bluuebunny/crossref_metadata_2025_split huggingface dataset:
Oral prostacyclin enhances the pulmonary vasodilatory effect of nitric oxide in children with pulmonary hypertension
Authors: Uese Kei-ichiro , Ichida Fukiko , Tsubata Shin-ichi , Hashimoto Ikuo , Hamamichi Yuji , Fukahara Kazuaki , Murakami Arata , Miyawaki Toshio 
Published: August 2008

AbstractTo evaluate the potential efficacy of the combined administration of inhaled nitric oxide and oral beraprost sodium in producing pulmonary vasodilation, we studied 20 patients with pulmonary hypertension during cardiac catheterization. We also evaluated some of the mechanisms of vasodilation under these circumstances by investigating the levels of cyclic GMP and AMP in the pulmonary venous blood. A significant decrease in pulmonary vascular resistance was observed after the administration of nitric oxide (−37 ± 6%, p<0.01), and more intense decrease was observed after the combined administration of nitric oxide and beraprost sodium (−46 ± 6%, p<0.05). In addition, the ratio of pulmonary-to-systemic resistance also decreased to a greater extent with combined administration than with nitric oxide alone (−34 ± 6% vs −42 ± 7%, p<0.05). Conversely, systemic vascular resistance showed no change, neither after loading with nitric oxide nor after combined administration. Although equivalent increases in levels of cyclic GMP were observed after inhalation of nitric and the combined administration (mean 78% vs 65%), greater increases in levels of cyclic AMP were observed with the combined use (mean 15% vs 69%). Combined treatment with nitric oxide and beraprost sodium may prove markedly beneficial in patients with primary and postoperative pulmonary hypertension who do not respond adequately to inhalation of nitric oxide alone or conventional modes of treatment.
The role of angiogenesis in the development of pulmonary arteriovenous malformations in children after cavopulmonary anastomosis
Authors: Marshall Blair , Duncan Brian W. , Jonas Richard A. 
Published: August 2008

AbstractPulmonary arteriovenous malformations are a frequent cause of progressive cyanosis after construction of a cavopulmonary anastomosis. Their formation complicates the management of children with single ventricle physiology after a bidirectional Glenn shunt or the Kawashima procedure. The key role of the liver in this phenomenon is suggested by the observation that providing modifications of the Fontan procedure which permit hepatic venous effluent to reach the pulmonary arterial circulation limit further development of the malformations. In addition, it is known that patients with end-stage hepatic failure develop pulmonary arteriovenous malformations that diminish after liver transplantation. We have begun purification of a factor derived from hepatocyte-conditioned media that is inhibitory for the proliferation of cultured endothelial cells. This substance is heat sensitive, and binds avidly to a copper-containing chromatography column. These clinical observations, and this preliminary experimental work, support the concept that hepatic-derived angiogenic factors may play a role in the development of pulmonary arteriovenous malformations after construction of cavopulmonary anastomoses.
Venous obstruction due to insertion of permanent transvenous pacemaker electrodes in children
Authors: Çeliker Alpay , Tokel Kürsad , Balkanci Ferhun , Çekirge Saruhan , Oto Ali , Özme Şencan 
Published: August 2008

AbstractTransvenous placement of pacemaker leads is the recommended method for permanent pacemaking in children. We evaluated 11 children with transvenous ventricular pacemaker leads by digital subtraction angiography in order to determine the incidence of venous obstruction. The ages at the implantation ranged from 4.5 to 11.2 years with a mean of 8.05±2.17 and a median of 7.9 years. The interval from implantation to the time of procedure ranged from 12 to 64 months, with a mean of 37.4±19.2 and a median of 45 months. Complete occlusion of the subclavian vein was demonstrated in two patients. We also showed significant stenosis of the venous system in five further patients. Of the 7 patients, 5 had an extensive venous collateral circulation. We did not observe obstruction of the superior caval vein, or superior caval venous syndrome. Although we did not demonstrate this syndrome, we propose future regular evaluation of all our children undergoing transvenous pacemaking. This point may be important when second or third leads are implanted.
Progression of congenital aortic stenosis in children beyond infancy: assessment using Doppler echocardiography
Authors: Goel Punit , Kumar Krishan , Kothari S.S. , Saxena Anita , Wasir Harbans S. 
Published: August 2008

AbstractCase records of 67 children who presented beyond infancy (57 male, 10 female) with congenital valvar aortic stenosis were reviewed to assess progression utilizing the Doppler derived peak gradient as the index of severity. Age at presentation ranged from 1–13 years, and mean follow-up was 67 ± 29 months (range 12–142 months). The patients were divided into three groups depending on the rate of progression. Those with the most rapid rate of progression were significantly older when aortic stenosis was detected and at the time of presentation compared to the other two groups, who progressed minimally or not at all (p < 0.002 and p < 0.01 respectively). There was no significant correlation between the progression of stenosis and other clinical or Doppler echocardiographic variables.
Cerebral abscess in children with cyanotic congenital heart disease
Authors: Baysal Kemal , Öztürk Fadil , Uysal Serap , Sancak Recep , Gürses Nuran 
Published: August 2008

AbstractThe cerebral abscess still occurs in children with cyanotic congenital heart disease. Mortality and morbidity have not yet been reduced in spite of new antibiotics and surgical techniques. We describe our experience with 185 patients having cyanotic congenital heart disease (117 with tetralogy of Fallot, 23 with complete transposition, 9 with tricuspid atresia, 4 with total abnormal venous return, 10 with double-inlet ventricle, 15 with double-outlet right ventricle and 7 with common arterial trunk) who have been followed regularly in our department. Their ages ranged between 3 years and 15 years. During the period of follow-up, cerebral abscess developed in 7 cases. Six of them had tetralogy of Fallot and one had complete transposition. One patient died before surgery could be performed. Operation was carried out in 6 patients and one died in the early postoperative stage. The others were as healthy as could be expected. Alpha-hemolytic streptococcus was isolated from the abscess in one patient, and Enterobacter in another. The abscesses were localized in the occipito-parietal region in 2 cases, the fronto-parietal region in 2 cases, and the parietal, temporal and occipital regions in one case each. Physical examination was normal apart from fever in 5 patients and, in these patients, the cause of fever could not be explained. Neurologic evaluations were all within normal range. If fever occurs unexpectedly in a patient with cyanotic congenital heart disease, therefore, it is important to rule out the possibility of a cerebral abscess.
Infectious endocarditis in an analysis of fifty-five episodes over a ten-year period
Authors: Wong Tracy , Fowler Rodney S. , Freedom Robert , Johnston Douglas H. 
Published: August 2008

AbstractMortality from infectious endocarditis in the general paediatric population had decreased significantly over the last two decades. This is attributable to the increased awareness of the health care team in recognizing the early signs of this once fatal disease such that early antibiotic intervention is possible. Two groups, however, remain at high risk for infectious endocarditis. Advances in cardiac surgery have dramatically improved the prognosis for survival in many children with otherwise fatal congenital heart diseases. This group of children, along with the seriously ill who have central venous catheters in place, are currently at the highest risk for development of infectious endocarditis.1-5
Clinical profile and prognosis of hypertrophic cardiomyopathy when first diagnosed in infancy as opposed to childhood
Authors: Comparato Calogero , Pipitone Salvatore , Sperandeo Velio , Mongiovì Maurizio 
Published: August 2008

AbstractOnly small groups of children, and especially infants, with hypertrophic cardiomyopathy have been analysed thus far. The clinical features, and the evolution, of this potentially heterogeneous disease, therefore, remain to be clarified. We describe the clinical profile of 35 patients with hypertrophic cardiomyopathy, 18 being younger than 1 year when first seen. Electrocardiography, chest radiography and echocardiography (m-mode, cross-sectional and color-Doppler) were performed in all patients. Ambulatory electrocardiographic monitoring was performed in 20 patients, and cardiac catheterization in 5 patients. The duration of follow-up was 6.6 years, and ranged from 1 month to 15 years. First-degree relatives of 9 infants and 13 children underwent complete echocardiographic evaluation.Inheritance, along with presence of congestive heart failure, cyanosis, cardiac enlargement and right ventricular hypertrophy proved to be different in infants who presented with hypertrophic cardiomyiopa-thy as compared with those presenting in childhood. In contrast, echocardiography showed that the distribution of ventricular hypertrophy was similar in the two groups. Intractable congestive heart failure, which was the commonest cause of death, was an ominous prognostic sign in infants. Older children, however, tended to die suddenly. Spontaneous resolution of severe ventricular hypertrophy occurred in four infants of non-diabetic mothers within their first 6 months of age. We conclude that hypertrophic cardiomyopathy may represent a constellation of disease entities. The observed spontaneous resolution in 4 infants may represent a new form of the syndrome which carries a good prognosis.
Comparison of right ventricular volumes by transthoracic and transesophageal echocardiography in patients with right ventricular volume overload
Authors: Gamillscheg Andreas , Jin Zhen , Skinner Jonathan , Schulze-Neick Ingram , Nürnberg Jan-Hendrik , Berger Felix , Uhlemann Frank , Lange Peter E. 
Published: August 2008

AbstractAssessment of right ventricular volume and function is important in most congenital heart diseases before and after corrective or palliative surgery. Since transthoracic echocardiography is often substituted by transesophageal echocardiography in the perioperative setting, it is useful to compare transesophageal echocardiography with transthoracic echocardiography as performed preoperatively. We compared right ventricular volumes as calculated using these two methods from a four-chamber view in 21 children and adults with atrial septal defect. For right ventricular end-diastolic volumes of less than 70 ml, and end-systolic volumes of less than 40 ml, a close correlation was found between the techniques (r=0.99 and r=0.91, respectively), with a small degree of underestimation by transesophageal echocardio­graphy. For values larger than 70 nil and 40 ml, respectively, correlation decreased (r=0.41 for end-diastolic volumes and r=0.48 for end-systolic volumes) and underestimation of volume by transesophageal echocardiography increased. Underestimation of right ventricular end-diastolic volumes increased with increasing body surface area (r=0.74), and with progressive right ventricular enlargement (r=0.63). In patients with a body surface area of more than 1m2, the largest end-diastolic right ventricular length determined by transthoracic echocardiography was significantly longer than that derived by transesophageal echocardiography (p<0.001), whereas in smaller patients there was no significant difference between the two methods (p>0.1). If right ventricular volumes determined by transthoracic echocardiography using a four-chamber view are substituted by those obtained with transesophageal echocardiography in serial haemodynamic evaluation of patients with atrial septal defect, different correlation equations and, consequently, a different degree of underestimation by transesophageal echocardiography must be considered for large and small volumes. This increasing underestimation of larger right ventricular volumes seems to be based on foreshortening of the long cross-sectional axis of the right ventricle as seen in the transesophageal four-chamber view.
Anomalous origin of the right coronary artery from the pulmonary trunk, echocardiographic and colour Doppler features
Authors: Barlow Amanda J. , Sandor George G.S. , Le Blanc Jacques G. 
Published: August 2008

AbstractAnomalous origin of the right coronary artery from the pulmonary trunk is a rare congenital anomaly. Diagnosis is usually made at autopsy or incidentally in asymptomatic adults, but cardiopul-monary arrest and sudden death have been reported. The angiographic findings have been well described but the echocardiographic findings are less well documented.This report describes the echo and colour Doppler findings in a 3-year-old child with an anomalous right coronary artery arising from the pulmonary trunk, as well as observations on myocardial perfusion demonstrated by nuclear angiography.
Neonatal hypertrophic cardiomyopathy with duct-independent systemic blood flow
Authors: Suda Kenji , Horton Darlene P. , Silverman Norman H. 
Published: August 2008

AbstractWe describe a newborn who had echocardiogaphic features of severe hypertrophic cardiomyopathy and duct-dependent systemic blood flow. Blood flow to the upper body could be maintained only by prostaglandin El, as in the case of hypoplastic left heart syndrome. Forward flow was gradually established from the left ventricle, and the patient weaned from prostaglandin El. Although the patient's mother had poorly controlled diabetes, the patient at 24 months of age shows residual but lessened ventricular hypertrophy and increased cavity size.
Discordant atrioventricular and ventriculoarterial connections with partially unguarded left-sided atrioventricular orifice and aortic atresia
Authors: Freund Matthias , Magener Achim , Schmidt Klaus G. 
Published: August 2008

AbstractCongenitally unguarded morphologically tricuspid valvar orifice is a rare abnormality, usually associated with pulmonary atresia. We describe such a case found in association with discordant atrioventricular and ventriculoarterial connections in which the aortic valve was atretic. Presentation was with respiratory distress due to severe cardiac failure after birth. Echocardiography demonstrated a dilated systemic ventricle with poor function, left atrial enlargement, and aortic atresia. The clinical findings were confirmed at autopsy.
Infantile scimitar syndrome with severe pulmonary hypertension: successful treatment with coil embolization of the systemic arterial supply to the sequestered lung
Authors: Pfammatter Jean-Pierre , Luhmer Ingrid , Kallfelz Hans Carlo 
Published: August 2008

AbstractScimitar syndrome is a rare cardiopulmonary malformation. Presentation in infancy is usually associated with pulmonary hypertension and severe symptoms. We discuss treatment of such an infant. Two abnormal systemic vessels supplying the sequestered lower part of the right lung were embolized using catheter-inserted coils. The patent arterial duct was surgically ligated. These procedures resulted in a significant reduction of the shunt and the level of pulmonary hypertension, as well as in an impresssive improvement of symptoms.
Balloon angioplasty for isolated coarctation of the aorta with mirror image right aortic arch in an infant
Authors: Tsubata Shinichi , Ichida Fukiko , Miyawaki Toshio 
Published: August 2008

AbstractWe report the first infant case with coarctation of the aorta and mirror image right aortic arch, who presented with severe myocardial dysfunction and underwent successful balloon angioplasty for relief of coarctation, avoiding anesthetic and surgical risk. A follow-up cardiac cathetelization revealed no residual pressure gradient and continued angiographic improvement with excellent restoration in left ventricular function.
Left ventricular fibroma in two children
Authors: Joo Chan Uhng , Kim Kong Soo , Yoon Hyang Suck 
Published: August 2008

AbstractPrimary cardiac tumors are rare in infancy and childhood. We describe the features of large intramural ventricular cardiac fibromas discovered in two children. In the first, a left ventricular apical tumor was discovered by means of echocardiography and left ventriculography in a symptomless 8-year-old boy. The intramural fibroma was successfully resected. In the other case, an 8-month-old female infant with severe congestive heart failure, sudden ventricular tachycardia and fibrillation occurred during left ventriculography. At necropsy a tumor was seen bulging in the anterior wall of the left ventricle and in the ventricular septum. Histology showed the tumor to be a fibroma.
Congenitally corrected transposition presenting at 24 years: errors of management
Authors: Gerlis L. M. , Egdell R. , Somerville Jane 
Published: August 2008

AbstractA 24-year-old female without previous symptoms or known heart disease consulted a general practitioner with dyspnoea which had slowly worsened over a period of two years. She was treated for asthma, without investigations, until she presented with rapid atrial fibrillation, chest pain and pulmonary oedema necessitating admission to hospital, where she was found to have “mitral” regur-gitation (of the left-sided atrioventricular valve) and congenitally corrected transposition. At operation the grossly regurgitant left-sided morphologically tricuspid valve was replaced by a “top-hat” inverted aortic valve homograft. The procedure resulted in intermittent heart block, needing a pacemaker, and continued myocardial failure which progressed with new right-sided valvar regurgitation. She died a few months later. Post mortem examination confirmed the double discordance, and showed a fistula around the tricuspid valvar prosthesis, left atrial thrombus and changes in the mitral valve. There was also an unsuspected area of old myocardial ischaemia, associated with severe hypoplasia of the circumflex branch of the right-sided coronary artery. This was considered probably to have been of embolic origin.
Pulsed Doppler echocardiographic assessment of patterns of venous flow after the modified Fontan operation: potential clinical implications
Authors: Kaulitz Renate , Luhmer Ingrid , Kallfelz Hans Carlo 
Published: August 2008

AbstractTo assess the effect of a modified Fontan operation on systemic venous blood flow and the hepatic circulation, we compared 11 patients having an atriopulmonary connection and 35 with total cavopulmonary anastomosis. The Doppler echocardiographic study of the caval venous, hepatic venous and portal venous flow was performed so as to calculate the pulsatility ratio and the variation of flow with respiration. All patients had undergone cardiac catheterization. In addition, we included specific laboratory investigations to assess function of various organs.Significantly lower maximum velocities of flow at inspiration (0.31±0.12 rn/sec vs 0.45±0.14 m/sec) and expiration (0.23±0.09 rn/sec vs 0.32±0.11 m/sec), less pulsatility (0.43 vs 0.16) and a lower ratio of systolic to diastolic velocity (1.22 vs 1.85) were found in the patients having a cavopulmonary as compared to an atriopulmonary anastomosis. Peak velocities of hepatic venous flow during inspiration and expiration were significantly lower in those with a cavopulmonary anastornosis (p=O.OOl and p<O.OOl, respectively). In these patients, forward flow was extremely dependent on respiration, with decrease or cessation of antegrade flow during expiration in 22 patients. The velocity of portal venous flow was also significantly lower in these patients, although the pulsatility ratio did not differ significantly between the groups (0.5 ± 0.21 and 0.57 ± 0.23, respectively). The ratio of inspiratory and expiratory velocities showed no significant difference between the groups, nor was there any correlation between the pulsatility ratio of the venous vessels or the ratio of peak flow velocities during expiration and the mean systemic venous/right atrial pressure on postoperative cardiac catheterization. Hypoproteinemia was found in 8 patients after total cavopulmonary anastomosis; 9 of 10 patients with protein C deficiency belonged to this group.The dependence of hepatic venous flow on respiration in the presence of a chronically elevated systemic venous pressure in patients after the total cavopulmonary anastornosis may influence hepatic function in the postoperative period.
Hemolysis associated with coil occlusion of the arterial duct
Authors: Pedra Carlos A. C. , Pedra Simone F. , Font Valmir F. 
Published: August 2008

AbstractSevere mechanical hemolysis ocurred in a 22 month old girl after placement of a 38-5-10 coil in the arterial duct. Shé had previously undergone percutaneous closure using the Rashkind technique 14 months before insertion of the coil, but remained with a moderate residual shunt. Surgical removal of the devices and division of the duct were required to abolish the hemolysis.
Mobility of the flap valve of the primary atrial septum in the developing human fetus
Authors: Firpo Cora , Zielinsky Paulo 
Published: August 2008

AbstractIt has already been suggested that redundancy of the primary atrial septum could be associated with primary fetal arrhythmias. There are few reports, however, concerning the mobility of the primary septum and its behavior during fetal life. In this study, fifteen fetuses without anatomic heart disease were serially evaluated by fetal echocardiography. The time interval between three separate examinations was at least two weeks. A redundancy index was calculated as the ratio between the maximum excursion of the flap valve of the septum and the maximal left atrium as seen in a four chamber view. The indexes observed in the three examinations were: 0.55 ± 0.15, 0.48 ± 0.13 and 0.41 ± 0.13, respectively. Analysis of variance for normal distribution and Tukey's test were performed. A significant decrease (p < 0.05) in the redundancy index was observed with increasing gestational age. We postulate that this finding may be related to the decrease in frequency of premature atrial beats throughout fetal life.
Transoesophageal echocardiographic assessment of obstruction to the pulmonary venous pathway in children with Mustard or Senning repair
Authors: Hashmi Aijaz , Hosking Martin , Teixeira Otto , Cornel Gary , Duncan Walter 
Published: August 2008

AbstractThe morphology and mechanism of obstruction to the pulmonary venous pathway in patients following either Mustard or Senning repair of complete transposition was assessed using transoesophageal echocardiography. Seven patients underwent catheterization and complete transoesophageal study in both transverse and longitudinal planes, followed by balloon dilation of the obstructed venous pathway in five of seven under transoesophageal echocardiography guidance. A complete scan of both systemic and venous pathway was obtained in all patients. Four patients with a Mustard repair were found to have a ‘tubular’ baffle, with stenosis resulting from a discrete wedge of tissue arising from the atrial free wall in association with fibrous adhesions to the baffle. In the three patients with a Senning repair the intra-atrial baffle showed a characteristic ‘peaked’ appearance, with stenosis of the venous pathway stenosis related directly to con tracture of the patch used to augment the atrial free wall. The mechanism of obstruction appears to be inherent to the different surgical techniques. Indwelling transoesophageal echocardiography provided immediate haemodynamic and morphologic assessment of the efficacy of dilation of the obstructed venous pathway.