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Biomedical subjects

S P Sanders

Publications and source records attributed to S P Sanders.

At least 19 recordsLinked to original sources

Cardiac structure and function in children with human immunodeficiency virus infection treated with zidovudine.

BACKGROUND: Abnormalities of cardiac structure and function are common in children infected with the human immunodeficiency virus (HIV). It is unclear, however, whether these abnormalities are attributable to the disease itself, associated infections, or possible cardiotoxic effects of the most commonly used treatment, zidovudine. METHODS: We performed echocardiography in 24 children with symptomatic HIV infection immediately before they started zidovudine therapy and a mean of 1.32 years after therapy began. Sixteen of these children were also studied a mean of 1.26 years before starting zidovudine treatment. Comparison groups included 27 age-matched children with symptomatic HIV infection who had not received zidovudine and 191 normal children. RESULTS: As compared with the normal children, the children treated with zidovudine had progressive left ventricular dilatation and an increase in ventricular-wall stress at end-systole (a measure of ventricular afterload); dilatation and stress were significantly elevated both before and during zidovudine treatment. The ratio of ventricular thickness to internal dimension was below normal before zidovudine treatment began (P < 0.001). After treatment with zidovudine, however, overall left ventricular mass was increased (P = 0.02), as was peak wall stress (a stimulus to ventricular hypertrophy) (P = 0.01). Ventricular contractility remained normal, but fractional shortening of the left ventricle was decreased (P = 0.004). No statistically significant differences were detected at follow-up in any of these measurements between HIV-infected children treated with zidovudine and those not so treated. CONCLUSIONS: Progressive left ventricular dilatation occurred in children with symptomatic HIV infection. Compensatory hypertrophy also occurred but was inadequate to maintain peak systolic wall stress within the normal range. The progressive elevation of ventricular afterload due to dilatation resulted in depressed ventricular performance, but intrinsic ventricular contractility remained normal. Zidovudine did not appear to worsen or ameliorate these cardiac changes.

Acquired Immunodeficiency Syndrome

Developmental modulation of myocardial mechanics: age- and growth-related alterations in afterload and contractility.

Somatic growth is associated with alterations in myocardial mechanics in children with heart disease and in most animal models of congenital heart disease. However, the effect of age and body size on myocardial contractility and loading conditions in normal infants and children is not known. Therefore, 256 normal children aged 7 days to 19 years (34% less than 3 years old) were evaluated with noninvasive indexes of left ventricular contractility and loading conditions. Two-dimensional and M-mode echocardiographic recordings of the left ventricle were obtained with a phonocardiogram, indirect pulse tracing and blood pressure recordings. Left ventricular dimensions, wall thickness and pressure measurements obtained from these data were used to calculate peak and end-systolic circumferential and meridional wall stress and mean and integrated meridional wall stress. Velocity of shortening adjusted for heart rate was compared with end-systolic stress to assess contractility independently of loading status. The subjects were stratified for gender and each of the derived variables was related to age and body surface area. Ventricular shape, assessed as the major/minor axis ratio, and the circumferential/meridional stress ratio were found to be invariant with growth. The ratio of posterior wall thickness to minor axis dimension did not change with age, despite the normal age-related increase in blood pressure. The increase in pressure despite unvarying ventricular shape and wall thickness/dimension ratio resulted in a substantial increase in wall stress that was most dramatic during the first few years of life. In association with the increase in afterload, systolic function decreased with age. However, the age-related decrease in the velocity of shortening was greater than that expected from the increase in afterload alone, indicating a higher level of contractility in infants and children during the first years of life than in older subjects. The process of normal growth and development, similar to that in children with heart disease, is associated with a rapid decrease in the trophic response to hemodynamic loads, resulting in an age-associated increase in wall stress. There is a similar but somewhat more rapid decrease in contractility, with the highest values seen in the youngest patients.

Adolescent

Fetal echocardiography: accuracy and limitations in a population at high and low risk for heart defects.

OBJECTIVE: Our objective was to assess the accuracy of prenatal echocardiography in detecting congenital heart defects in patients at high and low risk for structural cardiac anomalies. STUDY DESIGN: Sixty-nine consecutive fetuses with congenital heart defects who had had prenatal ultrasonography at greater than or equal to 18 weeks' gestation were evaluated to determine the accuracy of prenatal ultrasonography in identifying structural cardiac defects. Thirty-nine patients were at high risk and 30 patients were at low risk for cardiac anomalies. All fetuses were scanned with standard four-chamber and outflow tract views. Data concerning extracardiac anomalies and karyotypic abnormalities were tabulated. The accuracy of the four-chamber view alone in identifying congenital heart defects was evaluated. RESULTS: Fifty-seven of 69 fetuses (83%) were prenatally identified ultrasonographically as having a heart defect. There was no difference in the sensitivity of detecting cardiac anomalies between high-risk and low-risk groups. When the four-chamber view was used, only 63% of fetuses were recognized as having an abnormal heart. Extracardiac anomalies were noted in 36% and karyotypic abnormalities in 17% of patients. CONCLUSION: The four-chamber and outflow tract views done routinely in an ultrasonography laboratory seeing a mixed population of patients was successful in detecting 83% of fetuses with structural cardiac malformations. Because 43% of the fetuses with heart defects were referred for low-risk indications, systematic ultrasonographic examination of the fetal heart should not be reserved only for those at high risk.

Chromosome Aberrations

Bulboventricular foramen size in infants with double-inlet left ventricle or tricuspid atresia with transposed great arteries: influence on initial palliative operation and rate of growth.

Bulboventricular foramen obstruction may complicate the management of patients with single left ventricle. Bulboventricular foramen size was measured in 28 neonates and infants greater than 5 months old and followed up for 2 to 5 years in those patients whose only systemic outflow was through the foramen. The bulboventricular foramen was measured in two planes by two-dimensional echocardiography, its area calculated and indexed to body surface area. One patient died before surgical treatment. The mean initial bulboventricular foramen area index was 0.94 cm2/m2 in 12 patients (Group A) in whom the foramen was bypassed as the first procedure in early infancy. The remaining 15 patients underwent other palliative operations but the bulboventricular foramen continued to serve as the systemic outflow tract. There was one surgical death. Six (Group B) of the 14 survivors developed bulboventricular foramen obstruction during follow-up (mean initial bulboventricular foramen area index 1.75 cm2/m2). The remaining eight patients (Group C) did not develop obstruction during follow-up and had an initial bulboventricular foramen larger than that in the other two groups (mean initial bulboventricular foramen area index 3.95 cm2/m2). All patients with an initial bulboventricular foramen area index less than 2 cm2/m2 who did not undergo early bulboventricular foramen bypass developed late obstruction.(ABSTRACT TRUNCATED AT 250 WORDS)

Analysis of Variance

Diagnosis of anomalous left coronary artery by Doppler color flow mapping: distinction from other causes of dilated cardiomyopathy.

BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary trunk is difficult to diagnose reliably by two-dimensional echocardiography. Therefore, Doppler color flow mapping was tested in 29 patients with dilated cardiomyopathy or anomalous left coronary artery, or both. METHODS AND RESULTS: All patients with anomalous left coronary artery (10 patients) or dilated cardiomyopathy (27 patients) (excluding those with other known causes for cardiomyopathy) examined between January 1988 and May 1991 were identified. The direction of flow in the three main segments of the left coronary system was determined by Doppler color flow mapping. In all 10 patients with anomalous left coronary artery, flow mapping demonstrated an abnormal jet from the left coronary artery into the pulmonary trunk and retrograde flow in at least two segments of the left coronary system. The diagnosis was confirmed in all 10 patients at operation. Doppler color flow mapping, performed in 19 of the 27 patients with dilated cardiomyopathy, demonstrated anterograde flow in at least one segment of the left coronary system in 16 of the 19 patients; flow direction was not determined in the other 3 patients. Coronary artery anatomy was confirmed by aortic root or left ventricular angiography in 14 patients and at autopsy in 1 patient and was not directly confirmed in 4 patients. Left ventricular function spontaneously improved to normal in three of the latter four patients, a clinical course not consistent with anomalous left coronary artery. The left coronary artery appeared to arise from the aortic root by two-dimensional echocardiographic imaging alone in all patients with dilated cardiomyopathy and in 5 of 10 patients with anomalous left coronary artery (50% false negative diagnoses). CONCLUSIONS: Detection of an abnormal jet into the pulmonary trunk and retrograde flow in the left coronary system by Doppler color flow mapping is reliable for diagnosing anomalous left coronary artery whereas two-dimensional echocardiographic imaging alone is often inconclusive or misleading. Determining flow direction in the left coronary system in patients with dilated cardiomyopathy is useful for excluding anomalous left coronary artery but is technically more difficult to document in this condition than in anomalous left coronary artery.

Adolescent

Antiarrhythmic response to intravenously administered magnesium after cardiac surgery.

We evaluated the antiarrhythmic response to magnesium given intravenously to 40 patients who had had elective cardiopulmonary bypass for the surgical treatment of acquired cardiac disease. All 40 patients were found to have hypomagnesemia postoperatively as defined by a serum magnesium level of less than 1.8 mg/dL. The ventricular arrhythmias of these patients were categorized by a modified Lown grade classification. Magnesium sulfate (16 mEq, [2000 mg]) was administered, and the patients were reclassified according to their rate of ventricular ectopia. All arrhythmias of a higher grade responded to the treatment with a marked reduction in ventricular ectopia and were reclassified to a lower grade. This study supports the routine administration of magnesium sulfate, at a dose of 16 mEq, in postoperative cardiac patients who are hypomagnesemic.

Anti-Arrhythmia Agents

Natural history and patterns of recovery of contractile function in single left ventricle after Fontan operation.

BACKGROUND: Before the era of the Fontan procedure, the typical course of patients with single left ventricle (LV) consisted of heart failure and death during the second or third decade of life. Despite the advent of effective palliative therapy, ventricular dysfunction remains a significant clinical problem for these patients. METHODS AND RESULTS: To investigate the causes of ventricular dysfunction in these patients and to determine whether Fontan-type repair reverses deterioration of LV function, the ventricular dimensions, volume, shape, wall stress, and systolic function were determined by echocardiography in 84 patients 0.2-35 years old with double-inlet single LV or tricuspid atresia. Measurements were obtained in 67 patients after palliation (arterial shunt or pulmonary artery band) and in 47 patients a median of 4.4 years after a Glenn (n = 9) or a Fontan operation (n = 38). Before a Fontan procedure, ventricular volumes were 2 to 3 times normal. Ventricular afterload, assessed as circumferential and meridional end-systolic wall stress, became abnormal after 2 years of age. With age, LV shape changed progressively from ellipsoidal to spherical, as indicated by the decrease in long axis:short axis ratio from normal (1.9) toward unity. Concomitantly, the ratio of circumferential to meridional end-systolic wall stress fell from 1.3 to unity, the ratio of a sphere at equilibrium. This age-related change in shape and load occurred in concert with progressive deterioration of LV systolic function and contractility. Aortic oxygen saturation, an indicator of pulmonary blood flow and therefore volume work in single-ventricle physiology, was inversely and independently correlated with contractility. In the group of patients in whom a Glenn or a Fontan operation was performed at < 10 years of age, ventricular dimensions, volumes, and wall stress diminished and LV function and contractility improved after surgery (p < 0.001). In patients undergoing surgery after 10 years of age, few had improvement of LV function after surgery. Postoperative ventricular function and contractility were inversely related to age at surgery and to aortic oxygen saturation measured before surgery. CONCLUSIONS: Although Fontan-type repair of single ventricle early in life is associated with reversal of the abnormal contractile mechanics associated with age and volume load, this capacity for recovery diminishes with age at surgery.

Cardiac Surgical Procedures

Pulmonary valve-moderator band distance and association with development of double-chambered right ventricle.

Double-chambered right ventricle (DCRV), a form of right ventricular outflow obstruction that sometimes accompanies a ventricular septal defect (VSD), is associated with superior and rightward displacement of the septal insertion of the moderator band. It was hypothesized that this superior displacement is present and identifiable by echocardiography in patients with a VSD even before right ventricular outflow tract obstruction develops. Eight patients who had a previous echocardiographic study showing a VSD alone were echocardiographically diagnosed as having DCRV. Their initial echocardiographic studies were reviewed, and superior displacement of the moderator band was quantified by measuring the distance between the pulmonary valve and moderator band, normalized to tricuspid anulus diameter. These measurements were compared with those from the initial studies of the following 3 other groups: (1) an age-matched group of 10 patients with no structural heart disease; (2) an age-matched group of 10 patients with a VSD who did not develop DCRV; and (3) a group (not age-matched) of 10 patients with VSD and DCRV in whom subpulmonary obstruction was present on the initial study. The 8 patients who eventually developed subpulmonary obstruction had significant superior displacement of the moderator band at the time of their initial echocardiogram compared with that of the 2 age-matched control groups (p less than 0.01). In contrast, there was no significant difference in moderator band displacement between these patients and the 10 with DCRV who already had right ventricular outflow obstruction at their initial study (p = not significant).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

Late cardiac effects of doxorubicin therapy for acute lymphoblastic leukemia in childhood.

BACKGROUND: Cardiotoxicity is a recognized complication of doxorubicin therapy, but the long-term effects of doxorubicin are not well documented. We therefore assessed the cardiac status of 115 children who had been treated for acute lymphoblastic leukemia with doxorubicin 1 to 15 years earlier in whom the disease was in continuous remission. METHODS: Eighteen patients received one dose of doxorubicin (45 mg per square meter of body-surface area), and 97 received multiple doses totaling 228 to 550 mg per square meter (median, 360). The median interval between the end of treatment and the cardiac evaluation was 6.4 years. Our evaluation consisted of a history, 24-hour ambulatory electrocardiographic recording, exercise testing, and echocardiography. RESULTS: Fifty-seven percent of the patients had abnormalities of left ventricular afterload (measured as end-systolic wall stress) or contractility (measured as the stress-velocity index). The cumulative dose of doxorubicin was the most significant predictor of abnormal cardiac function (P less than 0.002). Seventeen percent of patients who received one dose of doxorubicin had slightly elevated age-adjusted afterload, and none had decreased contractility. In contrast, 65 percent of patients who received at least 228 mg of doxorubicin per square meter had increased afterload (59 percent of patients), decreased contractility (23 percent), or both. Increased afterload was due to reduced ventricular wall thickness, not to hypertension or ventricular dilatation. In multivariate analyses restricted to patients who received at least 228 mg of doxorubicin per square meter, the only significant predictive factors were a higher cumulative dose (P = 0.01), which predicted decreased contractility, and an age of less than four years at treatment (P = 0.003), which predicted increased afterload. Afterload increased progressively in 24 of 34 patients evaluated serially (71 percent). Reported symptoms correlated poorly with indexes of exercise tolerance or ventricular function. Eleven patients had congestive heart failure within one year of treatment with doxorubicin; five of them had recurrent heart failure 3.7 to 10.3 years after completing doxorubicin treatment, and two required heart transplantation. No patient had late heart failure as a new event. CONCLUSIONS: Doxorubicin therapy in childhood impairs myocardial growth in a dose-related fashion and results in a progressive increase in left ventricular afterload sometimes accompanied by reduced contractility. We hypothesize that the loss of myocytes during doxorubicin therapy in childhood might result in inadequate left ventricular mass and clinically important heart disease in later years.

Child

Two-dimensional echocardiography in the pre- and postoperative management of totally anomalous pulmonary venous connection.

The records of 23 infants who underwent surgical repair of isolated totally anomalous pulmonary venous connection were reviewed to assess the accuracy of pre- and postoperative echocardiographic diagnoses. Preoperative echocardiographic diagnoses were accurate in 22 of 23 patients, including the sites of connection of the individual pulmonary veins. Cardiac catheterization in 13 patients confirmed the echocardiographic findings. Analysis of multiple pre- and postoperative variables revealed no statistically significant difference between the infants with and without catheterization, although there was a tendency toward a higher mortality rate in the catheterized group. Postoperative echocardiographic examination revealed obstruction to pulmonary venous return in 7 of 19 patients. Catheterization confirmed the echocardiographic findings, localizing the obstruction in one patient. The size of the venoatrial anastomosis was measured on postoperative echocardiograms performed on 14 patients. The cross-sectional area of the anastomosis was less than 0.3 cm2/m2 of body surface area in the four patients with obstruction of the anastomosis, and greater than 0.95 cm2/m2 in all long-term survivors examined. Two-dimensional echocardiography with pulsed Doppler examination and Doppler color flow mapping is an excellent means of diagnosing totally anomalous pulmonary venous connection. The connections of the individual pulmonary veins can be identified in nearly all cases. Surgical repair can usually be undertaken on the basis of echocardiographic diagnosis alone. Echocardiography also provides an extremely accurate method of evaluating surgical repair and of identifying and localizing postoperative obstruction to pulmonary venous return.

Cardiac Catheterization

Double-inlet single left ventricle: echocardiographic anatomy with emphasis on the morphology of the atrioventricular valves and ventricular septal defect.

The echocardiographic anatomy of double-inlet single left ventricle was studied in 57 patients, aged 1 day to 27 years (mean 6 years); the variables examined included morphology, size and function of the atrioventricular (AV) valves and ventricular septal defect and their relation to pulmonary stenosis, aortic stenosis and aortic arch obstruction. The visceroatrial situs was solitus and the heart was in the left side of the chest in all 57 patients. A d-loop ventricle was present in 21 patients and an l-loop ventricle in 36. The great arteries were normally related (Holmes heart) in 8 patients and transposed in 49. In all hearts, the right AV valve was anterior to the left AV valve. In 53 patients, the tricuspid valve (right valve in d-loop and left valve in l-loop) was closer to and had attachments on the septum. The tricuspid valve straddled the outflow chamber in eight patients. No significant difference was noted in the mean AV valve diameter when comparing mitral and tricuspid valves within the same group or between the groups with a d- or l-loop ventricle. The right AV valve diameter had a significant direct correlation with the aortic valve diameter and the size of the ventricular septal defect regardless of ventricular loop. Both AV valves were functionally normal in 34 patients. Among patients with AV valve dysfunction, the tricuspid valve tended to be stenotic in patients with an l-loop ventricle and regurgitant in patients with a d-loop ventricle. Mitral valve dysfunction was uncommon. The ventricular septal defect (46 patients) was separated from the semilunar valves in 24 patients (muscular defect) and adjacent to the anterior semilunar valve as a result of hypoplasia or malalignment, or both, of the infundibular septum (subaortic defect) in 19 patients. Multiple defects were present in three patients. The defect was unrestrictive in 26 patients, restrictive in 23 and could not be evaluated in 8. Pulmonary artery banding had been performed in 8 of the 26 patients with an unrestrictive defect and in 10 of the 23 patients with a restrictive defect. Only 4 of 19 subaortic defects compared with 16 of 24 muscular defects were restrictive. The size of the defect was significantly correlated with the measured pressure gradient. Among patients with transposition, only 2 of 13 with pulmonary stenosis had a restrictive ventricular septal defect compared with 15 of 30 without pulmonary stenosis. In patients with transposition, the defect size was significantly smaller when coarctation was present.

Child

Straddling mitral valve with hypoplastic right ventricle, crisscross atrioventricular relations, double outlet right ventricle and dextrocardia: morphologic, diagnostic and surgical considerations.

The clinical, surgical and morphologic findings in five cases of a rare form of straddling mitral valve are presented. Three patients were diagnosed by two-dimensional echocardiography, cardiac catheterization and angiocardiography and two had diagnostic confirmation at autopsy. All five cases shared a distinctive and consistent combination of anomalies: 1) dextrocardia; 2) visceroatrial situs solitus, concordant ventricular D-loop and double outlet right ventricle with the aorta positioned to the left of and anterior to the pulmonary artery; 3) hypoplasia of right ventricular inflow (sinus) with tricuspid valve stenosis or hypoplasia; 4) large right ventricular infundibulum (outflow); 5) malalignment conoventricular septal defect; 6) straddling mitral valve with chordal attachments to the left ventricle and right ventricular infundibulum; 7) severe subpulmonary stenosis with well developed pulmonary arteries; and 8) superoinferior ventricles with crisscross atrioventricular (AV) relations. The degree of malalignment between the atrial and ventricular septa was studied quantitatively by measuring the AV septal angle projected on the frontal plane. The AV septal angle in the two postmortem cases was 150 degrees, reflecting marked malalignment of the ventricles relative to the atria. This AV malalignment appears to play an important role in the morphogenesis of straddling mitral valve. As judged by a companion study of seven postmortem cases, the more common form of straddling mitral valve with a hypertrophied and enlarged right ventricular sinus had less severe ventricular malposition than did the five rare study cases with hypoplastic right ventricular sinus. A competent mitral valve, low pulmonary vascular resistance and low left ventricular end-diastolic pressure were found at cardiac catheterization in the three living patients who underwent a modified Fontan procedure and are doing well 2.2 to 5.8 years postoperatively.

Adult

Predictors of survival in neonates with critical aortic stenosis.

BACKGROUND: Failure of infants with critical aortic stenosis to survive after adequate valvotomy despite a left ventricular size that appears to be adequate indicates that additional preoperative anatomic features may contribute to mortality. METHODS AND RESULTS: Discriminant analysis was used to determine which of several echocardiographically measured left heart structures were independent predictors of survival after valvotomy for neonatal critical aortic stenosis. It was possible to predict outcome after classic valvotomy (two-ventricle-type repair) with 95% accuracy based on mitral valve area, long-axis dimension of the left ventricle relative to the long-axis dimension of the heart, diameter of the aortic root, and body surface area. Left ventricular volume was not a major determinant in this study, in part because patients who had initial valvotomy had been preselected in favor of an adequately sized left ventricle. Patients with multiple small left ventricular structures were found to have significantly improved survival after initial Norwood operation. In contrast, balloon valvotomy with subsequent Norwood procedure was usually unsuccessful. CONCLUSIONS: The adverse effects of small inflow, outflow, and/or cavity size of the left ventricle are cumulative. The accuracy of prediction of outcome based only on preoperative anatomy indicates that adequacy of valvotomy is not generally a limiting factor for survival in this group of patients. It is possible to identify subjects whose chance of survival is better after a Norwood procedure rather than valvotomy, even if left ventricular volume is not critically small.

Aortic Valve Stenosis

Two-dimensional and Doppler echocardiographic and pathologic characteristics of the infantile Marfan syndrome.

Since the clinical, 2-dimensional and Doppler echocardiographic and pathologic findings in infantile Marfan syndrome have not been documented in detail, a study of 9 such infants was performed. The previously reported 64 cases were reviewed and the salient findings in 22 additional cases were discussed. The age at diagnosis in our 9 cases ranged from birth to 12 months (mean 2.7 months). Mitral valve prolapse was demonstrated in all, with mitral regurgitation in 8. Tricuspid valve prolapse was present in 8, with tricuspid regurgitation in 6. Marked aortic root dilatation was present in all, and was progressive. The aortic root assumed a "clover leaf" appearance in the parasternal short-axis view. Aortic regurgitation was documented initially in 1 patient, and developed during follow-up in 4 of 7 infants. Dilation of the pulmonary arterial root and pulmonary regurgitation were found in 3 of 7 infants. Severe heart failure associated with mitral or tricuspid regurgitation was present in 7 of the 9 patients. Four infants died during the first year of life. The salient pathologic features were myxomatous thickening and redundancy of the mitral and tricuspid leaflets, marked elongation of chordae tendineae and prominent dilatation of the aortic and pulmonary roots. Histologically, the collagen and elastic fibers were severely disrupted, disarrayed and fragmented with increased interstitial ground substance. These data document that infantile Marfan syndrome is characterized by clinical and morphologic features that are distinctly different from the classic syndrome seen in adolescents and adults. The aforementioned findings should facilitate early clinical and echocardiographic diagnosis of infantile Marfan syndrome.

Aorta

Results of the arterial switch operation in patients with transposition of the great arteries and abnormalities of the mitral valve or left ventricular outflow tract.

Between January 1983 and October 1989, 290 patients underwent an arterial switch operation for transposition of the great arteries; 30 (10.3%) of the patients had abnormalities of the left ventricular outflow tract or mitral valve, or both. These abnormalities included isolated pulmonary valve stenosis (n = 9), septal (dynamic) subpulmonary stenosis (n = 5), anatomic (fixed) subpulmonary stenosis (n = 7), abnormal mitral chordae attachments (n = 2) or a combination of abnormalities (n = 7). There were two early deaths, one of which was due to previously unrecognized mitral stenosis and a subpulmonary (neo-aortic) membrane and one late death due to presumed coronary obstruction. Of the nine patients with pulmonary valve abnormalities due to either a bicommissural (n = 5) or a thickened tricommissural (n = 4) valve, only one underwent valvotomy. Peak systolic ejection gradients in these nine patients measured preoperatively ranged from 0 to 50 mm Hg. At follow-up study 5 to 30 months postoperatively, the neo-aortic valve gradient was less than or equal to 15 mm Hg in all patients; three patients had mild neo-aortic regurgitation. Preoperative gradients may overestimate the degree of obstruction because of the increased pulmonary blood flow present in transposition. No patient with "dynamic" subpulmonary obstruction before the arterial switch operation had a surgical procedure performed on the left ventricular outflow tract; none had evidence of subaortic obstruction after the arterial switch.(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormalities, Multiple

Coronary artery pattern and outcome of arterial switch operation for transposition of the great arteries.

A successful outcome of an arterial switch operation (ASO) for dextro-transposition of the great arteries (D-TGA) depends in large part on the transfer of the coronary arteries to the neoaorta without distortion or narrowing. However, the origins and distribution of the coronary arteries are quite variable in D-TGA; therefore, the entire experience with ASO at the Children's Hospital in Boston was reviewed. From 1983 through November 1989, 314 patients underwent surgery for D-TGA with the intent to perform an ASO. An ASO was actually performed in 290 patients, and 20 patients died, with 12 deaths (4.2%) clearly related to problems with the coronary arteries (CA deaths). The ASO was aborted to a Senning operation in 24 patients (7.6%), primarily due to coronary anatomy. Eight coronary patterns were identified and related to the outcome of the ASO. In 182 patients with the "usual" coronary pattern for D-TGA (i.e., anterior descending and circumflex arteries from the left sinus and right coronary artery from the right sinus), five CA deaths occurred, and two patients had Senning operations. Sixty-seven patients had right coronary and circumflex arteries from the right sinus and anterior descending arteries from the left sinus. Three CA deaths occurred, and one patient had a Senning operation. Single right coronary artery was found in 14; 12 had ASOs with three CA deaths (25%); and two had Senning operations. One late death was due to diffuse narrowing of the single right coronary artery. Single left coronary artery occurred in 11 patients, and seven had ASO with no early but one late "sudden" death, whereas four had Senning operations.(ABSTRACT TRUNCATED AT 250 WORDS)

Child