bluuebunny/crossref_metadata_2025 huggingface dataset:
Catheter-based closure of atrial septal defects with a newly developed nitinol double disc: an experimental study
Authors: Bjørnstad Per G. , Smevik Bjarne , Fiane Arnt E. , Tønnessen Tor Inge , Fosse Erik
Published: August 2008

We report experimental use of a new device for transcatheter closure of atrial septal defects. The device was implanted in surgically created atrial septal defects in two pigs. The devices were easy to implant, and the procedures were short. Both devices were implanted correctly in the desired position, and closed the defect completely. No procedural complications nor problems were encountered. The technique is certainly worth trying in the clinical setting. The device appears to be particularly favourable for use in children because it can be implanted through a 7 French sheath.
Primary involvement of the mitral and aortic valves in Takayasu's arteritis
Authors: Bernstein Harold S. , Ursell Philip C. , Teitel David F.
Published: August 2008

A 3-year-old boy developed acute pulmonary edema following 6 months of recurrent fevers and thrombocytosis. Echocardiography demonstrated moderate-to-severe insufficiency of both mitral and aortic valves. Angiography and intravascular ultrasound were used to diagnose this unusual presentation of Takayasu's arteritis. To the best of our knowledge, this is the first histological demonstration of acute and chronic inflammatory changes in the mitral valve in these circumstances.
Supravalvar aortic stenosis due to organizing thrombus in an asymptomatic child
Authors: Elkins Louis W. , Muehrcke Derek D. , Jonas Richard A.
Published: August 2008

A large mass arising from the aortic valve caused supravalvar aortic stenosis in a 4-year-old girl. The differential diagnosis included an organized thrombus or myxoma. Following surgical excision, there was resolution of choreioform movements and tongue thrusting, which had been present for 1 week preoperatively.
Left atrial mass due to cancica endomyocarditis in a newborn
Authors: Şenocak Filiz , Akçayöz Ayşegül , Özkutlu Süheyla
Published: August 2008

We describe a case of Candida endomyocarditis in a neonate not diagnosed until autopsy. Echocardiography had demonstrated a left atrial mass. Since cardiac candidiasis seems to be occuring with greater frequency in neonatal intensive care units, echocardiography and blood cultures should be done to exclude this previously rare event.
Fatal outcome of pregnancy in the Eisenmenger syndrome
Authors: Brach-Prever Silvia , Sheppard Mary , Somerville Jane
Published: August 2008

A mildly symptomatic 28-year-old women with Eisenmenger syndrome became pregnant against advice, and presented at 17 weeks insisting on continuing the pregnancy. She underwent an emergency Caesarean section for pre-eclampsia at 26 weeks, delivering a live 620 g boy, who survived to leave the hospital. In the post-partum period, she presented increasing hypoxemia, despite treatment with noradrenaline, nitric oxide and prostacyclin. She died 2 weeks after delivery from pulmonary haemorrhage. Necropsy showed histological signs of severe pulmonary vascular disease, with evidence of diffuse fibrinoid necrosis, vasculitis and haemorrhage.
The diagnosis of innocent murmurs in childhood
Authors: Advani Najib , Menahem Samuel , Wilkinson James L.
Published: August 2008

Innocent murmurs are common in childhood. They require accurate diagnosis to avoid unnecessary anxiety and/or restrictions. With this in mind, we studied prospectively children diagnosed by a paediatric cardiologist as having innocent murmurs to review the necessity of investigations in excluding organic abnormalities. We included 63 children in the study. The majority had the so-called Still's, or musical/vibratory murmur. None had abnormal investigations, suggesting that such investigations are largely superfluous for the purpose of diagnosis in those patients with innocent murmurs seen by a paediatric cardiologist. They are often done, nonetheless, as part of the assessment and/or expectations of the parent or the referring physician.
Clinical and morphologic features of perimembranous ventricular septal defect with overriding of the aorta – the so-called Eisenmenger ventricular septal defect. A study making comparisons with tetralogy of Fallot and perimembranous ventricular defect without aortic overriding
Authors: Fukuda Toyoki , Suzuki Takaaki , Ito Tsutomu
Published: August 2008

The aim of our study was to elucidate the clinical and morphologic features of those perimembranous ventricular septal defects which extend between the ventricular outlets, particularly when found in association with anterior deviation of the muscular outlet septum and overriding of the aorta – the so called Eisenmenger ventricular septal defect. From 1990 through 1998, we have undertaken surgical correction in 203 patients with perimembranous ventricular septal defect. Of these, 15 patients had the Eisenmenger ventricular septal defect. We conducted retrospective analyses of the clinical records, catheterization data, and angiocardiographic and echocardiographic finding of these patients. Comparative studies were then made with the patients having tetralogy of Fallot, and those with simple perimembranous ventricular septal defects without overriding of the aorta. In the patients with the Eisenmenger ventricular septal defect, the extent of anterior deviation of the outlet septum was comparable with that seen in tetralogy of Fallot, but there was less rightward displacement of the aortic valvar orifice. In contrast to earlier investigators, however, we found evidence of progressive narrowing of the subpulmonary infundibulum in those with the Eisenmenger defect. These morphological features were reflected in the clinical features, since all patients showed evidence of increased pulmonary flow and congestive heart failure in early infancy, but with twothirds of them subsequently developing right-to-left shunting. We conclude, therefore, that the Eisenmenger ventricular septal defect is a discrete cardiac abnormality in which the morphologic substrate of anterior deviation of the outlet septum gives rise to a potential for progressive narrowing of the subpulmonary infundibulum. Surgical management, therefore, needs to take account of such narrowing as an additional cardinal morphologic feature.
Abnormal responses for blood pressure in children and adults with surgically corrected aortic coarctation
Authors: Hauser Michael , Kuehn Andreas , Wilson Neil
Published: August 2008

Background

Despite successful surgical repair of aortic coarctation, life expectancy is reduced, and up to one-third of patients remain or become hypertensive. So as to characterize the responses for blood pressure, we have studied 55 patients with surgically repaired coarctation. Their mean age was 11.3 ± 5.97 years. We documented maximal uptake of oxygen, anaerobic threshold, plasma renin activity and blood pressures during a Bruce protocol treadmill test. The velocity across the site of repair as imaged by crosssectional echocardiography was measured before and after exercise. We measured the changes in heart rate and blood pressure subsequent to an infusion of 1 ug per kg of isoprenalin, monitoring blood pressure over 24 hours in all patients.

Results

When compared with 40 healthy age-matched controls, the patients with coarctation had a normal exercise capacity. Resting systolic blood pressures above the 95th percentile were present in 45% of the patients. Exercise-induced hypertension, and an elevation in the average systolic 24 hour blood pressures, were observed, but less frequently than elevated baseline values, suggesting that socalled white-coat” hypertension may be present in this population. Abnormal reactions and elevation of plasma renin activity were related to a history of paradoxical hypertension at the time of surgery. Attenuation of the circadian rhythm for blood pressure was a frequent finding, and may have implications in the development of long-term damage to end-organs. A high correlation was found between mean systolic blood pressure measured by 24 hour monitoring and left ventricular hypertrophy (r=0.65, p<0.05).

Conclusions

Abnormalities in blood pressure occurred independently of significant mechanical obstruction. Despite successful surgical repair, abnormalities in the shape of the aortic arch, reduced sensitivity of baroreceptor reflexes, and neurohumoral factors may all contribute to the development of hypertension.
Outcomes for children with cardiomyopathy awaiting transplantation
Authors: Nield Lynne E. , McCrindle Brian W. , Bohn Desmond J. , West Lori J. , Coles Jhon G. , Freedom Robert M. , Benson Lee N.
Published: August 2008

Objective

To determine factors associated with outcomes after listing for transplantation in children with cardiomyopathies.

Background

Childhood cardiomyopathies form a heterogeneous group of diseases, and in many, the prognosis is poor, irrespective of the etiology. When profound heart failure develops, cardiac transplantation can be the only viable option for survival.

Methods

We included all children with cardiomyopathy listed for transplantation between 12/89 and 4/98 in this historical cohort study.

Results

We listed 31 patients, 15 male and 16 female, 27 with dilated and 4 with restrictive cardiomyopathy, for transplantation. The median age at listing was 5.7 years, with a range from fetal life to 17.8 years. Transplantation was achieved in 23 (74%), with a median interval from listing of 54 days, and a range from zero to 11.4 years. Of the patients, 14 were transplanted within 30 days of listing. Five patients (16%) died before transplantation. Within the Canadian algorithm, one of these was in the third state, and four in the fourth state. One patient was removed from the list after 12 days, having recovered from myocarditis, and two remain waiting transplantation after intervals of 121 and 476 days, respectively. Patients who died were more likely to be female (5/5 vs. 11/26; p=0.04) and to have been in the third or fourth states at listing (5/5 vs. 15/26; p=0.04). The use of mechanical ventricular assistance, in 10 patients, was not a predictor of an adverse outcome. While not statistically significant, survival to transplantation was associated with treatment using inhibitors of angiotensin converting enzyme, less mitral regurgitation, a higher mean ejection fraction and cardiac index, and lower right ventricular systolic pressure.

Conclusions

Children with cardiomyopathy awaiting transplantation have a mortality of 16% related to their clinical state at the time of listing.
Clinical and electrophysiologic evolution of the Wolff-Parkinson-White syndrome in children: impact on approaches to management
Authors: Vignati Gabriele , Balla Eva , Mauri Luigi , Lunati Maurizio , Figini Alberto
Published: August 2008

There is less certainty about the recommendations for radio-frequency ablation as a therapeutic option for Wolff-Parkinson-White syndrome in children as opposed to adults because of the different natural history and the age-related risks of ablation. To help decision-making, we evaluated the long term clinical evolution and electrophysiologic characteristics of pre-excitation in our series of children and young adults. We reviewed the clinical course of 109 patients below the age of 18 years who had been followed up over a period of 9+4 years, with a range from one to 27 years. This corresponded to 986 patient-years. We examined also the electrophysiologic data from 98 of those patients who underwent a transesophageal study.

At the discovery of pre-excitation, 59% of patients were asymptomatic, while 29% developed supraventricular tachycardia during follow-up. The peak incidence of the onset of supraventricular tachycardia occurred during infancy. These patients had the highest incidence of subsequent spontaneous disappearance of the tachycardia (53%), such a favourable evolution being encountered in only 12% of patients in whom the abnormal rhythm first appeared after 12 years of age. Chronic medical treatment was required in 47% of patients, and proved completely effective in 45% of cases. The potential to induce atrial fibrillation, and the incidence pre-excitation considered to be high risk, with the shortest pre-excited RR interval equal to or less than 220msec, was lowest in the group of patients aged less than 6 years of age, and highest in those older than 12 years of age (p <0.001). Pathways producing arrhythmia with high risk were more common in symptomatic (29%) than in asymptomatic patients (7%) (p<0.001). No mortality occurred. On the basis of our findings, we suggest that ablation should be avoided before the age of 5 or 6 years. Thereafter, the procedure should become the first line of treatment for symptomatic patients older than 12 years of age.
Radiofrequency catheter ablation for the treatment of supraventricular tachycardias in children and adolescents
Authors: Iturralde Pedro , Colín Luís , Kershenovich Sergio , Guevara Milton E. , Medeiros Argelia , Buendia Alfonso , Attie Fause
Published: August 2008

We report our experience in radiofrequency catheter ablation between April, 1992 and December, 1998, in which we treated 287 parients less than 18 years of age (mean 14.3±3.1 years) with supraventricular tachycardia. Accessory pathways were the arrhythmic substrate in 252 of the patients (87.8%), the patients having a total of 265 accessory pathways. Atrioventricular nodal re-entry was the cause of tachycardia in 26 patients (9.0%), while atrial flutter was detected in the remaining 9 patients (3.1%). We were able successfully to eliminate the accessory pathway in 236 patients (89%), but 25 patients had recurrent arrhythmias. Ablation proved successful in all cases of atrioventricular node re-entry tachycardia, the slow pathway being ablated in 25 patients, and the fast pathway in only one case. Recurrence of the arrhythmia occurred in three patients (11.5%). We performed a second ablation in these children, all then proving successful. The ablation was successful in all cases of atrial flutter, with one recurrence (11.1%). Overall, therefore, ablation was immediately successful in 271 patients (94.4%), with a recurrence of the arrhythmia in 29 cases (10.7%). The incidence of serious complications was 2.09% There was one late death due to infective endocarditis, 3 patients suffered complete heart block, 1 had mild mitral regurgitation, and 1 patient developed an haematoma in the groin. We conclude that radiofrequency catheter ablation can now be considered a standard option for the management of paroxysmal supraventricular tachycardias in children and young adults.
Successful palliation of Ebstein's malformation on the first day of life following fetal diagnosis
Authors: Deleon Mary Ann M. , Gidding Samuel S. , Gotteiner Nina , Backer Carl L. , Mavroudis Constantine
Published: August 2008

Abstract Severe Ebstein's malformation has a poor prognosis when diagnosed during the neonatal period. Surgical options that have proved successful in older patients have been associated with high mortality and morbidity in neonates. We report here our success with a policy of induction at term and immediate surgical intervention when Ebstein's malformation was diagnosed prenatally.
Abnormal pattern of flow in the superior caval vein in children after open heart surgery
Authors: Ichihashi Ko , Shiraishi Hirohiko , Momoi Mariko
Published: August 2008

Abstract To investigate the pattern of flow in the superior caval vein in children after open heart surgery, we examined the flow velocity in 37 partients who had undergone surgical correction of either ventricular or atrial septal defects. The control group consisted of 64 age-matched children. We used pulsed Doppler examinations to record the velocity curves. We measured the hightest and lowest peak flow velocities during systole and diastole, and calculated ratios for the various measurements.

We found that the velocity of the wave in systole is lower than that of the wave in diastole in the postoperative patients, which is contrary to the normal pattern. The velocity in systole increases more than that in diastole during inspiration, which is also diffetent from the normal pattern. The change in pattern of flow velocity cure during respiration was smaller in patients after surgery than in normal subjects, which might indicate that there is less reserve ability in the right atrium in postoperative patients.
Arrangement of the systemic and pulmonary venous components of the atrial chambers in hearts with isomeric atrial appendages
Authors: Min Jee-Yeon , Kim Chi-Yon , Oh Mee Hye , Chun Yi Kyeong , Suh Yeon-Lim , Kang I-Seok , Lee Heung-Jae , Seo Jeong-Wook
Published: August 2008

Background

The morphological definition of atrial chambers, and the determination of atrial laterality, are based on analysis of the structure of the atrial appendages. The systemic and pulmonary venous connections to the heart, nonetheless, are important in the management of patients having isomeric appendages. In this study, therefore, we analysed the morphology of the postero-superior walls of the atrial chambers so as to provide evidence concerning the morphogenetic background of those hearts, and to improve operative management.

Methods

We reviewed 15 autopsied specimens with isomeric right appendages, and 10 with isomeric left appendages, paying particular attention to the morphology of the systemic and pulmonary venous connections. The postero-superior walls of the atrial chambers can be made up of the atrial body, the systemic venous components, or the pulmonary venous component. We analysed the contributions made by each of these components.

Results

The postero-superior walls of the atrial chambers were markedly variable, but could be grouped into five patterns. Bilaterally well-developed systemic venous components and absence of the pulmonary venous component within the hypoplastic atrial body were present in 9 hearts with extracardiac pulmonary venous connections in the setting of right isomerism. Bilaterally well-developed systemic venous components, and a hypoplastic pulmonary venous component within the hypoplastic atrial body, were present in 5 hearts with intracardiac pulmonary venous connections in right isomerism. Bilaterally well-developed systemic venous components, and a hypoplastic pulmonary venous component within the sizable atrial body, were present in 1 heart with an intracardiac pulmonary venous connection in right isomerism. A well-developed pulmonary venous component within the atrial body, and hypoplasia of one systemic venous component, were present in 7 hearts with left isomerism. A well-developed pulmonary venous component within the atrial body, and hypoplasia of bilateral systemic venous components, were present in 3 hearts with left isomerism.

Conclusions

The postero-superior walls of the atrial chambers in hearts with isomeric atrial appendages can be analysed on the basis of a compound structure made of bilateral systemic venous components, a central pulmonary venous component, and the body of the atrium. Hearts with isomeric right appendages have absence or hypoplasia of the pulmonary venous component, while hearts with isomeric left appendages have hypoplastic systemic venous components.
Aortic dissection late after repair of congenital aortic coarctation
Authors: Peterson John , Moodie Douglas
Published: August 2008

Aortic dissection is one of several complications that can be seen late after repair of aortic coarctation. We report an unusual presentation of this complication, and review the pertinent literature.
Fetal echocardiographic features of twisted atrioventricular connections
Authors: Abdullah Mustafa , Yoo Shi-Joon , Hornberger Lisa
Published: August 2008

We present an example of corrected transposition with twisted discordant atrioventricular connections in which both fetal and postanatal echocardiograms were obtained. We correlate the fetal echocardiograms with the postanatal echocardiograms, placing emphasis on the echocardiographic clues to the diagnosis which were present in fetal life. The diagnosis should be suspected when the cardiac chambers and greatarteries show an unexpected spatial relationship for the given segmental connections, and when the axes of opening of the atrioventricular valves are not parallel.
Neuropathic pain syndrome as an occult manifestation of injury of the spinal cord after surgical repair of aortic coarctation
Authors: Beauchesne Luc M , Mailis Angela , Webb Gary D
Published: August 2008

Injury to the spinal cord injury with paraplegia, is a rare complication of surgical repair of aortic coarctation recognized immediately post-operatively. We present the case of a 41-year-old male undergoing surgery for restenosis at the site of a repair. Intra-operatively, he suffered inadvertent injury to an intercostal arterial branch during isolation of the aorta below the graft. Over the following months, he developed unusual symptoms involving the legs and genitourinary tract which, only after extensive investigations, were attributed to ischemic damage to the spinal cord related to the surgery. We suspect that similar syndromes reflecting injury to the spinal cord injury may be unrecognized following surgical repair of coarctation.
Right-to-left shunt through the cardiac veins after the Fontan procedure
Authors: Uemura Hideki , Yagihara Toshikatsu , Monta Osamu
Published: August 2008

We found right-to-left shunts through the cardiac veins postoperatively in 2 patients who had undergone the Fontan procedure. In one of the patients, channels were present through the cardiac veins independent of the coronary sinus. In the other patient, an atretic orifice for the coronary sinus, coupled with a persistent left-sided superior caval vein, complicated the postoperative course.
Non-confluent pulmonary arteries in a patient with pulmonary atresia and intact ventricular septum: ? a 5th aortic arch with a systemic-to-pulmonary arterial connection
Authors: Serra Astolfo , Chamie Francisco , Freedom R.M.
Published: August 2008

Major abnormalities of pulmonary circulation are uncommon in the patient with pulmonary atresia and intact ventricular septum. Non-confluent pulmonary arteries have only rarely been described in this setting. In this case report, we describe a patient in whom the pulmonary arteries are non-confluent, with the right pulmonary artery supplied through a right-sided arterial duct, and the left pulmonary artery most likely through a fifth aortic arch, thus providing a systemic-to-pulmonary arterial connection. We discuss the various forms of non-confluent pulmonary arteries in the setting of pulmonary atresia and intact ventricular septum.
Atrioventricular septal defect—the need for a flexible surgical approach in a lesion with markedly individual features
Authors: Lecompte Yves , Grupi Giancarlo
Published: August 2008

Hearts with biventricular connections and deficient atrioventricular septation are readily recognizable from their characteristic morphology.1As yet, however, there is still no consensus on precisely what to call them,2how to categorize them, and, perhaps most importantly, how to approach their surgical repair.
Epidemiology of cardiovascular malformations: The state of the art
Authors: Ferencz Charlotte , Villasenñor Adolofo Correa
Published: August 2008

A historical review highlights an interest in the etiology of birth defects over the course of several centuries and recalls specific hypotheses on the origin of cardiovascular defects postulated 60 years ago. The “State of the Art” of the epidemiology of cardiovascular malformations is characterized by an expanding base of knowledge which holds promise for eventual preventive interventions. Epidemiologic investigations conducted in the past decade have led to an increased understanding of the benefits and problems of studies of populations and of methodologic issues in surveillance of disease and study of risk factors. Comparative evaluations of reported studies to an ongoing population-based case-control investigation (the Baltimore-Washington Infant Study) demonstrate variations in prevalence by time and place, evidence for a strong genetic predisposition to cardiac maldevelopment and new hypotheses regarding environmental risks in the home and place of occupation.
Prevalence at birth, “natural” risk and survival with atrioventricular septal defect
Authors: Samaánek Milan
Published: August 2008

Prevalence of atrioventricular septal defect in liveborn infants at birth was calculated in Bohemia (population of 6.3 million). Of 664,208 liveborn infants, 4,409 had a congenital malformation of the heart and in 126 of them (0.19/1,000 live births, 2.9% of all heart defects) this was found to be an atrioventricular septal defect. The ratio between those with a common valvar orifice (complete form) versus those with separate right and left atrioventricular valvar orifices (incomplete form) was 1.14:1. The ratio of girls to boys was 1.17:1. Children born in March, July and October were more likely to have the defect (0.26/1,000 live births) than those born in May (0.10/ 1,000 live births). The defect was the eighth most frequent critical congenital cardiac malformation. Eight in each 100,000 liveborn children, and 3.56% of all patients with congenital cardiac disease, are at high risk of dying due to deficient atrioventricular septation, which in 82.5% resulted in the presence of a common atrioventricular valve (complete form). Unconditional death rate was studied in 1,008 liveborn children who died with a cardiac malformation before the age of 15 before the advent of cardiac surgery. Of these, 64 (6.35%) died with an atrioventricular septal defect which was complete in 5.95% and incomplete in 0.40%. All but one of 60 children with the complete form (common atrioventricular valve) died in the first year of life. The last child died between the age of 2 and 5 years. The estimate for natural survival was calculated using our own data on the prevalence and age distribution of mortality. Of all the liveborn children with atrioventricular septal defect, 91% are expected to survive the first month, 81% (78−85) the first trimester, 63% (56−69) six months, and 49% (35−54) the first year. The survival curve remains stable thereafter.
The morphology and diagnosis of atrioventricular septal defects
Authors: Anderson Robert H. , Baker Edward J. , Yen Ho Siew , Rigby Michael L. , Ebels Tjark
Published: August 2008

In this review, we discuss and describe those features which distinguish hearts with abnormal atrioventricular septation from the normal heart. The hearts, best described as atrioventricular septal defects, are unified by having a common atrioventricular junction guarded by a valve having five leaflets. The left component has three leaflets and cannot be interpreted in terms of a cleft in a normal mitral valve. The papillary muscles supporting this valve are also markedly dissimilar from the arrangement seen in normal hearts. The subaortic outflow tract is displaced superiorly compared to the normal heart, and is no longer wedged between the left valve and the septum. There is marked discrepancy in the inlet and outlet lengths of the ventricular mass, these dimensions being equal in hearts with normal atrioventricular septation. Although having the above features in common, atrioventricular septal defects show anatomic variations related to the arrangement of the bridging leaflets and their relationship to the septal structures. There may be a common valvar orifice or separate right and left orifices. The anatomic potential for shunting may be at atrial or ventricular levels, or both. Rarely, the septal structures may be intact. Other important features include ventricular dominance, the left ventricular outflow tract, and the disposition of the atrioventricular conduction tissues.
Surgical anatomy of the atrioventricular septum and atrioventricular septal defects
Authors: Henry G. William , Wilcox Benson R.
Published: August 2008

Defects of the atrioventricular septum impose changes in the septal architecture, atrioventricular valves, and conduction system that must be well understood for optimal repair. The atrioventricular node and bundles are shifted to more posterior and inferior positions that can be anticipated by the surgeon so as to maintain the integrity of atrioventricular conduction. The atrioventricular valve is a five leaflet structure that possesses a common orifice or separate orifices depending on characteristics of the superior and inferior bridging leaflets. These features are demonstrated by intraoperative photographs viewed from the surgeon's position.
The role of transesophageal echocardiography in the evaluation of patients with atrioventricular septal defect
Authors: Smallhorn Jeffery F. , Perrin Donald , Musewe Norman , Dyck John , Boutin Christine , Freedom Robert M.
Published: August 2008

Cross-sectional echocardiography has made a major contribution to the preoperative evaluation of the patient with an atrioventricular septal defect,1−5such that in many instances this modality has been the sole imaging technique used to formulate a medical or surgical plan6. With the advent of Doppler echocardiography additional hemodynamic information is now available, which enables the cardiologist and surgeon to evaluate the site and magnitude of associated atrioventricular valvar regurgitation, and obtain an accurate measurement of pulmonary arterial pressure along with an assessment of outflow tract obstruction, if present.
Atrioventricular septal defect in fetal life—a clinicopathological correlation
Authors: Cook Andrew C. , Allan Lindsey D. , Anderson Robert H. , Sharland Gurleen , Fagg Nuala L. K.
Published: August 2008

In a series of over 7,000 pregnancies referred for fetal echocardiography, atrioventricular septal defects were detected in 103 fetuses, (17% of 594 anomalies). A large proportion (42%) also had isomerism of the atrial appendages, abnormalities in arrangement and morphology of the thoracoabdominal organs, and a high incidence of associated cardiac malformations. When this was associated with abnormalities of rhythm, the fetus often showed signs of intrauterine congestive heart failure; hydrops, pleural effusion and ascites. Those with trisomic karyotypes (predominantly trisomy 21) were associated with usual atrial arrangement and concordant connections of the cardiac segments. Of the 103 fetuses, 53 were examined at autopsy. Extracardiac abnormalities were documented, and detailed morphologic examination and measurements of the heart were made. The ratios of inlet to outlet septal disproportion were not significantly different from those found in postnatal hearts. Again, as in postnatal life, those hearts with common atrioventricular orifice had a greater degree of ‘scooping’ of the ventricular septum compared to those with separate right and left atrioventricular valves. There were no significant differences in these findings in relation to the karyotype of the fetus. There was an increased incidence of ventricular dominance, and also of coarctation or hypoplasia of the aortic arch, in fetal hearts when compared to autopsied hearts obtained in postnatal life. In summary, the distinguishing morphological characteristics of atrioventricular septal defect are present from 16 weeks of gestation.
Surgery of the left atrioventricular valve and of the left ventricular outflow tract in atrioventricular septal defect
Authors: Ebles Tjark
Published: August 2008

Malfunctioning of the left atrioventricular valve has always been, and remains, the major incremental risk factor in the repair of atrioventricular septal defect. Now that the cardiac surgeon has ample time to assess the anatomy and function of the left valve, results have improved, but are still less than ideal. On the presumption that the anterior leaflet of the mitral valve is “cleft” in this anomaly, it used to be common practice to close the “cleft”. Currently, a substantial number of surgeons employ this technique, often irrespective of the individual anatomy, and in the majority of cases with success.
Technique of management of the left atrioventricular valve in the repair of atrioventricular septal defect with a common atrioventricular orifice
Authors: Laks Hillel , Capouya Eli R. , Pearl Jeffrey M. , Elami Amir , Drinkwater Davis C.
Published: August 2008

Despite improvement in the early outcome for patients undergoing complete repair of atrioventricular septal defects with a common atrioventricular orifice, the incidence of reoperation for failure of the repair of the left atrioventricular valve remains high. The surgical techniques employed in our most recent experience, including routine suture of the septal commissure and liberal use of valvar annuloplasty, were successful in achieving a very satisfactory competence of left atrioventricular valve with a iow incidence of reoperation for failure of the repair.
Management of the left atrioventricular valve in 95 patients with atrioventricular septal defects and a common atrioventricular orifice—a ten year review
Authors: Lacour-Gayet François , Comas Juan , Bruniaux Jacqueline , Serraf Alain , Losay Jean , Petit Jérôme , Dervanian Patrice , Planché Claude
Published: August 2008

During a 10 year interval, between January 1, 1981 and January 1, 1991, primary repair of atrioventricular septal defect with a common atrioventricular orifice was performed in 95 patients younger than 1 year. Patients with atrioventricular septal defect having two atrioventricular orifices and a small ventricular septal defect, and those with severe hypoplasia of the left ventricle, were not included in the present study. According to the presence of associated anomalies, patients were divided into those with simple forms (72%) and those with complex forms (28%). These included parachute deformity of the left atrioventricular valve (10 cases), multiple muscular ventricular septal defects (5 cases), low-lying infundibular stenosis of the right ventricle (4 cases), obstruction of the left ventricular outflow tract (3 cases), tetralogy ofFallot (2 cases) and hypoplasia of the left ventricle (2 cases).
Atrioventricular septal defect and the left atrioventricular valve at reoperation
Authors: Abbruzzese Pietro A. , Crupi Giancarlo , Tumbarello Roberto , Napoleone Alessandra , Merlo Maurizio , Parenzan Lucio
Published: August 2008

Between 1968 and December 1990, 29 patients underwent 33 reoperations for dysfunction of the left atrioventricular valve after correction ofatrioventricular septal defect. Repair of the valve was possible in all 10 patients who, initially, had a common atrioventricular orifice, using straightforward procedures such as closure of the septal commissure and annuloplasties according to Wooler. Additional valvar abnormalities (double orifice; fenestration of leaflets; malpositioned or malformed papillary muscles; and additional clefts) were rare in this group of patients. In contrast, these anomalies were frequent in the 19 patients who, initially, had separate right and left atrioventricular valves.
Technique of repair of atrioventricular septal defect with a common atrioventricular orifice
Authors: Merrill Walter H. , Hammon John W. , Bender Harvey W.
Published: August 2008

We advocate primary repair of atrioventricular septal defect with a common atrioventricular orifice in symptomatic children presenting with intractable congestive heart failure. The single patch technique has been utilized as our procedure of choice. A low operative mortality and a low incidence of residual ventricular septal defect and regurgitation of the left atrioventricular valve requiring reoperation has been achieved. This report emphasizes the technical details of the repair. Careful delineation of anatomic detail is considered to be of the utmost importance. Precise suture placement, tailoring the patch, and reconstruction of the left atrioventricular valve are essential to achieve complete closure of the atrial and ventricular septal defects and functional integrity of the left atrioventricular valve.
Replacement of the left atrioventricular valve after repair of atrioventricular septal defect
Authors: Kadoba Keishi , Jonas Richard A.
Published: August 2008

A retrospective analysis of 55 patients who underwent replacement of the left atrioventricular valve following surgery for atrioventricular septal defect with separate right and left atrioventricular valves or with a common valvar orifice has revealed a marked decrease in the need for valvar replacement over time. Between 1983 and 1987, the incidence of replacement following repair has been 3% in infants. Overall early mortality for the entire series, extending from 1970 to 1987, was 22%. Mortality in the first year of life was 58%, which was significantly greater than mortality beyond one year of age (14%, p<0.01). Obstruction of the left ventricular outflow tract was a frequent incremental risk factor for death during infancy. The incidence of complete heart block (25%) is disappointingly high, and may reflect distortion of the left atrioventricular junction in an attempt to place as large a prosthesis as possible. The incidence of complete heart block has not decreased with time. These results confirm the desirability of secondary valvoplasty procedures to enable a child to be larger before ultimate replacement of the valve is required.
Significance of left ventricular wall stress in infants with atrioventricular septal defect, common atrioventricular orifice and regurgitation across the left atrioventricular valve
Authors: Seguchi Masahi , Nakazawa Makoto , Oyama Kataro , Kawada Masa-aki , Kurosawa Hiromi , Imai Yasuharu
Published: August 2008

The outcome of primary repair in young infants having atrioventricular septal defect with a common atrioventricular orifice and regurgitation across the left atrioventricular valve is not yet satisfactory. We studied the significance of the characteristics of left ventricular volume and mass and the predicted wall stress for the outcome of repair in 13 infants with this lesion. Three patients died of left heart failure after operation, although neither residual shunting at ventricular level nor regurgitation across the left atrioventricular valve was present. End-diastolic volume and ejection fraction of the left ventricle were 228 ±66% and 0.65 ±0.06 of normal, respectively, with no difference between the survivors and non-survivors. End-diastolic thickness of the posterior ventricular wall, determined by echocardiogram, was within normal range for body size in all patients.
Transesophageal echocardiography in congenital heart disease
Authors: Stümper Oliver , Hess John , Godman Michael J. , Sutherland George R.
Published: August 2008

Transesophageal echocardiography has gained an established role in the evaluation and management of the adult patient with acquired cardiac disease. Unlike the transthoracic approach, the transesophageal approach provides excellent imaging of posterior cardiac structures in virtually all patients.
Morphology of atrioventricular septal defect and fetal hydrops in the fetal rat
Authors: Momma Kazuo , Ando Masahiko
Published: August 2008

We studied 28 fetal rats with atrioventricular septal defect with a common orifice produced from 300 fetuses treated with bis-diamine (200mg) on the ninth and tenth day of pregnancy. After undergoing rapid whole- body freezing on the 21st day, they were studied by means of serial cross-sectional photographs of the frozen thorax. Forty-one fetuses with normal hearts subsequent to treatment with bis-diamine served as controls. Atrioventricular septal defect was isolated in three, or was associated with either tetralogy of Fallot (n=14) or common arterial trunk (n=11). Fetal hydrops was associated with evidence of regurgitation of the atrioventricular valves. In a subgroup with larger right atrial volumes (increased by more than two standard deviation over the mean in the controls), the following parameters were significantly increased: subcutaneous edema, pericardial effusion, thickening of the left atrioventricular valve, cardiac chamber volumes and masses. The superior and inferior caval veins, and the descending aorta were significantly smaller in fetuses with atrioventricular septal defect than in controls. Fetal atrioventricular septal defect, therefore, was associated frequently with hydrops and increased volumes of the right atrium and both ventricles. Hydropic changes included cutaneous edema and increased pericardial, pleural, and peritoneal fluid.
Long-term follow-up of aortic coarctation in infants, children and adults
Authors: Mathew Prasad , Moodie Douglas , Blechman Gary , Gill Carl C.
Published: August 2008

We have studied 140 patients undergoing corrective surgery for aortic coarctation between 1952 and 1972, following them for a mean period of 23 years. Of the patients, 19 underwent surgery in infancy (age range 15 days to 190 days, mean 84 days), 52 during childhood, with a mean age of 11.8 years (range 1 years), and 69 as adults (age range 18−50 years, mean 30.5 years). All infants were symptomatic at presentation and fared poorly because of associated cardiac lesions. Their operative mortality was 11%, and mortality prior to discharge from hospital was 21%. Of the survivors, 38% required surgery for recurrent coarctation. There were three late deaths. Of the survivors, 90% were asymptomatic at long-term follow-up. Only six children (11%) were symptomatic at presentation. There was no operative or early postoperative mortality, and only three late deaths (6%). Of the 49 survivors, 96% were asymptomatic, and only six required antihypertensive medication. The recoarctation rate was 9% (four patients). In the group undergoing surgery as adults, 42% were symptomatic at presentation with 90% being hypertensive. There were two postoperative deaths and a late mortality of 22%, which was mainly related to the cardiovascular complications. None of the adults had suffered recoarctation, but 48% remained hypertensive. Our study confirms that the prognosis of patients with aortic coarctation presenting in infancy is related to presence of associated cardiac anomalies and operative complications. The prognosis in older patients, and particularly adults, is related to residual hypertension and resultant cardiovascular disease. Surgical correction reduces symptoms and improves life expectancy, regardless of the age at operation, with the best overall prognosis being obtained for those undergoing surgery as children.
Diagnosis and outcome of dilated cardiomyopathy in the fetus
Authors: Webber Steven A. , Sandor George G. S. , Farquharson Duncan , Taylor Glenn P. , Jamieson Sue
Published: August 2008

To identify the echocardiographic features and natural history of dilated cardiomyopathy in fetal life, we reviewed records of all fetal echocardiograms over the 10 year period 1980–1989 to identify cases of dilated cardiomyopathy without left ventricular outflow obstruction and conducted a retrospective review of the fetal echocardiograms and clinical course of those fetuses with dilated cardiomyopathy. In addition, all fetal losses, perinatal deaths and live-born infants presenting with dilated cardiomyopathy in the first seven days of life were identified from the British Columbia Pediatric Cardiovascular Pathology Registry and from inpatient records. The pathological findings and outcome for this group were also reviewed. Marked left ventricular dilatation and dysfunction without left ventricular outflow obstruction was diagnosed in five of 1,158 fetuses undergoing feta lechocardiography. These five fetuses were correctly distinguished from two others with critical aortic stenosis and severe left ventricular dysfunction. Two of the five fetuses had additional right ventricular dysfunction and dilatation and developed hydrops, while the remaining three with isolated severe left ventricular dysfunction did not and showed normal somatic growth. All fetuses were live-born with spontaneous labour occurring in four of five cases at a mean gestational age of 36.5 weeks. Four of the five infants died, with three deaths occurring in the first week of life. During the same 10 year period, two of 2450 autopsied stillbirths (both hydropic) were diagnosed with dilated cardiomyopathy. An additional 15 neonates (including the five identified by fetal echocardiography) were diagnosed in the first week of life with dilated cardiomyopathy, congenital myocarditis or endocardial fibroelastosis. Four were hydropic (all with associated right ventricular dysfunction). Only three of the 15 survive. Fetal echocardiography can accurately distinguish dilated cardiomyopathy from left ventricular outflow obstruction. In the absence of right-sided disease or premature closure of the oval fossa, severe left ventricular dysfunction is well tolerated in utero. The prognosis for fetal dilated cardiomyopathy is very poor.
Assessment of left ventricular mass and volume by cross-sectional echocardiography in newborns and infants with tricuspid atresia prior to surgical intervention
Authors: Vogel Michael , Skovaranek Jan , Bühlmeyer Konrad
Published: August 2008

Because left ventricular mass may be important in judging feasibility of Fontan type of palliation in tricuspid atresia, this study was undertaken to generate data on left ventricular mass, volume and mass to volume ratio in newborns and infants with tricuspid atresia, native pulmonary stenosis and concordant ventriculoarterial connections prior to any surgical palliation to obtain values for “normal” left ventricular dimensions in tricuspid atresia. The left ventricle was evaluated in the apical two and four chamber view. From these two perpendicular imaging planes, we calculated mass as difference between epicardial and endocardial volume x 1.05 (specific gravity of heart muscle). Mass divided by volume at end-diastole yields the index: mass to volume. Data from 23 newborns and infants with tricuspid atresia were compared to data from 30 age-matched controls with normal hearts. In both patient and control groups, growth of the left ventricle was not linear but related to the 1.4 (volume) or 1.25 (mass) power of body surface area. The equation best describing relation between left ventricular volume and body surface area in normals is volume = 60.7(body surface area)1.4−0.2 ml; in patients with tricuspid atresia volume calculates as 96.7(body surface area)1.4+0.9 ml. In relation to normal infants, infants with tricuspid atresia had a left ventricular volume of 167 (135–206)% of normal and a left ventricular mass of 163 (132–201)% of normal. Left ventricular mass assessed in normals calculates as mass = 59.9(body surface area)1.25+1.3 grams; in patients with tricuspid atresia it calculates as mass= 117.5 (body surface area)1.25−0.9 grams. The index of left ventricular mass to volume in patients with tricuspid atresia measured 1.32, not significantly different from controls, in whom this index calculates as 1.34.
Ectopic accessory liver masquerading as spleen in a case with isomerism of the right atrial appendages
Authors: Ho Siew Yen , Trotter Simon E. , Redington Andrew N.
Published: August 2008

We report a case of an infant with isomerism of the right atrial appendages in whom a spleen-like nodule was found at post-mortem. The nodule, on histology, was found to be a fragment of liver. The cardiac lesions were typical of those associated with right isomerism, a complex generally described as the “asplenia syndrome.” The presence of either a spleen, or a nodule masquerading as a spleen, does not preclude an isomeric arrangement of the atrial appendages nor its commonly associated constellation of cardiovascular anomalies.
The role of color-flow mapping in confirming the association of divided left atrium with an atrioventricular septal defect
Authors: Cazzaniga Mario , Gómez Manuel Casanova , de Melo Walquiria Domínguez
Published: August 2008

We describe the unusual association of a divided left atrium (cor triatriatum) with an atrioventricular septal defect with separate atrioventricular orifices in an eight-year-old asymptomatic girl. The diagnosis had been made by cross-sectional echocardiography combined with Doppler technology and color-flow mapping. These methods together demonstrate both the anatomic and hemodynamic findings.