Prevalence of epilepsy in Bogota, Colombia.

A combined epidemiologic, clinical, and electroencephalographic study of epilepsy was undertaken in Bogota by the Neurological Institute of Colombia, the Colombian Institute of Health, and the Department of Health of the City of Bogota. The prevalence of epilepsy on December 31, 1974, was 19.5 per 1,000, the highest rate for general populations reported in the literature. The prevalence was significantly higher for females than for males, 22.9 per 1,000 compared with 15.5 per 1,000.

Growth response of endothelial and smooth muscle cells to a collagen matrix throughout development.

The in vitro response of chick embryo endothelial and smooth muscle cells from aorta explants, induced to grow on a collagen type I matrix, was investigated at different stages of development. At early stages of embryonic development both cell types reacted with collagen as a substratum. At later stages, the endothelial cells had a lower response, whereas the response of muscle cells remained constant. Further evidence is given for the spatial-specific behaviour demonstrated by endothelial cells, which remained on the surface while smooth muscle cells invaded the matrix. Results further highlight the possible role of endothelial cell polarity on the cell-collagen interaction and growing response.

Development of the aorta in the chick embryo: structural and ultrastructural study.

A structural and ultrastructural study was designed to analyze systematically the cellular events which take place in the aortic wall between days 7 and 21 of chick embryo development. Between days 7 and 18, increase in total diameter, number of cell layers, and aortic wall thickness are highly correlated, whereas between days 18 and 21 the total diameter increase is correlated mainly with an increase in vessel lumen diameter. Cell layers of smooth muscle cells showing an immature or synthetic phenotype arise from progressive association and organization of mesenchymal cells originated from an endothelial activation process in which a hyaluronic acid-rich extracellular matrix seems to be involved. It is suggested that the process of endothelial activation takes place between days 7 and 18 of embryonic development provided that within that period the typical cellular events which are involved in such a process take place (hypertrophy, reorientation, invagination, mitotic activity, acquisition of migratory appendages, endothelial detachment and incorporation into adjacent spaces). This endothelial activation has been recognized as a selective multiphasic process required for the transition of endothelial cells into mesenchyma.

Surgical and medical results of complete atrioventricular canal: a ten year review.

The files of 121 patients who presented to Children's Hospital of Michigan over the last 10 years with complete atrioventricular (AV) canal were reviewed to evaluate long-term management and overall outcome. Of 121 patients, 70 underwent corrective surgery, 21 (30%) of whom died perioperatively. The surgical mortality rate was 13% when patients with hypoplastic left or right ventricle (n = 6), double-orifice mitral valve or extreme deficiency of mitral tissue (n = 5), and pulmonary vascular obstructive disease (n = 5) were excluded. Of the 49 patients who survived operation, 36 are in New York Heart Association class I, 1 patient requires a pacemaker and 3 died late. In 34 of the 51 patients (28%) who did not undergo operation, pulmonary vascular obstructive disease developed; it occurred within 12 months in 10 patients (8%). Eight other patients who did not undergo operation died before planned surgery (age 1 to 9 months). Although surgical prognosis in good candidates is acceptable, the overall prognosis for children with complete AV canal is guarded because of the risk of early death or early pulmonary vascular obstructive disease and frequently unfavorable anatomy.

Fatal air embolism during thoracotomy for gunshot injury to the lung. Report of a case.

Fatal coronary air embolism occurred during thoracotomy in a patient with a gunshot wound involving the hilum of the right lung. Embolism was observed during a second period of failure of heart action. Evidently, air entered the pulmonary veins from the bronchus, which was receiving positive-pressure ventilation. The literature contains reports of only 3 similar cases, but we suspect that air embolism may be responsible for death and morbidity in additional cases in which accidental or iatrogenic lung trauma has produced a pathway between the bronchial tree and the pulmonary veins.

Stenosis of the coronary sinus ostium. An unusual site of obstruction in total anomalous pulmonary venous drainage.

A patient with total anomalous pulmonary venous connection to the coronary sinus and stenosis of the right atrial opening of the coronary sinus is described. While the initial studies were compatible with increased pulmonary blood flow, subsequent evaluation demonstrated severe pulmonary venous obstruction. Successful operative correction was performed.

Results of total correction of tetralogy of Fallot with complete atrioventricular canal.

Four patients with tetralogy of Fallot (TF) and complete atrioventricular canal (CAVC) underwent successful intracardiac repair. All patients had undergone preliminary palliative shunts. Associated cardiovascular lesions included muscular ventricular septal defect (VSD) and left superior vena cava (one patient), bilateral main pulmonary artery stenosis (one patient), and right aortic arch (two patients). The diagnosis was suspected clinically by electrocardiographic evidence of right ventricular hypertrophy and left anterior hemiblock, by echocardiographic findings suggestive of CAVC with aortic dextroposition, and by the association with trisomy 21. The diagnosis was confirmed by cardiac catheterization and cineangiography. Total correction consisted of closure of the VSD and atrial septal defect (ASD), reconstruction of the atrioventricular valves, and relief of the right ventricular outflow tract obstruction (RVOTO). There were no early or late postoperative deaths. Surgically induced complete heart block did not occur. One patient underwent successful reoperation for residual VSD and right ventricular outflow aneurysm. Late hemodynamic evaluation revealed good results in all patients. We recommend a staged treatment plan consisting of preliminary systemic--pulmonary artery shunting for symptomatic children under 4 to 5 years of age and total correction for older children. With proper preoperative diagnosis and accurate intracardiac repair, good results may be obtained after total correction of TF and CAVC.

Intrapericardial teratoma in infancy.

Intrapericardial teratoma is a rare, congenital, pedunculated tumor, usually attached to the ascending aorta and associated with massive pericardial effusion, cardiac compression, and severe cardiorespiratory distress in infants and young children. Echocardiography and cardiac cineangiography are diagnostic and should be used promptly. The tumor is generally benign and early surgical removal is curative. The case reports of two infants who underwent successful surgical removal of their lesions are presented.

Results of two-stage surgical treatment of tetralogy of Fallot.

One hundred nine consecutive patients underent 115 palliative shunts between 1971 and 1979 for the initial management of symptomatic tetralogy of Fallot. A single Blalock-Taussig shunt was done in 78 patients, including 38 infants below 12 months of age, with two deaths (2.5% mortality rate). Thirty-two patients underwent a Waterston shunt, with one death (3.1% mortality rate). The total early shunt mortality rate was 2.7% (three deaths among 109 patients). There was one later noncardiac death and one instance of nonfatal brain abscess between the two stages. Fifty-nine of the previously shunted patients have undergone total correction at a mean postshunt interval of 37.1 months, with one early postoperative death (1.6% mortality rate). The mean patient age at total repair was 4.8 years. The two-stage combined operative mortality rate was 3.6% (four deaths among 109 patients). The Waterston shunt is considered undesirable because it complicated the total repair and was associated with a high incidence of residual right pulmonary artery obstruction at postoperative cardiac catheterization. The Blalock-Taussig shunt had a low mortality rate, high long-term patency rate, absence of interval complications, and no adverse effect at the time of total correction. It is considered the shunt of choice in all symptomatic infants and small children with tetralogy of Fallot. Our experience suggests that, at the present time, the two-stage surgical approach compares favorably with primary total correction, especially in infants under 1 year of age.

Changes in plasma fibronectin in children after elective repair of congenital heart defects.

Plasma fibronectin is an attachment protein important for maintaining capillary integrity and host defense mechanisms. Depletion of plasma fibronectin has been shown to occur in adults after septic shock, major trauma, and burns. Limited laboratory and clinical studies suggest a correlation between decreased plasma fibronectin levels and increased pulmonary capillary permeability and tissue perfusion. Mild and transient plasma fibronectin depletion has been observed in adults after cardiovascular operations. We measured plasma fibronectin by immunoturbidometric assay in 20 children (age 6 months to 12 years) undergoing repair of congenital heart defects. Plasma fibronectin levels immediately after operations and daily thereafter were compared with the preoperative values. Plasma fibronectin declined on postoperative days 1, 2, 3, 4, and 5 (p < 0.05). A nadir was reached on day 3 with a tendency toward recovery thereafter. Patients with a therapeutic intervention score of more than 35 had greater magnitude of plasma fibronectin decline than those with a score of less than 35 at 24 hours after the operation (p < 0.005). We conclude that (1) significant and prolonged plasma fibronectin depletion occurs after cardiovascular operations in children; and (2) postoperative plasma fibronectin depletion is associated with increasingly complex surgical intervention. Reduced plasma fibronectin synthesis and more extensive operations for congenital heart defects are likely reasons for children being more susceptible than adults to plasma fibronectin depletion after cardiovascular operations.

Early and late results of total correction of tetralogy of Fallot.

Two hundred and nine patients underwent total repair of tetralogy of Fallot without congenital pulmonary atresia from 1971 to 1979. The age of the patients ranged from 22 months to 27 years (mean 6.8 years). Previous palliative shunts were present in 130 patients (62.2%). The right ventricular outflow tract obstruction was relieved by infundibulectomy in 31 patients (14.8%), by transannular patching in 142 patients (67.9%), and by insertion of a right ventricular--to--pulmonary artery prosthetic conduit in 10 patients (4.7%) with associated coronary artery anomalies. A porcine valve was inserted after transannular patching only in patients with undersized pulmonary arteries, unilateral pulmonary artery, or congenitally absent pulmonary valve. There were 10 early postoperative deaths (4.7%). Previous palliative shunts did not adversely affect early postoperative mortality rate. Surgically induced permanent complete heart block occurred in one patient (0.4%). Six patients (3%) died in the late postoperative period, three of them of unrelated causes. Late results were good in 170 patients (87%), fair in 10 patients (5.1%), and poor in 15 patients (7.6%). Poor results were due to residual stenosis at the origin of the pulmonary arteries in 13 patients (6.5%) and to residual ventricular septal defect in two patients (1%). The mean cardiothoracic ratio was 0.61 for patients with poor results and 0.54 for the entire group. Pulmonary valvular insufficiency was well tolerated postoperatively in the absence of distal pulmonary artery obstruction. This experience supports a policy of aggressive relief of the right ventricular outflow tract obstruction including liberal use of transannular patching and, when indicated, extensive reconstruction of the pulmonary artery branches. We also recommend a two-stage treatment program for symptomatic infants with unfavorable anatomy consisting of initial Blalock-Taussig shunt followed by total repair at about 3 years of age.

Surgical management of congenital vascular rings.

Case histories of 53 patients with surgically treated vascular rings are presented. The diagnosis was strongly suggested in all patients by symptoms of tracheosophageal obstruction and was confirmed by barium esophagogram, which showed typical bilateral and posterior indentations in the esophagus. Angiography was rarely used although it is helpful in doubtful cases. Endoscopic studies were considered unnecessary and potentially harmful. A left posterolateral thoractomy afforded ample access for division of the ring in all patients. Two patients died who were operated upon during the early experience. There were no postoperative deaths among the last 45 consecutive patients. All survivors had excellent symptomatic relief although complete resolution of symptoms took several months in some patients. All survivors had excellent symptomatic relief although complete resolution of symptoms took several months in some patients. The importance of early diagnosis and prompt surgical division is emphasized.

Classic shunting operations for congenital cyanotic heart defects.

A total of 297 consecutive classic systemic artery-pulmonary artery shunts were performed in 281 patients over the past 9 years. This experience includes 200 Blalock-Taussig shunts (67.3%), 84 Waterston shunts (28.2%), and 13 Potts shunts (4.3%). The overall early mortality after the Blalock-Taussig shunt (5.5%) was significantly lower (p less than 0.02) than for the Waterston shunt (13%). The early mortality after either the Blalock-Taussig or Waterston shunt was not statistically related to the age of the patient at operation. Both the Blalock-Taussig and Waterston shunts demonstrated a higher mortality (p less than 0.02) among patients in Group II (complex defects) than in patients in Group I (tetralogy of Fallot and pulmonary atresia with ventricular septal defect [VSD]). However, the Blalock-Taussig shunt had a significantly lower (p less than 0.04) probability of early postoperative death than the Waterston shunt among patients with complex lesions. The Blalock-Taussig shunt also revealed a lower incidence of important late postoperative complications and did not affect adversely the hemodynamic result after intracardiac correction, as evidenced by late postoperative cardiac catheterization. This study also confirms the feasibility of the Blalock-Taussig shunt during the neonatal period without significantly increased age-related risk of postoperative death (p = 0.13) and with good early and late long-term patency rates.

Primary cardiac tumors in children.

Case histories of five children with primary cardiac tumors are presented. The clinical and pathological features of the most frequent cardiac neoplasms in childhood are also reviewed. Cardiac tumors should be suspected in any child with unexplained heart murmurs, congestive heart failure, or cardiac arrhythmias. Echocardiography, cardiac catheterization, and biplane cineangiograms provide diagnostic confirmation. Prompt surgical resection is indicated in all symptomatic patients and in those with significant inflow and outflow tumor-related obstruction. Complete resection is not possible in many patients with cardiac rhabdomyoma or lipoma but removal of the obstructive portion of the tumor frequently provides long-term symptomatic relief.

Results of the Mustard operation for dextro-transposition of the great arteries.

One hundred twenty patients with dextro-transposition of the great arteries (TGA) underwent intra-arterial baffle repair using thin Dacron fabric from 1971 to 1979. The ages of the patients ranging from 29 days to 17 years (mean age 28 months). Thirty-five patients had undergone 49 preliminary palliative operations. Early postoperative mortality was 4.8% for patients with simple TGA but was higher among patients with associated ventricular septal defect (26%), VSD and left ventricular outflow tract obstruction (28.5%) and intact ventricular septum with left ventricular outflow tract obstruction (12.5%). Early nonfatal postoperative complications included low cardiac output (23.3%), respiratory insufficiency (35.8%), junctional rhythm (34.1%), superior vena caval (SVC) obstruction (9.1%), and chylothorax (7.5%). Late postoperative mortality for all groups was 7.5% (nine patients). Late cardiac dysrhythmias occurred in 33 patients (40.7%). Normal sinus rhythm has been preserved in all patients since direct, high SVC cannulation was instituted. Late postoperative hemodynamic and angiographic evaluation in 61 patients revealed severe to total SVC obstruction in 12 patients (20.2%) and pulmonary venous obstruction in four patients (6.5%). Among the latter, two patients died following reoperation and another patient died without operation. SVC obstruction was clinically important in only three patients, two of whom have undergone successful reoperation. All other late survivors are acyanotic and clinically well. The Mustard operation has dramatically improved the survival rate and quality of life for patients with TGA. However, postoperative caval and pulmonary venous obstruction are problems which require additional technical modifications and stimulate the search for alternative corrective operations.

Extracorporeal membrane oxygenation for the circulatory support of children after repair of congenital heart disease.

We have treated 39 infants and children with congenital heart disease with extracorporeal membrane oxygenation during the past 5 years. Thirty-six were treated for low cardiac output or pulmonary vasoreactive crisis after repair of congenital heart defects. Twenty-two (61%) survived. Most patients were cannulated from the neck via the right internal jugular vein and the right common carotid artery. Six patients were cannulated from the chest, including three who had separate drainage of the left side of the heart with a left atrial cannula. Two of these patients survived and were the only survivors of the nine patients cannulated in the operating room because they could not be weaned from cardiopulmonary bypass after open cardiac operations. We also reviewed 312 patients (the predictor study series) having open cardiac operations before the availability of extracorporeal membrane oxygenation; 27 of these patients died. Data were collected at 1 and 8 hours postoperatively to determine if any parameters might predict early mortality. With these parameters used as criteria, patients who went on extracorporeal membrane oxygenation were as sick as those who died before extracorporeal membrane oxygenation was available. The most common complication was bleeding related to heparinization. The mean transfusion requirement in survivors was 1.50 +/- 1.13 ml/kg/hr, 5.63 +/- 7.0 ml/kg/hr in the nonsurvivors, and 7.46 +/- 8.29 ml/kg/hr in those cannulated in the operating room because they could not be weaned from bypass. Four children had intracranial hemorrhage, and two of them died. There was one late death. Nine of the 22 survivors are entirely normal. All survivors who do not have Down's syndrome are considered to have normal central nervous system function. We conclude that extracorporeal membrane oxygenation can improve survival in patients with both pulmonary artery hypertension and low cardiac output after operations for congenital heart disease.

Down's syndrome, complete atrioventricular canal, and pulmonary vascular obstructive disease.

We reviewed our experience over a 10-year period to determine whether children with Down's syndrome and complete atrioventricular canal develop pulmonary vascular obstructive disease earlier than children with normal chromosomes and this defect. Comparisons were made between Down's syndrome and normal chromosome children regarding (1) pulmonary blood flow and pulmonary vascular resistance at initial catheterization, (2) operability as related to elevation in pulmonary vascular resistance, and (3) age at diagnosis of fixed pulmonary vascular obstructive disease. The 45 patients with Down's syndrome catheterized under 1 year of age had a lower mean pulmonary blood flow (3.2 versus 5.7; p = 0.0001) and higher mean pulmonary vascular resistance (8.3 versus 4.6 Wood units.m2; p = 0.0003) than their 34 normal chromosome counterparts. When all ages were included, 38 of 81 (47%) of the children with Down's syndrome and 32 of 40 (80%) of the normal children were considered operable. Non-Down's syndrome patients who had operations had a higher pulmonary blood flow (5.8 versus 3.3; p = 0.004) and lower pulmonary vascular resistance (3.6 versus 6.0 Wood units.m2; p = 0.005) than Down's syndrome patients. Of the 34 patients who did not have operations because of pulmonary vascular obstructive disease, 31 had Down's syndrome. In 10 of 81 children with Down's syndrome, fixed pulmonary vascular obstructive disease was diagnosed before the age of 1 year, while this was found in none of 40 normal children. Our data demonstrate that Down's syndrome patients with complete atrioventricular canal have a greater degree of elevation of pulmonary vascular resistance in the first year of life and more rapid progression to fixed pulmonary vascular obstructive disease than children with normal chromosomes.

Classic shunting operations as part of two-stage repair for tetralogy of Fallot.

One hundred forty-nine consecutive patients with tetralogy of Fallot, with or without pulmonary atresia, underwent Blalock-Taussig or Waterston operation for initial palliation. Of these patients, 45 were less than 6 months old, and 63 were less than 1 year old. The type of shunt, and the presence or absence of pulmonary atresia did not have a significant effect (p greater than 0.2) on hospital mortality. Parametric analysis showed a significant effect of age (p = 0.03), the risk of hospital death being 6% at 1 month of age, 4% at 3 months, 3% at 6 months, and 2.5% at 12 months. No late deaths occurred before the age of 3 years. Six patients (4.2% of the hospital survivors) required another operation before they were 3 years old. Severe arm ischemia occurred after a Blalock-Taussig shunt in 1 infant with Down's syndrome.

Surgical treatment of cor triatriatum.

Six patients with cor triatriatum underwent surgical correction. They ranged for 1.5 to 93 months old (mean, 22 months). Congestive heart failure was present in 3 patients. Cardiomegaly and increased pulmonary vascularity were evident roentgenographically in all patients. Cardiac cineangiography demonstrated the subdividing left atrial membrane in 5 patients and suggested the correct diagnosis by revealing an abnormal configuration of the left atrium in the other patient. The opening in the anomalous left atrial membrane was stenotic in every instance. The proximal left atrial chamber communicated with the right atrium through an atrial septal defect in 5 patients and with the systemic venous circuit through a persistent left superior vena cava in the other patient, in whom the atrial septum was intact. A right atrial-transseptal approach provided ample exposure for complete excision of the obstructing membrane and repair of the atrial septum in all patients. One patient died of low cardiac output during the early postoperative period. The other 5 are alive and well at an average of 48 months after operation.

Concomitant changes in endothelial cell junctions and extracellular matrix components in the chick embryo aorta.

According to previous studies, a process of endothelial activation seems to be occurring in the chick embryo between days 7 and 18. Also, endothelial cells respond to collagen as a substratum between 12 and 18 days, and this response diminishes until it almost disappears after birth. In the present study, aortas from chick embryos (days 7 to 21), and from chicks (14 days posthatching) were used. The results obtained by the freeze-fracturing technique, showed that between days 12 and 14 the intramembranous particles were aggregated into linear or clustered arrays in the fracture P-face of endothelial cells. This could signify that some kind of gap junction-like coupling may occur between adjacent endothelial cells. Our results also indicate that in advanced stages (21-day-old chick embryos and 14-day-old chicks) the growth of small aggregates into larger aggregates or plaques could occur. In addition to gap junctions, the presence of macular and linear tight junctions, reported as focal tight junctions (day 14 of development) macular and linear tight junctions with free-ending strands oriented parallel to one another (21 days) and smooth contoured ridges (14 days post-hatching) were observed. This sequence of changes may represent a development from linear to macular, to a more occluding arrangement, and may also reflect an endothelial cell polarization. Histochemical study of proteoglycans was done by using cuprolinic blue according to the critical electrolyte concentration method. Cuprolinic blue-positive granular, elongated and microfibrillar materials were found in the subendothelial region, forming a meshwork that occupies the extracellular space. Qualitative and quantitative changes were observed both in proteoglycans and in other extracellular matrix components throughout development, suggesting an increase in extracellular matrix complexity. These results lead us to suggest that the assembly of a more complex extracellular matrix, concomitantly with the formation of intercellular junctions during development, might influence the polarization of endothelium in the aorta of the chick embryo.