Ebstein's anomaly: echocardiographic and clinical features in the fetus and neonate.

To determine which morphologic features are associated with early death, the complete echocardiograms and medical records of 16 consecutive patients with Ebstein's anomaly and concordant atrioventricular connections who presented in the fetal (n = 5) or neonatal (n = 11) period were reviewed. The cohort was classified into two groups on the basis of survival at 3 months. Group 1 consisted of seven patients who died at less than or equal to 3 months of age, and Group 2 consisted of the nine surviving patients. Comparing Groups 1 and 2, the respective incidence rates of morphologic features that correlated with early death (p less than 0.05) included tethered distal attachments of the anterosuperior tricuspid leaflet (86% versus 11%), right ventricular dysplasia (86% versus 0%), left ventricular compression by right heart dilation (71% versus 11%) and the area of the combined right atrium and atrialized right ventricle being greater than the combined area of the functional right ventricle, left atrium and left ventricle (57% versus 0%) measured in the apical four chamber view. Right ventricular dysplasia was present in all patients with marked right atrial and atrialized right ventricular enlargement, in 86% of patients with tethered anterior leaflets and in 83% of those with left ventricular compression; 86% of patients with right ventricular dysplasia had tethered distal attachments. In conclusion, echocardiography defines morphologic features in the fetus and neonate that are highly predictive of death by 3 months of age.

Malaligned outlet septum with subpulmonary ventricular septal defect and abnormal ventriculoarterial connection: a morphologic spectrum defined echocardiographically.

A retrospective analysis of 62 echocardiograms from 22 patients with malaligned outlet septum, subpulmonary ventricular septal defect and abnormal ventriculoarterial (VA) connection was performed and the results were confirmed by intraoperative inspection, cardiac catheterization and angiography, or pathologic specimen analysis in 21 of the 22 patients. The purpose of this study was to determine the relative incidence of the primary morphologic features; the correlation between the presence or absence of mitral valve to pulmonary valve fibrous continuity and the type of VA connection; the types and incidence of additional cardiovascular anomalies; and the correlation between the determination of the VA connection by echocardiography versus that ascertained at intraoperative and autopsy inspection. A spectrum of VA connections was found, ranging from double outlet right ventricle (23%) at one extreme, through an intermediate type (64%) in which the pulmonary artery was overriding the ventricular septum, thereby having no consistent and predominant connection to either ventricle, to a discordant VA connection (13%) at the opposite end of the spectrum. All three types of VA connection occurred whether or not there was mitral valve to pulmonary valve fibrous continuity. Additional cardiovascular anomalies were present in 95% of patients, the most common being aortic arch obstructive lesions in 59%, anomalous attachments of the atrioventricular valve tension apparatus in 55% and subaortic stenosis in 50%. A method of echocardiographic determination of the VA connection that correlated with that determined intraoperatively and from pathologic specimen analysis is described.

Intraoperative transesophageal echocardiography of atrioventricular septal defect.

To determine the accuracy and utility of single-plane transesophageal echocardiography in analyzing atrioventricular (AV) septal defect, intraoperative transesophageal echocardiography was performed before and after institution of cardiopulmonary bypass in 16 patients (age 24 days to 14 years, weight 3 to 47 kg). Prebypass transesophageal echocardiography (including two-dimensional echocardiography, Doppler color flow mapping and pulsed wave Doppler ultrasound) correctly diagnosed divided AV valve, common AV valve and unbalanced AV valve, as well as atrial or ventricular septal defect, or both, in all cases. It correctly analyzed AV valve regurgitation in all 10 patients with right and all 14 with left AV valve regurgitation and correctly analyzed 30 of 33 additional cardiac anomalies. Transesophageal echocardiography was able to detect the absence of normal pulmonary venous connections but failed to demonstrate all of the complex anomalous pulmonary venous connections in three patients with atrial isomerism. Postbypass transesophageal echocardiography documented the absence of a significant residual shunt in 11 of 11 patients undergoing corrective surgery and verified residual AV valve regurgitation in 7 of 9 patients with tricuspid regurgitation and 11 of 13 with mitral regurgitation. Transesophageal echocardiographic information that altered or refined the surgical treatment was obtained in 5 (31%) of 16 patients. Epicardial and transesophageal echocardiography results were concordant in all 13 patients in whom both were performed. Transesophageal echocardiography provides useful and accurate imaging of the important two-dimensional, pulsed wave Doppler ultrasound and Doppler color flow mapping features in AV septal defect.

Intraoperative echocardiography in infants and children with congenital cardiac shunt lesions: transesophageal versus epicardial echocardiography.

To determine the utility and limitations of intraoperative transesophageal echocardiography in infants and children with congenital intracardiac shunts, intraoperative transesophageal (n = 50) and epicardial (n = 49) echocardiograms were performed before and after cardiopulmonary bypass in children from 4 days to 16 years old and 3 to 45 kg in body weight. A miniaturized transesophageal probe (6.9 mm maximal diameter) was used in 36 patients weighting less than or equal to 20 kg. Epicardial imaging was performed with a 5 MHz precordial probe. The intraoperative transesophageal echocardiographic findings before and after cardiopulmonary bypass were correct and complete in 94% of patients. Transesophageal echocardiography correctly identified atrial septal defects, most types of ventricular septal defects, anomalous pulmonary veins, atrioventricular septal defects, tetralogy of Fallot, truncus arteriosus and double inlet ventricles. It failed to provide a correct diagnosis in only three patients, all of whom had doubly committed subarterial ventricular septal defects. Epicardial echocardiography identified all cases that had a doubly committed subarterial ventricular septal defect. A correct and complete intraoperative diagnosis was obtained with the use of epicardial imaging in 92% before and after cardiopulmonary bypass, but this technique required interruption of surgery and could not be completed in three patients because of induced arrhythmias and hypotension. These results demonstrated that intraoperative transesophageal echocardiography consistently defined important morphologic, color and pulsed Doppler ultrasound features of most congenital shunt lesions. Lesions that involved the right ventricular outflow tract are sometimes difficult to image with uniplane transesophageal echocardiography. There were no complications in any of the 50 subjects.

Extended aortic valvuloplasty for recurrent valvular stenosis and regurgitation in children.

Recurrent significant aortic valvular stenosis or regurgitation, or both, after balloon or open valvotomy in pediatric patients often necessitates aortic valve replacement. In an attempt to preserve the aortic valve, we performed extended aortic valvuloplasty in 21 children with recurrent aortic valve stenosis or regurgitation from January 1989 to March 1993. Previous related procedures were one open aortic valvotomy or more (n = 15), balloon valvotomy (n = 4), balloon valvotomy after surgical valvotomy (n = 1), and repair of iatrogenic valve tear (n = 1). Mean age at the time of the extended aortic valvuloplasty was 6 +/- 3.4 years. Mean pressure gradient across the aortic valve was 56 +/- 12 torr. Regurgitation was moderate (grade 2 to 3) in nine and severe (grade 4) in 12 patients. Extended aortic valvuloplasty techniques consisted of thinning of valve leaflets (n = 15), augmentation of scarred and retracted leaflets with autologous pericardium (n = 11), resuspension of the augmented leaflet (n = 14), release of the rudimentary commissure from the aortic wall (n = 5), extension of the valvotomy incision into the aortic wall on both sides of the commissure (n = 20), patch repair of the sinus of Valsalva perforation (n = 1), reapproximation of tears (n = 5), and narrowing of the ventriculoaortic junction (n = 2). No operative deaths occurred. The postoperative mean pressure gradient, assessed by most recent Doppler echocardiography or cardiac catheterization at a follow-up of 18 +/- 6 months, was 19 +/- 6 torr (p < 0.01 versus the preoperative gradient). Aortic regurgitation was absent in 13, mild in 6, and moderate-to-severe, necessitating subsequent aortic valve replacement, in 2. This short-term experience indicates that extended aortic valvuloplasty is a safe and effective surgical approach that minimizes the need for aortic valve replacement in children with significant recurrent aortic valve stenosis or regurgitation.

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.

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)

Resternotomy in patients with valved conduits adherent to the sternum.

Twenty-two patients with valved conduits adherent to the sternum underwent resternotomy. Mean age was 10 +/- 6 years, and mean conduit age was 4 +/- 4 years. Diagnoses were D-transposition (7), truncus arteriosus (7), univentricular heart (6), Taussig-Bing anomaly (1), and corrected transposition (1). The majority of patients (68%) had reoperation for outgrown or degenerated conduits. In 17 patients, the sternum was opened with a chisel. Two of these patients sustained conduit neointimal collapse from manipulation, and 3 had conduit tear requiring immediate cardiopulmonary bypass through the femoral vessels. In the last 5 patients, the sternum was opened above and below the conduit, and the inner table was chiseled and left attached to the conduit avoiding injury and undue conduit manipulation. Cardiopulmonary bypass and operation were carried out uneventfully. We believe that the recent technique described provides a safe alternative approach to valved conduits adherent to the sternum.

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.

Alternative techniques for surgical management of recoarctation.

OBJECTIVE: To evaluate the different surgical options in patients with recoarctation and minimal collaterals. METHODS: Thirty-three cases operated on between January 1980 and January 1995 were reviewed. Initial repair was end-to-end anastomosis in 16 patients, subclavian artery aortoplasty in 10, synthetic patch aortoplasty in 4 and bypass conduit in 3 patients. Age at reoperation was 7.5 +/- 5.2 years (1-17 years). Pressure gradient was 20-48 Torr (33 +/- 9). Upper extremity resting or exercise systemic hypertension was present in all. In 18 patients recoarctation was repaired using subclavian artery aortoplasty (n = 15) or synthetic patch aortoplasty (n = 3); alone in 9, with temporary heparinized bypass in 2, or in addition to placement of ascending aorta to descending aorta conduit as a permanent bypass through a left thoracotomy in 9. In 13 patients a conduit was interposed between ascending aorta and descending aorta through a right thoracotomy. In one patient recoarctation segment was patched on cardiopulmonary bypass through a midsternotomy. RESULTS: There was no mortality or complications. All patients had no echocardiographic pressure gradients across recoarctation on 5 +/- 3.4 years follow-up. Persistent systemic hypertension following recoarctation repair was present in 3/8 patients (37%) operated on at age greater than 10 years, but has been resolved in all 25 patients less than 10 years of age (P = 0.02). CONCLUSIONS: Use of ascending aorta to descending aorta conduit, either alone through a right thoracotomy, or as permanent bypass in combination with patching the recoarctation through a left thoracotomy provides safe and excellent relief of obstruction.

Unusual atrial septal anatomy resulting in an interatrial chamber: the true triatrial heart?

We report two patients who were found to have nearly identical, very peculiar atrial septal anatomy. The septum actually consisted of two distinct septa with discrete defects creating an interatrial chamber. The orifice from the left atrium was unrestrictive, but the orifice to the right atrium was restrictive. Overall, there was net left-to-right shunting. This finding represents a clinical dilemma: Left untreated, the interatrial chamber might be a nidus for thrombus formation, but attempting device closure might result in incomplete obliteration of the chamber, also resulting in potential locus for clot formation. Clot formation might lead to systemic embolization. Angiographic findings are correlated with echocardiographic findings. Embryology and treatment options are considered.

Color Doppler flow mapping of the patent ductus arteriosus in very low birthweight neonates: echocardiographic and clinical findings.

Forty-eight preterm infants (mean birthweight 1.0 +/- 0.3 kg; mean gestational age 28 +/- 3 weeks) underwent serial echocardiograms and physical examinations in order to determine the correlation between color Doppler flow mapping (CDFM) results and physical findings of a patent ductus arteriosus (PDA), the predictive value of early CDFM as an indicator of subsequent requirement for treatment of a PDA, and to determine the direction and duration of ductal shunting and the rate of ductal closure and opening. CDFM analysis and cardiac physical examination of left-to-right ductal shunting were usually concordant in infants with a large PDA shunt, the most reliable physical finding being increased precordial activity. CDFM studies on day 2 or 3 of postnatal life had prognostic value with regard to subsequent need for closing the PDA. Additional findings included the absence of right-to-left PDA shunting in infants < 1 kg and < 28 weeks gestation and the absence of ductal reopening in infants in whom it had closed spontaneously. After complete PDA closure using indomethacin, subsequent ductal reopening is uncommon, except in infants < 25 weeks gestation and < 700 g bodyweight.

The effect on the hemodynamic stability of varying calcium chloride administration during protamine infusion in pediatric open-heart patients.

IMPLICATIONS: We conducted a randomized study in 147 pediatric patients undergoing cardiopulmonary bypass to determine when there are any differences in hemodynamic effects if CaCl(2) 20 mg/kg and protamine 5mg/kg are mixed together and infused over 10 min versus administering half of the calcium dose (10 mg/kg) as a bolus followed by a 10-min infusion of protamine 5 mg/kg and CaCl(2) 10mg/kg.

Intraoperative transesophageal echocardiography of ventricular septal defect.

The accuracy and limitations of intraoperative two-dimensional (2-D) and color Doppler flow mapping transesophageal echocardiography (TEE) of ventricular septal defect (VSD), before and after cardiopulmonary bypass, were analyzed in 62 children. Twenty-one patients had an isolated VSD, and 41 had a VSD plus additional cardiac anomalies. Two-dimensional and color Doppler flow mapping TEE were performed with a miniaturized 5-MHz single (transverse) plane transducer in the 51 of 62 patients weighing less than 20 kg. The remaining 11 were monitored using a single plane adult probe (n = 4) and a biplane (transverse plus longitudinal) probe (N = 7). Prebypass TEE provided a correct diagnosis in 57 of 62 cases (92%) and corrected an erroneous preoperative transthoracic echocardiographic diagnosis in three of 62 cases (5%). Single plane TEE diagnosis was erroneous in five patients: four with doubly-committed subarterial VSD and one with multiple small apical muscular defects and pulmonary hypertension. Biplane TEE (transverse longitudinal) provided clear and complete imaging of the right ventricular outflow tract in all seven cases in whom it was used. Postbypass TEE showed absence of a hemodynamically significant residual VSD in 30 of 40 patients (95%) who underwent VSD patch closure, prospectively identified two of 40 with significant residual VSD, and accurately measured the color Doppler jet width of all residual VSDs. We conclude that hemodynamically significant VSDs can be identified immediately after cardiopulmonary bypass based on the width of the residual VSD color Doppler flow map jet. Therefore, 2-D and color Doppler flow mapping TEE provide an accurate diagnosis in most cases of VSD but may miss doubly-committed subarterial and apical muscular VSD unless biplane TEE is used.

Intraoperative echocardiography for evaluation of congenital heart defects in infants and children.

To determine the accuracy, utility, and limitations of intraoperative transesophageal echocardiography (TEE) in infants and children, we performed prebypass and postbypass TEE in 90 children undergoing surgical repair of congenital heart lesions, comparing the results to those obtained using intraoperative epicardial echocardiography and pre- and postoperative precordial echocardiography. Patients ranged in age from 4 days to 21 yr (mean 4.1 yr) and in weight from 3 to 68 kg (mean 15.4 kg). Prebypass, we obtained high-quality, two-dimensional TEE images in 86 patients, with correction of the preoperative precordial diagnosis in 3 and confirmation of the preoperative diagnosis in the rest. Adequate epicardial images were obtained in 78 patients, with confirmation of the preoperative diagnosis in all. Shunt lesions that were well delineated prebypass by both TEE and epicardial imaging included interatrial, interventricular, and atrioventricular septal defect lesions. TEE failed to detect the exact size and location of lesions involving the right ventricular outflow tract, i.e., doubly committed subarterial (supracristal) ventricular septal defects. Regurgitant lesions (n = 30) were identified and their severity evaluated in all patients by both TEE and epicardial imaging. Obstructive lesions (n = 33), excluding those involving the right ventricular outflow tract, were well defined by both echocardiographic approaches. Postbypass, we obtained high-quality, two-dimensional, color and Doppler TEE images in 86 patients and epicardial images in 78 patients.(ABSTRACT TRUNCATED AT 250 WORDS)