Predictors of coronary risk in Kawasaki disease.
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Child↗
Biomedical subjects
L N Benson
Publications and source records attributed to L N Benson.
Changes in pericardial surface pressure during pulmonary hypertensive crises after cardiac surgery.
Reactive pulmonary hypertension in children after surgical repair of large left-to-right shunts is an important cause of postoperative low cardiac output. Low output may be caused by impaired left ventricular filling from right ventricular dilatation. We studied 12 children during the first 72 hr after surgery by measuring right ventricular pericardial surface pressure with a flat balloon and compared the results with acute changes in pulmonary arterial pressure (PAP). All 12 children (ages 4 to 14 months) had preexisting large left-to-right shunts with systemic PAPs. We found a highly significant linear correlation between acute rises in PAP and rises in the pericardial pressure (PP). The regression equation was PAP = 1.48(PP) + 5.7, R2 = .61, p less than .001. There was an average of 15.6 pulmonary hypertensive episodes per patient during the 72 hr study period, but only 9% of these episodes were associated with either no change or a fall in systemic pressure. We conclude that changes in right ventricular pericardial pressure reflect acute changes in PAP.
Heart Defects, Congenital↗
Age-related differences in the response to myocardial ischemic stress.
Clinical experience indicates that the risk of reparative cardiac operations is increased in the neonatal period relative to that in older infants and children. Age-related differences in the susceptibility to myocardial ischemic dysfunction were evaluated by comparison of left ventricular function and metabolism in neonatal (mean age = 7 days) and weanling (mean age = 96 days) piglets. Six animals in each group supported on cardiopulmonary bypass were subjected to (1) 120 minutes of hypothermic crystalloid cardioplegic arrest (CP-120) and (2) 15 minutes of normothermic ischemic arrest (NA-15) after a 60 minute interval of reperfusion. Left ventricular systolic and diastolic function was measured after each intervention via endocardially implanted ultrasonic dimension crystals in a septolateral minor-axis position. In both groups, systolic dysfunction was evidenced by an increase in the dimension-axis intercept (p = 0.001), but not the slope of the end-systolic pressure-dimension relation. Left ventricular end-diastolic stiffness, expressed as left ventricular end-diastolic pressure versus Lagrangian strain, increased to a similar degree in both groups (p = 0.001). Adenosine triphosphate levels declined significantly (p = 0.001) in both groups in response to the ischemic interventions with no evident intergroup differences. Lactate levels increased significantly during the course of the experiment (p = 0.04); however, the increases were greater (p = 0.009) at all intervals in the neonatal group. This study demonstrates age-related metabolic differences in response to ischemia consistent with a greater dependence on glycolysis in neonatal myocardia. However, the fact that discriminating age-related differences in left ventricular function were not evident suggests that factors other than young age per se account for the increased surgical mortality in the neonatal period.
Adenosine Triphosphate↗
Radionuclide angiographic evaluation of ventricular function in isolated congenitally corrected transposition of the great arteries.
Eight asymptomatic patients (mean age 19 years, range 7 to 32) with congenitally corrected transposition of the great arteries (CCTGA) underwent equilibrium gated radionuclide angiocardiography at rest and during supine bicycle exercise to assess systemic (morphologic right) and pulmonary (morphologic left) ventricular function. Five patients had normal intracardiac hemodynamic values, 2 had trivial atrioventricular valve regurgitation and 1 patient had trivial pulmonary ventricular outflow tract obstruction. Average exercise duration was 11 +/- 1 minute, with limitation due only to fatigue. At peak exercise, heart rate increased 225% and systolic blood pressure 152% over the rest value. Pulmonary ventricular ejection fraction at rest was 51 +/- 3% (mean +/- standard error of the mean); it did not change significantly at peak stress, 53 +/- 2%. Systemic ventricular ejection fraction was 48 +/- 4% at rest and increased to 64 +/- 4% at peak exercise (p less than 0.01). Count-based volume changes for the pulmonary chamber showed no significant change in end-diastolic or systolic counts at peak exercise (109 +/- 8% and 106 +/- 9% of rest value, respectively). However, end-diastolic counts decreased 13% (87 +/- 3% of rest value) and end-systolic counts 34% (62 +/- 7% of rest value) at peak exercise in the systemic ventricle. These data suggest normal systemic and impaired pulmonary ventricular function in patients with congenitally corrected transposition of the great arteries unaccompanied by significant associated lesions. These findings have important clinical implications in the setting of complex congenital heart disease in patients in whom a morphologic right ventricle functions as the systemic pumping chamber. Despite the pulmonary ventricular dysfunction, symptoms were not apparent at rest or during exercise.
Adolescent↗
Cerebral complications following balloon angioplasty of coarctation of the aorta.
A 10-month-old male child underwent balloon angioplasty for a recurrent coarctation of the aorta. Postprocedural hemorrhagic cerebral infarction led to the child's death. At necropsy, hypoplasia of the left posterior communicating artery of the circle of Willis was found. The implications of such variations in cerebral blood flow circulation on the angioplasty technique are presented and discussed.
Angioplasty, Balloon↗
Subaortic stenosis, the univentricular heart, and banding of the pulmonary artery: an analysis of the courses of 43 patients with univentricular heart palliated by pulmonary artery banding.
Subaortic stenosis is well known to complicate the clinical course of patients with single ventricle or univentricular hearts, and we have previously suggested that the development of subaortic stenosis in such patients may be causal to and/or accelerated by previous banding of the main pulmonary trunk. To further define the relationship between banding of the pulmonary artery in patients with univentricular hearts and the development of subaortic stenosis, we examined the morphologic substrate and timing of the development of subaortic stenosis in 43 patients seen at our institution from January 1, 1970, through June 30, 1985. These 43 patients include all patients in this period with an unequivocal univentricular heart whose longitudinal data was available for follow-up. We excluded patients who died within 1 week of surgery, patients lost to follow-up, and patients with evidence of subaortic stenosis before banding. Thirty-one of 43 patients (72.1%) developed subaortic stenosis subsequent to banding of the main pulmonary artery. The mean age at banding of those patients who developed subaortic stenosis was 0.21 years, and subaortic stenosis was recognized at a mean age of 2.52 years. For the specific cohort of patients whose ventricular morphology was a main chamber of left ventricular type supporting the pulmonary artery and a rudimentary right ventricle supporting the transposed aorta (32 patients), 27 developed subaortic stenosis (84.4%). Subaortic stenosis in the classic form of single ventricle usually results from progressive restriction of a wholly muscular interventricular communication. Banding of the pulmonary artery by producing myocardial hypertrophy undoubtedly accelerates the potential for subaortic stenosis in these patients.(ABSTRACT TRUNCATED AT 250 WORDS)
Cardiomyopathy, Hypertrophic↗
Balloon dilatation for superior vena caval obstruction after the senning procedure.
A 3-month-old infant is presented who developed acute superior vena caval obstruction after atrial surgery for transposition of the great arteries. Balloon dilation angioplasty resulted in a decrease in caval-atrial gradient. The application of balloon angioplasty in this setting is discussed.
Angioplasty, Balloon↗
Pulmonary valve morphology after balloon dilatation of pulmonary valve stenosis.
Percutaneous balloon valvuloplasty (BV) for pulmonic valve stenosis (PS) is increasingly becoming a nonsurgical alternative in patient management. However, the mechanism by which BV dilates the obstructive lesion has not been firmly established. We have had the opportunity to examine the effects of BV in the setting of PS and present two illustrative cases documenting the morphology after BV of the stenotic bicuspid and tricuspid pulmonary valve.
Adolescent↗
Thrombolytic therapy for superior vena caval thrombosis following superior vena cava-pulmonary artery anastomosis.
Immediate postoperative thrombosis of left superior vena cava-left pulmonary artery anastomosis in a modified Fontan procedure for single ventricle and pulmonary artery stenosis is described. Before thrombolytic therapy with streptokinase is initiated, confirmation of thrombosis by venography is mandatory to lessen the risk of cardiac tamponade or hemothorax. By this technique major surgical intervention is avoided, but close attention to the dose of streptokinase and the coagulation profile is essential. Measures to be taken if bleeding occurs with streptokinase therapy are described.
Adult↗
Surgical correction of pulmonary atresia and ventricular septal defect with large systemic-pulmonary collaterals.
A two-stage surgical procedure was performed in a young adult with pulmonary atresia and a ventricular septal defect with upper lobe pulmonary artery flow from confluent central pulmonary arteries and lower lobe pulmonary blood flow originating from two large systemic-pulmonary collaterals. Initially a Dacron Y graft was anastomosed between the ascending aorta and the collaterals and a graft to the left pulmonary artery. At the second operation, continuity was established between the right ventricle and the Y graft by using a valved Dacron conduit and the ventricular septal defect was closed.
Adult↗
Radionuclide assessment of right and left ventricular function during bicycle exercise in young patients with cystic fibrosis.
Right and left ventricular performance during exercise was evaluated in 31 patients with cystic fibrosis using equilibrium radionuclide cineangiography. There were 21 males and 10 females (mean age 17, range 10.4 to 34.9 yr) with a wide range of clinical involvement. Thirteen patients had arterial desaturation at rest and an additional 4 became desaturated with exercise. Nine patients (29%) were unable to increase their right ventricular ejection fraction more than 5% during exercise. Left ventricular ejection fraction at rest was normal (greater than 55%) in all but 2, and with exercise, performance was impaired in 9 (29%). No relationship was found between resting or exercise radionuclide ejection fraction, M-mode echocardiography or pulmonary function studies. This study suggests that both right and left ventricular dysfunction may occur during stress in patients with cystic fibrosis without other conventional clinical signs or symptoms. Radionuclide cardiac angiography appears to be a safe method of assessing cardiac response to exercise in patients with chronic lung disease, and may be useful in evaluating therapeutic interventions.
Adolescent↗
Portacaval shunt in patients with familial hypercholesterolemia.
Portacaval shunt was performed in ten patients with homozygous and two with heterozygous familial hypercholesterolemia (FH). Total serum cholesterol was lowered by 20% to 55.4% during follow-up periods of 14 months to almost 9 years, with commensurate decreases in LDL cholesterol. The effect on HDL cholesterol and triglyceride levels was variable. Tendinocutaneous xanthomas diminished or disappeared. Growth and development in children proceeded or accelerated. There was no detectable emotional or intellectual deterioration. Hepatic failure did not occur, although blood ammonia concentrations and serum alkaline phosphatase levels increased relative to preoperative values. Cardiac symptoms were often improved, but evidence of reversal of cardiovascular lesions was inconclusive. Three patients with pre-existing heart disease died of cardiac complications after 4 months, 18 1/2 months, and 30 months. Portacaval shunt has been effective therapy for patients with FH who were refractory or intolerant to medical treatment; it should be performed before the development of irreversible cardiovascular damage.
Adolescent↗
Assessment of right ventricular function during supine bicycle exercise after Mustard's operation.
Right ventricular (RV) performance during supine bicycle exercise was evaluated by gated equilibrium nuclear angiography in 19 clinically well children with d-transposition of the great arteries (d-TGA), 6.4 +/- 2.7 years after Mustard's operation. Comparisons were made between rest and peak exercise. The mean resting ejection fraction was 44 +/- 12% (range 30-75%) and was unchanged at peak exercise. Eight children had a normal ejection fraction response, whereas 11 children had either no increase or a decrease in ejection fraction. Relative end-diastolic volumes decreased from resting values in all patients who had an abnormal ejection fraction response. Among patients whose ejection fraction increased, the end-diastolic volume increased in three, decreased in four and was unchanged in one at peak exercise. Heart rate increased 84% (range 52-135%) and systolic blood pressure increased 16% (range 0-28%) at peak exercise. There was no correlation between exercise response and age at surgery or interval since surgery. These data indicate that clinically well children after Mustard's procedure may have abnormal right ventricular function under stress, raising concerns about the ability of the right ventricle to function as the systemic ventricle.
Adolescent↗
Prostaglandins and the ductus arteriosus.
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Animals↗
Role of prostaglandin E1 infusion in the management of transposition of the great arteries.
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Ductus Arteriosus, Patent↗
Left ventricular hemodynamic effects of rapid, in utero intravascular transfusion in anemic fetal lambs.
We investigated the acute hemodynamic effects of in utero fetal intravascular transfusion in anemic fetuses. A conductance catheter technique of measuring left ventricular (LV) volume and pressure-volume analysis was employed in six anemic ovine fetuses of 131 days gestation (range 131-133 days). The mean fetal weight at necropsy was 3,795 +/- 166g. An intravascular transfusion of 120 ml packed maternal red cells was given over 12 minutes (rate of 2.6 +/- 0.1 ml/kg/min) increasing the hematocrit from 22.5 +/- 1.3% to 41.8 +/- 0.8%. The infusion of packed red cells leads to an approximate increase in blood volume of 30%. Heart rate, left ventricular stroke volume, and contractility, as assessed by end-systolic elastance, did not change significantly with transfusion. Left ventricular afterload, as assessed by effective arterial elastance, increased from 11.4 +/- 1.4 to 18.6 +/- 2.8 (P < 0.01) mmHg/ml with transfusion, returning to baseline levels by 60 minutes posttransfusion. During the transfusion, end-diastolic pressure increased from 4.1 +/- 0.4 to 10.6 +/- 0.8 (P < 0.01) mmHg and end-diastolic volume increased from 2.0 +/- 0.3 to 2.9 +/- 0.5 ml/kg. However, by 60 minutes posttransfusion, end-diastolic pressure had returned to baseline levels, whereas end-diastolic volume remained elevated at 3.2 +/- 0.6 ml/kg. We conclude that the persistent increase in end-diastolic volume implies a possible increase in LV diastolic compliance.
Anemia↗
Pulmonary artery tears following balloon valvotomy for pulmonary stenosis.
Tears involving the anterior supravalvar annulus developed in 2 children and an infant following percutaneous balloon pulmonary valvotomy using oversized balloons. The 3 patients had angiographic features of three different types of stenotic valves: usual pulmonary valve stenosis in 1, a form of dysplastic pulmonary valve with supravalvar narrowing in a second, and a doming valve in a neonate. All had a successful reduction in right ventricular outflow tract gradient following the procedure. The pulmonary arterial tears were not associated with balloon rupture or clinical symptoms. It is postulated that the relative deficiency of elastic fibers in the supravalvar commissure makes this site relatively vulnerable to intimal tearing.
Catheterization↗
Creation and enlargement of atrial defects in congenital heart disease.
Transcatheter creation and enlargement of interatrial defects (IAD) may improve hemodynamics; however, procedural outcomes have not been well defined. Hospital records were reviewed for children who underwent percutaneous procedures to create and enlarge an IAD and were grouped as follows: (1) right and (2) left heart obstructive lesions, (3) left atrial (LA) decompression during left heart assist, (4) failing Fontan circulation, and (5) miscellaneous. Forty-five children (mean age, 3.4 +/- 4.7 years; 30 (67%) male) were identified. In group 1 (n = 6), all achieved endpoints of right atrial (RA) decompression (n = 2), improved left ventricular filling (n = 3), or improved arterial saturations (n = 1). In group 2 (n = 18), mean LA pressure decreased (21 +/- 6 to 13 +/- 5 mmHg, p < 0.001) and arterial saturations increased (61 +/- 13% to 78 +/- 11%, p < 0.001). All except 2 patients achieved definitive repair, further palliation (n = 9), or heart transplantation (HTX) (n = 7). In group 3 (n = 5), the LA was decompressed (21 to 13 mmHg, p = 0.03) in all, and all except 1 patient survived to HTX (n = 2) or full recovery (n = 2). In group 4 (n = 11), of 7 patients with a low cardiac output syndrome after surgery, despite improved atrial shunting, 3 died and 1 required a HTX. In group 5 (n = 5), RA decompression (n = 1) or improved arterial saturation (n = 4) was achieved in all. Overall, 5-year HTX free survival was 75%. Mechanical ventilation before the procedure (p < 0.001), the need for a blade septostomy (p = 0.002), and higher LA pressures after the procedure (p = 0.04) independently predicted mortality or the requirement for HTX. Transcatheter optimization of an atrial communication can help optimize treatment strategies and has a low procedural risk.
Cardiac Catheterization↗