bluuebunny/crossref_metadata_2025_split huggingface dataset:
Balancing pulmonary and systemic arterial flows in parallel circulations: the value of monitoring system venous oxygen saturations
Authors: Riordan Christopher J. , Randsbaek Flemming , Storey John H. , Montgomery William D. , Santamore William P. , Austin Erle H. 
Published: August 2008

AbstractAccurate bedside assessment of the ratio of pulmonary to systemic flow (Qp/Qs ratio, referred to as “the flow ratio” or “the ratio”) plays an important role in the management of many congenital heart defects, especially the complexes unified by univentricular atrioventricular connections. Arterial oxygen saturation can be a misleading measure of the ratio, and may not reflect derangements until they are quite large. Theoretical analysis suggests that systemic venous oxygenation may be a better indicator of the ratio. To examine this, we created a widely patent atrial septal defect in neonatal piglets (weight =4–6.5 kg, n=6). Snares aruond the aorta and pulmonary trunk were adjusted to alter the flow ratio from 0.1 to 6.5. Venous oxygen saturations, measured in the mid-inferior caval vein, were at a maximum at a ratio about 1, and declined rapidly with increases or decreases in the ratio beyond a limited range. The venous oxygen saturation was found to vary much more than arterial oxygen saturation, with arterial oxygen saturation only falling when the ratio dropped below 0.5. Oxygen delivery (Oxygen Content x Cardiacoutput) was found to parallel closely systemic venous oxygen saturation, and was at a maximum at the same ratio that produced a maximum value of systemic venous oxygen saturation. The study suggests that systemic venous oxygen saturation provides a better estimate than does systemic arterial oxygen saturationof the flow ratio and oxygen delivery. Interventions that maximize systemic venous oxygen saturation should maximize oxygen delivery, and determination of systemic venous oxygen saturation should be a helpful addition in managing children with a number of congenital heart defects.
Pulmonary atresia with intact ventricular septum: management of the coronary arterial anomalies
Authors: Dyamenahalli Umesh , Hanna Brian D. , Sharratt Geoffrey P. 
Published: August 2008

AbstractOver a period of 12 years, we encountered 30 cases of pulmonary atresia with intact ventricular septum. The overall mortality was 53% (16 patients). Coronary arterial abnormalities were detected in 16 patients and, in 10 of these, the coronary arterial circulation was partially or totally dependent on the right ventricle. The mortality in this group was 80%. The strategies for management are reviewed in 6 cases to try to define the best approach to diagnosis, and to assess the benefits of the various modesof treatment used for specific coronary arterial anomalies. Two cases (2 and 3) underwent occlusion of a fistula from the right ventricle to a coronary artery using embolization coils. This modality is considered to be appropriate and useful when the coronary circulation supplied by the fistula is not dependent on flow from the right ventricle. The dependency of the coronary arterial circulation on the right ventricle was extensive in two cases (Case 4 and 6), and complete in one because of atresia of the orifices of the coronary arteries (Case 5). These three patients died. Cardiac transplantation is suggested as the most appropriate treatment in this group. In two cases (1 and 6), there was a supernumerary vessel connecting the right ventricle to the pulmonary trunk. These connections do not appear to be of therapeutic concern, as they do not seem to contribute to myocardial blood supply. The selection of the most appropriate protocol for management of patients with coronary arterial anomalies depends upon a thorough and aggressive approach to investigation.
Successful dilation of superior cavel venous obstruction following repair of totally anomalous pulmonary venous connection
Authors: Shrivastava S. , Radhakrishnan S. , Chandra Y. Vijaya 
Published: August 2008

AbstractWe describe post azygos obstruction of the superior caval vein following repair of a mixed variety of totally anomalous pulmonary venous connection. Balloon dilation was immediately successful, and has provided persistent relief at a follow-up of 20 months.
Absent pulmonary valve in tricuspid atresia with left ventricular outflow tract obstruction and patent arterial duct
Authors: Lemke Robert P. , Giddins Neils G. , Odim Jonah N.K. 
Published: August 2008

AbstractWe describe a neonate with tricuspid atresia, absent pulmonary valve, right ventricular hypoplasia, small venrricular septal defect, left ventricular ourflow tract obstruction and a patent arterial duct. These finding were diagnosed by echocardiography and confirmed by cardiac catherterization and postmortem examination. This is a unique report of a functionally single ventricle variant of absent pulmonary valve syndrome and biventricular outlet obstuction.
Sequential segmental analysis - description and categorization for the millennium
Authors: Anderson Robert H. , Yen Ho Siew 
Published: August 2008

AbstractThe concept of sequential analysis of congenitally malformed hearts is now widely accepted, although some aspects remain controversial. In this review, we analyse the evolution of the scgmental approach, showing how our own concepts of analysis have chaanged little since our initial description, heart dicrates the presence of the atrial, ventriclar, and arterial segments, Analysis depends upon recognizing the morphologic differences of the components within each segment, and then how these parts are joined together across the atrioventicular and ventriculo-arterial Junctions. The distinction of chambers and arterial trunks follows the principle of the morphologic method, namely that scructures be identified on the basis of their own intrinsic morphology, and not according to other features which themselves might be variable. Differentiation of junctional arrangements as opposed to segmental stucture resolves ongoing controversics such as the nature of “univentricular hearts”.Analysis in the fashion proposed shows that there is nothing intrinsically complicated with malformed hearts.
Morphology of hearts undergoing Fontan repair
Authors: Murari Vivek , Sharma Rajesh , Airan Balram , Bhan Anil , Choudhary Shiv K. , Saxena Anita , Kothari Shyam S. , Reddy Subhash C. B. , Venugopal Panangipalli 
Published: August 2008

AbstractBackgroundNotwithstanding the controversies evoked by the term “single ventricle”, most patients with this condition would undergo the Fontan procedure. In addition, there is a large group of patients in whom a biventricular repair would be abandoned in favour of a univentricular one because of the presence of unfavourable morphologic features. There is a need for a uniformly acceptable system of nomenclature that would permit precise description and classification of hearts with complex malformations to facilitate reporting and help in understanding the reasons for choosing a univentricular repair.MethodsEchocardiographic, angiographic and operative records of 240 patients undergoing the Fontan procedure were analysed.ResultsOut of the 104 patients with univentricular atrioventricular connections, 2 ventricles were discernible in all but 3 patients. A Fontan repair was performed in 136 patients with biventricular atrioventricular connections because of the presence of a hypoplastic ventricle in 52 patients and a non-committed ventricular septal defect in the remaining 84.ConclusionsThe Fontan operation is probably the only definitive treatment option for patients with univentricular atrioventricular connections. The decision to perform a univentricular repair in preference to a biventricular one in hearts with biventricular atrioventricular connections is based on the presence of a hypoplastic ventricle or a non-reroutable ventricular septal defect. This decision is subjective. In hearts with discordant atrioventricular connections and pulmonary stenosis, we prefer the Fontan operation to the classical repair.
Anomalies of the aortic arch and arterial duct induced by small doses of bis-diamine in fetal rats
Authors: Momma Kazuo , Ando Masahiko 
Published: August 2008

AbstractDeletion of chromosome 22 and DiGeorge syndrome are associated with inappropriate development of the neural crest and abnormalities of the ventricular outflow tracts. Bis-diamine induces comparable cardiac anomalies by inhibiting the normal development of the neural crest. In order to clarify the effect of damage on the development of the neural crest and its relation to the cardiac anomalies, the effects of giving a small dose of bis-diamine were compared with the results of a large dose of bis-diamine. A small dose of bis-diamine (20 mg), therefore, was administered to 40 pregnant rats on the 10th day of pregnancy, and 330 fetal hearts were studied on the 21st day with the rapid whole-body freezing method. Intracardiac anomalies were rare, being found in only 55 fetuses. The anomalies discovered were: aberrant origin of the subclavian artery in 88 fetuses, right aortic arch in 26 fetuses, interruption of the aortic arch in 23 fetuses, and high location of the aortic arch in 8 fetuses. Anomalies of the arterial duct were also common; with a vascular ring formed by the aortic arch and contra-lateral duct being seen in 21 fetuses, isolation of the right subclavian artery associated with either bilateral ducts (9 fetuses) or a right duct and absent left duct (9 fetuses), absent duct with tetralogy of Fallot in 7 fetuses, and with common arterial trunk in one fetus. In conclusion, a small dose of bis-diamine induced extra cardiac anomalies of the aortic arch and arterial duct, indicating a greater susceptibility of the aortic arch and the duct to inappropriate formation of the neural crest cells in the early developing embryo. Clinically, these anomalies of the aortic arch and the duct should be searched for in all patients suspected of having deletion of their 22nd chromosome.
The spectrum of pulmonary input impedance in children with complete transposition after the arterial switch operation
Authors: Murakami Tomoaki , Nakanishi Toshio , Nakazawa Makoto , Momma Kazuo , Imai Yasuharu 
Published: August 2008

AbstractWe evaluated the physical characteristics of the pulmonary trunk in 32 patients with complete transposition after the arterial switch operation, calculating the pulmonary input impedance. In 6 of the patients, the characteristic impedance was equal to, or less than, the value in patients with ventricular septal defect. In those patients with an intact ventricular septum, or those with a ventricular septal defect who had undergone the arterial switch operation or banding of the pulmonary trunk before 3 months age, the frequency of the first modulus minimum also agreed with the values obtained in patients with ventricular septal defect. The value was high, however, in patients with complete transposition with ventricular septal defect who had undergone an arterial switch operation or banding of the pulmonary trunk after 4 months of age. Our data suggest decreased distensibility of the proximal pulmonary trunk after the arterial switch operation, and mild changes in the pulmonary vasculature in patients with complete transposition with ventricular septal defect who had undergone either an arterial switch operation or banding of the pulmonary trunk after 4 months of age. Long-term follow-up is needed clarify whether or not these features affect right ventricular function by means of an increase in afterload.
Balloon dilation for postoperative pulmonary stenosis following surgical creation of an intrapulmonary coronary arterial tunnel
Authors: Mori Yoshiki , Nakanishi Toshio , Nakazawa Makoto , Momma Kazuo , Imai Yasuharu 
Published: August 2008

AbstractBalloon dilation was performed in four patients with postoperative pulmonary stenosis who had undergone surgical creation of a coronary arterial tunnel in the pulmonary trunk. Two patients had complete transposition in whom the arterial switch operation had been performed using the modified Aubert method. The other two patients had anomalous origin of the left coronary artery from the pulmonary trunk treated with the Takeuchi procedure. Balloon dilation was performed at 11 locations. The pressure gradient decreased from 48 ± 22 to 24 ± 14mmHg (p<0.01), and the diameter of the narrowest segment increased from 5.3 ± 2.5 to 7.5 ± 2.8mm (p<0.01), respectively. Of the 11 procedures, 8 (73%) were judged successful with use of the criterion of success as a greater than 50% decrease in pressure gradient, and/or a greater than 50% increase in diameter. The inflated balloon must have compressed the coronary arterial tunnel in the pulmonary trunk, but there was no apparent myocardial damage in any patient, although transient and mild ST-T changes appeared on electrocardiographic monitoring during the procedure in 2 patients. Rupture of the wall of the pulmonary trunk occurred in two patients, one of whom required elective surgical intervention. These data suggest that balloon dilation should be performed with caution for management of postoperative pulmonary arterial stenosis in patients with a surgically created intrapulmonary coronary arterial tunnel, since tearing the wall of the pulmonary trunk may occur.
Factors that predict epinephrine induced constriction of the persistent arterial duct
Authors: Tomita Hideshi , Fuse Shigeto , Chiba Shunzo 
Published: August 2008

SummaryWe studied factors that predict in children catecholamine induced vasoconstriction of the persistent arterial duct. Epinephrine at 2.0–10.0 (4.9 ± 1.3, mean ± standard deviation) μg/kg was injected intravenously into 30 patients with persistent arterial duct whose age ranged from 1 to 91 (27 ± 26) months. In 11 patients aged 10–66 (34 ± 23) months (responders), a continuous murmur had almost completely disappeared on auscultation, and both the diameter and the shunt flow area of the arterial duct had become smaller as shown by Doppler echocardiography. On the other hand, there was no such change in 19 patients aged 1–91 (23 ± 27) months (non-responders). Although there was no significant difference in the age, the body weight, the minimal diameter of the arterial duct, or the doses of epinephrine between responders and non-responders, the gestational age at birth was slightly less (p=0.09) and the birth weight was significantly smaller in responders than in non-responders (p<0.05). The persistent arterial duct of those who had a history of low birth weight always responded to epinephrine. In infants younger than 6 months, the persistent arterial duct was never constricted by epinephrine regardless of the birth weight and gestational age. In low birth weight infants, the vascular smooth muscle of the persistent arterial duct can usually constrict beyond infancy. There may be some age dependent difference in responsiveness to epinephrine until 6 months of age.
Chylothorax after surgery for congenital heart disease in children
Authors: Pedra Simone Rolim Fernandes Fontes , Pedra Carlos Augusto Cardoso , Fontes Valmir Fernandes , Santana Maria Virgínia Tavares , Paulista Paulo Paredes 
Published: August 2008

AbstractWe reviewed retrospectively the clinical and surgical data from 9 children with postoperative chylothorax secondary to cardiac surgery for congenital heart disease seen over a 3 year period. Mean age was 26 months and 6 patients were female. The procedures performed were 2 Blalock-Taussig shunts, 6 Bidirectional Glenn anastomoses and 1 modified Fontan procedure. Diagnosis of chylothorax was made by classical means. The overall incidence of chylothorax was 0.8%, with the relative incidences being 2.5% for Blalock-Taussig, 2.7% for Fontan, and 11.7% for the Glenn procedures. All patients were initially treated conservatively with chest tube drainage and hypercaloric diet suplemented with medium chain triglycerides. Parenteral nutrition was needed in 2 patients, one because of sepsis and the other because of progressive malnourishment. High output fistulas and long periods of drainage were noted after cavo-pulmonary procedures. Up to 12% weight loss was observed in 6 patients, one patient was successfully treated of pneumonia and another died ofPseudomonas aeruginosasepsis. Two patients required chemical pleurodesis because of unabated drainage, with the others being successfully treated conservatively. Chylothorax is a common complication after bidirectional Glenn procedures, and is associated with considerable morbidity and mortality. Earlier operative intervention may be required in these patients. Pleurodesis is a simple, safe, and effective procedure to stop chylous flow.
Beneficial effect of an oral analog of prostacyclin on pulmonary hypertension secondary to congenital heart disease
Authors: Ichida Fukiko , Uese Kei-ichiro , Tsubata Shin-ichi , Hashimoto Ikuo , Hamamichi Yuji , Fukahara Kazuaki , Murakami Arata , Miyawaki Toshio 
Published: August 2008

AbstractTo determine whether a newly synthesized oral analog of prostacyclin, called beraprost sodium, could cause pulmonary vasodilation, we studied its hemodynamic effect on pulmonary hypertension of children, comparing it to other vasodilatory agents, such as nitric oxide and tolazoline. We studied 20 children (mean age 24 months) having pulmonary hypertension secondary to congenital heart disease. A single oral dose of beraprost sodium resulted in an appreciable reduction of pulmonary vascular resistance (mean 34%), which was comparable to that induced by inhalation of nitric oxide and intravenous delivery of tolazoline (mean 41% and 31%, respectively). The results suggest that beraprost sodium may serve as a novel and safe vasodilator for the screening of pulmonary vasoreactivity, as well as the treatment of pulmonary hypertension in children.
Formation of thrombus and thromboembolism after the bidirectional Glenn anastomosis, total cavopulmonary connection and the Fontan operation
Authors: Hanséus Katarina , Björkhem Gudrun , Jögi Peeter , Sonesson Sven-Erik 
Published: August 2008

AbstractAlthough patients undergoing surgery with the Fontan procedure or its modifications are increasingly recognised to be at risk for thromboembolism, further knowledge is needed to minimise this complication and its sequels. To address this issue, we reviewed 100 patients operated with the Fontan procedure, the bidirectional Glenn anastomosis and/or the total cavopulmonary connection to describe our incidence and clinical characteristics of postoperative formation of thrombus. Symptomatic thrombosis or cerebrovascular accidents were found in 5 patients. Asymptomatic thrombus were found in another 5 patients. Three patients had venous thrombi. In 6 patients the thrombosis was found on the arterial side. In all these cases, the thrombus was located in the stump of the divided pulmonary trunk. In 3 of these patients, sudden onset of hemiparesis preceded the diagnosis of the thrombus while the remaining 3 patients were asymptomatic. In 3 cases, the formation of thrombus in the stump of the divided pulmonary trunk occurred after a bidirectional Glenn anastomosis. One patient developed severe neurological symptoms 2 months after a total cavopulmonary connection. No thrombus was found, but the patient had a small right-to-left shunt and embolization could not be excluded. The incidence of thrombosis after Fontan-type surgery in this study is 10%. Although not all episodes of thrombosis are symptomatic, there is a significant risk of severe sequels due to embolization to the pulmonary or cerebral circulations. The stump of the divided pulmonary trunk is one of the main sites for intracardiac formation of thrombus, even after a bidirectional Glenn anastomosis.
Surgical repair of interrupted aortic arch with ventricular septal defect
Authors: Allan Lindsey D , Apfel Howard D , Levenbrown Yosef , Quaegebeur Jan M 
Published: August 2008

AbstractBackgroundInterrupted aortic arch is often associated with subaortic narrowing and hypoplasia of the aortic orifice. The best surgical strategy for the management of these additional lesions is a matter of current debate.Methods and ResultsBetween 1986 and 1996, 19 patients underwent repair of interrupted aortic arch with closure of ventricular septal defect in a single stage, with no attempt at subaortic resection, irrespective of the dimensions of the left ventricular outflow tract. There was no perioperative hospital mortality, and all patients were alive at 1 year. Follow-up ranges from 0.75 −10 years, with a mean 4.2 ± 3.0 years. Seven patients (37%) have required reintervention for relief of subaortic stenosis, 2 of whom have died.ConclusionsOur results suggest that primary one-stage biventricular repair can be accomplished with low perioperative mortality without addressing the subaortic region. Further long-term follow-up will determine whether this is accomplished at the expense of later morbidity and mortality.
Cavo-pulmonary anastomosis excluding the portal venous return: an alternative for patients with protein-losing enteropathy secondary to the Fontan procedure
Authors: Barbero-Marcial Miguel , Riso Arlindo , Atik Edmar , Tanamati Carla , Reis Humberto , Sobrinho Veloso , Jatene Adib 
Published: August 2008

AbstractSevere protein losing enteropathy occurred in 3 of our 128 patients undergoing the Fontan procedure in the period of 1982 to 1994. The complication occurred 4, 4.5 and 5 years after the procedure, respectively. One patient had double inlet left ventricle while the other two had tricuspid atresia. All had initially undergone an atrio-pulmonary connection, and the duration of the enteropathy was 12, 13,5 and 15 months. All patients were in functional class IV of the New York Heart Association. The presence of protein losing enteropathy was confirmed by determining levels of fecal alfa-1 antitrypsin, enteric clearance of 51 Cromo labeled albumin, and endoscopy with histopathological study of the small bowel.They underwent further surgery to convert the atrio-pulmonary to a cavo-pulmonary anastomosis which excluded all the hepatic veins. A bovine pericardial baffle was sutured in the inferior caval vein so as to leave the hepatic venous flow draining to the right atrium and thence through an atrial septal defect to the left atrium and systemic circulation. The inferior caval venous flow was directed through an intra-atrial tunnel to the right pulmonary artery, and the superior caval vein was anastomosed to the superior aspect of the right pulmonary artery, thus completing the total cavo-pulmonary anastomosis with exclusion of the portal venous flow.Postoperatively, there was total regression of the clinical and laboratory findings. All patients are now in functional class I or II of the New York Heart Association. They have mild to moderate cyanosis up to three and a half years from the reoperation. We recommend the technique as an alternative for patients with severe protein losing enteropathy occurring after the Fontan procedure.