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Biomedical subjects

L N Benson

Publications and source records attributed to L N Benson.

At least 145 records · Page 8Linked to original sources
PubMed1988

Correlation of right ventricular volume using axial angulated ventriculography to known right ventricular cast volumes in infants and children with congenital heart disease.

To calculate right ventricular (RV) volumes from biplane cineangiography obtained in nonstandard views, regression equations were developed from RV casts of known volume. Volumes were calculated using Simpson's rule from casts ranging from 2 to 42 ml from 25 postmortem specimens with various congenital heart diseases. The casts were divided into 2 groups: group 1 (n = 15) with abnormal or group 2 (n = 10) with normal RV hemodynamic measurements. Biplane cinegrams were taken in the anterolateral, anterior and long axis oblique, hepatoclavicular and sitting up projections. The true volume of each cast was determined from its weight and specific gravity. Excellent correlations were obtained between measured and true volumes (r = 0.92 to 0.96) in all projections, although each projection overestimated the true volume (slope value less than 1). The regression equations obtained from group 1 were not statistically different from those in group 2 in any view. Although the application of different regression equations is required in measuring RV volumes by multiple angulated angiography, these regression equations appear not to be affected by the hemodynamic state of the ventricle. These results are important in assessing RV volume in pediatric patients with congenital heart disease using axial angulated ventriculography.

Cineangiography↗
PubMed1988

Angiographic features associated with percutaneous balloon valvotomy for pulmonary valve stenosis.

Changes in the mobility of the pulmonary valve were determined by a retrospective review of right ventricular cineangiograms from 25 balloon pulmonary valvotomy (BV) procedures in 23 infants and children. The angiographic changes were compared with the post-BV catheter and Doppler pressure gradients across the right ventricular outflow tract. Angiographic features felt to indicate valve tearing were present following 17 of 25 procedures and included increased excursion or straightening of leaflets, localized change in leaflet motion (flail leaflet), and the presence of an additional contrast jet through the valve. There was no statistically significant relationship between any of the angiographic parameters and the pressure data. Most patients with marked increase in angiographic valve mobility had low residual right ventricular to pulmonary artery gradients. However, the absence of angiographic change was not always associated with a high residual gradient.

Catheterization↗
PubMed1988

Thrombolytic therapy for femoral artery thrombosis after pediatric cardiac catheterization.

Femoral artery thrombosis remains a well-known complication after cardiac catheterization. A study was undertaken to assess the efficacy of thrombolytic therapy for this complication. A total of 526 consecutive infants and children were prospectively evaluated after cardiac catheterization, and the medical charts of 42 patients who required femoral artery thrombectomy between 1975 and 1985 were reviewed. In the prospective study, patients were given a bolus injection of heparin, 150 U/kg, at the time the artery was entered. Patients with persistently absent or diminished pulse 2 hours after catheterization received a second bolus injection of 50 U/kg followed by an infusion of 20 U/kg/hr heparin for a maximum of 48 hours. If the affected leg pulse was absent or reduced and the systolic Doppler blood pressure was less than two thirds that of the unaffected leg, thrombolytic therapy was begun. In the 42 patients with surgical thrombectomy, there were no serious complications of surgery. Forty-five of the 526 patients (8.6%) had a decreased or absent pulse after catheterization. Of these 45 patients, 32 (71.1%) improved with systemic heparinization only. Thirteen patients (28.9%) had a persistently absent pedal pulse suggesting femoral artery thrombosis, despite continuous heparinization. Eleven patients were successfully treated with thrombolytic therapy and two required surgical thrombectomy. Intraarterial balloon dilatation procedures were performed in 8 of these 13 patients. Prothrombin time was prolonged (11.5 +/- 1.06 to 52.3 +/- 40.4 seconds; p less than 0.025) and fibrinogen levels were significantly reduced (2.25 +/- 0.79 to 1.52 +/- 0.52 gm/dl; p less than 0.01) during therapy. There were no serious complications, although four patients bled from the groin entry site.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1988

Cardiac manifestations in disorders of fat and carnitine metabolism in infancy.

The prognosis of patients with cardiomyopathy associated with hypocarnitinemia is uncertain. Cardiac hemodynamics, histologic findings and response to oral L-carnitine therapy were retrospectively evaluated in 11 children with cardiomyopathy associated with abnormal carnitine metabolism. Three had systemic carnitine deficiency, two familial hypocarnitinemia with neutropenia, three transient neonatal hypocarnitinemia and three a carnitine insufficiency syndrome. Six had a hypertrophic and five a dilated cardiomyopathy. Hypotonia was present in seven (64%). The cardiothoracic ratio was greater than 0.60 in eight (73%). The most frequent abnormality on the electrocardiogram was ST-T wave inversion in the left precordial leads with various degrees of left ventricular hypertrophy. Echocardiographically, two patients with hypertrophic cardiomyopathy had decreased left ventricular function and two patients with dilated cardiomyopathy had increased thickness of the left ventricular wall. Histologic evaluation (two autopsies and one endomyocardial biopsy) revealed striking lipid accumulation within hypertrophied myocytes. Six of eight patients on carnitine replacement therapy had improvement echocardiographically during a 3 month to 2 year follow-up period. In summary, both hypertrophic and dilated cardiomyopathy can result from abnormal carnitine metabolism. The determination of plasma carnitine concentrations and fatty acid metabolism by-products should be performed in all patients with either form of cardiomyopathy of unknown etiology because carnitine supplementation may lead to improvement.

Biopsy↗
PubMed1988

Successful use of intraaortic balloon pumping in a 2-kilogram infant.

Severe low cardiac output of unknown cause that did not respond to inotropic and ventilatory support developed in a 2-kg infant. Intraaortic balloon pumping was used successfully to acutely improve her cardiac function and peripheral perfusion and to decrease the requirement for inotropic medications. We describe the technique for insertion and the equipment used, as well as our experience with intraaortic balloon pumping in children.

Cardiac Output, Low↗
PubMed1988

Repair of tricuspid atresia: utility of right ventricular incorporation.

During a 10-year period, 62 patients underwent the following modifications of the Fontan operation for repair of tricuspid atresia: direct atriopulmonary connection (N = 15), atriopulmonary connection using a conduit (N = 5), direct atrioventricular (AV) connection (N = 22), and AV connections with a valved conduit (N = 20), including 2 with combined Fontan-arterial switch procedures. The overall hospital mortality was 16.1% (10/62) (70% confidence limits, 11.2 to 22.4%). By multivariate analysis, the risk factors for early and late death included increasing right atrial pressure after repair, use of an atriopulmonary connection, and previous pulmonary artery banding (all variables, p less than 0.05). Postoperative catheterization was performed in 22 patients including 15 with AV valved-conduit connections. Right ventricular (RV) work based on pulmonary artery pressure minus right atrial pressure was correlated with the preoperative RV to left ventricular volume ratio computed from the four-chamber angiographic projection (p = 0.025), and was appreciable only with ratios exceeding about 30%. In 6 of 19 eligible patients, severe conduit obstruction has developed. Considering the survival data, the risk of reoperation, and postoperative hemodynamic findings, analysis of our experience supports the preferential use of nonvalved AV connections in most patients with tricuspid atresia and ventriculoarterial concordance.

Adolescent↗
PubMed1988

Cardiopulmonary adaptation at rest and during exercise 10 years after Mustard atrial repair for transposition of the great arteries.

Discordance exists between apparently reduced systemic right ventricular function and the reported asymptomatic state of many patients after atrial repair for transposition of the great arteries. To evaluate this clinical observation, cardiopulmonary response to exercise in 17 asymptomatic patients with no significant postoperative hemodynamic abnormalities was assessed by upright bicycle ergometry according to a modified Jomes protocol 11.5 +/- 1.5 years after Mustard atrial repair. Seventeen age- and sex-matched normal adolescents constituted the control group. Incremental exercise was performed to determine maximum work capacity, heart rate, blood pressure, oxygen saturation, and minute ventilation. Cardiac output was computed at rest and during steady-state exercise by a carbon dioxide rebreathing method at 50% of the maximum workload achieved during incremental exercise. Height and weight were similar in patients and controls (p greater than .05). Resting pulmonary function variables were normal in all subjects. At peak exercise, respiratory quotient was greater than 1 in both patients and controls (1.12 +/- 0.09 and 1.09 +/- 0.08 respectively, p greater than .05). Patients achieved a lower peak heart rate (172 +/- 14 vs 185 +/- 11 beats/min, p less than .01), lower maximum work capacity (2.3 +/- 0.6 vs 3.3 +/- 0.7 W/kg, p less than .01). The ratio of minute ventilation at peak exercise to maximum resting voluntary ventilation was normal (less than or equal to 80%) in both groups. The ventilatory equivalent for oxygen was similar at rest, but significantly higher in patients than in control subjects at peak exercise (42 +/- 7 vs 36 +/- 5, p less than .006).(ABSTRACT TRUNCATED AT 250 WORDS)

Adaptation, Physiological↗
PubMed1988

Inadequate myocardial protection with cold cardioplegic arrest during repair of tetralogy of Fallot.

Postoperative low cardiac output is the most common cause of death in patients undergoing elective repair of tetralogy of Fallot. The incidence is much higher than in elective adult bypass operations for coronary artery disease. To explain this difference, we investigated 16 children having elective repair of tetralogy (mean age 6.3 years). Myocardial biopsy specimens obtained during bypass before arrest, at the end of cold arrest by blood cardioplegia, and after 30 minutes of reperfusion were studied for adenosine triphosphate and lactate levels. Myocardium was submitted for microscopic study shortly after the onset of ischemia. The operation was successful in reducing right ventricular-pulmonary artery gradients from 82 +/- 28 to 9 +/- 1 mm Hg, yet seven patients required significant inotropic support (dopamine, greater than 5 micrograms/kg/min) for more than 24 hours and 12 patients needed prolonged use of digoxin and diuretics for right ventricular failure. Tissue levels of adenosine triphosphate and lactate in the tetralogy groups were compared with those in 20 adults with coronary artery disease having similar myocardial protection techniques. Adenosine triphosphate levels in the tetralogy group decreased during cross-clamping (41 +/- 8 minutes) from 24 +/- 3 to 16 +/- 2 mmol/kg dry weight (mean +/- 1 standard error), with a marked further drop after reperfusion to 9 +/- 2 mmol/kg (p less than 0.01). Adenosine triphosphate levels in the group with coronary disease also decreased from 20 +/- 1 to 16 +/- 1 mmol/kg after a longer cross-clamp time (70 +/- 17 minutes) but remained at 15 +/- 2 mmol/kg after reperfusion. Tissue lactate levels in the tetralogy group rose markedly during ischemia and remained elevated after reperfusion. In contrast, lactate levels in the group with coronary disease rose moderately during ischemia and returned to normal early on reperfusion. Microscopic study revealed focal myocyte necrosis in tetralogy of Fallot. Our findings, which demonstrate inadequate myocardial protection of patients with tetralogy during repair, with depression of adenosine triphosphate and increased lactate during ischemia and reperfusion, suggest a defect in oxidative metabolism. The drop in adenosine triphosphate after reperfusion in the patients with tetralogy implicates reperfusion injury as a mechanism of myocardial damage.

Adenosine Triphosphate↗
PubMed1987

Results with the Mustard operation in simple transposition of the great arteries 1963-1985.

From May 1963 to December 1985, 329 infants and children underwent repair of simple transposition of the great arteries (TGA) using Mustard's technique. To assess improvement, the patients were separated into two groups by date of operation: Group I, May 1963 to December 1973 (N = 106), and Group II, January 1974 to December 1985 (N = 223). The operative mortality was 11 (10.4%) in Group I and two (0.9%) in Group II. The 10-year actuarial survival rate was 73.4% in Group I and 93.7% in Group II. Baffle complications, similar in both groups, were identified in 81 patients; 19 were major, causing death or requiring reoperation. By latest electrocardiogram, 21 of 45 (46.7%) of Group I patients and 129 of 180 (71.7%) of Group II patients were in normal sinus rhythm. Late ambulatory electrocardiography, however, revealed that a majority of patients had sinus node dysfunction or other dysrhythmias. Right ventricular (RV) angiography revealed definite diminution of RV contractility in 14 (11%) of 126 children. At late follow-up, 113 of 148 (76%) children were in New York Heart Association (NYHA) Class I and 35 of 148 (24%) were in NYHA Class II. Thirty-five (21%) patients were on cardiac medication chiefly for dysrhythmia management. There has been significant improvement in the early and late mortality with the Mustard operation, and serious baffle complications are infrequent. Dysrhythmias continue to be a major problem but the surviving patients are clinically well and relatively few have significant RV dysfunction.

Actuarial Analysis↗
PubMed1987

The dysplastic pulmonary valve: echocardiographic features and results of balloon dilatation.

The feasibility of using balloon dilatation to relieve stenosis caused by dysplasia of the pulmonary valve was assessed in seven patients (five female, mean age two years) with angiographically confirmed dysplasia who were identified among 38 patients with pulmonary valve stenosis selected for balloon dilatation over a two year period. The clinical features in three patients were consistent with Noonan's syndrome. In all patients the gradient across the valve was assessed by cross sectional echocardiography and Doppler echocardiography before cardiac catheterisation. Balloon dilatation was performed by conventional techniques. In one patient, who had balloon dilatation in the operating room before surgical valvectomy, the diameter of the valve orifice increased from 3 mm to 10 mm. Inspection showed a tear along the anterior commissure. The mean (SD) pressure gradients between the right ventricle and pulmonary artery before and immediately after dilatation in five patients were not significantly different (58 (28) and 47 (12) mm Hg) respectively. There was no overall significant change in the degree of stenosis when four of these patients were examined by Doppler echocardiography six months after operation (44 (17) mm Hg), although one patient (case 5) did show a significant reduction in gradient. This patient had angiographic and echocardiographic features of dysplasia and commissural fusion. Several echographic features were common to all patients and distinguished them from cases of typical pulmonary valve stenosis. These were: pronounced thickening of leaflets; leaflet immobility in diastole and systole; no dilatation of the sinuses of Valsalva in diastole, and supra-annular narrowing. These poor results of balloon dilatation suggest that commissural fusion is not an important mechanism for causing stenosis in the dysplastic pulmonary valve. When dysplasia of the pulmonary valve is identified clinically and echocardiographically, balloon dilatation is unlikely to improve haemodynamic function; it should be attempted if commissural fusion is present.

Angioplasty, Balloon↗
PubMed1987

The morphology of the right ventricular outflow tract after percutaneous pulmonary valvotomy: long term follow up.

Twenty nine patients (19 male, mean (SD) age 6.25 (0.5) years (range 0.16-15 years] with typical pulmonary valve stenosis were treated by balloon dilatation of the pulmonary valve. They were studied by echocardiography before the procedure, immediately after it, and at follow up (mean (SD) 10.2 (5.6) months, n = 18). The morphology of the pulmonary valve, the right ventricular-pulmonary artery gradient, and ratio of the systolic to diastolic endocardial dimensions (infundibular ratio) were examined. No patient had pulmonary regurgitation before the study. The valve gradient was significantly reduced (47%) from a mean (SD) of 72 (31) to 37 (23) mm Hg with no short term change in cardiac index after dilatation with a balloon with a mean (SD) diameter that was 118 (10.8)% of the valve annulus. The infundibular ratio was unchanged by the procedure (0.49 (0.11) (n = 21) before dilatation and 0.47 (0.14) (n = 16) after dilatation). In twenty seven patients the commissure of the pulmonary valve was seen to be torn after dilatation. Two patients with bicuspid valves had flail leaflets. Doppler examination at follow up showed mild pulmonary insufficiency in all 29 patients; the mean (SD) valve gradient (31 (+/- 21) mm Hg) at follow up was no different from the gradient found immediately after the procedure and infundibular ratio (0.58 (0.15) was not abnormal. These data indicate that commissural tears are the primary mechanism of valve disruption and demonstrate that the dynamic right ventricular outflow tract obstruction relaxes and gradient reduction persists at follow up.

Adolescent↗
PubMed1987

Left ventricular geometry and function in adults with Ebstein's anomaly of the tricuspid valve.

We postulated that the abnormal shape, size, and function of the right heart and adjoining ventricular septum in adults with Ebstein's anomaly of the tricuspid valve might in turn alter the shape and function of the left ventricle. Seven adult patients with uncomplicated Ebstein's anomaly were studied. Left ventricular geometry was determined by two-dimensional echocardiography. Left ventricular function was assessed by treadmill exercise and radionuclide angiography at rest and with exercise. Paradoxic ventricular septal motion was consistently present. Left ventricular eccentricity (ratio of two minor axes in the short-axis view) was uniformly abnormal, averaging 1.35 +/- 0.23 (normal = 1.02 +/- 0.05). The ratio of right to left ventricular cavity size averaged 1.70 +/- 0.44 (normal 0.65 +/- 0.30), and tricuspid valve displacement into the right ventricular cavity averaged 52% (normal 8%). Functional right atrial size averaged 27.6 +/- 5.2 cm2 (normal right atrial area = 13.1 +/- 2.2 cm2). Resting left ventricular ejection fractions were below 50% in all but two patients. In response to Bruce protocol exercise stress, there were consistently appropriate increments in heart rate, blood pressure, and peak double product and, with one exception, radionuclide left ventricular ejection fraction. There were significant correlations between tricuspid valve displacement and functional right atrial size versus resting left ventricular ejection fraction and left ventricular eccentricity. These data support the hypothesis that derangements in right heart morphology and function in Ebstein's anomaly contribute to significant alterations in left ventricular geometry, but the geometric alterations are associated with tangible but less significant changes in left ventricular systolic function.

Adolescent↗
PubMed1987

Validation of Doppler-derived pulmonary arterial pressure in patients with ductus arteriosus under different hemodynamic states.

Twenty-nine patients with a patent ductus arteriosus (PDA) in isolation (n = 17) or in combination with other lesions (n = 12) underwent simultaneous hemodynamic assessment and evaluation of PDA flow velocity by the Doppler method. The accuracy with which Doppler velocity across the PDA predicted pulmonary arterial pressure and the influence of PDA size and shape on the Doppler velocity-pressure relationship were examined. Seventy percent had a cone-shaped PDA (narrowest at the pulmonary artery end), and the remainder were tubular. Narrowest PDA diameter ranged from 1.5 to 9 mm (mean 3.5 mm). Peak systolic and mean pulmonary arterial pressure ranged from 10 to 116 and 8 to 72 mm Hg, respectively. Twenty-one patients (group 1) had left-to-right shunting only. The following variables showed significant correlation in this group: peak instantaneous systolic aortic-to-main pulmonary arterial (MPA) pressure gradient and maximum Doppler velocity across the PDA (slope = 1.03, SEE = 13 mm Hg, r = .94, p less than .001), mean aortic-to-MPA pressure gradient and mean Doppler velocity (slope = 1.06, SEE = 10 mm Hg, r = .95, p less than .001), and end diastolic aortic-to-MPA pressure gradient and minimum Doppler velocity (slope = 1.12, SEE = 8 mm Hg, r = .96, p less than .001). Eight patients (group 2) had bidirectional shunting. In this group peak instantaneous aortic-to-MPA pressure gradient significantly correlated with maximum Doppler velocity measured from the left-to-right shunt (slope = .70, SEE = 2 mm Hg, r = .92, p less than .002) and mean pressure gradient correlated with mean Doppler velocity (slope = .83, SEE = 3 mm Hg, r = .78, p less than .003). Right-to-left Doppler velocities showed no correlation with pressures. In six patients with pulmonary hypertension Doppler velocity changes accurately predicted the effect of pulmonary vasodilation on pulmonary arterial pressure. Doppler velocity of PDA flow reliably predicts pulmonary arterial pressure over a wide range of pressures and PDA shapes and sizes.

Adolescent↗
PubMed1987

Surgical experience with the modified Fontan procedure.

During a 10 year period 109 patients (3 months to 47 years old) underwent modifications of the Fontan procedure for repair of classic tricuspid atresia (TA) (n = 58), univentricular atrioventricular connection (UVH) (n = 38), or other complex malformations (CM) (n = 13). Among patients with TA, an atriopulmonary connection was used in 19 (33%) and incorporation of the right ventricle with the Björk modification and with a right atrial-to-right ventricular valved conduit was used in 20 (34%) and in 19 (33%), respectively. Three of the latter 19 also underwent a combined Fontan-switch procedure. The hospital mortality rate was 13.8% (70% confidence limits, 9.3% to 18.3%) for patients with TA, 28.9% (70% confidence limits, 21.3% to 37.0%) for patients with UVH, and 7.7% (70% confidence limits, 0% to 15.4%) for patients with CM. Multivariate analysis identified with the following variables as risk factors for both early and late deaths: diagnosis of UVH, previous pulmonary artery banding (PAB), and postrepair right atrial pressure, and, in patients with TA, the use of a direct atriopulmonary connection (all variables, p less than .05). Morphometric lung biopsy scores were not different in patients with PAB, implicating the role of ventricular hypertrophy rather than pulmonary vascular disease as the mechanism for the adverse effect of PAB. Right atrial pressure was a predictor of serious late cardiac symptoms, which were present in 10% of eligible patients (p = .032). This review demonstrates a survival advantage with modifications of the Fontan procedure that incorporate the hypoplastic right ventricle in patients with TA.(ABSTRACT TRUNCATED AT 250 WORDS)

Actuarial Analysis↗
PubMed1987

Impaired left ventricular postischemic function and metabolism in chronic right ventricular hypertrophy.

Chronic right ventricular hypertrophy (RVH) has been shown to produce changes in left ventricular diastolic properties but minimal effects on left ventricular systolic function. We studied the effects of chronic pressure overload RVH on left ventricular systolic function before and after reversible hypothermic global ischemia. RVH was induced by pulmonary artery banding (PAB) in newborn piglets (5 to 7 days). At 2 months of age the PAB group (n = 6) and a control group (n = 8) were subjected to cardiac arrest on cardiopulmonary bypass with cold crystalloid cardioplegia (10 degrees C) for 2 hr and were reperfused for 1 hr. Left ventricular function was assessed by a conductance catheter in the left ventricle measuring the end-systolic pressure-volume relationship (Emax). Preischemic and postischemic Emax were the same in the control group (4.1 +/- 0.4 mm Hg/ml before vs 4.1 +/- 0.4 mm Hg/ml after ischemia), but significantly different in the PAB group (4.7 +/- 0.5 mm Hg/ml before vs 2.97 +/- 0.7 mm Hg/ml after ischemia, p less than .05). There also was a marked drop in ATP and phosphocreatine (CP) content in the PAB group during ischemia (ATP, 20 +/- 2 mmol/kg dry wt before vs 10 +/- 2 mmol/kg dry wt after ischemia, p less than .05; PC, 26 +/- 3 mmol/kg dry wt before vs 11 +/- 1 mmol/kg dry wt after ischemia, p less than .05). In the control group there was no change in ATP content and, although CP did drop by end-ischemia, there was complete recovery by 1 hr of reperfusion but minimal CP recovery in the PAB group.(ABSTRACT TRUNCATED AT 250 WORDS)

Adenosine Triphosphate↗
PubMed1987

Evidence of myocardial free radical injury during elective repair of tetralogy of Fallot.

Evidence of human myocardial oxygen-mediated free radical injury (OFRI) during cardiovascular repair is lacking. We studied six cyanotic children (ages 3.5 to 5.5 years) during elective repair of tetralogy of Fallot. Right ventricular biopsy specimens, obtained on bypass just before aortic cross-clamping, at the end of 40 +/- 8 min (mean +/- SD) cold arrest with blood cardioplegia, and during reperfusion, were analyzed for hydroxy conjugated dienes, a chemical signature of free radical injury. Structural confirmation of the dienes was done by mass spectroscopy. Conjugated dienes were detected before ischemia on bypass, at end-ischemia, and during reperfusion. The products identified are 9 and 13 OH isomers of linoleic acid and 5, 8, 9, 11, and 15 OH isomers of arachidonic acid. Microscopic examination of right ventricular biopsy specimens obtained on bypass just after cross-clamping showed focal myocyte necrosis. The hydroxy conjugated diene levels in control canine hearts subjected to 105 min of cardiopulmonary bypass without aortic cross-clamping were not detectable. Our results support the occurrence of oxygen-mediated free radical injury during elective repair of tetralogy of Fallot. The presence of conjugated dienes before aortic cross-clamping suggests oxygen toxicity with reoxygenation from cardiopulmonary bypass.

Animals↗