Estimation of circumferential fiber shortening velocity by echocardiography.

The M-mode and two-dimensional echocardiograms of 40 young patients were analyzed to compare the mean circumferential fiber shortening velocity (Vcf) of the left ventricle calculated separately by two methods. The mean circumferential fiber shortening velocity was derived from the M-mode echocardiogram as minor axis shortening/ejection time and derived from the two-dimensional echocardiogram as actual circumference change/ejection time. With computer assistance, circumference was determined from the short-axis two-dimensional echocardiographic images during end-diastole and end-systole. Good correlations were obtained between the left ventricular diameter derived by M-mode echocardiography and the vertical axis during end-diastole (r = 0.79) and end-systole (r = 0.88) derived by two-dimensional echocardiography. Likewise, high correlations were noted between diameter and circumference in end-diastole (r = 0.89) and end-systole (r = 0.88). However, comparison of Vcf obtained by M-mode echocardiography with that obtained by two-dimensional echocardiography showed only fair correlation (r = 0.68). Moreover, the diameter/circumference ratio determined in end-diastole and end-systole differed significantly (p less than 0.001), possibly owing to the change in geometry of the ventricular sector image during systole. Although Vcf derived by M-mode echocardiography is a useful index of left ventricular performance, it does not truly reflect the circumference change during systole.

Comparative response of right and left ventricles to volume overload.

The cardiac volume data of 49 normal children were compared with those of 23 with secundum atrial septal defect and 24 with patent ductus arteriosus. Significantly smaller ventricular end-diastolic volumes were observed in the normal infants than in older children (right ventricle 53.9 versus 75.5 cm3/m2; left ventricle 46.7 versus 63.6 cm3/m2). "Distensibility" of the right ventricle (DRV), left ventricle (DLV) and left atrium increased normally with age. DRV and DLV were similar shortly after birth; thereafter, DRV increased more rapidly than DLV (mean DRV 12.7; mean DLV 7.8 cm3/m2 per mm Hg, P less than 0.001). In both atrial septal defect and patent ductus arteriosus, the ipsilateral (involved) ventricles had increased volume, increased output, normal ejection fraction and increased distensibility. The contralateral (left) ventricle in atrial septal defect was smaller than normal (39.6 versus 49.7 cm3, P less than 0.001), and had a smaller ejection fraction (0.63 versus 0.71, P less than 0.01) and output (3.70 versus 4.57 liters/min per m2, P less than 0.005). In contrast, the contralateral (right) ventricle in patent ductus arteriosus remained normal. Left atrial maximal volume was larger than normal in atrial septal defect (46.6 versus 35.9 cm3/m2, P less than 0.001). The left atrial and left ventricular volumes in patent ductus arteriosus were, respectively, 152 and 142 percent of normal, indicating comparable response to the volume load. The left head changes in atrial septal defect may be related both to a functionally restrictive defect and to the difference in distensibility of the ventricles.

Surgical correction of truncus arteriosus in infancy.

An 8 week old infant with severe heart failure from type 1 truncus arteriosus underwent successful corrective surgery employing the Rastelli procedure with use of deep hypothermia and total circulatory arrest. Postoperative hemodynamic studies showed complete closure of the septal defect, disappearance of truncal stenosis, but presence of mild porcine valve stenosis. This procedure is possible even in very small subjects and is preferable to palliative pulmonary arterial banding.

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.

Endocardial cushion defect associated with cor triatriatum sinistrum or supravalve mitral ring.

Clinical and angiographic or autopsy data, or both, on three children with a subdivided left atrium (cor triatriatum) and an associated endocardial cushion defect are reviewed. (One child had ostium primum defect, and two had complete atrioventricular [A-V] canal.) A fourth patient demonstrates the difficulties in differentiating subdivided left atrium from supravalve mitral stenosis in the presence of an endocardial cushion defect. The clinical findings are greatly influenced by the endocardial cushion defect. A pressure gradient between the pulmonary wedge and (left or right) ventricular end-diastolic pressures in patients with an endocardial cushion defect indicates pulmonary venous obstruction and should alert one to the possibility of these combined lesions. The exact diagnosis is made with injections of angiographic contrast medium into the proximal and distal left atrial chambers, to documented the respective relations of the pulmonary veins, left atrial appendage and A-V valves to these atrial chambers. All three patients with an endocardial cushion defect and a subdivided left atrium had an associated patent ductus arteriosus. The common association of subdivided left atrium with intracardiac, pulmonary venous and aortic anomalies is again demonstrated.

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.

Right juxtaposition of the atrial appendages.

We present an infant with right-sided juxtaposition of atrial appendages who had open heart surgery for ventricular septal defect and patent ductus arteriosus. Of 12 cases thus far reported, ventricular d-loop was observed in nine, and normal position of great vessels in four. Contrary to previous views, this condition may not be accompanied by severe conotruncal anomalies.

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.

Arterial prosthesis of microporous expanded polytetrafluoroethylene for construction of aorta-pulmonary shunts.

A new arterial prosthesis made of polytetrafluoroethylene (OTFE) was evaluated in 10 infants with complex cyanotic congenital heart disease. All grafts used were 4 mm. in diameter and varied in length from 0.8 to 6 cm. The grafts were anastomosed to the main pulmonary artery or its bifurcation in 8 infants and to the right and left pulmonary arteries in one each. There were two early deaths, one of which was related to shunt failure. The advantages of a shunt to the main pulmonary artery are obvious, and the intraoperative procedure is facilitated with the prosthesis. Follow-up averages 9 months in the 8 survivors, and the patients have nearly doubled their preoperative weight. A shunt murmur is present in each case and the children have mild-to-moderate cyanosis at rest. Repeat aortograms in 2 patients, 8 and 10 months postoperatively, show a smooth graft without luminal narrowing. The aortic oxygen saturations were 73 per cent. The PTFE graft and/or anastomoses will not grow with the growth of the child and therefore may not accommodate growth by increased flow. This may prove to be a limiting factor in its long-term use in fants. We reserve the use of these grafts for infants with complex cyanotic defects undergoing emergency surgery or in older children in whom a conventional shunt is not possible or a previous shunt has failed.

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.

Surface cooling (20 degrees C) and circulatory arrest in infants undergoing cardiac surgery. Results in ventricular septal defect, complete atrioventricular canal, and total anomalous pulmonary venous connection.

During a six-year period, 46 severely symptomatic infants (average age, 5.1 months) underwent correction of ventricular septal defect (22 patients), total anomalous pulmonary venous connection (13 patients), and complete atrioventricular canal (11 patients), with the use of surface cooling to 20 degrees C. Cardiac repair was performed during circulatory arrest, and rewarming was performed with a pump oxygenator. Ten patients undergoing repair of ventricular septal defects were studied hemodynamically at 21 degrees C, before repair and at 37 degrees C after rewarming. Heart rate, left ventricular systolic pressure, maximum dp/dt, cardiac index, stroke work, and oxygen consumption were reduced substantially at 21 degrees C. Systemic vascualr resistance was increased at 21 degrees C. All changes were reversible with repair and rewarming. A protocol for hemodilution and crystalloid volume loading was devised to maintain urine output after early patients were noted to demonstrate renal dysfunction. With this protocol, survival rates were 89% for patients with ventricular septal defects, 67% for those with atrioventricular canal defects, and 85% for those with total anomalous pulmonary-venous connection.

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.

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.

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)

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.

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.

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.

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.

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.