-Taussig shunt by balloon angioplasty within 12 hours of its construction
Authors: Galal M. O. , Attas K. , Baslaim G. 
Published: August 2008

AbstractAn infant developed severe desaturation within a few hours of construction of a modified Blalock-Taussig shunt. Echocardiography revealed that the shunt had become occluded, and this was confirmed angiographically. At catheterisation, therefore, we passed a 0.014”percutaneous transluminal coronary angioplasty wire through the occluded shunt into the right pulmonary artery and then dilated the shunt successfully using a 5 mm coronary angioplasty balloon. Six weeks later, the shunt remained patent.
Endovascular stenting as an emergency treatment for neonatal coarctation
Authors: Fink Christoph , Peuster Matthias , Hausdorf Gerd 
Published: August 2008

AbstractWe describe a neonate with critical coarctation of the aorta. Since treatment with Prostaglandin El had failed to reopen the arterial duct, and surgery was deemed to be associated with an unacceptably high risk in this unstable neonate, it was decided to perform balloon dilation of the coarctation as a palliative procedure to stabilize the patient. Balloon angioplasty failed to reduce the pressure gradient across the coarctation, so a stent was implanted retrogradely as an emergency procedure. Subsequently, the stented segment was resected surgically and end-to-end anastomosis created without complications at seven months of age.
Recommendations of the British Paediatric Cardiac Association for therapeutic cardiac catheterisation in congenital cardiac disease
Authors: Qureshi Shakeel A. , Redington Andrew N. , Wren Christopher , Ostman-Smith Inga , Patel Raman , Gibbs John L. , de Giovanni Joe 
Published: August 2008

AbstractThe aims of these recommendations are to improve the outcome for patients after, and to provide acceptable standards of practice of, therapeutic cardiac catheterisation performed to treat congenital cardiac disease. The scope of the recommendations includes all interventional procedures, recognising that for some congenital malformations, surgical treatment is equally as effective as, or occasionally preferable to, interventional treatment. The limitations of the recommendations are that, at present, no data are available which compare the results of interventional treatment with surgery, and certainly none which evaluate the numbers and types of procedures that need to be performed for the maintenance of skills. Thus, there is a recognised need to collect comprehensive data with which these recommendations could be reviewed in the future, and re-written as evidence-based guidelines. Such a review will have to take into account the methods of collection of data, their effectiveness, and the latest developments in technology. The present recommendations should, therefore, be considered as consensus statements, and as describing accepted practice, which could be used as a basis for ensuring and improving the quality of future care.
Evolving management for critical pulmonary stenosis in neonates and young infants
Authors: Cheung Yiu-fai , Leung Maurice P. , Lee Jan W. T. , Chau Adolphus K. T. , Yung Tak-cheung 
Published: August 2008

AbstractOver the years, management of critical pulmonary stenosis in young infants has evolved from surgical reconstruction of the right ventricular outflow tract and closed pulmonary valvotomy to transcatheter balloon valvoplasty. Our study aimed at evaluating how the changing policy for management had affected the immediate and long term outcomes of babies with this cardiac lesion. Interventions were made in 34 infants at a median age of 8.5 days (2–90 days). Reconstruction of the right ventricular outflow tract reconstruction was performed in 10 patients, closed pulmonary valvotomy in 13, and balloon valvoplasty in 11. Initial procedure-related mortality was 50%, 15% and 0% respectively. Multivariate analysis revealed transannular patching of the right ventricular outflow tract, and male sex, to be significant factors for death. For the 27 survivors, the ratio of right ventricular to systemic systolic pressure decreased from 1.6 ± 0.3 to 0.3 ± 0.2 after reconstruction of the outflow tract, 1.8 ± 0.5 to 0.8 ± 0.4 after closed valvotomy, and 1.8 ± 0.6 to 0.9 ± 0.3 after balloon valvoplasty. The decrease was significantly greater after patch reconstruction (p=0.025) that required no further reinterventions. The overall rate of reintervention for the survivors was 37% (10/27). The freedom from reintervention after closed valvotomy was 82%, 64% and 51% at 1, 5 and 10 years respectively. The figure remained at 78% at both 1 and 5 years (p=0.66) after balloon valvoplasty. The higher reintervention rate for closed valvotomy corresponded to the significantly greater residual gradient across the pulmonary valve noted on follow-up (p=0.01). Reinterventions included balloon dilation (n=6), reconstruction of the outflow tract (n=4), and 1 each of ligation of an arterial duct and systemic-pulmonary arterial shunting. The risk factor for reintervention was a hypoplastic right ventricle. In conclusion, transcatheter balloon valvoplasty appears to be the optimum initial approach in view of its low mortality, efficacy at relieving the obstruction, and low rate of reintervention.
Growth of children with a functionally single ventricle following palliation at moderately increased altitude
Authors: Day Ronald W. , Denton David M. , Jackson W. Daniel 
Published: August 2008

AbstractThe bidirectional Glenn and Fontan procedures are empirically performed as interim and definitive procedures in children with a functionally single ventricle. The optimal stage of palliation, nonetheless, remains unknown. During childhood, growth is a fundamental measure of response to therapy. Growth may be influenced by the degree of cyanosis, the volume load on the ventricle, and cardiac performance. Thus, the weight and stature of children with a functionally single ventricle who underwent a bidirectional Glenn procedure or a Fontan procedure were studied to determine the effect of each intervention on growth. Z scores for weight and stature were retrospectively determined prior to palliation, at yearly intervals for 4 years, and from long-term measurements until 18 years of age in all patients with at least 2 years of observation following palliation. Growth was evaluated in 54 patients with a bidirectional Glenn procedure, and 65 patients with a Fontan procedure. The Z scores for weight were improved after each method of surgical palliation. Stature, however, was improved only following the bidirectional Glenn procedure. Growth was impaired in patients who developed protein losing enteropathy. Weight improved only during the initial 2 years after the Fontan procedure in patients who had a surgical fenestration. Over the long-term, patients who underwent a Fontan procedure were more likely to have a Z score less than −2.0 for weight and stature than patients who underwent only a bidirectional Glenn procedure. Late mortality and the incidence of heart transplantation were increased in patients who experienced a decrease in their rate of growth, defined as a negative change of more than one Z score in weight or stature, following the Fontan procedure. In conclusion, at moderately increased altitude, children with a functionally single ventricle grow more appropriately following the bidirectional Glenn procedure than following the Fontan procedure. A decrease in the rate of growth is associated with a poor prognosis following the Fontan procedure.
Similarities and differences in the exercise performance of patients after a modified Fontan procedure compared to patients with complete transposition following a Senning operation
Authors: Buheitel Gernot , Hofbeck Michael , Gerling Stephan , Koch Andreas , Singer Helmut 
Published: August 2008

AbstractObjectiveTo investigate cardiopulmonary performance in patients after a Fontan procedure, comparing it to patients following a Senning operation.MethodsWe studied 21 children, with a mean age of 11.1 years, after a total cavopulmonary anastomosis, comparing them to 13 with complete transposition after a Senning procedure, having a mean age of 11.8 years, and 21 control subjects with a mean age of 11.2 years. All were tested on a bicycle ergospirometer.ResultsPeak consumption of oxygen, maximal work rate, peak oxygen pulse and endexpiratory pressure of carbon dioxide at a work rate of 1.5 Watt/kg were lowest in patients with a modified Fontan procedure, and highest in the control group (p≤ 0.0278). Production of carbon dioxide, and minute ventilation at a work rate of 1.5 Watt/kg, was highest in the patients after Fontan procedure, and lowest in the healthy subjects (p≤ 0.0163). Production of carbon dioxide per single breath was lower in those having a Fontan procedure (28.9 ml) than in the two other groups (35.1 ml; p = 0.0243). The tidal volume showed no significant differences between the three groups.ConclusionsThe reaction to exercise was identical qualitatively in both groups of patients, and comparable to the behaviour of patients with chronic heart failure. Quantitatively, the results of the patients following a Senning procedure lay between those of control subjects and those who had undergone a Fontan operation. The only exception was dead space ventilation, where the patients after a Fontan procedure differed from the two other groups because of their increased ventilation-perfusion mismatch.
Immediate and early results of closure of moderate to large patent arterial ducts using the new Amplatzer™ device
Authors: Marwah Ashutosh , Radhakrishnan S. , Shrivastava Savitri 
Published: August 2008

AbstractObjectiveOur aim was to assess the immediate and short term results of closure of moderate and large patent arterial ducts using the self-expanding and repositionable Amplatzer device.MethodWe attempted closure in 25 patients (10 Females and 15 males) using the Amplatzer occluder. Their median age was 48 months with a range from 8months to 26years and median weight of 14kg with a range from 4.5kg to 48kg. The mean ductal diameter was 4 (S.D 1.51mm). A 6F/7F long sheath was used to deliver the device. Follow up was performed with colour- flow mapping of the pulmonary trunk within 24 hours, at 3 months, and 6 months of closure.ResultsOf the 25 patients, the device was placed successfully in 23. Concurrent angiography showed immediate closure in 12 patients, while 8 had trivial shunting and 3 had mild shunting. Within 24 hours, Doppler examination revealed complete closure in all but three patients, who had a mild residual shunt. Two attempts were unsuccessful. Both these patients underwent successful surgical ligation. All except one patient were discharged on the next day. Of the 23 patients, 15 (65%) have been followed up for 3months, while 8 (35%) have completed 6 months of follow-up. Of the three patients initially with mild residual flow, two had completely closed at 3 months The one remaining patient is yet to be seen at the 3 month follow-up. Thus, at 3 months, all patients studies had shown complete closure.ConclusionAntegrade transcatheter closure using the Amplatzer duct occluder is an efficacious treatment for bigger patent arterial ducts. Long-term follow-up is necessary to show sustained benefits and confirm the absence of side effects.
Surgical approach to complicated cervical aortic arch: anatomic, developmental, and surgical considerations
Authors: McElhinney Doff B. , Thompson LeNardo D. , Weinberg Paul M. , Jue Kenneth L. , Hanley Frank L. 
Published: August 2008

AbstractBackgroundAbnormalities of brachiocephalic arterial branching and arch laterality are common in patients with a cervical aortic arch. In addition, structural anomalies of the arch such as obstruction, aneurysms, and tortuosity are found in a significant number of cases.MethodsBetween 1990 and 1998, 6 patients underwent surgery for an obstructed right cervical arch. A significant obstruction was present at the transverse or distal arch in all patients, and was recurrent after previous repair in 2. In 1 patient, there was also a multi-lobed aneurysm of the aortic segment contiguous to the obstruction, and in 2 there was marked tortuosity of the arch. In all cases, the order of origin of the head and neck vessels was abnormal, and obstruction of 1 or more brachiocephalic vessels was found in 3. A vascular ring was present in all patients, with a right aortic arch and aberrant left subclavian artery in 4 patients and a double aortic arch with a dominant right cervical arch in 2. The descending aorta was circumflex (left-sided) in 3 patients. Three patients were repaired through a standard right posterolateral thoracotomy, and 3 through a median sternotomy. Patch augmentation aortoplasty was used in 2 patients, a tube graft from the ascending to descending aorta in 2, end to side anastomosis of the descending aorta to the proximal arch in 1, and direct anastomosis to reconstruct an atretic left-sided component of a double arch in 1.ResultsRepair was successful in all cases, with no perioperative complications. At follow-up ranging from 1 to 9 years, all patients were alive and well, with no recurrence of arch obstruction or other significant complications. Fluorescentin situhybridization revealed microdeletion of chromosome 22q 11 in 1 patient (not performed in the others).ConclusionsStructural anomalies of the arch are relatively common in patients with a cervical aortic arch. Such abnormalities may be the result of hemodynamic conditions and/or abnormal vascular tissue related either to the cervical position of the arch or its embryologic precursors. Given the highly variable anatomy of patients with a complicated cervical aortic arch, surgical considerations will vary in kind.
Diagnosis of left juxtaposition of the atrial appendages in the fetus
Authors: Abdullah Mustafa , Yoo Shi-Joon , Lee Young-Ho , Smallhorn Jeffrey , Hornberger Lisa K. 
Published: August 2008

AbstractWe describe two cases of left juxtaposition of the atrial appendages in which fetal echocardiograms provided a clue to the diagnosis. Both cases were associated with complex cyanotic congenital heart disease. The clue to the diagnosis was found at the three-vessel view. Abnormal vascular spaces were seen on the left side of the cross-sections of the great arterial trunks.