bluuebunny/crossref_metadata_2025 huggingface dataset:
Assessment of interventricular septal motion by cross-sectional echocardiography in patients with isolated atrial septal defects within the oval fossa
Authors: Vogel Michael , Schulze Karin , Bühlmeyer Konrad
Published: August 2008

To assess whether paradoxical (systolic anterior) motion of the interventricular septum is a true abnormality of septal contraction caused by right ventricular volume overload, we examined the regional pattern of left ventricular contraction in 20 patients with an atrial septal defect within the oval fossa using a fixed and floating reference system of analysis of wall motion. The patients, with a median age of 6.8 (3.5–13.4) years, had a Qp/Qs ratio of 3.5:1 (1.2:1–8:1). They were studied by cross-sectional echocardiography four days (3–5) before and 14.5 (3–67) days after surgical closure of the atrial septal defect. The postoperative Qp/Qs ratio was 1.1:1 (0.9:1–2:1). Regional wall motion of the left ventricle was analyzed in the parasternal short axis view at the level of the mitral valve and papillary muscles as well as in the apical four-chamber view. The endocardium was digitized manually in end-systolic and end-diastolic frames. The center of mass of this figure was calculated and connected to an outside reference point in the right ventricle. With the floating system, both centers of mass and the reference lines were superimposed, thus correcting for movement of the heart inside the thorax. With the fixed system,both end-diastolic and end-systolic frames were measured separately without correcting for movement of the heart. The left ventricle was divided in eight segments in a clockwise fashion and regional change in area was measured and compared to 40 normal age matched controls. With the floating system, left ventricular regional wall motion was normal in all patients before and after closure of the atrial septal defect and, thus, was not influenced by the change in right ventricular volume load. With the fixed system, both before and after surgical closure of the atrial septal defect, left ventricular regional wall motion was reduced in the two segments representing the basilar and middle portion of the interventricular septum. The floating system of analysis of left ventricular regional wall motion has the ability to correct for movement of the heart, which makes the supposed “abnormal” systolic anterior motion in right ventricular volume overload, a condition prevalent in patients with atrial septal defect, disappear. Thus, we conclude that the so-called paradoxical septal motion in atrial defect is an artifact. This may be caused by an increased motion of the heart but is unrelated to the volume load of the right ventricle.
Interobserver variability of determination of the size of the left-to-right shunt in isolated atrial septal defects within the oval fossa by Doppler and cross-sectional echocardiography
Authors: Vogel Michael , Stern Heiko , Bühlmeyer Konrad
Published: August 2008

Although noninvasive estimation of the ratio of pulmonary to systemic flow by measuring the cross-sectional areas of the pulmonary and aortic valves and the respective time integrals of the velocity of blood is widely used to determine the amount of shunting in patients with left-to-right shunts, few data exist concerning interobserver variability. We assessed such variability of shunt calculations derived from Doppler echocardiography in 10 children aged six (3.5–11) years with an isolated atrial septal defect within the oval fossa. The ratio of flows was calculated by measuring stroke volumes over the pulmonary trunk and the aorta immediately before and at an average of seven days (5–11) following surgical closure of the atrial septal defect. Two independent observers measured the cross-sectional areas of the aortic and pulmonary orifices and the velocity time integrals and calculated the size of the shunt from these data. All measurements were performed five times, and the mean of these five measurements was calculated and compared for analysis. Before surgery, observer I had measured a ratio of pulmonary to systemic flow of 3.8 (2.1–6.2) and observer II of 3.5 (1.9–5). The mean interobserver difference in terms of the size of the shunt was 1.1 (or 30%). After surgery, observer I measured a ratio of 1.3 (0.8–1.7) and observer II of 1.1 (0.7–1.6). The cross-sectional area of the pulmonary valve was measured as 3.6 (2.6–4.9) cm2 and 3.4 (2.3–5.8) cm2 respectively, with a mean interobserver difference of 0.8 cm2 or 23%. For the velocity time integrals across the pulmonary trunk, the mean interobserver difference was 13% (4–38) before and 16% (0–55) after surgery. The respective variability for the aortic velocity time integrals was 11 % (4–46) before and 9% (0.5–23) after surgery. The variability of measurements of maximal flow velocity across the pulmonary trunk was 15% (4–38) before and 18% (1–77) after surgery. The respective variability for the aortic flow velocity was 11 % (2–28) before and 9% (4–23) after surgery. A paired t-test showed no significant differences for all parameters assessed. The difficulty of reproducing measurements of the pulmonary valve is an important factor affecting interobserver variability. The magnitude of interobserver difference was significantly related to the cross-sectional area of the pulmonary trunk (r=0.84; p<0.02). The interobserver variability may be considerable, and is large enough to influence clinical decisions. Thus, we conclude that a decision regarding need for surgery cannot be based solely on calculation of the shunt derived only from Doppler measurements in patients with isolated atrial septal defects within the oval fossa.
Defective regional myocardial development and vascularization in one variant of tricuspid atresia—clinical and necropsy findings in three cases
Authors: O'Connor William N. , Cottrill Carol M. , Marion Michael T. , Noonan Jacqueline A.
Published: August 2008

Five previously reported cases have established the combination of a small right ventricle (sometimes with Uhl's anomaly), imperforate tricuspid valve with fibrotic tensor apparatus, congenital absence of the pulmonary valve, and an intact interventricular septum with muscular subaortic stenosis as a rare variant of tricuspid atresia. In this study of three new autopsy cases, we additionally identified Ebstein's malformation of the imperforate tricuspid valve, partial Uhl's anomaly, regional dysplasia of right ventricular myocardium and thinning of the interventricular septum by intramyocardial sinusoids from the right ventricle. All three new cases had epicardial anomalies of the right coronary artery—a fistula to right ventricle, ostial stenosis and proximal arterial hypoplasia. Sinusoidal connections from the right ventricle to the right coronary artery and to the sclerotic left anterior descending artery were identified in serial sections of the right ventricle and septum. Myofiber disarray, with thick walled intramyocardial arteries and sinusoids from left ventricle, involved the bulging subaortic interventricular septum. These studies are consistent with the hypothesis that defective development of the the right ventricle, along with its blood supply and associated atrioventricular and arterial valves, may underlie this unusual form of congenital heart disease.
Atrioventricular septal defects with severe left ventricular hypoplasia—clinical findings and surgical options
Authors: Troconis Carlos J. , Di Donato Roberto M. , Marino Bruno , Vairo Ugo , Marcelletti Carlo
Published: August 2008

Right ventricular dominance was observed in 22 (6.6%) out of 332 patients with atrioventricular septal defects. Their mean age was 2.24 ± 2.73 months. Chromosomal anomalies were seen in only three patients (Down's syndrome in two and trisomy nine in one). Seventeen patients underwent surgical treatment. A pulmonary artery banding was performed in eight of them with one long-term survivor who underwent a successful staged orthoterminal repair. A Norwood-type of palliation was attempted in four without success. Biventricular repair was performed in four additional patients with two mid-term survivors, in one of whom the patch used for closure of the atrial septal defect was fenestrated. One patient underwent successful heart transplantation at seven months of age. Pathophysiological problems and surgical options are discussed.
The use of intraoperative epicardial echocardiography with color flow imaging during the repair of complete atrioventricular septal defects
Authors: Ungerleider Ross M.
Published: August 2008

The use of intraoperative echocardiography with Doppler color flow imaging has acquired increased popularity for early assessment of efficacy of repair of congenital heart defects soon after discontinuance of cardiopulmonary bypass. This technology has been found useful also for evaluating the anatomy of the lesion prior to repair. Furthermore, with the use of color flow mapping, additional anomalies, such as patent arterial duct or multiple ventricular septal defects, which might have been overlooked during the initial examination, can be easily diagnosed. Moreover, intraoperative echocardiography provides the quickest and most sensitive method for a surgeon to look for residual atrioventricular valves regurgitation and/or residual atrial or ventricular septal defects as well as for depressed ventricular contractility. Previous studies1–4have discussed specifically the applicability of intraoperative color flow imaging during the repair of atrioventricular septal defect with a common atrioventricular orifice using both epicardial and transesophageal methodology. Routine intraoperative echocardiography with Doppler color flow imaging has been used since March 1987 at Duke University Medical Center during repair of congenital heart defects. Our aim was to confirm the preoperative dignosis and to assess the quality of repair soon after discontinuance of cardiopulmonary bypass. Our overall experience now includes 612 patients of whom 239 or 39% were less than one year of age at the time of repair. This indicates our tendency to undertake early correction regardless of the age of patients and of the severity of the lesion.
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

The 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

Two 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

Using 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

Surgical 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

The 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

Obstruction 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

The 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

To 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

Although 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

The 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

The 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

The 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

We 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

The 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.
Magnetic resonance imaging in patients after a modified Fontan operation
Authors: Stern Heiko , Bauer Richard , Schrötera Gerrit , Sauer Ursula , Emmrich Peter , Bühlmeyer Konrad
Published: August 2008

Magnetic resonance imaging was performed in 26 patients who underwent a modified Fontan procedure. Their age ranged from 1.8 to 31 years with a mean of 12.2 years. A valveless anastomosis was performed between the right atrium and the rudimentary right ventricle in 12 patients and between the right atrium and the pulmonary arteries in 10 patients. A cavopulmonary connection was established in the remaining four patients. Spin echo and gradient echo scans of the heart were performed in orthogonal and angulated projections. The cross-sectional area of the atrioventricular, atriopulmonary or cavopulmonary anastomoses was measured using diameters in two orthogonal imaging planes. Recordings were examined for the presence of right atrial thrombosis, the site of drainage of the coronary sinus, compression of the pulmonary veins, as well as for the presence and extent of pericardial effusions. Cine recordings were used for the assessment of the pattern of flow within the right atrium. The cross-sectional area of the anastomoses could be determined in 24 of 26 patients. This was not statistically different between patients with different surgical procedures. Patients with a cavopulmonary connection, however, tended to have a smaller anastomosis (mean 1.4 cm2/m2BSA, S.D. 0.62) than patients with atrioventricular (mean 3.0 cm2/m2, S.D. 2.1) or atriopulmonary (2.4 cm2/m2, S.D. 1.1) connections. When compared to normal values for the size of the tricuspid valve, the size of the anastomosis was within the normal range in only four patients, it was larger in one and smaller in 19 patients. There were signs of right atrial thromboses in the scans in eight of 26 patients, as observed by two independent investigators. The site of drainage of the coronary sinus was imaged in 20 of 26 patients and was in accordance with the description of surgical procedure in eight. Compression of the right pulmonary veins by an enlarged right atrium was present in seven patients. This was severe in two children. Presence and extent of pericardial effusions could be adequately assessed in 11 of26 patients. Systolic regurgitation from the rudimentary right ventricle into the right atrium was shown in eight of 12 patients with an atrioventricular valveless anastomosis. Cine recordings revealed slow forward flow from the right atrium into the pulmonary arteries in seven of 22 patients, and there was a markedly altered pattern of intraatrial flow in two patients with anomalous systemic venous connections. Magnetic resonance imaging allows adequate examination of right atrial anatomy, determination of the size of the anastomosis with the pulmonary arteries, and semiquantitative assessment of pulmonary blood flow in the majority of patients after a modified Fontan procedure.
Echocardiographic measurements in adolescents based upon gender and sexual maturity rating
Authors: Pearl William , Stafford Elisabeth M. , Martinko Thomas , Villazana Robert , Lucker William , Schydlower Manuel , Imai Walter
Published: August 2008

M-mode echocardiograms were recorded from 298 healthy adolescents between 10 and 16 years of age. Nineteen measured and 10 calculated parameters were correlated with the subjects’ gender and sexual maturity rating. Significant (p<0.05) differences by gender were demonstrated for 21 of 29 parameters. Sexual maturity rating had a significant effect on four of 29 parameters for boys and on five of 29 parameters for girls, when controlling for body surface area. Fourteen of 29 parameters in boys and three of 29 parameters in girls showed a significant effect of sexual maturity rating when controlling for age. The male adolescent growth spurt in lean body mass is paralleled by a similar growth spurt in left ventricular volume.
Balloon atrial septostomy in the intensive care unit under echocardiographic control—nine years experience
Authors: Kakadekar Ashok P. , Hayes Alison , Rosenthal Eric , Huggon Ian C. , Baker Edward J. , Qureshi Shakeel A. , Tynan Michael
Published: August 2008

Between December 1982 and April 1991, balloon atrial septostomy was performed in the intensive care unit under echocardiographic control in 60 neonates. Of the patients, 58 had complete transposition. Two patients had double outlet right ventricle with a sub-pulmonary ventricular septal defect. Associated lesions included a patent arterial duct in 19 patients, ventricular septal defect in nine, obstruction of the left ventricular outflow tract in six, aortic coarctation in two and tricuspid atresia in one. The mean age at septostomy was four days (range 4 hours - 25 days) and the mean weight 3.19 kg (range 1.17–4.25 kg). In 39 (65%) patients, an infusion of prostaglandin was in progress prior to the septostomy and 22 (37%) were being ventilated. Standard subcostal four-chamber echocardiographic views were used to show the atrial septum and to guide the catheter used for septostomy. Venous access was obtained via the femoral vein in 43 (by percutaneous puncture in 40 and by cutdown in three) and the umbilical vein in 17. Transient atrial arrhythmias were common during the septostomy but no acute hemodynamic disturbances or deaths occurred during the procedure. The size of the atrial septal defect as measured by echocardiography after the septostomy ranged from three to 12 mm in diameter. In only one patient was this inadequate. Three (5%) patients died between two and 10 days after the septostomy, two due to necrotizing enterocolitis and one from persistent hypoxemia. One patient had a cerebral thrombosis and convulsions immediately after the septostomy but made a good neurological recovery. Corrective surgery was performed in 52 (86.6%), two (3.3%) had palliative surgery and two were considered unsuitable for total correction, of whom one has died. One patient died whilst awaiting correction. We conclude that balloon atrial septostomy using echocardiographic guidance can be safely and effectively performed in the intensive care unit.
Doppler evaluation of physiologic peripheral pulmonic stenosis in newborns
Authors: Friedman Deborah M. , Fernandes John , Rutkowski Monika , Danilowicz Delores
Published: August 2008

A common systolic ejection murmur of the neonate has been attributed to physiologic peripheral pulmonic stenosis. We investigated this auscultatory finding using duplex pulsed Doppler. Three groups of normal fuliterm neonates less than one week old were studied—10 without murmurs, 10 with grade 1/6 murmurs and nine with at least grade 2/6 murmurs. We measured the anatomical size and peak flow velocities in the main pulmonary artery and left and right branches, the peak velocity in the right ventricular outflow tract, and the bifurcation angle. Flow gradients were calculated as 4 (Vmax)2 Groups were compared by t-tests. A loud peripheral pulmonic stenosis murmur was associated with increased pulmonary artery velocities, with left pulmonary artery velocity the most discriminating variable (1.3 ± 0.29 vs 0.94 ± 0.19 m/s; p ≤ 0.05). Although the peak gradient never exceeded 12 mmHg, there was an increased gradient in the loud murmur group (8.7 ± 2.6 vs 5.7 ± 2.2 mmHg; p ≤ 0.05) which may even be underestimated by the lack of angle correction. The left pulmonary artery diameter was also larger in the loud murmur group, but there were no other anatomic or volumetric flow differences between groups. The soft murmur group could not be separated from normals. We conclude that Doppler techniques can confirm the physiologic basis of peripheral pulmonic stenosis murmurs.
Renal artery Doppler blood velocity waveforms in children with aortic coarctation
Authors: Friedman Deborah M. , Schacht Robert G. , Rutkowski Monika , Doyle Eugenie F. , Danilowicz Delores
Published: August 2008

Noninvasive translumbar renal artery Doppler blood flow patterns were obtained in 20 normal children, 11 with successfully repaired coarctation, and eight with native or restenosed coarctation. Native or restenosed lesions result in decreased relative systolic velocity and prolonged acceleration time. After successful surgery, the renal flow returns to near normal. This technique may provide a useful tool to explore and predict hemodynamic and renal physiologic changes, and follow the course of therapy.
Tricuspid valve endocarditis on a healthy valve—potentially lethal in infants and young children
Authors: Levin Solomon E. , Dansky Raymond , Benatar Avram , Milner Selwyn
Published: August 2008

Tricuspid valve endocarditis on a normal valve has been rarely documented in children. In all five previous cases the infecting organism was Staphylococcus aureus. We report five infants and children with tricuspid valve endocarditis, whose ages ranged from four months to four years. In all of them large vegetations were identified by two-dimensional echocardiography. Positive blood cultures were found in four of five patients. Staphylococcus aureus, Corynebacterium species, Haemophilus influenzae and Candida albicans were the respective isolates. There were three deaths, all relatively sudden, 10–33 days after commencement of antibiotic therapy. At the only post mortem carried out in the patient with Candida albicans endocarditis and Down syndrome, there were two large fungating masses straddling the tricuspid valve. They appeared to have obstructed the circulation. Microscopic examination of a lymph node and the thymus showed features consistent with an immune deficiency syndrome. One child has been lost to follow-up. In the remaining survivor, progressively severe tricuspid regurgitation with congestive cardiac failure, necessitated open heart surgery 13 months later. A homograft replacement of the destroyed septal leaflet was carried out. Tricuspid valve endocarditis in an infant or child is a serious and potentially lethal condition. Early surgical intervention to remove large obstructive vegetations appears to be warranted.
The cardiac conduction system in Hurler syndrome: pathological features and clinical implications
Authors: Chow Louis Tsun-cheung , Chow Wing-Hing
Published: August 2008

We studied the cardiac conduction system in a case of Hurler syndrome. There was dense fibrosis of the supporting matrix of the sinus node and accumulation of mucopolysaccharide in the nodal cells. The bundle branches showed prominent hydropic degeneration, being encased and punctuated by dense fibrous tissue. These changes in the conduction system may predispose to the development of arrhythmias, accounting for the sudden deaths in Hurler syndrome.
Replacement of the aortic valve in tetralogy of Fallot prior to intracardiac repair—a case report
Authors: Aru Giorgio M. , Cirio Emiliano , Martelli Valentino
Published: August 2008

A 21-year-old woman with tetralogy of Fallot, who had undergone a right Blalock-Taussig shunt at one year of age, developed endocarditis of the aortic valve. Septic thrombosis of the right pulmonary artery was diagnosed by intraoperative transesophageal echocardiography. Replacement of the aortic valve initially performed by a St. Jude Medical prosthesis, was unsuccessful due to immobilization of the valve occluder. Subsequent replacement of the St. Jude valve by a Carpentier-Edwards valve was uneventful. Thrombectomy and patch enlargement of the right pulmonary artery were also performed successfully. We suggest that the St. Jude Medical valve should be employed with caution for replacement of the aortic valve in patients with tetralogy of Fallot who have not undergone intracardiac repair. Transesophageal echocardiography is extremely useful in discovering undiagnosed associated lesion such as septic thrombosis of the pulmonary arteries.
Diagnostic aspects, carditis and other acute manifestations of streptococcal infection
Authors: de Carvalho Coelho Mota Cleonice , Meira Zilda Maria Alves , Graciano Rosangela Nicoli , Silva Marly Conceição
Published: August 2008

We have conducted, in two stages, a descriptive and comparative analysis of the diagnostic and evolutionary clinical aspects of rheumatic fever. The descriptive analysis was based on a sample of 226 patients seen between 1976–1986. The forms and frequency of the manifestations of rheumatic fever were studied, as well as the difficulties encountered in diagnosing mild cases and the risk of making a hasty diagnosis due to the high prevalence of the disease. Subsequent to the establishment of an outpatient clinic for referral of patients with rheumatic fever, as part of a study and control program for the disease, 98 patients were seen in the period 1988–1991. In order to compare the profile of the manifestations in the acute phase with evolutionary aspects over the course of the disease, a subgroup of 61 children, treated in the period 1983–986 was selected to obtain equivalence in size of sample and time of follow-up (p = 0.08). The comparative analysis revealed that no modifications have occurred over the period of study in the profile of the manifestations during the acute phase, and no statistically significant differences have occurred in age at first attack, severity ofcarditis, or in the type ofvalvar and articular involvement. Evolutionary data after rigid control of primary and secondary prevention, however, showed a marked reduction in the incidence of recurrence (p = 0.036), the frequency of hospital admission (p = 0.001) and deaths (p = 0.024). The main factors modifying the evolution of the disease were analyzed. The action of the health team had a fundamental importance in the control of recurrence. This fact is especially important for developing countries, where a reduction of the prevalence of the disease can only be achieved through major structural socioeconomic changes in the community.
Echocardiographic diagnosis of rheumatic fever and rheumatic valvar disease
Authors: Medeiros Caio César Jorge , de Moraes Alvaro Villela , Snitcowsky Rachel , Graziosi Pedro Mauro , de Figueiredo Neto José Albuquerque , Filho José Rodrigues Parga , Bellotti Giovanni , Pileggi Fulvio
Published: August 2008

We studied 56 children echocardiographically, ages four to 15 years (mean nine years) with acute rheumatic fever to determine the extent of cardiac involvement. All had a clinical diagnosis of carditis. Mitral regurgitation was always present and was associated with aortic regurgitation in 53.6% and tricuspid regurgitation in 32.1%. Mitral valvar prolapse was observed in 18 patients 5(32.1%) and could not be differentiated from myxomatous prolapse in 10 (17.8%). Cordal rupture was detected in seven patients (12.5%), three of whom required surgical treatment. Other findings concerning the mitral valve were vegetations in two patients (3.6%), commissural fusion without stenosis in 36(64.3%), and mitral stenosis in four (7.1%). Heart failure was usually secondary to valvitis rather than myocarditis, and led to surgical treatment in seven patients. The myocardial function was depressed in only two patients. In both, the ejection function returned to normal after medical treatment. Echocardiography provides important information on the involvement of the heart in acute rheumatic fever, helping to determine prognosis and the results of treatment.