bluuebunny/crossref_metadata_2025 huggingface dataset:
Achieving optimal pulmonary blood flow in the first-stage of palliation in early infancy for complex cardiac defects with hypoplastic left ventricles
Authors: Kitagawa Tetsuya , Katoh Itsuo , Fukumura Yoshiaki , Yoshizumi Masanori , Masuda Yutaka , Hori Takaki
Published: August 2008

The aim of the study was to determine the optimal size and technique for construction of the systemic-to-pulmonary arterial shunt which will provide suitable pulmonary blood flow in first-stage Norwood palliation for hypoplastic left heart syndrome in neonates. Our clinical experience suggested that an arterial oxygen tension of about 30 mm Hg immediately after cardiopulmonary bypass, with the patients being ventilated at the lowest possible mean airway pressure with an FiO2of 1.0, provided a suitable pulmonary-to-systemic flow ratio. We also aimed to clarify the characteristics of pulmonary blood flow in accordance with the size of the shunt and the change in the pulmonary vascular resistance in a simplified rigid model of the Norwood procedure. A hole of2.0 mm diameter proved adequate to provide a suitable pulmonary blood flow of 200−300 mlx002F;min in the presence of a pressure gradient of 20−40 mm Hg between the systemic and pulmonary circulations in neonates weighing 3 kg. A short central shunt with a prosthesis of4 mm in diameter produced an excessive flow of pulmonary blood. Our data suggest that using a smaller shunt than that commonly used is necessary to decrease the early and intermediate postoperative mortality. A prosthesis of 3.0 or 3.5 mm in diameter arising from the brachiocephalic artery would be acceptable and can be recommended for first-stage Norwood palliation in small infants, especially in view of the operative difficulties encountered in taking down the shunt at the time of subsequent operations.
Use of the bidirectional cavopulmonary shunt in patients under one year of age
Authors: Calderón-Colmenero Juan , Ramírez Samuel , Rijlaarsdam Maria , Buendia Alfonso , Zabal Carlos , Zarco-Martínez Ernesto , Attie Fause
Published: August 2008

Between January 1990 and March 1992, a bidirectional cavopulmonary shunt was performed in seven patients under one year of age for palliation of complex cyanotic congenital heart disease. Five cases had tricuspid atresia with a restrictive ventricular septal defect. One case presented with complete mirror imagery, right-sided heart, double inlet and double outlet right ventricle with pulmonary stenosis. Another case had isomerism of the left atrial appendages with common atrioventricular valve and double outlet right ventricle. All had a pulmonary arterial index greater than 250 mm/m2(Nakata index). Age and weights before surgery averaged five months (one to 11 months) and 5.6 kg (range 2.8 to 8.5 kg) respectively. Preoperative mean arterial oxygen saturation measured by cardiac catheterization was 60.05±14% and postoperative mean arterial oxygen saturation on room air was 79.85±6.03% (p<0.05). There were no deaths, although postoperative complications occurred in three patients, namely pleural effusion and pneumothorax. Mean follow-up was 10±4.2 months. The cardiothoracic ratio decreased in all cases. On subsequent echocardiographic studies with pulsed Doppler sampling, low velocity systolic-diastolic forward flow was registered in the pulmonary trunk. The velocity of flow increased clearly during inspiration. The bidirectional cavopulmonary shunt can be considered as primary palliation for complex cardiac malformations with restricted pulmonary flow independent of age and body weight.
Criteria for selection of balloon valvoplasty for treatment of aortic stenosis in neonates
Authors: Donti Andrea , Bonvicini Marco , Gargiulo Gaetano , Frascaroli Guido , Picchio Fernando M.
Published: August 2008

In 10 neonates with critical aortic stenosis who were treated with balloon dilation, we investigated retrospectively the predictive value for mortality of three echocardiographic parameters: early diastolic mitral valvar diameter, left ventricular end-diastolic diameter, and diameter of the aortic root. Valvoplasty was technically successful in each patient and the peak systolic ejection gradient decreased from 85±42 to 22±13 mm Hg, but clinical success was achieved in only six neonates, with four dying. The diameter of the aortic root was similar in survivors and non-survivors. The mitral valvar diameter and the left ventricular end-diastolic diameter, in contrast, were significantly smaller in non-survivors. The mitral valvar diameter and the left ventricular end-diastolic diameter, in contrast, were significantly smaller in non-survivors. The association of a mitral valvar diameter equal to, or less than, 9 mm with a left ventricular end-diastolic diameter equal to, or less than, 14 mm identified clearly all those who did not survive. In the future, we will recommend patients with these anatomical features for primary Norwood palliation. Neonates with a mitral valvar diameter equal to or greater than 12 mm and a left ventricular end-diastolic diameter equal to or greater than 17 mm, in contrast, are good candidates for balloon dilation. All our patients with these anatomical features survived and are doing well at follow-up (30±14.8 months). Simple echocardiographic measurements, therefore, can help in predicting outcome and choosing the best treatment in neonates with critical aortic stenosis.
Prospective study on a new therapeutic strategy for infants and children with aortic coarctation
Authors: Ino Toshihiro , Nishimoto Kei , Akimoto Katsumi , Ohkubo Mataichi , Yabuta Keijiro , Watanabet Mikio , Hosodat Yasuyuki
Published: August 2008

There is little information on the indications for balloon dilation angioplasty for aortic coarctation. To determine the indications for balloon dilation in aortic coarctation, we evaluated prospectively the outcome of children treated according to our therapeutic protocol. The protocol included patients with postoperative recoarctation and isolated native coarctation with risks for surgical repair as conditions for which balloon intervention was absolutely indicated, and native coarctation without a significant arterial duct as a condition possibly qualifying for balloon dilation. Patients with duct-dependent coarctation and diffuse hypoplasia of the aortic arch were not considered to require balloon intervention and were treated surgically. We entered 47 patients into this protocol between January 1988 and December 1993. In 21, dilation was performed successfully and provided significant relief of pressure gradients. The peak systolic pressure gradients across the coarcted site in native coarctation and postoperative recoarctation decreased significantly from 40±19 to 13±10 mm Hg (p<0.0001) and 38±24 to 14±75 mm Hg (p<0.01) immediately after the procedure, respectively. Similarly, the diameters of the coarcted site increased significantly from 3.8±1.8 to 6.6±2.43 mm (p<0.0001) and 4.6±1.9 to 6.0±2.0 mm (p<0.005), respectively. Five patients with native coarctation, and one with postoperative recoarctation, developed restenosis during the period of follow-up and were treated successfully with repeat dilation. It may be possible to change the therapeutic strategy for aortic coarctation with advances in the use of balloon dilation. Our protocol for this technique appears to be reasonable, but further evaluation of its indications is needed.
Configuration of right ventricular pressure curves and infundibular stenosis after balloon pulmonary valvoplasty
Authors: Nakanishi Toshio , Tsuji Tohru , Nakazawa Makoto , Momma Kazuo
Published: August 2008

We evaluated the clinical significance of the configuration of right ventricular pressure curves after balloon valvoplasty in 35 patients with pulmonary valvar stenosis. Right ventricular pressures were measured with a fluidfilled catheter. We divided the subjects into two groups according to the pressure curves seen after balloon valvoplasty. In eight patients, two peaks were found in the curves, with the higher peak occurring at late systole. The remaining 27 patients had a single peak observed during early to mid systole. In all patients in the group with a single peak, the ratio of ventricular pressures decreased by more than half, and no residual narrowing was seen in right ventricular outflow tract, the diameter after valvoplasty increasing by more than half over the diameter before the procedure. In contrast, in five of eight patients in the group with double peaks in the pressure curves, the ratio between ventricular pressures remained higher than 0.5, and the diameter of the right ventricular infundibulum was reduced to less than half the diameter prior to balloon valvoplasty. In three of these patients with double peaked pressure contours, to whom propranolol was administered intravenously, the pressure configuration changed to one with a single peak and the ventricular pressure ratio fell to below 0.5. The degree of obstruction of the right ventricular infundibulum also decreased. These data suggest that a high right ventricular pressure and two peaks in the pressure curve with the higher peak at late systole after balloon valvoplasty indicate, first, the presence of a significant narrowing in the right ventricular outflow tract and, second, effective balloon valvoplasty.
Aortic run-off in children with arterial shunts or persistent arterial duct—characteristics of flow detected by Doppler techniques in the descending aorta
Authors: Ward Cameron J. B. , Sholler Gary F.
Published: August 2008

Flow in the descending aorta was studied to characterize aortic run-off in patients with shunts at the arterial level. Pulsed Doppler examination of descending aortic flow was performed with simultaneous estimation of ratios of shunt-to-systemic flow (shunt flow ratio) at cardiac catheterization. We studied 14 patients with shunt-dependent circulations, eight patients with isolated persistence of the arterial duct, 11 normals and five patients immediately after umbrella occlusion of their persistent arterial duct. Parameters of diastolic flow were standardized to systolic parameters. The ratio of diastolic velocity to the time integral (velocity/time integral retrograde diastolic flow ÷ velocity/time integral systolic flow) ranged from 1×10−1−10.8×10−1. Shunt flow ratios ranged from 0.1−3.9. Pandiastolic retrograde flow was seen in 21 of 22 patients with shunts at the arterial level (the ratio was 0.1 in the remaining patient). No normal patient exhibited pandiastolic retrograde flow. Of the five patients undergoing ductal closure, three exhibited persistent pandiastolic retrograde aortic flow following effective occlusion. The ratio of diastolic velocity to the time integral correlated with the ratio of shunt flow (r=0.76, standard error 0.7). A diastolic velocity/time integral ratio of greater than 8.5×10−1 (sensitivity 67%, specificity 94%) selected patients with shunt flow ratio greater than two, and a diastolic velocity/time integral ratio of less than 5×10−1 (sensitivity 100%, specificity 94%) selected shunt flow ratios of less than 0.5.
The impact of severe congenital heart disease on physical and psychosocial functioning in adolescents
Authors: Bjørnstad Per G. , Spurkland Ingrid , Lindberg Harald L.
Published: August 2008

A study was made of 26 adolescents, aged from 13 to 18 years, with different types of severe congenital heart disease, assessing their somatic condition and its impact on mental health and psychosocial functioning. Physical capacity was reduced, more pronounced in girls than in boys, along with an increased rate of psychiatric problems. Associations were found between physical capacity and psychosocial functioning, and between psychosocial functioning and chronic family difficulties. On the other hand, half the patients studied achieved fair function, both physically and with regard to their mental health. The degree of reduced physical capacity is an important etiological factor for impaired mental health in patients with congenital heart disease. This knowledge should be taken into account when dealing with this group of patients. A good social network seems to be a protective factor. It will probably be rewarding to give patients and their families not only an optimal medical follow-up, but also psychological, practical and financial support to improve or create such a network.
A morphometric study of the human fetal heart with perimembranous ventricular septal defects
Authors: Alvarez Luis , Aránega Antonia , Saucedo Roberto , Marchal Juan A. , González Amanda R. , Hernández María J.
Published: August 2008

Morphometric methods were used to study hearts with perimembranous ventricular septal defects in 22 human fetuses and neonates weighing from one to 3.8 kg (2.221±0.852 kg). All specimens displayed concordant atrioventricular and ventriculoarterial connections, and all had perimembranous ventricular septal defects. A total of 17 interventricular parameters (nine in the right ventricle and eight in the left) were measured in each specimen. The values were compared with patterns of normality established by Rowlatt and her colleagues in hearts from 83 children ranging in age from birth to 15 years. These patterns were found to be less reliable than our own data from fetal and perinatal hearts from children ranging in age from birth to one month. Our morphological data, when appropriately interpreted, have immediate clinical and surgical applications in the treatment of fetal and perinatal cardiovascular disorders.
The combined use of amiodarone and class 1-C antiarrhythmic agents in children with intractable supraventricular tachyarrhythmias
Authors: Balaji Seshadri , Case Christopher L. , Gillette Paul C.
Published: August 2008

Combined antiarrhythmic drug therapy is an occasionally necessary but problematic approach to the child with recalcitrant supraventricular tachycardia. There is little experience with the combined use of amiodarone and class 1-C agents (flecainide, propafenone and encainide) in children. To judge the efficacy and safety of this combination, we reviewed the case notes and results of investigation in all nine children with supraventricular tachycardia who received such therapy between 1984 and 1993. These nine children received combined therapy on 12 occasions. Five were infants with either atrioventricular reentrant tachycardia (n=3) or atrial ectopic tachycardia (n=2), and four were older children with atrial flutter seen after a Fontan procedure. Amiodarone was combined with flecainide on eight occasions, with propafenone on three occasions, and with encainide on one occasion. Both infants with atrial ectopic tachycardia were successfully controlled, but only one of three infants with atrioventricular reentrant tachycardia had successful control on combination therapy. In three of the four patients with atrial flutter, the combination was useful in reducing the number of arrhythmic episodes. Three infants suffered side effects. At electrophysiologic study to judge efficacy, ventricular tachycardia was induced in two patients (one infant and one Fontan patient), necessitating a change in the 1-C agent. One patient had skin rash due to flecainide and was placed on propafenone with success. One Fontan patient died of complications after an elective surgical procedure. No deaths occurred attributable to proarrhythmia. Thus, combined therapy with amiodarone and 1-C agents was found to be safe and fairly effective in children with certain types of intractable supraventricular tachycardia.
Ruptured aneurysm of the aortic sinus of Valsalva—difficulties in establishing the diagnosis
Authors: Holdright Diana R. , Brecker Stephen , Sheppard Mary
Published: August 2008

Aneurysms of the aortic sinus of Valsalva are rare and generally do not produce symptoms unless the aneurysm ruptures. When rupture occurs, the clinical findings depend to an extent on the site of the aneurysm and the cardiac chamber into which the aneurysm ruptures. We report a case of acute rupture of an aneurysm of the right aortic sinus, which was misdiagnosed as a ventricular septal defect. The clinical condition of the patient deteriorated rapidly, and transcatheter closure of the defect with an umbrella was associated with a fatal outcome.
Non-surgical reconstruction of the right ventricular outflow tract in acquired infundibular atresia
Authors: Rosenthal Eric , Qureshi Shakeel A. , Tynan Michael
Published: August 2008

A 33-year-old woman known to have tetralogy of Fallot and a straddling tricuspid valve had bilateral systemic-to-pulmonary arterial shunts constructed at the age of six months. She subsequently developed infundibular atresia. Antegrade flow of blood from the right ventricle to pulmonary trunk was re-established by percutaneous laser perforation of the outflow tract, later followed by implantation of a stent.
Co-existence of quadricuspid aortic and pulmonary valves in an adolescent with rheumatic heart disease
Authors: Attie Fause , Zabal Carlos , López-Valle Hilda R.
Published: August 2008

We describe a male adolescent with co-existence of quadricuspid aortic and pulmonary valves and rheumatic heart disease. As far as we know, the presence of quadricuspid aortic and pulmonary valves in the same patient has not previously been described. Furthermore, the presence of rheumatic heart disease in this case makes it a true rarity.
Fistula between the right pulmonary artery and the left atrium—an unusual cause of cyanotic heart disease
Authors: Saatvedt Kjell , Stake Gunnar , Lindberg Harald
Published: August 2008

A fistula from the pulmonary artery to the left atrium is a very rare congenital anomaly. The communication, almost exclusively arising from the right pulmonary artery, may give rise to a variety of symptoms with cyanosis as the predominant sign. To our knowledge only seven neonates have been reported with this anomaly, and five of them died postoperatively. We present another two neonates with such a fistula which was closed by simple ligation using a transsternal approach and cardiopulmonary bypass. Both patients had an uneventful postoperative course and are alive and well at the age of two and three years, respectively.
Horseshoe lung associated with anomalous pulmonary venous connection without pulmonary hypoplasia
Authors: Corno Antonio , Rosti Luca , Machado Ivan
Published: August 2008

Horseshoe lung is an exceedingly rare congenital malformation, characterized by unilateral pulmonary hypoplasia together with a midline isthmus producing fusion of the tissues of the lower lobes. It is frequently associated with other cardiac and extracardiac anomalies. We report an infant with a variant of the horseshoe lung with partial anomalous venous connection of intracardiac type, ventricular septal defect, and persistent left superior caval vein. There was no pulmonary hypoplasia.
Successful treatment of leakage of serous fluid following construction of a polytetrafluorethylene central shunt
Authors: Masiello Paolo , Panza Antonio , Di Benedetto Giuseppe
Published: August 2008

A central polytetrafluorethylene shunt was constructed in a 13-year-old boy after previous placement of a Waterston-Cooley shunt. Because of excessive leakage of serous fluid from the prosthesis, the boy required a second operation when the shunt was wrapped with absorbable collagen hemostat (Novacol FibrillarTM). The leakage stopped suddenly and the boy was discharged home after one week. Follow-up at four months shows no pericardial effusion and good function of the shunt.
Catheter closure of a residual aortopulmonary window after corrective surgery
Authors: Gildein H. P. , Mocellin R.
Published: August 2008

Apart from its original use for closing an open duct, the Rashkind ductal occluder has been used to close a variety of other defects. We report successful use of this device to close a residual aortopulmonary window persisting after corrective surgery had been carried out at the age of six months.
Recanalization of the arterial duct after initial total occlusion with a Rashkind umbrella
Authors: Bjørnstad Per G. , Smevik Bjarne
Published: August 2008

We report two patients in whom an initially completely closed arterial duct reopened 6–12 months after implantation of the Rashkind USCI®umbrella. We are not aware of any other reports of this occurrence. No obvious reason could be found for the recanalization.
Relation of left ventricular mass, volume and mass/volume ratio in children with aortic stenosis and/or insufficiency
Authors: Li Jennifer S. , Snider A. Rebecca , Zreik Hani , Armstrong Brenda E. , NeSmith James W. , Bengur A. Resai
Published: August 2008

To determine if symptoms and electrocardiographic abnormalities relate to left ventricular mass, volume, and mass/volume ratio in children with aortic stenosis and/or insufficiency, we examined 42 patients (aged 11±6 years) with cross-sectional and Doppler echocardiography. Clinical symptoms included exertional chest pain, shortness of breath, exercise intolerance, congestive heart failure and syncope. Electrocardiographic abnormalities were defined as the presence of both left ventricular hypertrophy and ST-T wave changes. Left ventricular volumes and mass were measured from echocardiograms using paired orthogonal apical views and biplane Simpson's and truncated ellipsoid models, respectively. The peak and mean pressure gradients across the aortic valve, the aortic valvar area, and the degree of aortic insufficiency were determined using echo/Doppler techniques. Eighteen patients (ages 10±7 years) had pure aortic stenosis (peak gradient 52±29 mm Hg, mean gradient 28±18 mm Hg, and valve area 0.86±.46 cm2/m2). Of these 18, seven had symptoms and seven had an abnormal electrocardiogram. Twenty-four patients (aged 12±6 years) had aortic stenosis and aortic insufficiency (peak gradient 36±18 mm Hg, mean gradient 19±9 mm Hg, 18 with 1−2+ aortic insufficiency and six with 3−4+ aortic insufficiency). Of these 24, 10 had symptoms and 10 had an abnormal electrocardiogram. When symptomatic and asymptomatic patients were compared using an unpaired Student's t-test, symptomatic patients with pure aortic stenosis and combined aortic stenosis and insufficiency had left ventricular hypertrophy and inappropriately increased mass/volume ratios. However, due to differences in left ventricular volume, symptoms and electrocardiographic abnormalities occurred at a higher mass/ volume ratio in pure aortic stenosis (2.7±0.6) than in combined aortic stenosis and insufficiency (1.7±0.3).
Modulation of the viability of immature cardiac myocytes by cardiac fibroblasts after hypothermic preservation—its values as a technique for evaluation of storage solutions
Authors: Orita Hiroyuki , Fukasawa Manabu , Uchino Hideaki , Fukui Kana , Kohi Minoru , Washio Masahiko
Published: August 2008

We evaluated the modulation of the viability of immature cardiac myocytes by cardiac fibroblasts after hypothermic preservation using three types of storage solutions—saline, University of Wisconsin solution, and MCDB 107 medium. Cardiac myocytes and fibroblasts were isolated from neonatal rat ventricles, and cultures of myocytes only or co-cultures with fibroblasts (myocyte: fibroblast 2:1) were established. On the fourth day of culture, the cultures were incubated at 4 °C for 6, 12, 18 and 24 hours in the different storage solutions. Enzymes were measured in the storage solutions immediately before and after hypothermic incubation. The cultures were then incubated for an additional 24 hours at 37 °C to evaluate the recovery of the myocyte beating rate. The myocyte beating rate in the co-culture groups showed significantly higher recovery ratios than the corresponding groups in which only myocytes were cultured. Complete recovery was observed in the group co-cultured in MCDB medium 24 hours after hypothermic incubation (83.4% of control—beating rate prior to hypothermic incubation) compared to the other co-cultured groups (15.4, 0%, respectively). Release of enzymes in the co-cultures was significantly suppressed compared to the cultured myocytes, and the greatest suppression was found after 24 hours of incubation in MCDB medium (CPK: 36.6 mIU/flask, LDH: 281.2 mIU/flask) compared to the other two co-cultured groups (CPK: 181.1, 281.1; LDH: 501.7, 773.2). Cardiac fibroblasts diminished myocytic injury after hypothermic preservation using various storage solutions, in which MCDB 107 medium showed the best overall protective effect. Thus, cardiac fibroblasts may play an important role in controlling myocytic viability under various hypothermic conditions.
Open heart surgery without homologous blood transfusion and subsequent use of recombinant erythropoietin to treat postoperative anemia
Authors: Vogel Michael , Kirchhoff Antje , Bühlmeyer Konrad , Bourlon François
Published: August 2008

The purpose of our study was to assess the effect of administration of erythropoietin on postoperative anemia in patients with congenital heart disease who had undergone open heart surgery without the use of homologous blood. One intravenous and one subcutaneous dose of 300 units of erythropoietin per kg body weight was given to 10 patients at a mean of five (3–8) days after surgery. Another group of 10 patients, who had undergone open heart surgery but were not given erythropoietin postoperatively, were used as controls. There was a significant fall in all patients in hemoglobin from 13.6±1.3 gm/dl prior to surgery to 9.8±1.6 gm/dl after surgery. In patients treated with erythropoietin, there was an immediate significant rise in hemoglobin and hematocrit. Hemoglobin rose by 14% on the seventh day after treatment (the twelfth day after surgery) and by 25% on the tenth day after treatment (the fifteenth day after surgery). It had increased by only 8% 15 days after surgery in the controls. These findings suggest a beneficial effect of administration of recombinant erythropoietin on postoperative anemia in children who had undergone open heart surgery without the use of homologous blood.
A new surgical approach for preparation of candidates for the Fontan operation with acquired systemic-pulmonary collateral arteries
Authors: Matsuki Osamu , Yagihara Toshikatsu , Kawashima Yasunaru
Published: August 2008

A new surgical technique is described to reduce the flow through acquired systemic-pulmonary collateral arteries, a complication which has been recognized as a risk factor in patients undergoing the Fontan operation. When systemic-pulmonary collateral arteries have developed subsequent to previous construction of an ipsilateral Blalock-Taussig shunt or a Glenn anastomosis, and are identified by angiography in advance of the Fontan operation, the technique is indicated as a preparative procedure. It consists of rethoracotomy aimed at isolating the lung from the thoracic wall (peeling), and wrapping of the upper half of the lung with expanded polytetrafluoroethylene sheets to avoid redevelopment of collateral vessels into the lung (wrapping). This decreases the systemic-collateral flow through small vessels which cannot be handled by conventional embolization using catheterization techniques, and permits the Fontan operation to be performed more safely.
Aortic insufficiency associated with complex cardiac anomalies
Authors: Folliguet Thierry A. , Laborde François , Macé Loїc , Dervanian Patrice , Dibie Alain , Grinda Jean-Michel , Neveux Jean-Yves
Published: August 2008

The purpose of this study was to determine the cause, onset, clinical outcome and treatment of aortic insufficiency developing during treatment of complex cardiac anomalies. Aortic insufficiency associated with ventricular septal defect is a well-recognized entity. Very few studies, in contrast, have addressed the development of aortic insufficiency in the presence of more complex cardiac anomalies. Ten patients were selected from two clinical centers. All patients were diagnosed with a major cardiac anomaly requiring surgical treatment. Each patient developed aortic insufficiency requiring a surgical treatment of the valve after correction or palliation of their cardiac defect. There were six males, and mean age for the series was 17 years (range 5.5 to 33 years). All patients were initially operated for repair (eight patients) or palliation (two patients) of their congenital anomaly. Five patients had tetralogy of Fallot, one had tetralogy associated with pulmonary atresia, and two patients had double outlet right ventricle, and two had complete transposition. The degree of aortic insufficiency, the amount of pulmonary stenosis present before the original procedure and the diameter of the aortic root measured before the aortic surgical procedure on the valve were assessed by angiography and echocardiography. The mean time between the last surgical procedure and the appearance of aortic regurgitation was three years, with a mean time between the last intervention and the aortic procedure of 7.3 years. The etiology of the insufficiency was explained by prolapse of an aortic leaflet in three patients. Four patients had annular dilation with central regurgitation, two patients had traumatic perforation of a leaflet, one patient had lesions consistent with endocarditis. Eight patients had dilation of the aortic root. Surgical procedures included five replacements of the aortic valve, three aortic valvoplasties, one aortic valvar replacement associated with a Glenn shunt, and one Bentall procedure. One patient died two months following an aortic valvar replacement. All patients were assessed by transthoracic echocardiography before discharged. The two main causes of aortic regurgitation, leaflet prolapse and annular dilation are age-related complications. Most patients had dilation of the aortic root from longstanding increases in aortic flow. Early repair of congenital anomalies may decrease these complications.
Unicommissural aortic valve in neonates and its association with other congenital heart disease
Authors: Durán Ana C. , Daliento Luciano , Frescura Carla , Stellin Giovanni , Sans-Coma Valentín , Angelini Annalisa , Milanesi Ornella , Thiene Gaetano
Published: August 2008

The morphology of the aortic valve was studied in 1,022 heart specimens belonging to the collection of the Institute of Pathological Anatomy, University of Padua. Twenty specimens were found to have a unicommissural aortic valve, characterized by the presence of a single leaflet with only one functional commissure; however, the presence of two raphes enabled the recognition of a basically three-sinus arrangement. Age and gender were known in 19 cases: 14 male and five female, mean age nine days. In 19 cases, the unicommissural valve was dysplastic and resulted in a severe congenital aortic valvar stenosis. Only in two hearts was the unicommissural nature of the aortic valve an isolated finding; among the remaining 18, left ventricular fibroelastosis in 11, malformation of the mitral valve in 11, hypoplasia of the left ventricle in eight, ventricular septal defect in four, mitral atresia in three, and subaortic fibrous diaphragm in one. Furthermore, seven (35%) of these 20 unicommissural aortic valves were associated with coarctation of the aorta. Statistical analysis shows that this association is not a random event. Our findings support the hypothesis that the unicommissural aortic valve originates from the early fusion of the three mesenchymal valvar cushions or leaflet primordia. Although the present data do not exclude the possibility that reduction of the blood flow through the aorta during fetal life may play a role in the formation of the unicommissural aortic valve, they rather point in the direction that another etiologic factor, such as an anomalous migration of neural crest cells, may be responsible for the fusion of the valvar cushions.
Cross-sectional echocardiographic delineation of coronary arterial origins in complete transposition
Authors: Kosutic Jovan
Published: August 2008

The role of cross-sectional echocardiography is demonstrated in defining the anatomy of the coronary arteries in 29 patients with complete transposition (concordant atrioventricular and discordant ventriculoarterial connections). The study revealed a relationship between some of the less frequent patterns and the spatial relationship of the arterial trunks, along with the presence or absence of a ventricular septal defect. It suggests that a side-by-side relationship of the arterial trunks due to infundibular maldevelopment may be related to some of the less common patterns.
The carotid arterial approach for balloon dilation of critical aortic stenosis in neonates—immediate results and follow-up
Authors: Giusti Sandra , Borghi Adele , Redaelli Sofia , Bonhoeffer Philipp , Spadoni Isabella , Macrì Rocco , Carminati Mario
Published: August 2008

Balloon dilation of the aortic valve was performed in 20 consecutive neonates with critical aortic stenosis using an approach achieved by cutting down on the right carotid artery. The age of the patients ranged from one to 25 days (mean seven days) and their weight from 2.1 to 4.0 kg (mean 3.16 kg). All patients were evaluated before cardiac catheterization with cross-sectional and Doppler echocardiography so as to keep the catheterization procedure as short as possible. Balloon dilation was accomplished in all patients. The only complication was an apical perforation by the guide wire in two cases. The ensuing pericardial effusion was immediately drained with pericardiocentesis and the subsequent course of the procedure was uneventful. Immediate results showed dramatic improvement in cardiovascular conditions. The transvalvar pressure gradient fell from 80±40 to 27±20 mm Hg (p<0.001). Left ventricular ejection fraction evaluated by echocardiography increased from 30±21% before dilation to 54±18% 24-48 hours after the procedure (p<0.001). In all patients, the procedure was free from vascular complications. Aortic regurgitation was documented after the procedure in 11 patients, being severe in one, moderate in five and trivial in five. Seven patients died, although in only one was the death related directly to the procedure itself. Six patients died because of associated lesions despite an immediate satisfactory result of the balloon valvoplasty. The 13 surviving patients are doing well, and are receiving no medications. During a mean follow-up of 25 months (range 2-54 months), four patients have developed restenosis. One underwent surgical valvotomy at one year of age. The second was successfully redilated through the same approach at two months of age. The other two have a significant gradient, as assessed by Doppler measurements (60 and 70 mm Hg), with normal systolic ventricular function. Two patients have moderate aortic regurgitation. Balloon dilation achieved through cutdown on the right carotid artery is a safe and effective alternative to surgery in neonates with isolated aortic stenosis. The unfavorable results are mainly due to associated anomalies.
Right ventricular “overhaul”—an intermediate step in the biventricular repair of pulmonary atresia with intact ventricular septum
Authors: Pawade Ash , Mee Roger B. B. , Karl Tom
Published: August 2008

The management of pulmonary atresia with intact ventricular septum remains controversial. The plan for management at our institution is based on the echocardiographic identification of a well-developed infundibulum. Neonates with such a well-developed infundibulum are prepared for a biventricular repair, whereas those without an infundibulum are groomed for a Fontan operation. In all, 48 neonates with the lesion were admitted to our unit between 1980 and 1992. In 31 neonates with a well-formed infundibulum, the initial palliation consisted mainly of pulmonary valvotomy without cardiopulmonary bypass and construction of a polytetrafluoroethylene shunt from the left subclavian artery to the pulmonary trunk. In most of these patients, growth of the right ventricle could be promoted by establishing antegrade flow through the ventricle. In seven patients in this group, nonetheless, the right ventricle failed to grow satisfactorily. In this subgroup, additional intermediate procedures were performed on the apical component and infundibulum of the ventricle together with the tricuspid and pulmonary valves which could collectively be termed as “right ventricular overhaul.” Five of these patients have gone on to further successful biventricular repairs at a median interval of 24 months after the overhaul. There was no operative mortality in this group. Following the final biventricular repair, there have been no late deaths or reoperations over a total follow-up of 145.4 patient months (mean 22.2 months).
Cardiac rhabdomyoma in childhood tuberous sclerosis
Authors: Choi Jung Yun , Bae Eun Jung , Noh Chung Il , Yoon Yong-Soo , Hwang Yong Seung
Published: August 2008

We studied 52 children in order to assess the prevalence, natural history, and electrophysiologic effects of cardiac rhabdomyoma in tuberous sclerosis. Their ages ranged from one day to 12 years (median age 1.33 year). The tumors in the heart were found in 22 patients through cross-sectional echocardiography. The overall prevalence of cardiac rhabdomyoma was 42%. The prevalence of cardiac tumors in patients first seen in infancy was 77%, and that in patients over one year of age was 31%. Those with cardiac tumors diagnosed in infancy had more tumors per person than did the patients diagnosed after infancy, and the tumors were smaller in the patients diagnosed over one year of age. Follow-up cross-sectional echocardiography showed that almost all tumors showed spontaneous regression. Complete regression occurred more commonly in younger patients, and in those with smaller tumors. About 70% of patients with cardiac tumors had either normal findings or infrequent premature contractions on 24hour Holter monitorings. The rest of the patients with cardiac tumors showed either frequent premature contractions, atrioventricular block, or atrial tachycardia, but antiarrhythmic medication was rarely required. Cross-sectional echocardiography, therefore, seems to be the best diagnostic tool for screening cardiac rhabdomyomas in patients with tuberous sclerosis. The tumors may be managed more conservatively in view of their high rate of spontaneous regression and rather rare association with symptomatic arrhythmias.
Neonatal tricuspid insufficiency—a Doppler echocardiographic study of 49 cases
Authors: Senocak Filiz , Özkutlu Süheyla
Published: August 2008

We analyzed tricuspid insufficiency noninvasively using cross-sectional and continuous wave Doppler echocardiography in 49 newborn infants free of structural cardiac and pulmonary disease. In 41(83.6%) of the cases, mild insufficiency was detected using the continuous wave Doppler technique. All the infants with valvar insufficiency had normal electrocardiographic tracings and, in 25 (61%) of them, there was no audible murmur. We detected transient ductal patency in five (12.1%). The ratio of right ventricular preejection period to ventricular ejection time was in the normal ranges (below 0.05) in 39 (95.1%) of the neonates. Of the 30 cases who underwent continued surveillance over a mean period of 37.9 days, the incompetence disappeared in 15 (50%). Our findings show that, in neonates, the lack of a murmur does not eliminate the possibility of neonatal tricuspid incompetence. Furthermore, abnormalities such as postnatal transient pulmonary hypertension, fetal closure of the arterial duct and perinatal asphyxia cannot readily explain the finding of tricuspid incompetence.
Rotation of the umbrella, an important factor for residual patency of arterial ducts closed with the Rashkind device
Authors: Bjørnstad Per G. , Stensæth Knut-Haakon
Published: August 2008

To check a possible mechanism for residual patency of arterial ducts closed with umbrellas, we studied retrospectively all 25 implantations of the Rashkind device carried out in our institution. In all instances, the smaller umbrella had been used. Residual patency occurred in 40%. There was a significant correlation (p<0.001) between the way the umbrella was rotated and the rate of residual patency. In the larger subgroup, the residual patency was 75% when two arms were pointing towards the aortic blood stream. In contrast, when only one arm pointed upwards, there was only one patient (8.3%) with residual shunt. This potential precipitating mechanism should be considered when implanting the Rashkind devices in future.
Isolation of the left pulmonary artery and ventricular septal defect—successful staged management
Authors: Milanesi Ornella , Stellin Giovanni , Zucchetta Pietro
Published: August 2008

We present a case of anomalous origin of the left pulmonary artery from a left-sided brachiocephalic artery via an arterial duct in a patient with a right aortic arch. Associated intracardiac anomalies were a large perimembranous ventricular septal defect and the persistence of a small vertical vein, connecting the pulmonary veins normally incorporated into the left atrium to the brachiocephalic vein. Spontaneous closure of the arterial duct occurred in the first days of life and was responsible for the circulatory “isolation” of the left pulmonary artery. The clinical course, surgical strategy and possible embryological explanation are described.
Surgical repair of unilateral pulmonary venous stenosis
Authors: Sakamoto Yoshimasa , Kurosawa Hiromi , Hashimoto Kazuhiro
Published: August 2008

We describe a two-year-old boy suffering from pulmonary hypertension due to pulmonary venous stenosis. Previous reports suggest that it is not easy to diagnose this entity during life, and most cases are diagnosed at operation or autopsy. Our patient was diagnosed relatively early in life and treated successfully by pericardial patch venoplasty. Histologic examination of the lung biopsy performed at operation was considered to be useftil prognostically. The minimal pulmonary vascular changes noted were consistent with a good outcome after the surgical repair.
Isolated noncompaction of ventricular myocardium—detection during fetal life
Authors: Kohl Thomas , Villegas Maria , Silverman Norman
Published: August 2008

Isolated noncompaction of ventricular myocardium is a rare cardiomyopathy with persistence of a myocardial architecture such as seen early in fetal development with numerous prominent trabeculations and deep intertrabecular spaces. Symptoms are related to cardiac failure, arterial embolism, and severe arrhythmias. Familial recurrence has been described. We describe a case where isolated noncompaction of ventricular myocardium could be recognized on an ultrasound study at 23 weeks gestation, thus supporting the developmental hypothesis.
Aorto-left ventricular tunnel in association with hypoplastic left heart syndrome—recognition by transesophageal and transthoracic echocardiography
Authors: Birk Einat , Silverman Norman H. , Vidne Bernardo A.
Published: August 2008

We describe a neonate who presented with aortic atresia, a hypoplastic left ventricle and aorto-left ventricular tunnel. These findings, recognized by transthoracic and transesophageal cross-sectional echocardiography complemented by Doppler color flow imaging, were confirmed at surgery by direct inspection. This complex of associations has not been described before and suggests a primary abnormality of the left ventricle because diastolic filling from the aorto-left venticular tunnel and mitral flow should tend to ensure fetal left ventricular growth.
Prenatal diagnosis of an obstructive mediastinal bronchogenic cyst
Authors: Patel Chandrakant R. , Muise Kevin L. , Levine Marc M.
Published: August 2008

We report a patient with a mediastinal cyst compressing the left atrium that was diagnosed prenatally by fetal echocardiography. Postnatally, attempted aspiration guided by computerized tomography was unsuccessful. Because of respiratory distress, the cyst was removed surgically. Pathological examination revealed a bronchogenic cyst.
Rheumatic pneumonia with carditis and arthritis in a three-year-old girl
Authors: Saraiva Lurildo Ribeiro , Carneiro Regina Walmsley , Willcox Glauco
Published: August 2008

We describe a three-year-old girl in whom arthritis developed ten days after the onset of illness, followed by clinical features consistent with rheumatic carditis and pneumonia. Treatment with prednisone proved satisfactory. This case illustrates the persistence of patterns of rheumatic fever in northeast Brazil which are most unusual in developed countries
Transposition of the great arteries {S,D,D} and absent proximal left pulmonary artery
Authors: Kholwadwala Dipak , Parnell Vincent A. , Cooper Rubin S.
Published: August 2008

while preferential blood flow to the rightpulmonary artery has been described in transposition of the great arteries with or without obstruction of the left ventricular outflow tract, this disparity of pulmonary blood flow is not present in newborns.1We report a newborn with transposition in whom there was discontinuity of the pulmonary arteries and ductal blood supply to the left pulmonary artery. To our knowledge, this entity has not been described in newborns with transposition of the great arteries {S,D,D}.
Prostaglandins in congenital heart disease—potential for confusion
Authors: Madar R. John , Donaldson Tim J.D. , Hunter Stewart
Published: August 2008

the use of prostaglandians in maintaining the patency of the arterial duct in congenital heart disease is well established. Intravenous1-3 and ora12–4 administration has been used, although for acute use intravenous and possibly intraosseous5 routes are favored. Both prostaglandin E1 (alprostadil—ProstinVR: Upjohn)1,4 and prostaglandin E2 (dinoprostone—Prostin E2: Upjohn)2,3 are used for this purpose, although only prostaglandin E1 is licensed for this indication in the United Kingdom. Prostaglandin E1 costs approximately 8 times more than prostaglandin E2 (£56.96 versus £7.43 per vial).
The Centre and the Periphery: some reflections on the nature and conduct of architectural research
Authors: Hawkes Dean
Published: August 2008

This paper, which is based upon a talk given to the Martin Centre Research Society at the Department of Architecture at Cambridge in October 1994, examines the continuing relevance of the model of architectural research which was proposed at the Oxford Conference on architectural education in 1958. It suggests that the Oxford model, with its roots in the procedures of the sciences, in which ‘fundamental’ research precedes ‘development’, before leading into practical application, fails to account for the role of the designer in the evolution of the state of architecture. It also proposes that developments in architectural education, in particular the growth of the unit system of studio instruction, have added a further element to the productive and investigative potential of the schools of architecture, and that this must be accounted for in any valid model of research.
Can design be called research?
Authors: Yeomans David
Published: August 2008

As funding for research in British universities is now dependent upon an assessment of research productivity (made every four years), departments of architecture are finding that design, which used to be considered as the equivalent of research for architectural teachers, may not be regarded as such for the purposes of this assessment. The arguments both for and against considering design as a research activity are discussed, as is the need for a recognised way of grading the quality of work. Suggestions are made for the direction in which design as research in schools of architecture might take.
Wealden poolhouse: reflections on language and architecture
Authors: Hogben Gavin
Published: August 2008

An account is given of the recently completed Wealden poolhouse, and an argument is made for embracing indeterminacy in language and architecture
A tapestry in the landscape
Authors: Beigel Florian , Christou Philip
Published: August 2008

A concise report about the landform, architectural, and detail design concepts for the recently completed modernisation of the Bishopsfield Housing Estate in Harlow, Essex. The objective was to develop proposals for new interventions which are directly based on, and give emphasis and new life in a contemporary way to the architectural and urban design conceptions of the original architects and town planner.