bluuebunny/crossref_metadata_2025 huggingface dataset:
The middle aortic syndrome: an important feature of Williams' Syndrome
Authors: Radford Dorothy J. , Pohlner Peter G.
Published: August 2008

The 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

Background

The 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.

Methods

Eight 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.

Results

3D 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.

Conclusions

3D 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

Objectives

We 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.

Background

Abnormal 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.

Methods

We 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.

Results

The 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).

Conclusions

In 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

Deletions 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

A 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

In 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

An 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

We 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

The 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

Over 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

The 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

Objective

To investigate cardiopulmonary performance in patients after a Fontan procedure, comparing it to patients following a Senning operation.

Methods

We 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.

Results

Peak 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.

Conclusions

The 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

Objective

Our 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.

Method

We 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.

Results

Of 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.

Conclusion

Antegrade 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

Background

Abnormalities 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.

Methods

Between 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.

Results

Repair 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).

Conclusions

Structural 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

We 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.
Balloon aortic valvotomy through a carotid cutdown in infants with severe aortic stenosis: results of the multi-centric registry
Authors: Robinson Blair V. , Brzezinska-Rajszys Grazyna , Weber Howard S. , Ksiazyk Joanna , Fricker F. Jay , Fischer Donald R. , Ettedgui José A.
Published: August 2008

Objectives

The purpose of this study was to evaluate the short and intermediate term results of infants who have undergone balloon aortic valvotomy from the carotid arterial approach, and to identify risk factors in those infants who had a poor outcome.

Methods

Between 1988 and 1999, balloon aortic valvotomy was attempted at four centres in 95 infants with severe aortic stenosis. Echocardiographic and hemodynamic data, and outcome, were analysed retrospectively.

Results

Valvotomy was accomplished in 92 of the 95 infants, with a median age of 5 days, a range from 0 to 191 days, and weighing 3.4 kg, with a range from 1.0 to 6.5 kg. Major procedural complications occurred in 10 infants. Post-procedural aortic regurgitation was severe in 5 patients. There were 13 early deaths, and 4 late deaths. The period of mean follow-up has been 2.1 years, with a range from 0 to 9.3 years. The actuarial survival at 3 years was 76 ± 6%. Further interventions were needed in 19 patients, giving a 3-year freedom from reintervention of 67 ± 6%. The 51 infants who were duct-dependent were further analyzed, and found to have a higher mortality (38%) compared to those infants not dependent on the arterial duct (5%). Risk factors for a poor outcome in the duct-dependent infants were mitral stenosis (p<0.005), a left ventricle which did not form the cardiac apex (p<0.005), and an aortic valve with a diameter of less than 6 mm (p<0.05).

Conclusions

This multi-centric registry shows good results in the intermediate term for treating infants with severe aortic valvar stenosis with balloon valvotomy through a carotid arterial cutdown. Infants dependent on prostaglandin had a worse outcome, especially if they had any of the identified risk factors.
Embryology of congenital ventriculo-coronary communications: a study on quail-chick chimeras
Authors: Männer Jörg
Published: August 2008

Ventriculo-coronary arterial communications are rare congenital heart defects which have been explained traditionally on the basis of abnormal persistence of such communications found in the normal developing heart. Recent studies, however, have suggested that these embryonic communications might be an incidental finding rather than a normal feature. Thus, it has been suggested that congenital ventriculo-coronary communications do not represent remnants of normal embryonic vessels, but rather represent acquired lesions. In the present study, hearts were constructed in embryonic chicks in which the coronary vasculature was almost completely derived from a quail-donor. After immunohistochemical staining of the quail-derived coronary endothelium, chimeric hearts were analysed with respect to the presence of embryonic ventriculo-coronary communications, and with respect to the origin of these structures from either coronary arteries or endocardium. The results demonstrate the normal presence of ventriculo-coronary communications in avian embryonic hearts. They show, furthermore, that these structures are of coronary endothelial origin. The findings are in accord with the traditional view on the pathogenesis of congenital ventriculo-coronary communications. The roles of elevated ventricular pressure, abnormal remodelling of the developing myocardium, and of abnormal growth of the coronary vasculature are discussed relative to the pathogenesis of congenital ventriculo-coronary communications.
Psychological distress and styles of coping in parents of children awaiting elective cardiac surgery
Authors: Utens Elisabeth M. , Versluis-Den Bieman Herma J. , Verhulst Frank C. , Witsenburg Maarten , Bogers Ad J.J.C. , Hess John
Published: August 2008

Aims

We sought to assess the level of psychological distress, and the styles of coping of, parents of children with congenital heart disease. The study was based on questionnaires, which were completed, on average, four weeks, with a range from 0.1 to 22.1 weeks, prior to elective cardiac surgery or elective catheter intervention.

Methods

We used the General Health Questionnaire, and the Utrecht Coping List, to compare scores from parents of those undergoing surgery, with scores of reference groups, and with scores of the parents of those undergoing intervention.

Results

Overall, in comparison with our reference groups, the parents of the 75 children un dergoing surgery showed elevated levels of psychological distress, manifested as anxiety, sleeplessness, and social dysfunctioning. They also demonstrated less adequate styles of coping, being, for example, less active in solving problems. With only one exception, no differences were demonstrated in parental reactions to whether cardiac surgery or catheter intervention had been planned. The mothers of the 68 patients who were to undergo cardiac surgery, however, reported greater psychological distress and manifested greater problems with coping than did the fathers.

Conclusion

Elevated levels of psychological distress, and less adequate styles of coping, were found in the parents of patients about to undergo cardiac surgery, especially the mothers, when compared to reference groups. Future research should investigate whether these difficulties persist, and whether this will influence the emotional development of their children with congenital cardiac malformations.
Is there a possible role for haemostasis in the development of perigraft reaction complicating the modified Blalock Taussig shunt?
Authors: Salzer-Muhar Ulrike , Pabinger-Fasching Ingrid , Zacherl-Wightman Sophie
Published: August 2008

The perigraft reaction is an unusual complication found in patients in whom a modified Blalock Taussig shunt has been created using a polytetrafluoroethylene graft. We found that, in two infants, consistent laboratory findings during such a perigraft reaction were hypofibrinogenemia, increased levels of thrombin-antithrombin III complex, prothrombinfragment 1 and 2 and products of degradation of fibrin. Normalization of the levels of fibrinogen produced resolution of the perigraft reaction.
Severe haemolysis after transcatheter closure of a patent arterial duct with the new Amplatzer™ duct occluder
Authors: Godart François , Rodés Josep , Rey Christian
Published: August 2008

Severe mechanical haemolysis occurred in an 11-month-old boy after implantation of the new Amplatzer duct occluder. Temporary balloon occlusion of the aortic ampulla was performed 4 days after the initial procedure leading to prompt abolition of the haemolysis.
Congenitally corrected transposition with pulmonary atresia and intact ventricular septum
Authors: Patel Chandrakant R. , Spector Michael L. , Zahka Kenneth G.
Published: August 2008

We describe a patient with the rare association of the heart in the left chest, congenitally corrected transposition, pulmonary atresia and an intact ventricular septum. There were associated fistulous communications between the morphologically left ventricle and the coronary arteries. Diagnosis was made by echocardiography, and subsequently confirmed by cardiac catheterization.
Rapid recurrence of an inflammatory myofibroblastic tumor in the right ventricular outflow tract
Authors: Hartyánszky István L. , Kádár Krisztina , Hubay Márta
Published: August 2008

We describe an unusual case of rapid recurrence of a previously excised inflammatory myofibroblastic tumor of the right ventricular outflow tract in a 5-month-old infant. The infant is asymptomatic 18 months after the second surgery. The very rare cardiac involvement, and the early relapse of the inflammatory pseudotumor, to the best of our knowledge, is a unique combination.

The inflammatory myofibroblastic tumor, as known as a pseudotumor or plasma cell granuloma is an uncommon reactive lesion with unknown aetiology. It is found most commonly in the lung and a number of visceral organs, such as the spleen, liver, ileum, salivary glands, urinary bladder, larynx or brain or in the retroperitoneum and lymph nodes. To our knowledge only 9 cases have been published of such tumor arising within the heart.1–6
Pulmonary arterial thrombosis in a neonate with homozygous deficiency of antithrombin III: successful outcome following pulmonary thrombectomy and infusions of antithrombin III concentrate
Authors: Roman K. , Rosenthal E. , Razavi R.
Published: August 2008

We report a newborn male who presented with severe central cyanosis on the third day of life. Partial thrombotic obstruction of the pulmonary trunk secondary to Antithrombin III (homozygous defect of heparin binding site) deficiency was subsequently diagnosed. Surgical thrombectomy, and infusions of Antithrombin III concentrate, led to a successful outcome. We postulate that intrauterine thrombosis occurred to give this unusual presentation.
Banding of a patent arterial duct to palliate complete transposition in a compromised neonate
Authors: Drummond-Webb Jonathan J. , Imamura Michiaki , Mee Roger B.B.
Published: August 2008

In a hemodynamically compromised neonate with complete transposition and intact ventricular septum (concordant atrioventricular and discordant ventriculo-arterial connections), it became necessary to control severe congestive heart failure. The arterial switch operation had been delayed because of intracranial bleeding and subsequent coagulopathy. The cardiac failure was reversed by surgical banding of the patent arterial duct. The arterial switch was performed successfully one week later.
Vascular aneurysm producing divided right atrium in a patient with pulmonary atresia and intact ventricular septum
Authors: Wood John C. , Reyes-Mugica Miguel , Kopf Gary
Published: August 2008

We describe a patient with pulmonary atresia and intact ventricular septum in whom the right atrium was divided by a vascular aneurysm located in the right atrioventricular groove. We postulate that the structure represents an aneursymally dilated right coronary artery taking anomalous origin from the pulmonary trunk, with fistulous communication to the right atrium. We discuss the findings relative to concepts of development of the coronary arteries in normal hearts and in pulmonary atresia with an intact ventricular septum.
Biventricular repair of lesions with straddling tricuspid valves using techniques of cordal translocation and realignment
Authors: Reddy V. Mohan , Liddicoat John R. , McElhinney Doff B. , Brook Michael M. , van Son Jacques A.M. , Hanley Frank L.
Published: August 2008

Surgical Management of straddling tricuspid valve and associated defects is a complex problem. Between August 1992 and August 1995, 5 patients with major straddling of the tricuspid valve underwent a complete or partial biventricular repair. All patients had either an inlet ventricular septal defect (n=4) or a ventricular septal defect with an inlet component (n=1), Co-existing cardiac lesions included hypoplasia of the right ventricle (n=3), discordant ventriculoarterial connections (n=1), tetralogy of Fallot (n=1), and multiple muscular vetricular septal defects (n=2). At the time of presentation to our institution, two of these patients had previously been palliated in preparation for a Fontan procedure, having undergone construction of a bidirectional superior cavopulmonary shunt. One patient was referred specifically for a Fontan procedure. The tricuspid valve was repaired by transecting all of the straddling cords and reattaching them in the right ventricle or onto the right side of the patch used to close the ventricular septal defect. Associated procedures included closure of the septum in all patients, an arterial switch procedure in one, repair of tetralogy of Fallot in one, and construction of a bidirectional superior cavopulmonary shunt in one. There has been no early or late mortality. Complete heart block requiring insertion of a pacemaker occurred after surgery in three patients. At a median follow-up of 32 months, functional integrity of the tricuspid valve is well maintained, with only one patient having moderate tricuspid regurgitation. None of the patients are receiving any cardiac medication.
Prenatal diagnosis of aneurysm of the arterial duct: postnatal management and literature review
Authors: Better Donna J. , Timchak Donna M. , Allan Lindsey D
Published: August 2008

Aneurysm of the arterial duct is well described when presenting in infants and children. The case we present were detected by fetal echocardiogram in late pregnancy, indicating the congenital nature of this lesion and excluding widely accepted explanations of the pathogenesis. Spontaneous closure may occur more frequently than the literature suggests. We recommend a conservative approach in asymptomatic patients.
Mitral value anomalies in tricuspid atresia: an autopsy study of 54 hearts
Authors: Pinto Robin J. , Deshpande Jaya , Dalvi Bharat V
Published: August 2008

Anatomic study of 54 autopsied cases of tricuspid atresia revealed mitral valvar anomalies in 9 (16.7%). The morphologic findings in these 9 cases are presented and compared with 45 cases having a normal mitral valvar apparatus. The anomalies included a cleft valve in 2, parachute valve in 2, straddling valve in 3, and a valve with dual orifice in 2.Two of these cases had more than one anomaly. Data from echocardiography and cardiac catheterisation, when available, had failed to diagnose the anomalies during life. The anomalies were more commonly associated with discordant ventriculo-arterial connections (33.3% versus 13.3%, p < 0.01), were associated significantly with a large ventricular septal defect (33.3% versus 4.4%, p < 0.01), and with pulmonary hypertension (33.3% versus 2.2%, p < 0.01). Two of 3 post operative deaths among the 9 were due to hemodynamic consequences arising from the uncorrected mitral valvar anomaly (parachute valve in both ). Diagnosis of these anomalies prior to surgical correction is imperative, as their presence may necessitate repair or replacement of the mitral valve. Failure to do so may result in increased perioperative mortality, congestive cardiac failure and arrhythmias at a later date. Mitral valvar anomalies may be a contributory factor to the early onset of cardiac failure seen in patients with tricuspid atresia undergoing palliative shunts.
Digital subtraction angiography, magnetic resonance imaging and echocardiographic findings in patients with an anomalous subaortic left brachiocephalic vein
Authors: Nii Masaki , Matsuoka Suguru , Mori Kazuhiro , Hayabuchi Yasunobu , Tatara Katsuyoshi , Kuroda Yasuhiro
Published: August 2008

We evaluated 6 patients with an anomalous subaortic left brachiocephalic vein. All cases were associated with some form of congenital heart disease: 4 patients had coexistent tetralogy of Fallot, one had congenitally corrected transposition, and the other an atrial septal defect with azygos connection of the inferior caval vein. Our incidence of an anomalous subaortic left brachiocephalic vein in patients with congenital heart diseases was 1.1% (6/527). It was observed in 4 out of 117 patients with tetralogy of Fallot (3.4%). Doppler echocardiography was useful in establishing the diagnosis. Furthermore, both digital subtraction angiography and magnetic resonance imaging demonstrated well the anomalous course of the vein, and were useful in making an accurate diagnosis.
Cardiac effects of carnitine deficiency induced by antibiotics containing pivalic acid in children
Authors: Abrahamsson Kate , Mellander Mats , Eriksson Bengt O. , Holme Elisabeth , Jodal Ulf , Lindstedt Sven
Published: August 2008

We studied echocardiographically 17 children with depletion of carnitine due to treatment over 1–3 years with antibiotics containing pivalic acid. When the children were depleted of carnitine, the left ventriclar posterior wall was significantly thickened in diastole when compared to a reference group. Six months later, after normalisation of the concentrations of carnitine, the left ventricular posterior wall was significantly thinner and did not differ from the reference group. We conclude that drugs containing pivalic acid, when given for several months, decrease the stores of carnitine to levels where cardiac involvement occurs.
Prognostic features in childhood idiopathic dilated cardiomyopathy
Authors: Rheuban Karen S. , Cregan-Lambert Donna , Gutgesell Howard P.
Published: August 2008

The clinical course, noninvasive findings and outcome of 25 infants and children with idiopathic dilated cardiomyopathy were reviewed retrospectively to identify factors predictive of outcome both at diagnosis and at short-term follow-up. Patients, stratified by clinical status at last visit, were assigned to groups encompassing those who were asymptomatic and receiving on medication, those patients who were symptomatic or needed medication to control symptoms, and those who were dead, awaiting heart transplantation, or had undergone transplantation. Older age at diagnosis was strongly associated with poor clinical outcome (p=0.005), as was the presence of arrhythmias at the time of diagnosis (p=0.008) and at short-term follow-up-(p=0.003). Echocardiographic studies at diagnosis failed to predict eventual clinical outcome, although patients who ultimately recovered and did not need medications tended to demonstrate improvement in or resolution of echocardiographic abnormalities within 6 months of diagnosis.
Natural history of congenital aortic valvar stenosis: an echo and Doppler cardiographic study
Authors: Király Petèr , Kapusta Livia , van Lier Henk , Hofman Anke Otten , Daniëls Otto
Published: August 2008

Abstrat

The availability of echo Doppler cardiography enables monitoring of the natural course of congenital aortic valvar stenosis more adequately than before. Between 1986 and 1993, 129 children with such stenosis were examined echocardiographically, 83 of them over a prolonged period with repeated studies of at least one per year. The pressure gradient between the left ventricle and ascending aorta, as well as the left ventricular wall thickness, were measured. When the pressure gradient detected increased to more than 60 mmHg, the patients were treated by balloon valvoplasty or surgical valvotomy. The last examination prior to intervention was taken as the final measurement.

We found a significant increase (≥10 mmHg) in the measured pressure gradient in 40 of the 83 patients (48%). The onset of severe stenosis was not observed at a specific age, rather the stenosis was found suddenly to increase in severity at any age. The gradients measured in children younger than 2 years of age, however, increased twice as rapidly as those measured in the older children. A high gradient was not always accompanied by evidence of left ventricular hypertrophy.

Based on our findings, we suggest that, since a sudden increase in severity of aortic valvar stenosis might be expected, the pressure gradient should be measured once every 6 months in children younger than 2 years of age and once a year after that age
Effects of triiodothyronine supplementation following modified Fontan procedure
Authors: Mainwaring Richard D. , Lamberti John J. , Nelson Jerald C. , Billman Glenn F. , Carter Thomas L. , Schell Kenneth H.
Published: August 2008

Patients undergoing the modified Fontan procedure may develop low cardiac output postoperatively. Since thyroid hormone has important effects on cardiovascular function, the present study was undertaken to evaluate the influence of triiodothyronine supplementation. Ten consecutive patients under-going the Fontan procedure were administered intravenous triiodothyronine (0.4 mcg per kg) following surgery. Clinical outcome and thyroid hormone profiles were assessed and then compared to a previous series of patients undergoing the Fontan procedure who had not received triiodothyronine supplementation. Both groups initially demonstrated marked decreases in serum free triiodothyronine levels. The group which received triiodothyronine supplementation demonstrated a more rapid return of serum triiodothyronine levels to baseline [259±17 vs 121±15 pg/dl (p<0.05) on the fifth postoperative day and 336±18 vs 178±12 pg/dl (p<0.05) on the eighth day]. In addition, patients receiving supplemental triiodothyronine demonstrated more rapid recovery of total triiodothyronine, free thyroxine, total thyroxine and thyroglobulin levels. The group which received triiodothyronine supplementation had a shorter length of hospital stay [9±2 vs 14±3 (p<0.05)] as compared to patients who did not receive exogenous triiodothyronine. The results of this study demonstrate that triiodothyronine supplementation aids in the recovery of thyroid hormone levels following Fontan procedure. This endocrinologic finding correlated with improved clinical outcome.
Infectious endocarditis in children: changing pattern in a developing country
Authors: Sharma Mukti , Saxena Anita , Kothari Shyam S , Subash C.B. , Reddy D.M. , Venugopal P. , Wasir H.S.
Published: August 2008

Background

There is a reported change in the profile of infectious endocarditis and a reduction in its mortality in the developed world. We present our experience of infectious endocarditis in children seen in the last 5 years in the developing world.

Methods

Records of 43 consecutive children with infectious endocarditis admitted to this centre were analysed retrospectively. Diagnosis was based on presence of any two of the following: fever with no extracardiac features; vegetations on echocardiography; Positive blood culture with no extracardiac focus; and embolic episodes.

Results

The age at diagnosis ranged from 40 days to 16 years (mean 8.5 years); Of the patients 3 were under 2 years of age; 28 were males and 15 females. Congenital heart disease was the underlying cause in 32 (74%), and rheumatic heart disease in 11 children. All except the youngest presented with fever. Blood cultures were positive in 16 (37%). Vegetations were detected by cross-sectional echocardiography in all except 1 child. After treatment 31 (72%) responded to a combination of penicillin and aminoglycoside. Emergency surgery was undertaken in 9 (21%). Three patients (7%) died, and all of these had fungal endocarditis.

Conclusions

Blood cultures give a low yield, but cross-sectional echocardiography is a sensitive tool in the diagnosis of infectious endocarditis. Most children respond to penicillin and an aminoglycoside. An aggressive surgical approach in complicated cases lowers the mortality.
Univentricular repair for complex double right ventricle and transposed great arteries
Authors: Serraf Alain , Jonas Richard A , Burke Redmond P , Castaneda Aldo R , Mayer John E
Published: August 2008

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

In 3 patients with isomeric morphologically left atrial appendages, univentricular atrioventricular connection, concordant ventriculoarterial connections, bilateral superior caval veins, with the left one draining via the coronary sinus, together with absence of any communicating vein, interruption of inferior caval vein with drainage via a right-sided (n=2) or left-sided (n=l) azygos vein, the hepatic venous blood was rerouted via the large coronary sinus into the pulmonary arterial circulation. In a fourth patient with similar pathology, having interruption of the left-sided inferior caval vein with drainage to the left-sided superior caval vein via a left-sided azygos vein and a large communicating vein, the pathway from the left superior caval vein to the coronary sinus was correspondingly small. An extracardiac conduit was therefore constructed between the hepatic veins and the left pulmonary artery so as to reroute the hepatic venous blood into the pulmonary arterial circulation. At a mean follow-up of 8.5 months, all patients are clinically well and none of them have developed pulmonary arteriovenous malformations. To avoid the latter complication in Fontan physiology, especially in the setting of an interrupted inferior caval vein with drainage via the azygos vein, we believe that it is preferable to reroute the hepatic venous blood into the pulmonary circulation.