bluuebunny/crossref_metadata_2025 huggingface dataset:
Spectrum and influence of hypoplasia of the left heart in Neonatal aortic coarctation
Authors: Tani Lloyd Y. , Minich L. Luann , Hawkins John A. , McGough Edwin C. , Pagotto Luciana T. , Orsmond Garth S. , Shaddy Robert E.
Published: August 2008

Obstruction of the left ventricular outflow tract may be associated with hypoplasia of the left heart, which importantly influences the options for treatment. Although the influence of the size of the left heart on the outcome for critical aortic stenosis has been described, less is known about the spectrum of such hypoplasia seen with neonatal aortic coarctation, and how this influences outcome. To determine, first, the spectrum and influence of hypoplasia of the left heart in neonatal coarctation, second, if the previously described critical values for adequacy of the left heart in neonates with critical aortic stenosis are applicable to neonates with coarctation, and, third, if any of the variables or associated abnormalities are risk factors for recoarctation, we studied 63 neonates who underwent repair of coarctation. From the initial echocar diogram, we measured multiple structures in the left heart, and calculated a score for adequacy as has been done for critical aortic stenosis. The sizes were compared to previously reported minimal values. We then analyzed the influence of the variables and the associated anomalies on outcome. There were no deaths. There was a broad spectrum of sizes that did not correlate with the need for re-intervention. The calculated score for adequacy would have predicted survival in only 56% of the patients, and 73% of the neonates had at least one parameter measured in the left heart below the previously reported minimal values. There is, therefore, a broad spectrum of sizes for the left heart in neonates with aortic coarctation that is not predictive of outcome. Minimal sizes, and the score for adequacy used for critical aortic stenosis, are not applicable to neonates with coarctation.
Impact of dynamic 3D transoesoesophageal echoardiography in the assessment of atrial septal defects and occlusion by the double-umbrealla device(CardioSEAL)
Authors: Maeno Yasuki V. , Benson Lee N. , Boutin Christine
Published: August 2008

Occlusion of the atrial septal defects in the oval fossa by interventional catheterization has progressed, but still has limitations. Three-dimensional (3D) echocardiography can provide unique views unavailable by cross-sectional imaging. The objective of this study was to define the clinical application of 3D echocardiography in the assessment and monitoring of transcatheter occlusion of atrial septal defects. Three-dimensional echocardiography was attempted prior to occlusion of atrial septal defects in 41 patients (median age 8.6 years). Serial cross-sectional images were acquired by multiplane transoesophageal echocardiography and displayed by means of computer reconstruction. Dynamic 3D echocardiographic images of defects in the oval fossa were obtained in 40 of 41 patients (98%). Volume-rendering demonstrated the anterosuperior rim in 36 (90%) and the inferoposterior rim in 24 (60%), but failed to reveal small additional fenestrations in six. Sizes measured by 3D echocardiography were significantly larger than those provided by cross-sectional transoesophageal echocardiography (p =;0.007), but differed little from those obtained with balloon sizing (p =; 0.6). After occlusion, 3D echocardiography showed positions of all arms of the device in 20 of 24 cases. Location of any protruding arms, or residual defects, were also clearly revealed.Three-dimensional images obtained in 12 patients during deployment of the double-umbrella device were useful in monitoring its position (single-frame) and for explaining the mechanism of protrusion. Current 3D echocardiography provides clinically relevant information for selection of patients for closure of atrial septal defects by interventional catheterization and when monitoring during implantation. Information obtained by this technique can clarify the mechanism of deployment of the device and closure of the defect, therefore influencing outcomes.
Resonance imaging of a ruptured aneurysm of the sinus of Valsalva
Authors: Roche Kevin J. , Genieser Nancy B. , Ambrosino Michael M.
Published: August 2008

Ruptured aneurysm of an aortic sinus of Valsalva is a rare cause of left-to-right shunting. We show how resonance imaging can be used to make the diagnosis. This technique can successfully characterize the shunt as well as determine the presence of associated anomalies, such as ventricular septation and aortic regurgitation. It may be the only study required prior to therapeutic intervention.
Superior caval venous syndrome after atrial switch procedure: relief of complete venous obstruction by gradual angioplasty and placement of stents
Authors: Michel-Behnke Ina , Hagel Karl-Jürgen , Bauer Jürgen , Schranz Dietmar
Published: August 2008

Superior caval venous syndrome is one of the late problems known to occur after Mustard repair of complete transposition. Reoperation may leave residual stenosis, and carries substantial risk for the patient. It is now feasible to use intravascular stents to overcome systemic venous baffle obstructions, and such an approach is probably more effective. The purpose of our study therefore, was to assess immediate and medium term results of inserting stents subsequent to gradual balloon enlargement of acquired atresia of the intraatrial baffle in patients who had undergone an atrial switch operation. We investigated five patients with complete obstruction of the superior caval venous pathway at perforation of the atretic segment was achieved using a guide wire technique. The procedure was successful in all patients. Gradual angioplasty was performed and intravascular stents were implanted. The pressure in the superior caval vein dropped to normal values, symptoms improved, and the patency of the newly created venoatrial communication was proven at mid-term follow-up. Thus critical obstructions at the superior caval venous pathway after the Mustard procedure can be reopened by interventional catheterization. Implantation of balloon-expandable intravascular stents is safe and effective in the acute relief of the obstructions, but careful long-term follow-up is mandatory.
Congenitally corrected transposition with atrioventricular septal defect
Authors: Attie Fause , Iturralde Pedro , Zabal Carlos , Rijlaarsdam Maria , Buendia Alfonso , Colin Luis , Vargas-Barron Jesus , Muñoz-Castellanos Luis
Published: August 2008

We describe 4 cases of congenitally corrected transposition associated with atrioventricular septal defect, diagnosed by echocardiography and angiocardiography. Two had usual atrial arrangement and two had mirror imaged atrial arrangement. All cases were associated with subpulmonary valvar stenosis. All patients presented with cyanosis and were in sinus rhythm. Atrioventricular septal defect with common atrioventricular junction was easily diagnosed on the basis of a common atrioventricular valve permitting interatrial and interventricular communications. All patients had balanced right and left ventricles. The echocardiographic recognition of the ventricles was based on the presence of the moderator band within the morphologically right ventricle, the characteristics of the apical septal trabeculations, and the shape of the ventricles. Angiocardiographic recognition of the ventricles was achieved on the basis of right and left ven-triculography.

In one case with usual atrial arrangement, we recorded two His bundle potentials, one anteriorly and another posteriorly. Atrial stimulation revealed blocked atrioventricular conduction at the level of the pos terior bundle, and normal atrioventricular conduction through the anterior bundle. In both cases with atrial mirror-imagery, only a posterior His bundle potential was found, with normal atrioventricular conduction revealed by atrial stimulation

The clinical course with this combination depends on the other lesions present in addition to the common atrioventricular valve. Our electrophysiological studies show that the conduction system in presence of a common atrioventricular valve is as expected for congenitally corrected transposition with two atrioventric ular valves.
Echocardiographic follow-up of percutaneous closure of the arterial duct in infants
Authors: Piñeda Luis Fernández , Torrealday Hugo , Cañete Ramón Bermúdez , Azcárate María J. Maitre , Jiménez Manuel Quero
Published: August 2008

Since February 1990, we have attempted nonsurgical occlusion of persistent patency of the arterial duct using the Rashkind double-disk occluding device, of either 12 or 17 mm diameter. Results are presented from 31 patients (23 female and eight male, ages 15 months to 16 years). M-Mode, cross-sectional, Doppler and color Doppler echocardiographic studies were made prior to closure, in the following 24 hours, and six months later. Left ventricular diastolic dimension and shortening fraction, systolic intervals, and left atrium/aortic ratio were evaluated by M-Mode echo. We examined carefully the location of the device relative to the left pulmonary artery and descending aorta, also checking for disturbed flows and residual shunts. These studies showed a clear tendency for the left ventricular diameter to decrease, and revealed a significant normalization of the left atrium/aortic ratio. Successful closure of the duct was achieved in 27 cases. Follow-up studies six months later showed residual shunting in four cases. In one patient, a second device was implanted with an excellent result. At the immediate follow-up, turbulences were noted in the area of the device (nine cases) and in the left pulmonary artery (seven cases). Distortion of the anatomic orientation of the left pulmonary artery was observed in three cases in which we discovered 20 mm Hg systolic gradients. In our opinion, echocardiography is the best technique with which to follow-up these patients. The high sensitivity of color Doppler echocardiography revealed minimal distortion of the left pulmonary artery, probably generated by regional anatomic adjustment to the insertion of the device.
Echocardiographic diagnosis of inferior sinus venosus defects
Authors: Ettedgui José A. , Siewers Ralph D. , Zuberbuhler James R. , Anderson Robert H.
Published: August 2008

Sinus venosus defects are relatively uncommon forms of interatrial communication in which one of the caval veins has a biatrial connection, and partial anomalous pulmonary venous connections are the rule. The variety related to the inferior caval vein is much the rarer of the two forms, and here the defect is posterior and inferiorly placed. To determine whether an inferior sinus venosus defect could be identified preoperatively, we reviewed retrospectively the available echocardiograms and cineangiograms of the five patients with an inferior sinus venosus defect who underwent surgical repair between January 1983 and December 1991. A cross-sectional echocardiogram had been performed preoperatively in four of these patients, but the correct diagnosis had been made in only one case. Retrospective study, however, showed that the extent of the true atrial septum and the nature of the junction of the inferior caval vein with the atriums could be evaluated from the subcostal position in three of these four patients. Each of the three had an interatrial communication located posteriorly and inferiorly adjacent to the atrial connection of the inferior caval vein, along with abnormally connected right pulmonary veins. The oval fossa was intact and the coronary sinus was normal in all. Cardiac catheterization had been performed in three of the five patients. Anomalous return of right pulmonary veins was noted in each, and the posterior and inferior location of the interatrial communication was evident in two. An interatrial communication adjacent to the atrial connection of the inferior caval vein, along with anomalous connection of the right-sided pulmonary veins, are the diagnostic features of the inferior variety of sinus venosus defects. These features can be demonstrated by cross-sectional echocardiography from the subcostal position.
Primary pediatric cardiac tumors: a 17 year experience
Authors: Sallee Denver , Spector Michael L. , van Heeckeren Daniel W. , Patel Chandrakant R.
Published: August 2008

We reviewed 22 cases of primary pediatric cardiac tumors followed at our institution from January 1981 through November 1997, analyzing them by subtype, age and manner of presentation, location, associated findings, interventions, and clinical course. Rhabdomyomas were the most common (11), followed by intrapericardial teratomas (2), myxomas (1), fibromas (1), hemangiomas (1), mesotheliomas (1), and rhabdomyosarcomas (1), with 4 undetermined tumors. The majority (77%) of tumors were diagnosed before the age of one year, including six prenatally. The most common presentations were murmurs (5), arrhythmias (5), and abnormal screening fetal ultrasound examinations (4). Tumors were located most frequently in the right ventricle (13) and left ventricle (7), with multiple tumors being present in 10 cases. Eight patients (36%) had associated arrhythmias or conduction abnormalities, and of the 11 patients with rhabdomyomas, tuberous sclerosis was diagnosed in 8. Eight patients underwent cardiac catheterization, including two for electrophysiologic study with radiofrequency ablation, and seven patients had complete or partial tumor resection. The follow-up period ranged from 2 months to 15 years, and there were 3 tumor-related deaths. Therefore, despite the benign histology of most primary pediatric cardiac tumors, there may be significant associated morbidity and occasional mortality. As echocardiographic techniques such as fetal ultrasonography have continued to improve, however, these cardiac tumors have increasingly been detected early before significant symptoms develop.
Thrombotic occlusion of the main stem of the left coronary artery in a neonate
Authors: Abdurrahman Luby , Schwartz Steven M. , Beekman Robert H.
Published: August 2008

Thrombotic coronary arterial occlusion, and myocardial infarction, are rare in the newborn. We report such a happening presenting shortly after birth with cardiogenic shock, no left ventricular output and a systemic circulation dependent on flow from a patent arterial duct.
Simultaneous surgical treatment of atrial septal defect and atrial flutter using a simple modification of the atrial incision
Authors: Henglein Dagmar , Cauchemez Bruno , Bloch Gérard
Published: August 2008

The reentrant circuit of common atrial flutter is known to located in the right atrium between two anatomical barriers. Recent electrophysiologic studies have defined the tricuspid annulus as the anterior barrier, and the terminal crest and its continuation as the eustachian ridge as the posterior barrier. Construction of a bidirectional block to conduction between these two barriers by means of lesions created with radiofrequency current have been shown to be effective in ablating the flutter. We now find that surgical creation of such a block to conduction between the barriers by a simple modification of the atrial incision line is equally effective. In a 6-year-old boy, who was admitted to our hospital for closure of an atrial septal defect and treatment of sustained atrial flutter, the atriotomy was performed perpendicular to the terminal groove and extended towards the tricuspid annulus, placing some crythermal lesions between the end of the incision and the annuals. The special defect was closed using a Dacron patch. The child was free of arrhythmia both during the postoperative stay and over the initial three months of follow-up. We conclude that this simple modification of the atrial incision line provides cure of atrial flutter in children who require atriotomy for repair of congential cardiac anomalies. It may also be beneficial in preventing ‘incisional’ reentrant tachycardia.
Multiple rhabdomyomas in monozygotic twins
Authors: Zamith Marina Maccagnano , Brunoni Décio , Ebaid Munir
Published: August 2008

Rhabdomyoma is the most frequent primary cardiac tumor seen during infancy. We report multiple rhabdomyomas diagnosed in twins in the neo natal period. To the best of our knowledge, this is the first instance of this happening.
Paediatric cardiology at the edge of the millennium and quality assessment
Authors: Daniëls Otto
Published: August 2008

To achieve good and responsible medical care, not only is it necessary to legislate to control training, the institutes responsible for training, and continuing medical education, but it is also necessary to document ratings of mortality and morbidity as well as measures of workload for those providing care. We, as an Association for Paediatric Cardiology, must be more active in this process, and in politics, so as to convince the patients and the authorities that these processes are necessary, and to obtain the financial requirements to permit them to be achieved. Much has still to be done.
Self-efficacy and physical activity in adolescents with trivial, mild, or moderate congenital cardiac malformations
Authors: Bar-Mor Galit , Bar-Tal Yoram , Krulik Tamar , Zeevi Benjamin
Published: August 2008

Our purpose was to examine the cognitive processes that influence involvement in physical activity among 100 adolescents, 55 boys and 45 girls, ranging in age from 12 to 18 years, with trivial, mild, or moderate forms of congenital cardiac disease. We hypothesized, first, that the severity of the congenital cardiac malformation itself has an indirect effect on self-efficacy regarding physical activity, and that the relationship between the two is mediated by the recommendations of the cardiologist and the attitude of the mother. Second, we argued that self-efficacy serves as a mediating variable between the recommendations of the cardiologist and the attitude of the mother, on the one hand, and involvement in physical activity, on the other. The results confirmed both hypotheses. In a population of adolescents with trivial to moderate congenital cardiac malformations, beliefs in self-efficacy, rather than severity of the disease, were the most influential factors in determining whether or not adolescents will engage in sports or other physical activities. We also demonstrated the importance of the role played by the recommendations of the cardiologist in determining both the attitudes of the mother and the belief in self-efficacy of the adolescents.
Cardiac surgery in a girl with trisomy 13
Authors: Strømme Petter , Thaulow Erik , Geiran Odd
Published: August 2008

We describe a profoundly retarded infant girl with multiple anomalies caused by trisomy 13. Due to heart failure, which was resistant to medical treatment, we closed successfully a ventricular septal defect at three months of age. She died at 10 months of age. Despite the short survival, we believe that the patient benefitted significantly from the surgical repair of her cardiac defect.
Patency and development of stenosis in modified Blalock-Taussig shunts constructed using expanded polytetrafluoroethylene
Authors: Godart François , Qureshi Shakeel A. , Simha Ary , Fagg Nuala , Deverall Philip B. , Anderson David , Baker Edward , Tynan Michael
Published: August 2008

Construction of a modified Blalock-Taussig shunt using polytetrafluroethylene is frequently used for palliation in infants with complex cyanotic heart disease. This study was performed to assess angiographically the late patency rate of such prosthetic shunts. A modified Blalock-Taussig shunt had been constructed in 82 children and, of these, 63 (77%) patients had post-operative angiography and were included in this study. There were 29 males and 34 females, ranging in age from 1 day to 164 months. A second shunt was needed in 7 patients. Thus, 70 shunts were studied. Of the shunts, 16 were 4 mm in diameter, 28 shunts 5 mm in diameter, and 26 shunts 6 mm in diameter. Postoperative angiography was performed on 1 day to 132 months (median 39 months) after construction of the shunts. Acute post operative occlusion occurred in 2(3%) patients, who had 5 and 6 mm shunts, respectively. At the latest follow-up (median 39 ± 26 months), 6(9%) late occlusions were noticed. Overall patency was 89% for all the shunts, with 94% of the 4 mm shunts, 85% of the 5 mm and 88% of the 6 mm shunts being patent. Regression analysis showed a progressive reduction in diameter of the graft with time. This narrowing of the graft may have been related to the formation of a ‘neointima’ due to fibrous hyperplasia.
Surgical treatment in infancy of ductal aneurysm associated with a vascular ring
Authors: Hartyánszky István L. , Szolnoki Judit , Mózes Gèza
Published: August 2008

A 5-week-old male, born with an aneurysm of the arterial duct forming part of a vascular ring, underwent successful surgery without cardiopulmonary bypass. From our search of the literature, it seems, that this combination of rare congenital malformations has not previously been reported.
Atrioventricular septal defect with tetralogy of Fallot
Authors: Dalvi Bharat , Gupta Krishnagopal , Sharma Satyavan
Published: August 2008

We present a case of atrioventricular septal defect associated with tetralogy of Fallot which was diagnosed by cross-sectional echocardiography and angiography. The diagnosis was confirmed at necropsy. This case is unusual for the absence of Down's syndrome. We discuss the role of clinical, echocardiographic, hemodynamic and angiographic studies.
Selective coronary angiography in tetralogy of Fallot
Authors: Sharma Satyavan , Sundaram Usha , Loya Yunus , Desai Dhruman
Published: August 2008

Selective coronary angiography was performed in 110 cases (aged 1–45 years) of tetralogy of Fallot. Anomalies of surgical importance were seen in 26 cases (23.6%). In 17 (15.4%) these anomalies existed alone, and in nine (8.2%) they coexisted with relatively minor anomalies. Isolated minor anomalies involving the terminal branches existed in 15 cases (13.6%). The coronary arterial anatomy was normal in the other 69 (62.7%) cases. Our study shows that coronary angiography can safely be performed in patients with tetralogy of Fallot, providing the surgeon with vital information for subsequent planning of the time and type of surgery required.
New imaging modalities in absent pulmonary valve syndrome
Authors: Fletcher Scott E. , Greene Craig A. , Wiles Henry B.
Published: August 2008

Tetralogy of Fallot with absence of the leaflets of the pulmonary valve is often complicated by marked respiratory involvement. Newer imaging modalities such as magnetic resonance imaging and color Doppler echocardiography underscore the inter-relationship of the cardiorespiratory systems in this disorder.
Divided left atrium with absence of the interatrial septum in monozygotic twins
Authors: Cochrane Andrew D. , Menahem Samuel , Mee Roger B.B.
Published: August 2008

Division of the left atrium is a rare congenital lesion, but one which is amenable to complete surgical correction. We describe twin brothers, demonstrated to be monozygotic, who presented in infancy with cardiac failure, pulmonary congestion and mild cyanosis. Both were found to have systemic and pulmonary venous return to a common atrial chamber, separated from the left atrial appendage and the vestibule supporting the mitral valve by a typical muscular diaphragm with a single small orifice. In one twin, there was also persistence of the left superior caval vein, while, in the other twin, a remnant of the primary atrial septum was present. Excision of the diaphragm and neoseptation of the atrial chambers was performed in both infants. At 10 years after surgery, there was normal growth and development in both twins, with no evidence of recurrent obstruction.
Vertebral arterial steal following surgical repair of neonatal coarctation
Authors: Sreeram Narayanswami , Brown Christopher , Walsh Kevin
Published: August 2008

A five day old infant underwent repair of coarctation by resection and end-to-end anastomosis of the proximal and distal segments of the arch. Four weeks later, cross-sectional Doppler echocardiography showed a tight recoarctation proximal to the origin of the left subclavian artery. A dilated proximal left subclavian artery was demonstrated, with continuous flow from it into the descending aorta. These findings were confirmed at catheterization. This “vertebral arterial steal” disappeared following transcatheter balloon angioplasty.
Total correction of tetralogy of Fallot with pulmonary atresia associated with an unusual aortopulmonary collateral artery
Authors: Hashimoto Kazuhiro , Kurosawa Hiromi , Tatara Akira
Published: August 2008

We report a patient with tetralogy of Fallot and pulmonary atresia who displayed an atypical aortopulmonary collateral artery extending from the ascending aorta to the pulmonary trunk. The internal thoracic artery, instead of the right subclavian artery (which was aberrant), was used as the source ofblood during unifocalization of the right pulmonary arteries to avoid the potential formation of a vascular ring. Unifocalization in the left lung was carried out by enlargement with a patch of the previously stenotic Blalock-Taussig shunt. The palliation set the scene for final intracardiac repair achieved one month after unifocalization of the right sides. The postoperative course was good and uneventful.
The application of transthoracic dynamic three-dimensional echocardiography by computer-controlled parallel slicing in patients with fixed subaortic obstruction
Authors: Vogel Michael , Lösch Shideh , Bühlmeyer Konard
Published: August 2008

A 64-element phased-array echocardiographic transducer mounted on a sliding carriage was used transthoracically in 11 patients with subaortic obstruction caused by various different morphological substrates. The transducer is moved in a plane from the outflow tract to the apex of the heart in steps of 0.5 to 1.3 mm and records a tomographic slice of the heart at each step. Parallel images are recorded at a frame rate of 25–30 images per second and triggered to heart rate and respiration. A complete cardiac cycle is recorded at each level. The images are digitized and stored in the image-processing computer, which reconstructs the anatomic structures of the heart in a three-dimensional format by means of different gray-scales. Good quality echocardiographic pictures were obtained in 10 of the 11 patients and three-dimensional reconstructions were possible in those 10 patients. The technique is able to display the heart in real-time in any desired plane, and in up to five planes simultaneously, without the need to change the position of the transducer on the chest. Thus the best views with which to display the substrate of subaortic stenosis could be selected. The left ventricular outflow tract could also be displayed in a view similiar to the one obtained by the surgeon during operative procedures for resection of the subaortic stenosis through an incision in the aortic root. Image acquisition took three to five minutes, and three-dimensional reconstruction of various cardiac structures took 20–90 minutes. Display of the subaortic area in three-dimensional format may enhance our understanding of cardiac anatomy and identify the different morphological lesions in the left ventricular outflow tract which cause subaortic obstruction.
Sizes of the descending aorta and pulmonary arteries in infants with atrioventricular septal defects
Authors: Ahn Jae H. , Knott-Craig Christopher J. , Elkins Ronald C. , Lane Mary M. , Overholt Edward D. , Ward Kent E. , Razook Jerry D.
Published: August 2008

The descending aorta appears small relative to unusually large pulmonary arteries in infants with atrioventricular septal defects. To determine whether this discrepancy in great arterial size influences the outcome after repair of atrioventricular septal defects, we reviewed our experience with 34 infants younger than one-year-old undergoing primary repair. There were four early deaths (11.8%), one in 16 patients with normal chromosomes (6.3%), and three of 18 with Down's syndrome (16.7%). There was no difference between survivors and non-survivors in terms of either the size of the aorta at the level of the diaphragm (descending aortic index, 149±34 versus 139±46 mm2/m2), the pulmonary arterial index (675±170 versus 745±182 mm2/m2), or the ratio of the descending aorta to the pulmonary trunk arterial (4.85±1.6 versus 5.93±2.8). Compared with survivors, non-survivors had higher ratios of vascular resistance (0.48±0.42 versus 0.23±0.14, p<0.05), and tended to have larger preoperative cardiothoracic ratios (0.63±0.05 versus 0.55±0.08, p=0.12) Compared to patients with normal chromosomes, the patients with Down's syndrome tended to have smaller descending aortas (140±34 versus 159±34 mm2/m2, p=0.12), higher ratios of vascular resistance (0.32±0.2 versus 0.18±0.1, p=0.08), and higher mean pulmonary arterial pressures (47±11 versus 39±18 mm Hg, p=0.12), suggesting that pulmonary vascular obstructive disease may be more severe in patients with Down's syndrome. The size of the descending aorta and pulmonary arteries do not influence outcome after repair of atrioventricular septal defect in infants.
Divided left atrium (cor triatriatum sinistrum) presenting with syncope
Authors: Camerieri Angelo , Pongiglione Giacomo , Bertolini Alberto
Published: August 2008

Syncope has never been described as the presenting symptom of divided left atrium (cor triatriatum sinistrum). We have now encountered this mode of presentation in an otherwise asymptomatic patient. An upright tilt test elicited syncope prior to surgical correction, but failed to induce any symptom, even subsequent to infusion of isoproterenol, after surgery.
Tetralogy of Fallot with coarctation of the aorta
Authors: Yoshigi Masaaki , Momma Kazuo , Imai Yasuharu
Published: August 2008

We report a two-year-old boy with a rare combination of tetralogy of Fallot and aortic coarctation. The obstruction in this patient had an hourglass-like configuration, with a pressure difference of 15 mm Hg. There was a right aortic arch, bilateral brachiocephalic arteries, and persistent left superior caval vein. Percutaneous transluminal balloon angioplasty was performed to lower the resistance to the left ventricular ejection, considered a risk factor for subsequent corrective surgery of tetralogy. Six months later, he underwent combined repair of tetralogy of Fallot and coarctectomy with end-to-end anastomosis, thus avoiding the future potential for restenosis. During surgery, a ligament was found extending from the right subclavian artery to the distal side of the coarctation. The perioperative course was uneventful, indicating the success of the preoperative balloon angioplasty. We speculate that the embryogenesis of the coarctation could be related to an abnormal involution of the aortic arches.
Endocarditis in the young
Authors: Al Jubair Khalid , Al Fagih Mohamed R. , Al Yousef Saad , Ali Khan Mohamed A. , Sawyer William
Published: August 2008

Between February 1980 and February 1992, 28 children (17 males and 11 females) were treated for endocarditis on congenital cardiac lesions, most commonly the mitral valve (32%). Their ages ranged between four months and 14 years (mean 8.2 years). The most common infecting organism (in 25% of patients) was Staphylococcus aureus. Patients were divided into two groups. In the first, made up of eight patients, cardiac surgery was undertaken within one week of the start of antibiotic therapy. The second group of 20 patients was further divided into a group of seven patients who underwent surgery during the initial, active stage of endocarditis because of failure of medical management and a group of 13 patients who initially received antibiotic therapy alone. In this last group, three infections with the same organism recurred within two months and surgical intervention was necessary in the active phase of the recurrence. Subsequently, surgery for “healed” endocarditis was undertaken in three more patients. Mortality in the 21 patients undergoing surgery was 19%—one of eight (12.5%) in those undergoing surgery within one week of starting treatment and two of 10 in the remaining patients. Overall, three patients (15%) died from those in whom surgery was not undertaken within one week of the start of treatment. There were also fewer pre- and postoperative complications as well as a lower mortality rate in those undergoing early surgery. We conclude that early surgical intervention, in our hands, is the most appropriate management for endocarditis in congenital cardiac lesions.
Echocardiographic assessment of left ventricular function at rest and during exercise in postoperative patients with tetralogy of Fallot
Authors: Norgård Gunnar , Andersen Kai , Vik-Mo Harald
Published: August 2008

Digitized M-mode echocardiograms of left ventricular function were obtained in 34 patients subsequent to surgical repair of tetralogy of Fallot and in 34 healthy subjects at rest. In 16 patients and 16 controls, studies were also made during submaximal semisupine bicycle exercise. At rest, the peak ejection rate and fractional shortening were slightly reduced in the patients, whereas peak filling rate was comparable in patients and controls. Pulmonary regurgitation did not seem to influence left ventricular function. During exercise, however, peak filling rate was reduced in the patients compared to the healthy subjects. At peak exercise, the peak filling rates were 27.8±6.3 cm•s−1 and 34.1±3.4 cm•s−1 in patients and controls, respectively (p<0.01). No differences were found in heart rate, fractional shortening, peak ejection rate or blood pressures between patients and controls throughout the exercise test. When the subjects were subdivided by median age, the oldest patients had reduced normalized peak filling rates throughout the exercise test, whereas no differences were found between younger and older healthy subjects. Thus, it is suggested that the reduced left ventricular peak filling rate found during exercise is caused by subclinical myocardial dysfunction which seems to be related to myocardial protection at surgery and the period of follow-up.
Reconstruction of the mitral valve in children— long-term follow-up in 63 cases
Authors: Attie Fause , Zabal Carlos , Juárez Alejandro , Buendía-Hernández Alfonso , Barragán Rodolfo , Calderón Juan , Mini-Miranda Miguel
Published: August 2008

Between June 1985 and June 1991, 63 children underwent surgical reconstruction of the mitral valve for rheumatic (46 cases), congenital (12 cases) and myxoid (five cases) disease. The ages ranged from two to 18 years (mean 14.1±3.7 years). Valvar dysfunction was classified according to its pathophysiological abnormalities. A group of four cases presented with regurgitation secondary to lesions located in the valvar structures but with normal motion of the leaflets. In a second group of 14 cases, mitral regurgitation was due to prolapsed leaflets because of lesions located mainly in the subvalvar structures. A third group was formed by 35 patients with mitral regurgitation with restricted motion of the leaflets due to lesions in the valvar and subvalvar structures. The final group, of 10 cases, presented with mitral stenosis. The overall surgical mortality rate was 4.7% (3/63), and follow-up data were available in all survivors from one to 96 months (mean 33.4±25.4). Four cases underwent reoperation due to residual incompetence, one case due to bacterial endocarditis, and two more are scheduled for replacement of the valve due to unfavorable evolution, giving an overall rate of reoperation of 4.3% per patient/year. One patient died in the period following valvar replacement (late mortality rate of 0.6% per patient/year). Thromboembolism occurred in four cases in the absence of anticoagulation; three of them were in atrial fibrillation (late thromboembolic rate 2.4% per patient/year). Prior to surgery, 28 cases were in functional class II of the New York Heart Association, 34 patients were in class III and one patient in class IV. At the end of the follow-up period, 49 patients were in class I, seven in class II and four in functional class III (p<0.0001). The cardiothoracic ratio before surgery ranged from 0.40 to 0.81 (mean 0.60±0.07) and, after surgery, the values ranged from 0.40 to 0.79 (mean 0.55±0.07) (p<0.0001). Randomized late echocardiographic evaluation in 24 cases revealed residual mild mitral regurgitation in 20 cases, moderate in two and severe in two. The latter are scheduled for valvar replacement. There were no significant differences in the surgical results among the four groups. Reconstruction of the mitral valve, therefore, provides stable functional results with low surgical and late mortality, as well as an acceptable rate of reoperation irrespective of the lesions of the valvar apparatus.
Pediatric cardiology in the Netherlands
Authors: Hess John
Published: August 2008

BEFORE GOING INTO THE SPECIFIC AREAS OF PEDIATRIC cardiology and pediatric cardiac cardiology and pediatric cardiac surgery, an overview of the general health care organization in the Netherlands might be helpful. The Netherlands, a country with about 15 million inhabitants, spent 47 billion Dutch guilders (US$22 billion) for general health care in 1991. This is about 8.5% of the gross domestic product. Of this, 61% was spent on in-hospital costs, 39% on extramural costs, including the financing of organizations that deal with preventive (primary) medicine, such as clinics for infants and school children which were visited regularly. Almost all inhabitants, 99.6%, are covered for the expenses of health care, 60% through the “Sick Fund,” a state-insurance that is obliged for everyone with a yearly income of less than US$30,000. Employers of these people have to deduct an amount of money from the monthly salary to be paid to the “Sick Fund.” In case of unemployment this money is deducted from the amount paid by the social security office. The remaining 40% has some form of private insurance, that covers health care. Both diagnostic and therapeutic approaches as the timing of these are completely similar and independent from the type of insurance that applies for the individual patient.
Transcatheter closure of the patent arterial duct as a day-case procedure
Authors: Galal Omar , Abbag Fuad , Fadley Fadel , Redington Andrew , Szurman Peter , Oufi Saud
Published: August 2008

We have now achieved transcatheter occlusion of the patent arterial duct as a day-case procedure in 26 patients. In all but two, who were judged not to be appropriate for the device, implantation was successful. Most of the patients arrived in the hospital at 8:00 a.m. and had to wait for a mean time of 154 minutes until they were taken for cardiac catheterization. The procedure, performed under sedation in 25 patients and under general anesthesia in two, took a mean of 91 minutes. The children were then monitored for two hours in the recovery room. The interval From the time of arrival in the Unit until waking was a mean of 196 minutes. Then, from the time of waking, a mean of 123 minutes elapsed prior to discharge. Admission of the patient was necessary in three instances (11.1%). Two patients vomited and remained too drowsy for safe discharge. A third patient had either occlusion or thrombosis of the femoral artery and received an infusion of heparin which necessitated admission overnight. All three patients were discharged the following morning. Patients from outside Riyadh stayed overnight in Riyadh before they went home. One patient complained of non-cardiac chest pains a few hours after discharge and was subsequently sent home. Total occlusion, as judged by mean follow-up of 13 months (5–26 months) using color flow Doppler was achieved in 19 of the 24 patients (79!) in whom devices were inserted. All patients are well and there are no complaints concerning the procedure done on a day-case basis. We conclude that the day-case protocol for transcatheter occlusion of the patent arterial duct is a safe and cost-effective option for selected patients.
Nitric oxide given via a head-box for persistent neonatal pulmonary hypertension
Authors: Martin Paul H. , Sreeram Narayanswami , Petros Andy J.
Published: August 2008

A neonate presenting with persistent pulmonary hypertension and severe hypoxemia was treated with nitric oxide administered into a head-box. Improvement of clinical, biochemical and echocardiographic parameters were observed during therapy, suggesting an acute decrease of pulmonary arterial pressure. It is feasible to administer nitric oxide to non-ventilated neonates provided all recommended safety precautions are observed
Concurrent percutaneous balloon dilation for combined severe valvar pulmonary and aortic stenosis in a child
Authors: Zeevi Benjamin , Zalzstein Eliahu , Berant Michael
Published: August 2008

CRITICAL STENOSIS OF BOTH PULMONARY AND AORTIC valves is an exceedingly rare congenital combination.1−3 In recent years, balloon dilation of these valves in isolation has been reported with good results in children, infants and even newborns.4−6 We now report successful concurrent percutaneous balloon dilation for severe congenital pulmonary and aortic valvar stenosis in an 18-month-old child.
The trilogy of double-chambered right ventricle, perimembranous ventricular septal defect and subaortic narrowing—a more common association than previously recognized
Authors: Ward Cameron J. B. , Culham J. A. Gordon , Patterson Michael W. H. , Sandor George G. S.
Published: August 2008

The records of nine patients with double-chambered right ventricle, ventricular septal defect and subaortic narrowing were reviewed to clarify the natural history of the components of this pathological combination. All nine patients presented with heart failure related to a perimembranous ventricular septal defect, which underwent spontaneous reduction in eight and closed in one. Echocardiographic diagnoses of double-chambered right ventricle and subaortic narrowing were delayed in eight of the nine cases. The ratio of the distances from the pulmonary valve to moderator band/tricuspid valve annulus (the displacement index) was less than one in all patients. Subaortic narrowing was always discrete and was present at the diagnosis of double-chambered right ventricle. The subaortic narrowing was always recognized echocardiographically, and was seen in eight of nine patients at angiography. The subaortic gradient was 0–20 mm Hg and the subpulmonary gradient 0–110 mm Hg at catheterization. Five patients demonstrated aortic regurgitation. Subaortic narrowing should be diligently sought among patients with double-chambered right ventricle, and vice versa, even in the absence of a ventricular septal defect which may have closed. Both the subaortic narrowing and subpulmonary obstruction may progress, but probably at different rates. Patients with a perimembranous ventricular septal defect, particularly with a displacement index of less than one, should be monitored carefully for evolving subpulmonary obstruction, subaortic narrowing and aortic regurgitation. The subpulmonary obstruction should be resected at the time of any other surgical intervention. The indications for resection of the subaortic narrowing remain ill-defined.
Depression in multiple sclerosis: Review and theoretical proposal
Authors: ARNETT PETER A. , BARWICK FIONA H. , BEENEY JOE E.
Published: September 2008

Because of its high prevalence and implications for quality of life and possibly even disease progression, depression has been intensively studied in multiple sclerosis (MS) over the past 25 years. Despite the publication of numerous excellent empirical research papers on this topic during that time, the publication of theoretical work that attempts to explain depression in a comprehensive way is scarce. In this study, we present a theoretical model that attempts to integrate existing work on depression in MS and provide testable hypotheses for future work. The model suggests that risk for depression begins with the onset of MS. MS results in disease-related changes such as increased lesion burden/brain atrophy and immunological anomalies that are associated with depression in MS, but explain only a relatively limited proportion of the variance. Common sequelae of MS including fatigue, physical disability, cognitive dysfunction, and pain, have all been shown to have an inconsistent or relatively weak relationship to depression in the literature. In the model, we propose that four variables—social support, coping, conceptions of the self and illness, and stress—may moderate the relationship between the above common MS sequelae with depression and help to explain inconsistencies in the literature. (JINS, 2008, 14, 691–724.)
On the establishment of new communities: Allerton Bywater and Osbaldwick Fields
Authors: Clelland Doug
Published: August 2008

Two competition designs have provided frameworks for ideas for the establishment of new communities. The projects provided the opportunity for the design team to consider core architecture (urban and architectural space) and its relationship with extended architectures (detailed design and construction). The building designs have been required to meet performance standards considerably in excess of recent UK norms. This paper describes the design approach and demonstrates how research (university and practice based) has contributed to the two submissions.
Urbanist rhetoric: problems and origins in architectural theory
Authors: Macarthur John
Published: August 2008

‘Urbanism’ has become a familar posture among architects, so familiar that it has recently become a target for ridicule. The actual developments of cities today make the neo-Sitte-esque contextualism of the 1970s look even more Utopian than the International Style. There are many and varied socio-economic and political determinants in many differing situations which might explain the hopes of the past and their distance from the realities of the present. However, much of the problem with urbanism is not to do with actual urban conditions or the success or failure of particular projects, but rather with how the concept of urbanism was framed in the architectural profession and academy. It ought still to be possible to develop a few operative concepts and a way of having a shared discourse on the architectural aspects of city sites. But at the moment we are caught between vast rhetorical claims for such work as ‘theory’; and a new naturalism that sees the city as generic global and beyond architecture. These notes are intended as a provocation both to the institutionalisation of urbanism and to the idea that it has become passé.
Delivering the well-tempered institution of 1873
Authors: Cook Jeffrey , Hinchcliffe Tanis
Published: August 2008

The Museum of Natural History, London, typified the state of environmental service design in large public buildings when construction started in 1873, as described in an earlier paper in arq, vol. 2. Its exemplary systems included both ventilation with heating and the architect's use of towers, especially the novel multi-sleeved versions which he described as ‘thermosyphonic’, as ventilation exhausts. This paper describes how, both in critical design decisions during construction and in physical adjustments made after its occupation in 1881, the Museum reveals both contemporary practices and the professional skills of its architect and engineer.
Strategies for central areas of Copenhagen
Authors: Sørensen Erik Christian
Published: August 2008

The planned closure of Copenhagen's commercial docks and railway yards presented the opportunity to consider not only possible uses for the vacated land but also the impact of these changes on the rest of the city. This paper is based on extracts from Urban Make, a record of a study made of this subject by a team of architects drawn from the architecture schools at Copenhagen and Århus, led by Erik Christian Sørensen. Reproduced here is an abbreviated account of the team's approach together with its proposals for three of the study areas and for reinforcing the link between the existing centre and the expanding Ørestad area across the harbour. Limitations on space preclude the inclusion of the remaining proposals and the studies made of other European cities, a series of essays on ‘The Commendable City’ and suggestions for reordering the city's streets and obtaining better value from investment in the public realm.