bluuebunny/crossref_metadata_2025_split huggingface dataset:
Arterial supply to the lungs in tetralogy of Fallot with pulmonary atresia or critical pulmonary stenosis
Authors: Yen Ho Siew , Catani Gualtiero , Seo Jeong-Wook 
Published: August 2008

SummaryThe arterial supply to the lungs in cases with tetralogy of Fallot with pulmonary atresia or critical pulmonary stenosis is mostly via the arterial duct or else by collateral arteries arising directly or indirectly from the aorta. Nine anatomical specimens with collateral arteries were studied by blunt dissection to determine the arterial supply to each pulmonary segment. The precise supply varied from case to case but, overall, nearly two-thirds of the pulmonary segments were connected to the central pulmonary arteries. A quarter were supplied exclusively by systemic-to-pulmonary collateral arteries, with the remainder having a dual supply. Coexistence of ductal connections and systemic-to-pulmonary collateral arteries was seen in one case. Anastomoses between vessels of different origins were observed up to the segmental level in eight of the nine cases. In four cases, the anastomoses formed arterial rings. The extent of pulmonary parenchyma supplied by arteries from different sources and the presence of arterial anastomoses in these malformations have implications on the planning of surgical repair.
Kawasaki disease—sudden death as the first presenting symptom
Authors: Chow Louis Tsun-cheung , Chow Wing-hing , Tse Christopher Chun-hing , Wong Eric Hon-man , Wong Koon-sang , Yip David Chi-pang 
Published: August 2008

SummaryTwo patients are described with sudden death as the first presenting symptom of Kawasaki disease. The first patient died of cardiac tamponade as a result of rupture of a coronary arterial aneurysm at the age of three months and nineteen days. The second patient, one and half years old, developed acute myocardial infarction secondary to coronary arterial aneurysm and thrombosis. The true nature of their underlying disease was realized only after postmortem examination. Their clinical signs and symptoms of Kawasaki disease might be so mild as to escape recognition. A high index of suspicion should be exercised by the clinician in order not to miss this condition, especially in places where Kawasaki disease is uncommon.
The early response of the systemic ventricle during transition to the Fontan circulation—an acute hypertrophic cardiomyopathy?
Authors: Penny Daniel J. , Lincoln Christopher , Shore Darryl F. , Xiao Han B. , Rigby Michael L. , Redington Andrew N. 
Published: August 2008

SummaryUsing combined epicardial echocardiography and high-fidelity intraventricular pressure recordings, the acute effects of transition to a Fontan circulation were studied in 10 patients. Measurements were made before and after cardiopulmonary bypass. The Fontan operation had no significant effect on load-dependent indices, or on a load independent index (Vmax) of ventricular systolic function. Large changes were observed in ventricular geometry. Maximum and minimum cavity dimensions were reduced, while maximum and minimum thicknesses of the posterior wall were increased.While simultaneous Doppler/intraventricular pressure measurements suggested that the compliance of the ventricle was unchanged, the pattern of atrioventricular flow changed from predominantly early diastolic (E wave) to predominantly atrial systolic (A wave) as a result of surgery in four patients. The time constant of ventricular relaxation was prolonged in all patients and five developed Doppler echocardiographic evidence of incoordinate relaxation of the ventricle, with intraventricular flow occurring during isovolumic relaxation. Thus, the Fontan operation causes acute hypertrophy of the systemic ventricle due to a sudden reduction in ventricular preload in the presence of a maintained shortening fraction. While global ventricular diastolic compliance may be unchanged by the operative procedure, filling of the ventricle during early diastole is altered as a result of incoordinate relaxation and a prolonged time constant of ventricular relaxation.
Preparation of the “pulmonary ventricle” for arterial switch by adjustable intravascular balloon outflow obstruction—an experimental approach in a lamb model
Authors: Bonhoeffer Philipp , Henry G William , Katayama Hiroshi , Krzeski Robert , Carminati Mario , Crupi Giancarlo , Parenzan Lucio 
Published: August 2008

AbstractSurgical banding of the pulmonary artery in patients with transposition of the great arteries leads to a rapid increase of the left ventricular mass. The purpose of this animal experiment was to induce ventricular systolic hypertension in the “subpulmonary ventriclerdquo; by intravascular obstruction to pulmonary flow. The first experiment was performed in an anesthetized animal with an open thorax. A balloon-tipped catheter was introduced into the femoral vein and advanced to the pulmonary artery. The balloon was positioned in the pulmonary trunk and was progressively inflated until a systemic pressure was achieved in the “pulmonary ventricle”. This was accomplished within 40 minutes without a significant reduction in cardiac output, which was continuously monitored by an electromagnetic flowprobe. In the second experiment an analogous procedure was performed in a closed chest, awake, spontaneously breathing animal. The catheter was maintained in proper place for 24 hours. Subsequent macroscopic examination of the heart demonstrated a remarkable increase in the ventricular mass. The results of this preliminary experiment demonstrate the possibility for creating “intraluminal pulmonary banding”. The potential advantages of this technique are the nonsurgical approach and the ability to adjust the grade of obstruction to pulmonary flow.
Obstructing balloon catheter for the induction of systolic hypertension in the pulmonary ventricle—an acute hemodynamic study in the piglet
Authors: Krzeski Robert , Katayama Hiroshi , Bonhoeffer Philipp , Lucas Carol L. , Long Walker A. , Parenzan Lucio , Henry G. William 
Published: August 2008

AbstractThe use of an obstructing balloon catheter positioned in the outflow tract of the pulmonary ventricle may be an alternative method for the adaptation of the left ventricle to an increased afterload prior to an arterial switch procedure in patients with complete transposition requiring a two-stage approach. We tested this approach in an acute hemodynamic study in a piglet model. Seven piglets were anesthetized with halothane and ventilated at FiO21.0. Through a median sternotomy, the great vessels were exposed. The right ventricular pressure and the aortic blood pressure were recorded using microtip manometers, cardiac output was measured by an electromagnetic flow probe on the ascending aorta. A 6F or 7F Berman angiographic catheter was placed in the lumen of the pulmonary artery and the balloon inflated to decrease cardiac output no more than 30%. The hemodynamic changes observed after balloon inflation included an 100% increase in systolic right ventricular pressure (control vs balloon inflation (mean±SE) 20.4 ± 1.5 mm Hg vs 46.6 ± 4 mm Hg, p<0.001), a slight reduction in systolic aortic pressure (75.5 ± 3.8 mm Hg vs 65.0 ± 5.8 mm Hg; p<0.05), and a reduction in cardiac output by 30% (1.1 ± 0.081/min vs 0.8 ±0.08 1/min, p<0.01). The systolic right ventricular to aortic pressure ratio was increased from 0.27 ± 0.02 to 0.69 ± 0.08, p<0.001. This study shows that a balloon catheter may be useful in creating systolic hypertension in the pulmonary ventricle in patients with complete transposition requiring a two-stage approach.
Concealed pulmonary venous obstruction in right atrial isomerism with pulmonary outflow tract obstruction—surgical management following modified Blalock-Taussig shunt
Authors: Chiu Ing-Sh , Wang Nan-Koong , Wu Mei-Hwan , Wu Fen-Fen , Hung Chi-Ren 
Published: August 2008

SummaryObstruction to the pulmonary venous return is a frequent associated anomaly in patients with isomerism of the right atrial appendages. Yet, preoperative diagnosis by means of either cross-sectional echocardiography or cardiac catheterization can be intriguing. Indeed, the presence of two morphologically right lungs reduce considerably the size of window for precordial echocardiography. Also, in the presence of severe pulmonary stenosis or atresia, it can be difficult at cardiac catheterization to enter the pulmonary trunk. In these patients, construction of a systemic-to-pulmonary artery anastomosis will almost inevitably result in pulmonary edema. Between May 1984 and December 1988, five patients with isomerism of the right atrial appendages, severely decreased pulmonary blood flow and concealed obstruction to the pulmonary venous return were admitted to our hospital. A modified Blalock Taussig shunt by interposition of a polytetrafluoroethylene prosthesis was performed in each patients and all of them developed pulmonary edema. Three patients died despite appropriate medical treatment. The remaining two patients were successfully treated by banding of the Blalock shunt. This was performed in the first patient at the time of the initial surgery, when prior to closure of chest, pulmonary edema became manifest. The second patient who developed pulmonary edema early postoperatively, underwent cardiac catheterization to confirm the clinical diagnosis of obstruction to the pulmonary venous return. Reduction of blood flow through the Blalock shunt with resolution of edema was initially achieved by means of a partially occluding balloon catheter. Pulmonary edema recurred one week later because of rupture of the balloon and the patient eventually underwent a successful banding of the Blalock shunt through a left thoracotomy. We conclude that preoperative assessment of the pulmonary venous return is mandatory in patients with right isomerism and reduced pulmonary blood flow. Construction of a modified Blalock-Taussig shunt in the presence of concealed obstruction to the pulmonary venous return will almost inevitably cause pulmonary edema. Banding of the Blalock shunt can be successful, as observed in our experience, for the management of this serious complication.
Successful radiofrequency catheter ablation of medically resistant reentrant supraventricular tachycardia in an infant
Authors: Case Christopher L. , Gillette Paul C. , Shuler C. Osborne 
Published: August 2008

SummaryThe feasibility and efficacy of radiofrequency catheter ablation of medically resistant supraventricular tachycardia is a promising technique with applications in the pediatric age group. The limitations of this procedure in the pediatric population, however, have yet to be delineated. We herein report a case of incessant reentrant supraventricular tachycardia in a two month old infant which was resistant to pharmacologic therapy, yet successfully treated by radiofrequency catheter ablation.
Flow visualization in anatomically accurate, flow-through models of the main pulmonary artery trunk
Authors: Carroll Sheri L. , Katayama Hiroshi , Henry G. William , Ferreiro Jose I. , Zalesak Rudy , Ha Belinda , Lucas Carol L. , Singh Megha , Lynch Patricia G. , Yoganathan Ajit P. 
Published: August 2008

AbstractTo study the effect of maturational geometric changes on flow characteristics in the pulmonary artery trunk, anatomically accurate, acrylic flow-through models were constructed from four flexible silicone rubber casts obtained in situ in lambs weighing 2.4, 7.8, 9.5, and 11.5 kg. A silicone rubber cast of the right heart was fabricated by injecting the superior caval vein in situ with liquid silicone rubber (Dow Corning's HS-II RTV, Midland, MI). Each cast was used as a template for a transparent acrylic mold of the pulmonary artery trunk and primary generation branches. The acrylic block was then fitted with a curved rigid Plexiglass inflow tube (to simulate the curvature of the right ventricle) just proximal to the pulmonary valve sinuses and mounted in a closed loop system driven by a variable speed pulsatile pump (to simulate physiological flow rates between 0.5 and 4.0 lmin−1) A blood analog solution of polystyrene beads (Rohm & Haas Amberlite, Philadelphia, PA) suspended in a 45% by weight glycerine solution was illuminated by a laser source (15 mwatts, Siemens, Germany) to trace the flow patterns. Two flow field planes of the main pulmonary artery trunk—one parallel, and one perpendicular, to the origins of the right and left arterial branches—were visualized and video recorded (Canon H660 8mm, Japan) for subsequent analysis. A prominent vortex, originating in the center of the main pulmonary artery and directed inferiorly toward the inner wall, was noted in the flow field plane perpendicular to the bifurcation in the 9.5 and 11.5 models. These characteristics were less developed in the 7.8 kg model and not present in the 2.4 kg model, possibly because the angle of curvature was less acute than in the larger models. In the flow field plane parallel to the bifurcation, the patterns were more complex, principally influenced by turbulence in the main pulmonary artery (which increased at higher flow rates) and the geometric changes in the branch pulmonary arteries.
Pulmonary vascular remodeling in rats with unilaterally banded lobar pulmonary veins
Authors: Cottrill Carol M. , O'Connor William N. , Fitz Robert , Gillespie Mark N. 
Published: August 2008

AbstractAlthough pulmonary vascular disease occurs in association with pulmonary venous obstruction, a model of pulmonary vascular lesions in this setting in small laboratory animals has yet to be described. The objective of this study was to determine if unilateral banding of pulmonary veins in the rat would produce pulmonary vascular disease that mimicked key aspects of the human disorder. Rats were anesthetized and, after mechanical ventilation and thoracotomy, one of the four pulmonary veins was banded to reduce its external diameter by approximately 65–75% to 0.8 mm. Additional animals, sham-operated controls, were treated identically except for venous banding. Mortality did not differ between groups and was less than 20%. Eight weeks after surgical preparation, moderate pulmonary hypertension was demonstrated with a mean pressure in the pulmonary artery of 24.9 mm Hg (range 19.25 – 30.0) in the banded group versus 18.7 mm Hg (range 16.6 – 19.3) for sham-operated animals. Pulmonary cineangiography in rats with banded pulmonary veins demonstrated marked pulmonary congestion and a prolonged residence time of contrast medium in the capillary circulation of the lung region subjected to banding. Angiographically, non-banded lungs were similar to shams. Histopathology of the vein-banded region revealed venous congestion, arterialization of veins, perivenular edema, and sparse to moderate inflammation. Prominent bronchial vessels, pulmonary arterial medial and occasional intimal thickening, and periarterial inflammation were also observed in banded regions. Similar inflammation around pulmonary arterial and pulmonary venous vessels was noted in contralateral lung lobes but not in sham-operated control rats. Morphometric evaluation of all muscular pulmonary arteries from 50–200μ external diameter accompanying airways to the level of terminal and respiratory bronchioles indicated that the medial area in lung regions subjected to venous banding was over three times greater than in shams. Pulmonary arteries from the contralateral, unbanded lungs of treated animals also exhibited a two-fold increase in medial area. These findings indicate that a modest degree of pulmonary venous banding in rats produces congestion and causes alterations in lung vessels which are reminiscent of those observed in humans with pulmonary venous outflow obstruction. Such a model in a cost-effective laboratory animal should be useful for delineating the specific mechanisms underlying these alterations.
Prognosis in asplenia syndrome—a population-based review
Authors: Webber Steven A. , Sandor George G.S. , Patterson Michael W.H. , Taylor Glenn P. , Wadsworth Louis D. , LeBlanc Jacques G. 
Published: August 2008

SummaryThe prognosis for the fetus or neonate with asplenia syndrome is unknown as population-based data are unavailable. Factors influencing survival in this syndrome were, therefore, assessed for the province of British Columbia by retrospective review of all cases diagnosed between 1970 and 1990. Particular attention was paid to the prognostic influence of palliative surgery, sepsis, extracardiac malformations, gastrointestinal disease, arrhythmias and sudden death. We identified 43 cases of asplenia syndrome, of which 40 were live-born (incidence 1/22,000 live‐births). Only one spontaneous death was noted during fetal life amongst approximately 5,000 fetal autopsies. Five cases had been diagnosed by fetal echocardiography, two of these pregnancies being terminated. Of the live-born infants, 28 (70%) underwent active management of their cardiac anomalies and of these, 14 are alive. Since 1980, 26 systemic-to‐pulmonary shunts have been constructed in 17 children with two operative deaths. Three children have had successful Fontan or bidirectional Glenn procedures. Systemic sepsis occurred in three children with no deaths. Three neonates underwent surgery for obstruction of the upper gastrointestinal tract without complication. Late sudden death occurred in four children with systemic-to-pulmonary shunts, none of whom had documented arrhythmias. Major extracardiac malformations were present in nine cases (21%), these contributing to death in two. Overall survival for all live-born infants is 35%. Sepsis and palliative surgery are no longer the major causes of death in asplenia syndrome, the current challenges being prevention of sudden death and the complexity of surgical repair of the cardiac malformations.
Extracardiac malformations in asplenia syndrome
Authors: Webber Steven A. , Taylor Glenn P. , Colwell Kim , Sandor George G.S. , Patterson Michael W.H. 
Published: August 2008

SummaryThe incidence and spectrum of extracardiac malformations in congenital asplenia syndrome were determined by review of43 cases (40 live-births) identified in the province of British Columbia over a 21 year period. Major extracardiac anomalies (excluding those of abnormal arrangement of the organs) were identified in nine cases (21%), and minor anomalies were present in an additional twelve infants (28%). Extracardiac anomalies were associated with one spontaneous stillbirth, one termination of pregnancy, and two early neonatal deaths. In an additional four newborns, they contributed to a decision not to palliate the complex cardiac anomalies. Gastrointestinal malformations led to neonatal intestinal obstruction in four infants. Of the 14 surviving children, one is severely handicapped due to a congenital anomaly of the central nervous system. The clinical significance of extracardiac malformations and their developmental implications are discussed.
Clinical course of atrial septal defect in children
Authors: Han Ling , Ettedgui José A. , Zuberbuhier James R. 
Published: August 2008

AbstractThe incidence of spontaneous closure of uncomplicated defects of the oval fossa was determined in cohort of 498 children. Surgical repair was accomplished in 428 at a median age of 4.5 years. Of the 70 who did not undergo surgery, 20 showed spontaneous closure or a decrease in size of the defect. The defect was unchanged in the other 50. The age at diagnosis, pulmonary-to-systemic flow ratio and size of the defect were not statistically significantly different in the changed and unchanged groups, and there was no difference in prevalence of symptoms, the triad of systolic and diastolic murmurs and abnormal splitting of the second sound, or of right ventricular enlargement by echocardiography. The rate of spontaneous diminution in size or actual closure of a defect in this series was 4%, and no clinical predictor of change in the size of a defect was identified. Thus, delay in repair of a large flow atrial septal defect in the hope of spontaneous closure is not justified, whether or not symptoms are present.
Pulmonary valvar involvement in rheumatic heart disease—a study of 100 post-mortem cases
Authors: Buendía Alfonso , Attie Fause , Zabal Carlos , Calderón-Colmenero Juan , Aranda Alberto , Reyes Pedro 
Published: August 2008

SummaryWe reviewed 100 postmortems of patients with pulmonary valvar involvement due to rheumatic heart disease. The patients were divided in two groups: the first group included 59 cases with acute rheumatic lesions of the pulmonary valve. At the time of death, the mean age in this group was 18.4 ± 9.7 years. The second group was formed by 41 patients with chronic rheumatic lesions of the pulmonary valve whose mean age at the time of death was 25± 16.5 years. The percentage of patients who died before the age of25 years was greater in the group with acute as opposed to chronic lesions (p=O.0003). The mean age of the first attack in those with acute lesions was 11.7±7.8 years, as compare to 13.8 ± 5.3 years in those with chronic lesions. All patients but one had at least one attack of the acute rheumatic process. There was no significant difference between the groups regarding the number of acute processes. The length of the acute rheumatic process was similar in both groups. Fifty-five cases having acute lesions, and 38 of those with chronic lesions suffered involvement of all cardiac valves. Three valves were affected in two hearts, while only one heart had two affected valves. Fifteen cases had an associated congenital defect of the pulmonary valve, and we found a significantly greater incidence of rheumatic pulmonary disease in the group with co-existing congenital lesions of the valve (p.O.00001). Three patients undergoing catheterization (21.4%) showed normal, or near normal, pulmonary arterial systolic pressure. Rheumatic damage to the pulmonary valve, therefore, exists in association with plurivalvar rheumatic disease. A congenital lesion of the pulmonary valve predisposes to its involvement in the rheumatic process.
Annual and seasonal variations in the prevalence of congenital cardiac malformations in live-born infants
Authors: Samánek Milan , Slavík Zdenek , Krejcir Miroslav 
Published: August 2008

SummaryThe annual and seasonal distribution of the prevalence of congenital cardiac malformations was calculated in 664,218 infants born from 1977 to 1984 in Bohemia (population of 6.3 million). All children who died were autopsied and those confirmed as having a malformation of the heart were included in our series. In total, 4,409 infants (6.64/1,000 live-births) were born with a cardiac defect. Annual variations were insignificant. The prevalence rate of cardiac malformations at birth was highest in October and lowest in December, June and July. It was not modified by differences in the birthrate. The peak of the prevalence coincided with the epidemics of influenza in early pregnancy. A nonuniform seasonal variation was found in individual forms of congenital malformations of the heart, indicating their possible etiological heterogeneity.