Barriers to prenatal detection of congenital heart disease: a population-based study.

OBJECTIVE: To evaluate the extent and determinants of missed prenatal detection of congenital heart disease (CHD) in a population-based setting. METHODS: This was a retrospective cohort study of cases with CHD, excluding minor defects, identified between 1997 and 2007 by a statewide surveillance program. We examined a comprehensive list of potential risk factors for which data were available in the surveillance database from abstracted medical charts. We analyzed the association of fetal, maternal and encounter factors with 1) whether a prenatal ultrasound was performed and 2) prenatal detection of CHD. RESULTS: CHD was detected prenatally in only 39% of 1474 cases, with no improvement in detection rate over the 10-year period. Among the 97% (n = 1431) of mothers who underwent one or more ultrasound examinations, 35% were interpreted as abnormal; fetal echocardiography was performed in 27% of the entire cohort. Maternal and encounter factors increasing the adjusted odds of prenatal detection included: family history of CHD (OR, 4.3 (95% CI, 1.9-9.9)), presence of extracardiac defects (OR, 2.7 (95% CI, 1.9-3.9)) and ultrasound location i.e. high risk clinic vs clinic (OR, 2.1 (95% CI, 1.3-3.1)). Defects that would be expected to have an abnormal outflow-tract view were missed more often (64%) than were those that would be expected to have an abnormal four-chamber view (42%). CONCLUSION: The majority of CHD cases over the 10-year study period were missed prenatally and detection rates did not increase materially during that time. The failure to detect CHD prenatally was related to encounter characteristics, specifically involving screening ultrasound examinations, which may be targeted for improvement.

Surgical repair is safe and effective after unsuccessful balloon angioplasty of native coarctation of the aorta.

Since 1985 balloon angioplasty, followed by surgical repair if angioplasty is unsuccessful, has been used as a treatment strategy for eligible children with discrete native coarctation of the aorta. Although balloon angioplasty has been successful in most patients, this strategy is appropriate only if surgery is safe and effective in children in whom angioplasty does not succeed. To address this issue, the surgical procedure and clinical outcome in 11 children who underwent surgery after unsuccessful balloon angioplasty (defined as a residual systolic gradient greater than 20 mm Hg in 10 and a saccular aneurysm in 1) were evaluated. Data for subjects were compared with data for a control group of seven children who had surgical repair of a discrete coarctation without prior angioplasty during the same time period. In the study group, balloon angioplasty was performed at 4.3 +/- 1.2 years of age, resulting in a balloon/isthmus ratio of 0.98 +/- 0.05 and decreasing mean peak systolic gradient from 54 +/- 3 to 27 +/- 2 mm Hg (p less than 0.001). Follow-up angiography (n = 7) or nuclear magnetic resonance imaging (n = 4) documented a discrete residual stenosis in 10 patients and a small saccular aneurysm in 1. Collateral circulation decreased in three patients. The subsequent surgical procedure and its outcome were similar in the study and control groups. Chylothorax was the only complication, occurring in one child from each group. No paraplegia or mortality occurred. Pathologic examination revealed irregular intimal surfaces with small flaps of intima in 5 of 10 resected specimens from the study group and in 2 of 6 from the control group.(ABSTRACT TRUNCATED AT 250 WORDS)

Quantitative assessment of ventricular function in children with single ventricles using the Doppler myocardial performance index.

Assessment of ventricular function in patients with functionally single ventricles and unusual ventricular geometry is difficult. The Doppler myocardial performance index (MPI) may provide a method of assessing function in these patients. The purposes of this study were to compare the MPI in normal children and in children with a functionally single ventricle, and to compare the MPI in patients with single ventricles before and after bidirectional cavopulmonary anastamosis (BCPA). Echocardiograms of all patients who underwent BCPA between January 1994 and December 1998 were reviewed before and after surgical palliation. Age-matched normal subjects (n = 30) served as controls. The MPI was calculated from Doppler tracings of ventricular inflow and outflow. Of the 60 patients with single ventricles who underwent BCPA, 41 had adequate preoperative Doppler studies to allow calculation of the MPI, and 21 of 41 had adequate studies before and after BCPA. The MPI was higher in patients with single ventricles than in normal controls (0.67 +/- 0.14 vs 0.32 +/- 0.10, p < 0.001). There was no significant difference in the MPI in patients with single ventricles before and after surgery (0.70 +/- 0.16 vs 0.66 +/- 0.15). However, patients who had a BCPA at < 1 year of age had a significant decrease in the MPI after surgery (0.71 +/- 0.10 vs 0.61 +/- 0.11, p = 0.01). Compared with controls, the MPI was significantly higher in patients with single ventricles suggesting an altered hemodynamic state consistent with decreased ventricular function. The MPI decreased in patients who underwent BCPA at < 1 year of age, suggesting an improvement in ventricular function. The MPI provides an objective method of assessing and following ventricular function in patients with single ventricles that is independent of ventricular geometry.

Abnormal Doppler pulmonary venous flow patterns in children after repaired total anomalous pulmonary venous connection.

Doppler echocardiography was used to evaluate pulmonary venous flow patterns in 16 children with repaired total anomalous pulmonary venous connection and in 16 age-matched normal controls. Using right upper pulmonary venous pulsed Doppler tracings, peak velocities and velocity time integrals were determined for ventricular systole, ventricular diastole, and atrial systole. Mitral inflow indexes and cardiac outputs were obtained. Patients with repaired total anomalous pulmonary venous connection and controls were similar in weight, heart rate, mitral inflow indexes, and cardiac output. In normal children, peak velocities were greater during ventricular diastole than systole, but velocity time integrals were greater during ventricular systole than diastole. Compared with normals, repaired patients had unobstructed flow patterns with increased peak velocities during ventricular diastole (0.92 +/- 0.35 vs 0.62 +/- 0.12 m/s) and atrial systole (0.27 +/- 0.12 vs 0.17 +/- 0.04 m/s). Velocity time integrals of repaired patients were increased during atrial systole (0.02 +/- 0.01 vs 0.01 +/- 0.03 m) but decreased during ventricular systole (0.08 +/- 0.03 vs 0.12 +/- 0.03 m). Systolic-to-diastolic ratios were decreased in repaired patients for peak velocity (0.56 +/- 0.20 vs 0.79 +/- 0.12) and velocity time integral (0.6 +/- 0.18 vs 1.48 +/- 0.35). Thus, pulmonary venous flow in normal children is greater during ventricular systole than during ventricular diastole. Repaired patients show a shift in forward flow from ventricular systole to diastole, with greater reversed flow during atrial systole.

Doppler evaluation of aortic regurgitation in children.

Doppler indexes have been used successfully to determine the severity of aortic regurgitation (AR) in adults but have not been evaluated systematically in children. To evaluate the accuracy of specific Doppler echocardiographic indexes in assessing the degree of AR in children, 30 children underwent 2-dimensional and Doppler echocardiography within 24 hours of angiography. Patients were divided into 4 groups based on the degree of angiographic AR. Color Doppler jet width, short-axis jet area, jet length, and maximum jet area were measured. AR slope was measured using continuous-wave Doppler. Flow in the abdominal aorta was evaluated using pulsed Doppler. Doppler indexes were compared with the angiographic grade of AR. Jet width and short-axis jet area were significantly different between groups and showed strong correlation with the angiographic grade. Holodiastolic flow reversal in the abdominal aorta separated 1+ to 2+ from 3+ to 4+ AR (100% sensitivity and 100% negative predictive value for 3+ to 4+ AR). Jet length, maximum jet area, and the ratio of reverse to forward abdominal aortic velocity time integrals correlated with angiography but showed little difference between groups that differed by only 1 angiographic grade. AR slope did not correlate with the angiographic grade. We conclude that in children, color Doppler jet width, short-axis jet area, and holodiastolic abdominal aortic flow reversal are the best predictors of angiographic severity. Use of these indexes may obviate the need for angiography to determine the degree of AR in children.

A novel approach to pediatric intraaortic balloon pump timing using M-mode echocardiography.

Timing balloon inflation and deflation is difficult in pediatric patients undergoing intraaortic balloon pumping, because the electrocardiogram and arterial pressure tracings are inadequate markers of the onset of diastole and systole. M-mode echocardiography provides a precise marker of aortic valve opening and closure, as well as balloon motion, and allows accurate timing by real-time adjustment of balloon inflation to diastole and deflation to systole.

Influence of left ventricular cavity size on interventricular shunt timing and outcome in neonates with coarctation of the aorta and ventricular septal defect.

In this study, we reviewed the records and echocardiograms of 39 consecutive patients with coarctation of the aorta and ventricular septal defect who underwent neonatal coarctation repair to examine the influence of left heart size on ventricular shunting and outcome. We found smaller left heart structures (initial mitral and aortic annular diameters) to be associated with diastolic interventricular shunting and to be predictive of the development of mitral or aortic and/or subaortic stenosis.

Usefulness of the preoperative tricuspid/mitral valve ratio for predicting outcome in pulmonary atresia with intact ventricular septum.

Although tricuspid valve z-scores have been used to predict outcome in pulmonary atresia with intact ventricular septum, they are statistically generated from local populations, and widespread generalization may not be appropriate. To determine if there are echocardiographic predictors of outcome that can be universally used, the records of all infants with this diagnosis since 1988 were reviewed for age, weight, type of surgery, and outcome. Preoperative and follow-up echocardiograms were reviewed for valve diameter and z-scores, and valve ratios were calculated. Thirty-six patients were divided into 2 groups: group 1 included 23 infants who had a successful biventricular repair; group 2 included the remaining 13 infants who did not have a successful repair. Preoperatively, both groups had similar ages, pulmonary, aortic, and mitral z-scores, and pulmonary/aortic ratios, but the patients in group 2 had significantly lower weight (3.5 +/- 0.6 vs 2.9 +/- 0.5 kg), tricuspid z-scores (-0.7 +/- 1.5 vs -2.3 +/- 1.2), and tricuspid/mitral ratios (0.8 +/- 0.2 vs 0.5 +/- 0.1). At similar follow-up, both groups of patients had similar weight, aortic and mitral z-scores, and pulmonary/aortic ratios, but group 2 infants had significantly lower pulmonary and tricuspid z-scores and tricuspid/mitral ratios. Compared with the preoperative echocardiograms, group 1 had significant increases only in pulmonary z-scores, and pulmonary/aortic and tricuspid/mitral ratios. Group 2 had no significant change in any echocardiographic variable. The tricuspid/mitral ratio was >0.5 in all group 1 infants, and in 6 of 13 group 2 infants (2 sepsis deaths, 4 palliations). Compared with a tricuspid valve z-score >-3, a tricuspid/mitral ratio >0.5 was a better predictor of biventricular repair. Thus, infants who have a successful biventricular repair have significantly greater preoperative weight, tricuspid valve z-scores, and tricuspid/mitral valve ratios. A tricuspid/mitral ratio >0.5 was the best predictor of a biventricular repair.

Usefulness of echocardiography for detection of coronary artery thrombi in patients with Kawasaki disease.

To evaluate the role of echocardiography for predicting and accurately detecting thrombi in patients with abnormal coronary arteries after Kawasaki disease, we reviewed the echocardiograms of 40 consecutive patients and compared echocardiographic findings with angiographic findings when available. Patients with Kawasaki disease who had coronary artery aneurysms > or =5 mm had significantly greater multivessel involvement, thrombi, and persistent coronary abnormalities than those with diameters <5 mm.

Size of ventricular structures influences surgical outcome in Down syndrome infants with atrioventricular septal defect.

To evaluate the relation between ventricular structure size and surgical outcome in Down versus non-Down syndrome infants with an atrioventricular septal defect, we reviewed the charts and echocardiograms of 44 consecutive infants (34 with Down syndrome) who underwent atrioventricular septal defect repair. Children with Down syndrome had significantly greater aortic valve diameters, left ventricular valve areas, and left/right atrioventricular valve area ratios as well as fewer adverse outcomes than non-Down syndrome children.

The management of severe subaortic stenosis, ventricular septal defect, and aortic arch obstruction in the neonate.

Neonates with ventricular septal defect and aortic arch obstruction frequently have subaortic stenosis resulting from posterior deviation of the infundibular septum. Because the aortic anulus is often hypoplastic, making direct resection of the infundibular septum through the standard transaortic approach difficult, the optimal method of repair is uncertain. From September 1989 through November 1991, seven patients with ventricular septal defect, coarctation (n = 4), or interrupted aortic arch (n = 3) and severe subaortic stenosis underwent repair with use of a technique that included transatrial resection of the infundibular septum. Their ages ranged from 5 to 63 days (median 15 days) and weights from 1.3 to 5.4 kg (mean 3.1 kg). Only one patient was older than 1 month. The systolic and diastolic ratios of the diameter of the left ventricular outflow tract to that of the descending aorta were 0.53 +/- 0.09 mm (standard deviation) and 0.73 +/- 0.11, respectively. At operation, the posteriorly displaced infundibular septum was partially removed through a right atrial approach by resecting the superior margin of the ventricular septal defect up to the aortic anulus. The resulting enlarged ventricular septal defect was then closed with a patch to widen the subaortic area. In each patient the aortic arch was repaired by direct anastomosis. All patients survived operation; there was one late death from noncardiac causes 3 months after repair. The survivors remain well from 3 to 14 months after repair (mean 8 months). All are in sinus rhythm and none has a residual ventricular septal defect. One patient underwent successful balloon dilation of a residual aortic arch gradient late after repair. No patient has significant residual subaortic stenosis, although one has valvular aortic stenosis. This series suggests that in neonates with ventricular septal defect and severe subaortic stenosis resulting from posterior deviation of the infundibular septum, direct relief can be satisfactorily accomplished from a right atrial approach. This method provides effective widening of the left ventricular outflow tract and is superior to palliative techniques or conduit procedures.

Cost and efficacy of surgical ligation versus transcatheter coil occlusion of patent ductus arteriosus.

OBJECTIVE: The purpose of this study was to compare cost and efficacy of surgical closure of patent ductus arteriosus using new critical pathway methods with outpatient transcatheter coil occlusion of patent ductus arteriosus. METHODS: Surgical techniques included a transaxillary, muscle-sparing thoracotomy, triple ligation of the patent ductus arteriosus, no chest tube, and discharge from the hospital within 24 hours. Transcatheter coil occlusion of patent ductus arteriosus was done as an outpatient procedure. Costs were compared with inclusion of all hospital and professional charges. RESULTS: From July 1994 until March 1996, 20 patients underwent coil occlusion of patent ductus arteriosus and 20 patients underwent surgical closure of patent ductus arteriosus. Duration of hospitalization was significantly less for the patients receiving coil occlusion (11 +/- 6 hours) as compared with that for the patients having surgical ligation (28 +/- 7 hours, p < 0.05). Total charges were similar for surgical ligation ($7101 +/- $408) as compared with those for coil occlusion ($7104 +/- $886, p > 0.05). Morbidity in coil occlusion included inability to occlude the patent ductus arteriosus in two patients (2/20, 10%) and residual patency in two patients (2/18, 11%). Morbidity in the surgical group included nausea and vomiting necessitating hospitalization for more than 36 hours in one patient (1/20, 5%), transient left recurrent laryngeal nerve palsy in one (1/20, 5%), and pneumothorax in two patients (2/20, 10%). There were no instances of residual patency in the surgical group. CONCLUSIONS: Transaxillary thoracotomy without tube thoracostomy and with critical pathway methods allows safe and effective ligation of a patent ductus arteriosus with early hospital discharge. This surgical method has similar cost, higher efficacy rate, and applicability in all patients as compared with newer transcatheter coil occlusion techniques for closure of a patent ductus arteriosus.

Left heart hypoplasia and neonatal aortic arch obstruction: is the Rhodes left ventricular adequacy score applicable?

OBJECTIVE: Although the influence of small left heart structures on outcome of a biventricular repair in neonatal critical aortic stenosis is well documented, little is known about its effect in neonates with aortic arch obstruction and coarctation. The purpose of this study was to evaluate the influence of small left heart structures on early and late results of repair and the ability to achieve a biventricular repair in neonates with coarctation and aortic arch obstruction. PATIENTS: Neonates included in this study had a left ventricular adequacy score (as proposed by Rhodes and associates for critical aortic stenosis) that would have predicted a need for a univentricular (Norwood) repair. All were ductus dependent but had antegrade ascending aortic flow and a small but nonstenotic aortic valve (<30 mm Hg gradient). Twenty neonates aged 10 +/- 9 days were identified for the study with weights averaging 3. 1 +/- 0.6 kg. Selected left heart measurements obtained by preoperative echocardiography included the following: aortic anulus 5.3 +/- 0.3 mm, mitral anulus 8.4 +/- 1.0 mm, transverse aortic arch 3.4 +/- 0.6 mm, and left ventricular volume 25 +/- 4 mL/m2. All patients underwent coarctation repair by resection and extended end-to-end anastomosis to enlarge the transverse arch as needed. Three patients underwent simultaneous pulmonary artery banding because of a hemodynamically significant ventricular septal defect. These 3 patients have subsequently had their defects successfully closed without mortality. RESULTS: There were no early or late deaths at a follow-up of 38 +/- 16 months after the operation. Three patients (3/20, 15%) have had to undergo reintervention with balloon aortoplasty because of recurrent coarctation (gradient > 20 mm Hg) in 2 and resection of subaortic stenosis in 1. Late follow-up in the remaining patients reveals 1 with moderate subaortic stenosis (gradient = 43 mm Hg), 2 with mild aortic stenosis (gradient < 30 mm Hg), and 2 with mild to moderate mitral stenosis. At late follow-up, 16 patients (16/20, 80%) are completely free of symptoms and 4 (4/20, 20%) have mild residual symptoms. CONCLUSIONS: Biventricular physiology can be successfully achieved in neonates with small left heart structures and aortic arch obstruction with minimal mortality and excellent late functional results. Standard echocardiographic measurements used to predict the need for a univentricular repair in critical aortic stenosis are not valid for the neonate with aortic arch obstruction.

Intermediate-term survival and functional results after arterial repair for transposition of the great arteries.

An assessment of late morbidity and mortality is essential before arterial repair can be considered truly corrective for patients with transposition of the great arteries. We describe the early and intermediate-term results in 126 patients who underwent arterial repair. Operation was performed at a median age of 6 days, with 76 patients operated on within the first 7 days of life. Coronary artery anatomy differed from the usual arrangement in 37 patients. Simultaneous procedures included ventricular septal defect closure (35) and repair of interrupted aortic arch (2) or coarctation (5). Hospital mortality was seven of 126 (5.5%), with three deaths among the most recent 100 patients (3%). There were one late, noncardiac death and one late death after reoperation. Reoperation for pulmonary artery stenosis was required in 10 of the first 63 patients (16%), all of whom underwent pulmonary artery reconstruction with separate patches for closure of the coronary excision sites. Of the last 63 patients, all of whom underwent pulmonary artery reconstruction with a single pantaloon-shaped pericardial patch, one (2%) required reoperation for pulmonary artery stenosis. Doppler flow studies and echocardiography performed in 115 of 119 surviving patients at a mean of 12 months after repair demonstrated normal left ventricular function, minimal left ventricular outflow gradients, and no more than trivial aortic regurgitation. Peak gradient across the right ventricular outflow tract was 19 +/- 3 mm Hg in patients with separate pulmonary artery patches and 5 +/- 2 mm Hg in those with a single pantaloon patch (p = 0.0001). Follow-up is 96% complete from 1 month to 8 years after operation (mean 2.5 years). The actuarial survival rate at 5 years, including operative mortality, was 92%. All patients are in sinus rhythm, and none requires antiarrhythmic medications. These data suggest that pulmonary artery reconstruction with a single pantaloon patch may be associated with a decreased requirement for reoperation. Intermediate-term survival and functional results are excellent after arterial repair for transposition of the great arteries.

Congenital heart disease with ductal-dependent systemic perfusion: Doppler ultrasonography flow velocities are altered by changes in the fraction of inspired oxygen.

BACKGROUND: An unfavorable balance in systemic and pulmonary perfusion may occur in neonates with ductal-dependent systemic perfusion while being treated with prostaglandin E1 before surgical palliation or transplantation. At our institution, we adjust the fraction of inspired oxygen, with supplemental nitrogen if needed, to control pulmonary vascular tone and maintain systemic oxygen saturations near 75%. METHODS: We performed a noninvasive Doppler ultrasound study in 10 patients to determine whether velocity time integrals in the descending aorta and middle cerebral arteries and the peak velocity at the foramen ovale were acutely altered by changes in the fraction of inspired oxygen. Measurements were performed after 10- to 15-minute intervals of breathing 14% to 19%, 21%, and 35% oxygen. RESULTS: Antegrade descending aortic velocity time integrals did not change significantly when these patients breathed different amounts of oxygen; however, the retrograde velocity time integral in the descending aorta and the peak velocity of left-to-right shunt at the foramen ovale increased with increasing amounts of inspired oxygen. The ratio of antegrade to retrograde velocity time integrals in the descending aorta was greater with supplemental nitrogen than with supplemental oxygen. Middle cerebral arterial velocity time integrals were not significantly greater with supplemental nitrogen than with supplemental oxygen (p = 0.061). CONCLUSIONS: Systemic and interatrial Doppler velocities are acutely influenced by the fraction of inspired oxygen in neonates with ductal-dependent systemic perfusion.

Intra-aortic balloon pumping in children with dilated cardiomyopathy as a bridge to transplantation.

Children with dilated cardiomyopathy awaiting transplantation who fail maximal pharmacologic therapy may benefit from intra-aortic balloon pumping. Between July 1993 and August 1999, a total of 4 children with dilated cardiomyopathy underwent pre-transplant balloon pumping for 6.0 +/- 5.8 (1 to 12) days. One child (pumped for 12 days) died awaiting transplant, and the remaining 3 were successfully transplanted. Intra-aortic balloon pumping timed precisely with M-mode echocardiographic markers offers a relatively simple and safe intermediate level of mechanical support for children with dilated cardiomyopathy who fail pharmacologic support.

Are screening echocardiograms warranted for neonates with meningomyelocele?

OBJECTIVE: To evaluate the incidence and types of congenital heart defects associated with meningomyelocele. DESIGN: All neonates who underwent meningomyelocele repair and had a perioperative echocardiogram from July 1990 to October 1998 were studied. Medical records were reviewed for age, weight, clinical cardiac examination results, meningomyelocele location, and associated noncardiac defects. Heart defects were identified from reviewing echocardiographic reports and videotapes. RESULTS: At meningomyelocele surgery, the 105 patients (53 female; 52 male) ranged in age from 1 to 20 days and in weight from 0.6 to 4.1 kg. Congenital heart disease was detected in 39 patients (37%). A secundum atrial septal defect was the most common defect (24%). A ventricular septal defect was found in 10 patients, 2 patients had anomalous pulmonary venous return, and 1 each had tetralogy of Fallot, bicuspid aortic valve, coarctation, and hypoplastic left heart syndrome. A patent ductus arteriosus and patent foramen ovale were not considered abnormal in these neonates. The cardiac examination was abnormal in only 5 of the 39 patients with heart defects (sensitivity = 13%). The presence of associated noncardiac defects (in addition to meningomyelocele) and location of the meningomyelocele (cervicothoracic vs lumbar) did not affect the incidence of heart disease. Of the patients with heart defects, girls were more frequently affected (25 of 39 vs 14 of 39, P<.05). CONCLUSIONS: Congenital heart defects are common in neonates, especially girls, with meningomyelocele and are unrelated to meningomyelocele location or associated noncardiac defects. Because the clinical examination is insensitive for detecting heart defects in this group, screening echocardiograms are warranted. This information has important implications for ventriculoatrial shunting, urinary tract instrumentation (antibiotic prophylaxis), and neurosurgical procedures (venous air embolism).

Absorbable polydioxanone suture and results in total anomalous pulmonary venous connection.

BACKGROUND: Despite theoretical advantages of absorbable suture in the growing vascular anastomosis, there has not been a documented advantage over nonabsorbable suture in preventing late anastomotic stenosis in total anomalous pulmonary venous connection (TAPVC). METHODS: We reviewed our experience from 1982 to 1994 with 65 hospital survivors of total TAPVC repair to examine the influence of suture type on survival and incidence of late pulmonary venous obstruction. From 1982 until 1988, we used continuous nonabsorbable polypropylene suture for the pulmonary venous-left atrial anastomosis in supracardiac, infracardiac, and mixed types of TAPVC: In 1989, we adopted a running absorbable polydioxanone suture technique. Cardiac catheterization and echocardiography were used to evaluate late pulmonary venous obstruction. RESULTS: Late pulmonary venous obstruction occurred in 17% (4/23) of survivors after repair with polypropylene suture compared with 3.2% (1/32) after repair with polydioxanone suture (p < 0.05). There were no instances of late pulmonary venous obstruction in the intracardiac TAPVC group (0/10). All late pulmonary venous obstructions occurred within 16 months after operation. The actuarial 3-year and 5-year freedom from late pulmonary venous obstruction was 100% for intracardiac TAPVC, 96% for the polydioxanone group, and 81% for the polypropylene group. Five patients died late (5/65, 7.7%), 3 in the polypropylene suture group (3/23, 13%) and 2 in the polydioxanone group (2/32, 6%). CONCLUSIONS: Continuous absorbable polydioxanone suture for the repair of TAPVC results in a low incidence of late pulmonary venous obstruction and death and appears to offer advantages over a continuous nonabsorbable suture. A continuous nonabsorbable suture may limit growth of a vascular anastomosis, particularly one involving a "low-pressure" anastomosis such as in the repair of TAPVC:

Neonatal piglet model of intraaortic balloon pumping: improved efficacy using echocardiographic timing.

BACKGROUND: Pediatric intraaortic balloon pumping (IABP) has met with little success because of technical difficulty in tracking rapid heart rates. This study was designed to evaluate the efficacy of M-mode echocardiography for IABP timing in a neonatal piglet model. METHODS: Two groups of piglets underwent mitral valve avulsion to create a model of shock. Group 1 (n = 8; mean weight, 7.7+/-1.8 kg) underwent IABP timed with both the ascending aortic pressure and M-mode echocardiogram. Group 2 (n = 6; mean weight, 7.5+/-1.4 kg) underwent two separate periods of IABP: one with echocardiographic timing and the second using standard timing points from the femoral arterial pressure tracing and electrocardiogram. Measurements included ascending aortic flow, left anterior descending arterial flow, ascending aortic pressure, left atrial pressure, and heart rate. RESULTS: Mitral valve avulsion produced a shock model with a significant decrease in mean aortic pressure and aortic flow and a significant increase in left atrial pressure and heart rate. Compared with the shock state, IABP in group 1 animals resulted in a significant increase in aortic flow (353+/-152 versus 454+/-109 mL/min; p < 0.05) and a significant decrease in left atrial pressure (23+/-6 versus 17+/-7 mm Hg; p < 0.05). Group 2 animals with echocardiogram-timed IABP had significantly increased aortic flow (365+/-106 versus 458+/-107 mL/min; p < 0.05) and mean aortic pressure (43+/-11 versus 52+/-8 mm Hg; p < 0.05). However, standard-timed IABP failed to show any improvement. CONCLUSIONS: In piglets with rapid heart rates, echocardiogram-timed IABP results in increased aortic flow and pressure and decreased left atrial pressure compared with standard-timed IABP.

Late results and reintervention after aortic valvotomy for critical aortic stenosis in neonates and infants.

BACKGROUND: Many centers have adopted balloon valvuloplasty for treatment of infants with critical aortic stenosis because of historically poor early results and a lack of long-term results with surgical valvotomy. We evaluated our results with open aortic valvotomy over the past decade, specifically examining factors influencing survival and reintervention in the current era. METHODS: From 1986 to 1996, 37 infants in the first 3 months of life underwent open aortic valvotomy for critical aortic stenosis. All patients underwent cardiopulmonary bypass, valvotomy, and valve debridement under direct vision with standard techniques. RESULTS: Early mortality was 11% (4 of 37, 70% confidence limit 7% to 20%) and all early deaths were in neonates less than 2 weeks of age. Late death occurred in 6 patients a mean of 10 +/- 12 months (range, 2 to 36 months) after valvotomy. Actuarial survival, including operative deaths was 92% +/- 6% at 1 month, 78% +/- 9% at 1 year, and 73.4% +/- 10% at 10 years. In a multifactorial regression analysis, the best predictors of death were the presence of endocardial fibroelastosis and small body surface area and the best predictor of the need for late reintervention was preoperative aortic annular size. Thirteen patients required reintervention: repeat operation in 7 patients, balloon valvuloplasty in 3 patients, and both balloon valvuloplasty and reoperation in 3 patients. Actuarial freedom from reintervention postoperatively is 97% +/- 3% at 1 month, 73% +/- 9% at 1 year, and 55% +/- 11% at 10 years. Reintervention was for recurrent left ventricular outflow obstruction in 9 patients and mixed aortic stenosis and aortic insufficiency in 4. Echocardiography 4.3 +/- 2.5 years after aortic valvotomy in survivors who have not required reintervention (n = 20) revealed a Doppler peak instantaneous systolic gradient of 37 +/- 14 mm Hg and mild or less aortic regurgitation in 16 patients and moderate aortic regurgitation in 4 patients. CONCLUSIONS: Current surgical results with critical aortic stenosis in the neonate and young infant are acceptable in terms of both late survival, reintervention, and functional results in the majority of patients. Newer interventions, such as balloon valvuloplasty, should be carefully evaluated for long-term results and should be compared more appropriately to current surgical results to determine the best treatment modality for the neonate and infant with critical aortic stenosis.