bluuebunny/crossref_metadata_2025_split huggingface dataset:
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

AbstractInjury 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

AbstractWe 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

AbstractMajor 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.
Epidemiology of cardiovascular malformations: The state of the art
Authors: Ferencz Charlotte , Villasenñor Adolofo Correa 
Published: August 2008

SummaryA 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

SummaryPrevalence 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

SummaryIn 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

SummaryDefects 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.
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

SummaryIn 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

SummaryMalfunctioning 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

SummaryDespite 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

SummaryDuring 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

SummaryBetween 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

SummaryWe 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

SummaryA 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

SummaryThe 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.
Morphology of atrioventricular septal defect and fetal hydrops in the fetal rat
Authors: Momma Kazuo , Ando Masahiko 
Published: August 2008

AbstractWe 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

AbstractWe 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

SummaryTo 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.