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Biomedical subjects

R A Arcilla

Publications and source records attributed to R A Arcilla.

At least 19 recordsLinked to original sources

Technical modifications for improved results in total anomalous pulmonary venous drainage.

To delineate factors that contribute to improved surgical outcome in patients with total anomalous pulmonary venous drainage, we reviewed the records of 52 consecutive patients. Venous drainage was supracardiac in 25 (48%), cardiac in 12 (23%), infracardiac in 10 (19%), and mixed in five (10%). Preoperative pulmonary venous obstruction was present in 18 patients (35%). Median age at the time of repair was 35 days and weight, 3.7 kg. Repair was performed with deep hypothermia, low-flow cardiopulmonary bypass, and occasional short periods of circulatory arrest. In patients with coronary sinus drainage, the veins were tunneled to the left atrium through an enlarged atrial septal defect, with a mortality of 8% (1/12) and no postoperative stenosis. The approach in patients with supracardiac, infracardiac, and mixed drainage varied with time. In 16 patients, the condition was managed by apical or right-sided exposure of the common vein, anastomosis of the common vein to the left atrium with continuous sutures, and primary closure of the atrial septal defect (type I repair). In the other 24 patients the common vein was approached from the right side through the right atrium and the interatrial septum. Common vein-left atrium anastomosis was performed with interrupted sutures and a piece of pericardium used to augment the anastomosis, prevent common vein distortion, and close the atrial septal defect (type II repair). Mortality in type I repair was 25% (4/16) and in type II repair, 4% (1/24). Follow-up was 7.86 +/- 4.0 years with no late deaths. Postoperative stenosis occurred in five of 14 (36%) patients who had type I repair versus two of 23 (9%) who had type II repair. Multivariate analysis showed that type I repair was a positive risk factor for hospital mortality (p = 0.05) and restenosis (p = 0.04). The technique of transatrial exposure of the common venous chamber, interrupted suturing of the common vein to the left atrium, and pericardial patch augmentation significantly improves survival and decreases risk of restenosis.

Anastomosis, Surgical

Extended aortic valvuloplasty: a new approach for the management of congenital valvar aortic stenosis.

A new technique for the treatment of congenital valvar aortic stenosis is described. It consists of augmenting the aortic cusp by extending the commissurotomy incision into the aortic wall around the leaflet insertion, mobilizing the valve cusp attachment at the commissures, and freeing the aortic insertion of the rudimentary commissure. The results of standard valvotomy performed on 48 patients (group 1) were compared with those of the new extended valvuloplasty carried out on 16 patients (group 2). The two groups were comparable in age at operation (2.7 +/- 2.1 years for group 1 versus 2.1 +/- 1.7 years for group 2; p = not significant) and in preoperative pressure gradient (58 +/- 25 mm Hg for group 1 versus 61 +/- 36 mm Hg for group 2; p = not significant). There was no operative mortality in either group. Follow-up is available on all patients, with a mean of 4.3 +/- 2.6 years for group 1 versus 1.7 +/- 0.5 years for group 2 (p = 0.05). There was one late death in group 1. Postoperative gradient was 47 +/- 13 mm Hg in group 1 versus 19 +/- 13 mm Hg in group 2 (p = 0.05). Moderate or severe regurgitation was present in 18 patients (38%) in group 1 and 2 patients (13%) in group 2 (p = not significant). Reoperation was needed in 8 patients (17%) in group 1 versus 2 patients (13%) in group 2 (p = not significant). The described valvuloplasty procedure addresses the unique pathological features of valvar aortic stenosis and provides better relief of the obstruction than the presently available techniques. Longer follow-up is needed to determine the late results of this approach.

Aortic Valve

Advantages of early relief of subaortic stenosis in single ventricle equivalents.

Thirteen patients with single ventricle equivalents and subaortic stenosis underwent relief of the stenosis and subsequent Fontan operation. Nine patients, group 1, had the obstruction relieved at 3.6 +/- 1.6 years of age whenever the pressure gradient became apparent. Four patients, group 2, had the subaortic stenosis operated on at the neonatal period, 10.5 +/- 10 days old, before hemodynamic evidence of obstruction. Preoperative pressure gradient across the outflow tract was 44.2 +/- 4.7 mm Hg in group 1 versus 4.7 +/- 5 mm Hg in group 2 (p = 0.002). Ventricular muscle mass was 186% +/- 18% in group 1 versus 114% +/- 5% of normal in group 2 (p = 0.0001), and mass/volume ratio was 1.12 +/- 0.62 in group 1 versus 0.62 +/- 0.16 in group 2 (p = 0.003). Relief of subaortic stenosis was achieved by proximal pulmonary artery to ascending aorta or aortic arch anastomosis and by systemic to distal pulmonary artery shunt. There was no hospital mortality or complication related to the procedure. At evaluation before Fontan operation, 4.3 +/- 1.6 years after relief of subaortic stenosis in group 1 and 3.2 +/- 0.9 years in group 2, the pressure gradient across the ventricular outflow tract was 4 +/- 3 mm Hg in group 1 versus 3 +/- 2 mm Hg in group 2 (p = not significant), ventricular muscle mass was 184% +/- 31% in group 1 versus 114% +/- 5% of normal in group 2 (p = 0.003), and the mass/volume ratio was 1.17 +/- 0.2 in group 1 versus 0.62 +/- 0.2 in group 2 (p = 0.003).(ABSTRACT TRUNCATED AT 250 WORDS)

Aorta

Surgical options in subaortic stenosis associated with endocardial cushion defects.

Over a 15-year period, 12 patients with endocardial cushion defects undergoing correction had subaortic stenosis requiring operative intervention. Ages ranged from 4 months to 17 years (mean, 7 +/- 6 years) and subaortic gradients from 15 to 100 mm Hg (mean, 60 +/- 25 mm Hg). Subaortic stenosis was due to discrete fibromuscular tissues in 7 patients, mitral valve malattachment in 3, and tunnel outflow in 2. In 2, the subaortic stenosis was clinically significant at the time of endocardial cushion defects repair, whereas in 10 it was noted 2 to 14 years postoperatively (mean, 6.3 +/- 5 years). Surgical relief of subaortic stenosis was accomplished by resection of muscle tissues in 7, apicoaortic conduit insertion in 2, modified Konno procedure (aortic valve preserved) in 2, and lifting of malattached mitral valve from the outflow in 1. There was no early death and one late death (infected conduit). Severe mitral insufficiency developed in the patient who had the mitral valve lifted and necessitated valve replacement. Postoperative echocardiographic gradient in 9 patients ranged from 0 to 36 mm Hg (mean, 10.5 +/- 14 mm Hg). Clinically significant subaortic stenosis has not developed in any patient in 15 years of follow-up (mean, 5 +/- 4 years). We conclude that in subaortic stenosis associated with endocardial cushion defects, resection is effective for discrete obstruction, whereas a modified Konno procedure is preferable for obstruction due to tunnel outflow or mitral valve malattachment.

Aortic Valve Stenosis

Conal enlargement for diffuse subaortic stenosis.

Twelve patients underwent conal enlargement for diffuse subaortic stenosis over a 3 1/2-year period. The subaortic stenosis was due to tunnel outflow in 11 and malattached mitral valve in one. Mean age was 4.4 +/- 4 years and mean subaortic gradient was 50 +/- 21 mm Hg. Three infants had a malalignment ventricular septal defect. In eight patients significant obstruction occurred 2 to 7 years (mean 4 +/- 2) after simple resection of subaortic stenosis (n = 2), ventricular septal defect closure (n = 2), ventricular septal defect closure and subaortic stenosis resection (n = 2), and canal repair (n = 2). In three infants the tunnel outflow distal to a malalignment ventricular septal defect was enlarged and closed with the defect. In three patients with subaortic stenosis proximal to a previously repaired ventricular septal defect, transatrial conal enlargement through the ventricular septal defect was performed. Another patient without a ventricular septal defect had transatrial conal enlargement. The remaining five patients had the modified Konno procedure. Two patients had postoperative complete heart block and one infant had insertion of an apicoaortic conduit for aortic anulus hypoplasia 9 months later. One patient died of pneumonia during the follow-up period. Postoperative echographic outflow gradients up to 3 1/2 years (mean 1.2 +/- 1) ranged up to 25 mm Hg (mean 7 +/- 11) and were mainly at the aortic level. The 11 surviving patients are doing well up to 3 1/2 years of follow-up (mean 1.5 +/- 1). We conclude that conal enlargement procedures with aortic valve preservation are preferable, effective, and can be safely performed for diffuse subaortic stenosis in infants and children.

Aortic Valve

Cardiovascular effects of hypertransfusion therapy in children with sickle cell anemia.

Thirteen children, age 1.9 to 14.8 years with documented sickle cell disease, underwent echocardiographic assessment of cardiac status while on and off periodic hypertransfusion therapy (HTX). Two to three units of washed packed red blood cells were transfused every 2-4 weeks in children with splenic sequestration crises, cerebrovascular accidents (CVA), aseptic necrosis of the femoral head, and miscellaneous complications of sickle cell disease to maintain hemoglobin (Hgb) concentrations of greater than or equal to 10 g/dl and % sickle hemoglobin (S Hgb) of less than or equal to 20%. This therapy administered over an average duration of 24 months resulted in normalization of left heart chamber enlargement and statistically significant decrease in heart rate, left ventricular mass, and cardiac output. Echocardiographically derived left ventricular function parameters remained normal on and off transfusion therapy. Changes in left ventricular diastolic dimension and cardiac output correlated with changes in % S Hgb (r = 0.59, p less than 0.001; and r = 0.54, p less than 0.001, respectively), and with changes in Hgb concentration (r = -0.78, r = -0.76, p less than 0.001). Expression of left heart abnormalities as a single composite function (Ydv), using multivariate regression analysis, allowed a comparison of cardiac status of 99 normal black controls, nontransfused sickle cell anemia (SCA) patients, and 13 study patients on and off HTX, and permitted serial assessment of cardiac status on and off treatment over 5 years in a single patient.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

Transatrial relief of diffuse subaortic stenosis after ventricular septal defect closure.

Transatrial enlargement of the left ventricular outflow tract for serious obstruction was performed in 3 patients with previous ventricular septal defect closure. Two patients had recurrent subaortic stenosis as resection had already been performed at initial operation. In all patients, the obstruction was located below the ventricular septal defect patch. Patch enlargement of the left ventricular outflow tract was carried out by opening the ventricular septal defect patch through the tricuspid valve and extending the incision downward through the area of obstruction and the left ventricular body. All patients had uneventful postoperative course and effective relief of left ventricular outflow tract obstruction. We feel that the approach is simple and effective; it avoids a right ventriculotomy and provides a viable option in certain patients with left ventricular outflow tract obstruction.

Aorta

Cardiac abnormalities in children with sickle cell anemia.

The cardiac status of 64 children (ages 0.2 to 18 yr) with sickle cell anemia documented by hemoglobin electrophoresis was evaluated by echocardiography. Left atrial, left ventricular and aortic root dimensions were significantly increased in over 60 percent of these children at all ages compared to values for 99 normal black (non-SCA) control subjects. Left ventricular wall thickness was increased in only 20 percent of older children with sickle cell anemia. Estimated LV mass/m2 and left ventricular cardiac index were increased compared to control subjects (p less than 0.001). Left heart abnormalities expressed as a single composite function, derived from multivariate regression analysis, correlated well with severity of anemia expressed as grams of hemoglobin (r = -0.52, p = less than 0.001) and with percentage of hemoglobin S (r = 0.51, p less than 0.001), but not to the same extent with age. Echocardiographically assessed left ventricular function at rest was comparable to that of control subjects. These data suggest that the major cardiac abnormalities in children are related to the volume overload effects of chronic anemia, and that in this age group, there is no evidence for a distinct "sickle cell cardiomyopathy" or cardiac dysfunction.

Anemia, Sickle Cell

Small aortic valve annulus in children with fixed subaortic stenosis.

Twenty-one hearts with fixed subaortic stenosis (FSAS) were examined pathologically. Thirty children with no hemodynamically significant heart disease, 31 children with valvar aortic stenosis, and 25 children with FSAS were studied by echo- and angiocardiography. The following conclusions were drawn: (1) Patients with FSAS often have abnormal aortic valve leaflets as well as small aortic valve annulus. (2) A small aortic annulus/descending aorta ratio is probably present at birth, and may decrease with increasing age. (3) In some patients with FSAS the aortic valve annulus is too small for simple resection of the fibroelastic tissue. A Konno operation is needed for these patients. (4) M-mode echocardiography has not been useful in identifying abnormally small aortic valve annulus in FSAS patients.

Adolescent

Congenital left atrial aneurysm in an infant.

Congenital left atrial aneurysm, without associated cardiac abnormalities, is a rare defect. We report the case of a large left atrial aneurysm filling almost the entire left hemothorax. This is the first report of this anomaly occurring in an infant less than 1 year of age.

Female

Aortic root replacement for complicated bacterial endocarditis in an infant.

We report the case of a 10-week-old girl with streptococcal aortic valve endocarditis that was resistant to medical management and complicated by aortic insufficiency, congestive heart failure, annular abscess, and a large periaortic pseudoaneurysm. She underwent successful aortic valve and root replacement (with coronary artery reimplantation) utilizing an aortic valve homograft (allograft). The patient recovered completely and was discharged 3 weeks postoperatively. To our knowledge, this is the youngest patient to receive an aortic homograft for bacterial endocarditis and annular abscess.

Aorta

Echocardiographic functions and blood pressure levels in children and young adults from a biracial population: the Bogalusa Heart Study.

M-mode echocardiograms were obtained on 651 healthy subjects, 7-22 years of age, whose diastolic blood pressure levels remained in the same height-, race-, and sex-specific decile during two biannual examinations. Echocardiographic measures of heart size and dynamics were compared across the total blood pressure distribution. Left ventricular stroke volume, cardiac output and ejection fraction, minor axis shortening, velocity of circumferential fiber shortening, and peripheral vascular resistance were correlated with blood pressure levels. There were positive correlations (p less than .001) of cardiac output and stroke volume with both systolic and diastolic blood pressure levels. Left ventricular output and stroke volume were associated with measures of body size, especially height, weight, ponderal index, and body surface area (p less than .001). The left ventricular output and stroke volume increased with age and with systolic blood pressure quintiles in the four race-sex groups. With adjustment for systolic blood pressure and measures of body size, white males had greater cardiac output (1.25 l/minute for ages 18-22 years, p = .01) and stroke volume than black males. Black males had higher peripheral resistance (4.5 mm Hg/(l/minute), p = .01) than whites. These results suggest that different hemodynamic mechanisms operate in the early phase of hypertension in blacks vs. whites in this population.

Adolescent

Horizontal ventricular septum with dextroversion: hearts with and without aortic atresia.

Two hearts with horizontal ventricular septum, dextroversion (situs solitus), ventricular septal defects, and malaligned great vessels are reported. One of the hearts had aortic atresia and the infant died; the other patient had a Fontan-type physiologic correction. Reviewing the literature, the following conclusions are drawn: (a) Hearts with horizontal ventricular septum and those with criss-cross atrioventricular connections may be the result of different degrees of rotation of the ventricular muscle mass. This rotation is not likely to be postseptational but preseptational. (b) Only those hearts with a complete 180 degrees rotation should be called criss-cross hearts. (c) Partial rotation results in a horizontal septum such that the right ventricle is invariably superior, regardless of atrioventricular concordance or discordance, situs solitus or inversus, or dextroversion. (d) Physiologic surgical correction is often possible but has to be tailored to the details of each heart.

Aorta

M-mode echocardiography in normal children and adolescents: some new perspectives.

Normal M-mode echocardiography values were determined using computer-assisted measurements of echocardiograms (ECHO) in 202 children and young adults 25 days to 23 years of age: 77 were female, and 125 were male and, reflecting the population served by our Center, 99 were black and 103 were white children. The values for left and right heart wall thicknesses and chamber sizes were graphically displayed as a function of body surface area, and with an illustration of the regression line and 2 standard deviation (SD) range of normal for each parameter. In addition, normal ECHO predicting equations for dimension and function parameters were derived using multiple linear regression analysis with age, height, weight, sex, race, and heart rate as independent variables. A comparison was made between the observed data and the data derived from the normal predicting equations for each of the parameters. Also, values obtained from these equations were compared to data generated from other published normal predicting equations. A description of the digitizer measurements, computer interfacing, and a sample ECHO report form utilizing the predicted normal ranges for each of the parameters is presented. We propose that quantitative M-mode echocardiographic reporting should be easily accessible to all pediatric cardiology laboratories.

Adolescent

Blood pressure and echocardiographic measures in children: the Bogalusa Heart Study.

M mode echocardiograms were obtained from 654 healthy subjects, 7 to 22 years of age, whose diastolic blood pressure levels remained in the same height-, race-, and sex-specific decile during two biannual examinations. Echocardiographic measures of heart size, obtained according to the recommendations of the American Society for Echocardiography, were compared across the entire systolic and diastolic blood pressure distributions. Echocardiographic indexes of left heart size varied as a function of both blood pressure levels and body size. Significant positive correlations were present between systolic blood pressure and different measures of left ventricular size. Left ventricular wall thickness in systole correlated with systolic blood pressure (r = .42, p less than .0001), and persistence of this relationship was noted after adjustment for body size. Left ventricular wall thickness in diastole correlated with blood pressure before adjustment (r = .31, p less than .0001), but the relationship was not significant after adjustment for body size. The ratio of left ventricular thickness to chamber size (systole) correlated with systolic blood pressure levels both before and after adjustment for body size (r = .20 and r = .22, p less than .001). Male subjects of both races demonstrated significantly higher adjusted left ventricular mass, left ventricular wall thickness, and left ventricular chamber size. Adjusted left ventricular wall stress was significantly related to both systolic (r = .14) and diastolic blood pressure levels (r = .14, p less than .001). Measures of left ventricular wall thickness increased throughout the entire blood pressure distribution, indicating a consistent trend rather than a threshold effect seen only in the highest blood pressure groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

Ventricular trabeculations in the chick embryo heart and their contribution to ventricular and muscular septal development.

Sixty-two chick embryo hearts were studied at incremental stages of development (Hamburger-Hamilton stages 16 to 39) by scanning electron microscopy following 3% glutaraldehyde fixation and critical point drying. Early in cardiac development, the primitive ventricle becomes homogeneously trabeculated with highly organized sheets of myocytes lined by endocardial cells, with the trabeculae generally oriented in the dorsoventral direction. Coalescence of these trabecular sheets begins at stage 26, initially at the area of the bulboventricular flange, and later proceeding caudally toward the floor of the ventricle. The fusion process is finished by stage 30, resulting in a muscular ventricular septum that has now divided the primitive ventricle into right and left ventricles. Further growth of the ventricular septum is by continued fusion of the adjoining trabecular sheets. Remnants of the apposing trabecular sheets are found in the solidified muscular septum in the form of endocardial channels. We suggest that persistent patency of these channels results in muscular ventricular septal defects.

Animals

Electrophysiologic abnormalities of children with ostium secundum atrial septal defect.

Sinus node (SN) and atrioventricular node (AVN) function were evaluated in 49 patients with secundum type atrial septal defect (ASD). Automaticity and conduction system function were assessed by intracardiac recording of the AH and HV intervals at rest, corrected SN recovery time, sinoatrial conduction time, AVN refractory period and the ability of the AVN to conduct rapidly paced atrial beats to the ventricles. Electrophysiologic abnormalities were found in 41% of the 34 patients who were studied before surgery. However, no preoperative abnormalities were present in children younger than 2.5 years. If only children older than 2.5 years were analyzed, the incidence of conduction abnormalities was similar for the patients studied before operation (62%) and those studied after operation (71%). The size and ejection fractions of the right and left ventricles, the magnitude of shunt flow and the size of the ASD did not differ between the patients with and those without electrophysiologic abnormalities. AVN dysfunction was present in 40% of the patients who were studied after surgical repair. While this frequency was more than twice the preoperative incidence of AVN dysfunction, it was not statistically significant. The data suggest that patient age is the major factor that influences the presence of conduction system dysfunction in patients with ASD.

Adolescent

Cardiovascular responses to PEEP and CPAP following repair of complicated congenital heart defects.

Fourteen infants and children ranging in age from 7 months to 8 years were studied in a hemodynamically stable condition following repair of various heart defects. Changes in cardiac index, stroke index, heart rate, systemic vascular resistance, mean arterial pressure, and central venous pressure were evaluated at levels of 0, 5, and 10 cm H2O using positive end-expiratory pressure (PEEP) in 14 patients and continuous positive airway pressure (CPAP) in 3 patients. No significant changes were found in any of the measurements taken at the different levels.

Blood Pressure