What is the optimal age for repair of tetralogy of Fallot?

BACKGROUND: Controversy regarding the timing for the repair of tetralogy of Fallot centers around initial palliation versus primary repair for the symptomatic neonate/young infant and the optimal age for repair of the asymptomatic child. We changed our approach from one of initial palliation in the infant to one of primary repair around the age of 6 months, or earlier if clinically indicated. We examined the effects of this change in protocol and age on outcomes. METHODS AND RESULTS: The records of 227 consecutive children who had repair of isolated tetralogy of Fallot from January 1993 to June 1998 were reviewed. The median age of repair by year fell from 17 to 8 months (P:<0.01). The presence of a palliative shunt at the time of repair decreased from 38% to 0% (P:<0.01). Mortality (6 deaths, 2. 6%) improved with time (P:=0.02), with no mortality since the change in protocol (late 1995/early 1996). Multivariate analysis for physiological outcomes of time to lactate clearance, ventilation hours, and length of stay, but not death, demonstrated that an age <3 months was independently associated with prolongation of times (P:<0.03). Each of the deaths occurred with primary repair at an age >12 months. The best survival and physiological outcomes were achieved with primary repair in children aged 3 to 11 months. CONCLUSIONS: On the basis of mortality and physiological outcomes, the optimal age for elective repair of tetralogy of Fallot is 3 to 11 months of age.

Pulsed Doppler echocardiography in the preoperative evaluation of total anomalous pulmonary venous connection.

Pulmonary venous flow was evaluated by pulsed Doppler echocardiography in 38 patients with total anomalous pulmonary venous connection. Twenty-nine of these 38 had no associated intracardiac anomaly (Group I), and 9 had complex intracardiac anatomy associated with low pulmonary blood flow (Group II). In Group I the drainage was infracardiac in nine, supracardiac in seven, intracardiac in eight and mixed in five. In both groups, in those with venous obstruction the flow in the individual pulmonary veins and ascending or descending vein was nonphasic, varying only with respiration. Flow in the absence of obstruction was phasic, varying with the cardiac cycle. Distal to a site of obstruction the flow was nonlaminar and of high velocity irrespective of the amount of pulmonary blood flow. The pulsed Doppler technique provides important physiologic information in the patient with total anomalous pulmonary venous connection before surgical intervention.

Serial analysis of regional left ventricular wall motion by two-dimensional echocardiography in patients with coronary artery enlargement after Kawasaki disease.

OBJECTIVES: This study was designed to assess the temporal relation between early coronary artery abnormalities and left ventricular function in Kawasaki disease. BACKGROUND: Although late segmental wall motion abnormalities may be seen in patients with Kawasaki disease who have coronary artery stenosis, the impact of early coronary artery abnormalities is unclear. METHODS: Regional left ventricular wall motion was assessed by two-dimensional echocardiography in 18 patients with Kawasaki disease and echocardiographic evidence of coronary artery enlargement at 3 weeks and 3 months and at either 6 or 12 months after the onset of fever. Four patients had a persistent left coronary artery aneurysm, four had regression of their aneurysm, two had persistent left coronary artery ectasia and eight had regression of ectasia. Left ventricular wall motion was assessed by measuring regional area change in parasternal and apical views. After planimetry of an end-systolic and an end-diastolic frame, the ventricle was divided into eight equal segments and the percent area change was calculated. A floating system correcting for translation and rotation was applied. The measurements in the patient group were compared with values previously obtained in 55 normal age-matched infants and children. RESULTS: A transient regional wall motion abnormality 3 and 6 months after the onset of fever was discovered in the inferolateral wall of one patient with a persistent left coronary artery aneurysm. One patient with regression of coronary artery ectasia had a persistent wall motion abnormality in the anterolateral left ventricular wall. There was no correlation between the extent of coronary artery enlargement and the presence or absence of wall motion abnormalities. CONCLUSIONS: These early changes are most likely secondary to associated myocarditis rather than coronary artery abnormalities.

Regional ventricular wall motion abnormalities in tricuspid atresia after the Fontan procedure.

OBJECTIVES: The purpose of this study was to determine whether wall motion abnormalities are present before or after the Fontan procedure in patients with a univentricular heart of the left ventricular type with an absent right atrioventricular valve connection (tricuspid atresia) and to assess the impact of such abnormalities on ventricular performance and clinical outcome. BACKGROUND: Normal systolic and diastolic ventricular function is critical for a successful Fontan repair. However, there have been no previous studies addressing the relation between regional ventricular function and hemodynamic factors. METHODS: Thirty-seven pediatric patients were studied with biplane ventricular cineangiography. There were 20 male and 17 female patients whose mean age at the time of the Fontan operation was 6.5 +/- 3.5 years (range 2.5 to 15.6). Eighteen patients were studied preoperatively, 25 at > 1 year postoperatively and 6 serially. Wall motion was assessed by a centerline method. Normal ranges for wall motion and other variables were established from 25 normal subjects. RESULTS: Wall motion abnormalities were observed in 2 of 18 patients preoperatively and in 11 of 25 patients postoperatively. Age at operation and ventricular volumes did not differ between postoperative patients who had normal (group I, 14 patients) or abnormal (group II, 11 patients) wall motion. However, ventricular mass and the mass/volume ratio were significantly greater and systolic variables and cardiac index were significantly lower in group II versus group I. Two patients in group I were considered to have a clinically poor outcome (persistent heart failure), and five in group II had heart failure, including one who died late. CONCLUSIONS: These observations suggest that postoperative regional wall motion abnormalities in this setting are not rare, may be related to excessive hypertrophy and may contribute to cardiac dysfunction and a poor clinical outcome.

Transcatheter patent ductus arteriosus occlusion: application in the small child.

OBJECTIVES: This study intended to evaluate application of transcatheter occlusion of the patent ductus arteriosus in children < 10 kg body weight. BACKGROUND: Transcatheter occlusion of the patent ductus arteriosus in the child weighing > 10 kg has been proved safe and effective. METHODS: We reviewed 74 consecutive patients weighing < 10 kg (median 8.1 kg, range 3.98 to 10) and aged 4 to 30 months (median 13 months) who underwent patent ductus arteriosus occlusion between June 1986 and November 1992. A modification of the delivery system to facilitate application in small children is described. RESULTS: A 12-mm device was implanted in 50 patients and a 17-mm device in 24. Three device embolizations occurred early in the experience, and one required removal because of hemolysis associated with a moderate residual shunt. One 17-mm device was removed at catheterization because of acute compromise to left pulmonary artery flow after implantation. Prevalence of residual shunting was 33% at 6 months, 20% at 12 to 18 months and 17% at 2-year follow-up and was not related to device, age, weight or size of the patent ductus. Altered flow to the left pulmonary artery was noted in seven patients (9.9%), with more significant compromise associated with the 17-mm device. CONCLUSIONS: Transcatheter patent ductus arteriosus occlusion is feasible in the small child < 10 kg, particularly with the use of a modified delivery system. However, implantation of a 17-mm device in this patient population may impair flow to the left lung and should be reserved for the symptomatic child.

Echocardiographic assessment of pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect.

OBJECTIVES: The purpose of this study was to determine the role of high resolution two-dimensional echocardiographic imaging and color flow Doppler study in assessing the pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect (VSD). BACKGROUND: Although echocardiography is a well established technique for assessing central pulmonary arteries in pulmonary atresia VSD and for determining the presence or absence of a patent arterial duct, few data are available on its role in patients whose source of blood supply is from collateral vessels. METHODS: Forty-two patients aged a few hours to 19 months (mean 29 days) were prospectively assessed by high resolution echocardiography, including color flow Doppler study, during a 4-year period ending in 1994, before any intervention other than intravenous administration of prostaglandins. Angiographic confirmation was available in 29 patients, including 18 (95%) of 19 with aortopulmonary collateral channels. RESULTS: A patent arterial duct was correctly identified as the sole source of pulmonary blood supply in 23 patients, whereas aortopulmonary collateral channels were detected in 19, with one of these having a small patent arterial duct and collateral channels. The patent arterial duct originated from the undersurface of the aorta in 16 (67%) of 24 patients and from the base of the brachiocephalic trunk in 7 (33%) of 24. All patients with a patent ductus as the sole source of pulmonary blood supply had confluent pulmonary arteries. Nonconfluent pulmonary arteries were present in six patients, with all but one having aortopulmonary collateral channels as the sole source of pulmonary flow. Aortopulmonary collateral channels were direct in 17 (89%) of 19 patients, whereas in 2 (11%) of 19, both direct and indirect collateral channels were present. Color flow Doppler study was accurate in determining the presence or absence, the side and the origin of the collateral channels in all patients, with the correct number being determined in 12 (67%) of 18. "Wash-in" to the hilar pulmonary arteries (retrograde color flow) was seen in 12 (92%) of 13 patients with collateral channels and confluent pulmonary arteries. Failure to identify a tiny central pulmonary artery occurred in one patient. CONCLUSIONS: High resolution imaging and color flow Doppler study provide good appreciation of the source of pulmonary blood supply in neonates and young infants with pulmonary atresia VSD.

Pulsed Doppler echocardiographic assessment of extraparenchymal pulmonary vein flow.

Extraparenchymal pulmonary vein flow profiles were assessed by pulsed Doppler echocardiography in three groups of patients. Group I comprised 41 normal patients. Group II comprised 16 patients, 10 of whom had nonpulsatile pulmonary artery flow (5 with a right atrium to pulmonary artery Fontan procedure and 5 with a Glenn shunt). Six patients with pulsatile pulmonary artery flow had simultaneous Doppler and left atrial pressure measurements during cardiac catheterization. Group III comprised one patient with pulmonary vein obstruction, six with a large left to right shunt at ventricular level and two with pulmonary vascular disease. In Group I, biphasic forward pulmonary vein flow occurring during ventricular systole and diastole was observed in 26 subjects, 15 others had triphasic flow. In those with triphasic flow, the ventricular systolic component was divided into early and late. Reversed flow in the pulmonary veins during atrial systole was seen in 36 of the 41 subjects. The flow pattern in Group II was identical irrespective of the presence of pulsatile or nonpulsatile flow. The two periods of ventricular systolic flow occurred during the a to c and c to x descent, with the ventricular diastolic flow occurring during the y descent. Variations in waveform were mirrored in the left atrial pressure. Neither increased nor decreased pulmonary artery flow substantially altered the pattern. Pulmonary vein obstruction produced a distinctive pattern of high velocity turbulent flow. This technique demonstrates that extraparenchymal pulmonary vein flow is dependent on left atrial pressure events. It has major potential applications in patients who are prone to develop pulmonary vein obstruction.

Echocardiographic analysis of regional left ventricular wall motion in normal children and neonates.

Because comprehensive normal data and the effect of observer variability for echocardiographic evaluation of regional left ventricular wall motion are not available in children or newborns, left ventricular wall motion was assessed by measuring regional area change. The study group comprised 55 infants and children with a normal heart: 15 neonates (greater than 1 week to less than 1 month old), 10 infants (greater than 1 month to less than 1 year old) and 30 children, 10 each in the age group greater than 1 year to less than 5 years, greater than 5 to less than or equal to 10 years and greater than 10 years. A combination of parasternal, apical and subcostal two-dimensional echocardiographic views was applied. After planimetry of an end-systolic and end-diastolic frame, the left ventricle was divided into eight equal segments and the percent area change calculated. Both a fixed reference and a floating system correcting for translation and rotation were applied. Intraobserver variability for percent area change measurements was 2.8 +/- 0.9% and 3.8 +/- 1% for observers 1 and 2, respectively. The mean interobserver difference of regional percent area change was 4.7 +/- 1.8%. Normal values for the eight anatomic segments were established in each echocardiographic imaging plane. The overall results were independent of the type of reference system utilized. The subcostal views yielded different results from their parasternal counterparts, probably because of differing imaging planes. These normal values establish a data base in the pediatric age range that can be used to detect abnormal segments in children at risk for developing regional left ventricular wall motion abnormalities.

Echocardiographic analysis of regional left ventricular wall motion in children after the arterial switch operation for complete transposition of the great arteries.

Regional left ventricular wall motion was assessed by two-dimensional echocardiography in 21 patients with complete transposition of the great arteries at a mean of 2.2 years (range 0.3 to 7) after an arterial switch operation. Fourteen patients had undergone a two-stage and seven patients a primary repair. Twenty patients were found to have echocardiographic images adequate for wall motion analysis. The results of these studies were correlated with thallium-201 myocardial perfusion scans. Left ventricular wall motion was assessed by measuring regional area change in parasternal and apical views. After planimetry of an end-systolic and an end-diastolic frame, the ventricle was divided into eight equal segments and the percent area change was calculated. Both a fixed reference and a floating system correcting for translation and rotation were applied. The measurements in the patient group were compared with normal age-matched values previously obtained in 55 normal infants and children. Wall motion abnormalities, found in seven patients, were located at the apex in three, at the inferior septum and anterolateral wall in one and the inferior, anterolateral and lateral walls in one patient each. All had a myocardial perfusion defect in a corresponding anatomic location. One patient with a small reversible perfusion defect at the basilar septum had normal regional wall motion. The sensitivity of detecting impairment of myocardial perfusion was 0.95. Wall motion abnormalities were found only in patients who had undergone a two-stage repair. Echocardiographic wall motion analysis can be used as a screening method to identify patients with suspected myocardial ischemia after the arterial switch operation.

Scimitar syndrome in infancy.

OBJECTIVES: The objectives of this study were to determine the anatomic and physiological factors most responsible for the severe symptoms and poor prognosis of infants with scimitar syndrome. BACKGROUND: Whereas the diagnosis of scimitar syndrome is often made incidentally in older children and adults who undergo chest radiography for diverse reasons, infants in whom the diagnosis is made typically present with severe symptoms and have a poor prognosis. METHODS: The clinical, catheterization and imaging data of 13 consecutive infants with scimitar syndrome who underwent cardiac catheterization in the 1st 6 months of life were reviewed, with emphasis on the pulmonary artery pressure, pulmonary and cardiovascular anatomy, therapeutic interventions and outcome. RESULTS: Twelve of the 13 infants had pulmonary hypertension at the time of diagnosis. Six patients died despite specific treatment. Eleven of 13 infants had associated cardiac malformations and 9 had large systemic arterial collateral channels to the right lung. Seven patients had anomalies involving the left side of the heart, especially varying degrees of hypoplasia of the left heart or aorta, and six of these patients died. Ten patients underwent surgical or transcatheter therapy in the 1st year of life. Systemic arteries to the right lung were ligated in three patients and occluded by transcatheter embolization in four. Balloon angioplasty was carried out in two patients, one with stenosis of the left-sided pulmonary veins and one with stenosis of the anomalous right pulmonary vein. The latter had placement of a balloon-expandable stent. In both patients, pulmonary vein stenosis progressed. Six patients had surgical repair of associated cardiovascular anomalies, and two required repair of extracardiac congenital anomalies. Occlusion of the anomalous systemic arteries was generally associated with clinical improvement, but congestive heart failure and pulmonary hypertension recurred in those patients with associated cardiovascular anomalies, whose condition subsequently responded after correction of the shunt lesions. CONCLUSIONS: The severe symptoms and pulmonary hypertension found in infants with scimitar syndrome have many causes. Anomalous systemic arterial supply, pulmonary vein stenosis and associated cardiovascular anomalies play a significant role, and the ultimate outcome of individual infants depends on the feasibility of treating these anomalies in early infancy.

Immunosuppressive therapy in the management of acute myocarditis in children: a clinical trial.

To assess whether steroid therapy influenced the clinical course of myocarditis in a pediatric population, findings in 13 consecutive infants and children (8 female, 5 male) with biopsy-proved myocarditis were reviewed. The mean age was 5.7 +/- 4.8 years (range 1.1 to 14.8). Congestive heart failure was present in all as were ST-T wave changes, cardiomegaly and pulmonary edema on chest roentgenogram. Echocardiography demonstrated pericardial effusion in five patients and mitral regurgitation in eight. Mean left ventricular ejection fraction was 34 +/- 12%. Prednisone was administered to all patients; one patient also received azathioprine. There was one death. All survivors showed clinical improvement with normalization of ECG changes, heart size and systolic function. No significant side effects occurred. Repeat myocardial biopsy in eight patients demonstrated improvement in all eight and elimination of the inflammatory infiltrate in six. Immunosuppressive therapy in this pediatric population appeared useful in improving the clinical course and cardiac function in acute myocarditis with no adverse side effects.

Percutaneous implantation of a balloon-expandable endoprosthesis for pulmonary artery stenosis: an experimental study.

Conventional therapy to treat peripheral pulmonary artery stenosis (surgery or balloon angioplasty) has been frustrating. Recently a variety of peripheral vascular stenoses, in which conventional approaches are disappointing, have become amenable to therapy with the use of a balloon-expandable endovascular stent. This experimental study was designed to assess the application of such a prosthesis in artificially created pulmonary artery stenoses. In 9 of 12 2-week old pigs, left pulmonary artery stenosis was surgically created (3.9 +/- 1.1 mm diameter and 7 +/- 1 mm Hg mean gradient). At 6.8 +/- 1 weeks of age (13 +/- 4 kg), percutaneous (femoral venous) implantation of a 3-cm long balloon-expandable (maximal diameter 18 mm) stent (three placed into normal pulmonary artery branches) using a 3-cm x 10-mm balloon dilating catheter was achieved without technical difficulties. Stenoses were enlarged to 8.3 +/- 1.4 mm with a decrease in mean gradient to 1 +/- 1 mm Hg that was maintained through 3.5 months of follow-up. Histologic and electron micrographic studies identified normal-appearing neoendothelial layering over stent struts without intraluminal or peripheral thrombus formation and nonobstructed side branching to lung subsegments. These findings support the application of this approach in the treatment of pulmonary stenosis that is not amenable to conventional therapy.

Balloon angioplasty of native coarctation: clinical outcomes and predictors of success.

OBJECTIVES: We sought to investigate the clinical impact of balloon angioplasty for native coarctation of the aorta (CoA) and determine predictors of outcome. BACKGROUND: Balloon dilation of native CoA remains controversial and more information on its long-term impact is required. METHODS: Hemodynamic, angiographic and follow-up data on 69 children who underwent balloon angioplasty of native CoA between 1988 and 1996 were reviewed. Stretch, recoil and gain of CoA circumference and area were calculated and related to outcomes. RESULTS: Initial systolic gradients (mean +/- SD, 31+/-12 mm Hg) fell by -74+/-27% (p < 0.001), with an increase in mean CoA diameters of 128+/-128% in the left anterior oblique and 124+/-87% in the lateral views (p < 0.001). Two deaths occurred, one at the time of the procedure and one 23 months later, both as a result of an associated cardiomyopathy. Seven patients had residual gradients of >20 mm Hg. One patient developed an aneurysm, stable in follow-up, and four patients had mild dilation at the site of the angioplasty. Freedom from reintervention was 90% at one year and 87% at five years with follow-up ranging to 8.5 years. Factors significantly associated with decreased time to reintervention included: a higher gradient before dilation, a smaller percentage change in gradient after dilation, a small transverse arch and a greater stretch and gain, but not recoil. CONCLUSION: Balloon dilation is a safe and efficient treatment of native CoA in children. Greater stretch and gain are factors significantly associated with reintervention, possibly related to altered elastic properties and vessel scarring.

Morphologic and hemodynamic consequences after percutaneous balloon valvotomy for neonatal pulmonary stenosis: medium-term follow-up.

OBJECTIVES: This study sought to evaluate ventricular and valvular morphologic changes, hemodynamic consequences and clinical outcomes of pulmonary balloon valvotomy performed in the neonatal period. BACKGROUND: Previous studies support percutaneous balloon valvotomy as the management option of choice for infants and children with valvular pulmonary stenosis. Less information is available to define the impact of valvotomy on the clinical course when performed in the neonatal period. METHODS: Patient records, catheterization data, cineangiograms and selected echocardiograms (initial and most recent studies) of 37 consecutive neonates undergoing attempted balloon dilation were reviewed. RESULTS: Dilation was accomplished in 35 (94%) of 37 attempts. Immediately after dilation, the transvalvular peak to peak systolic gradient decreased from 60 +/- 22 mm Hg (mean +/- SD, range 20 to 100) to 11 +/- 10 mm Hg (range 0 to 45) (p < 0.0001), and the right ventricular/aortic systolic pressure ratio decreased from 1.25 +/- 0.43 (range 0.5 to 2.6) to 0.66 +/- 0.22 (range 0.2 to 1) (p < 0.0001). Oxygen saturation measured by percutaneous oximetry increased from 80 +/- 7% to 92 +/- 4% (p < 0.0001). Three patients died (8%), and two required repeat balloon dilation. At the follow-up visit (median 31 months, range 6 months to 8 years), the estimated peak instantaneous Doppler gradient was 15 +/- 9 mm Hg (range 6 to 36). Thickening of valve leaflets, initially present in 93% of patients, was found in only 4%, and leaflet mobility improved in all. Hypoplasia of the right ventricle, initially present in 31%, was found in only 4% at the latest evaluation. Pulmonary annulus diameter Z score increased from -3 +/- 1.0 to 0 +/- 0.1 (p < 0.0001). Freedom from reintervention was 90%, 84% and 84% at 1, 2 and 8 years, respectively. CONCLUSIONS: These data support the application of balloon valvotomy as the initial intervention in the treatment algorithm for neonates with critical pulmonary valve stenosis. Medium-term follow-up observations demonstrate sustained hemodynamic relief and support maturation of the right ventricle and pulmonary valve annulus, with the expectation of a good long-term outcome.

Postoperative follow-up of fibromuscular subaortic stenosis.

OBJECTIVES: This study attempted to determine whether early subaortic resection at lower levels of obstruction reduces the rate of recurrence of subaortic stenosis or reduces secondary damage to the aortic valve, or both. BACKGROUND: Fibromuscular subaortic stenosis is a progressive condition, and at present it is unclear whether early operation reduces the recurrence rate along with decreasing the incidence of aortic insufficiency. METHODS: Thirty-seven patients with fibromuscular subaortic stenosis and no other significant cardiac abnormality who underwent open subaortic resection were evaluated. The preoperative, early and late postoperative catheterization or echocardiographic findings as well as the operative reports were reviewed. The median age at operation was 6.4 years (range 1.1 to 17.3). The entire group has been followed up postoperatively for a median of 5.2 years (range 1.1 to 11). Mean systolic gradients across the left ventricular outflow tract were used for the purpose of this study. RESULTS: There was a significant correlation between the preoperative mean systolic gradient and the incidence of preoperative aortic regurgitation and late postoperative aortic valve thickening as well as the incidence and degree of late postoperative aortic regurgitation. Late postoperative gradient and degree of aortic regurgitation correlated significantly with the follow-up interval. Aortic regurgitation was progressive in some patients despite subaortic resection. A preoperative mean gradient > 30 mm Hg provided a reasonable cutoff for the likelihood postoperatively of needing a reoperation, having a postoperative shelf, a thickened aortic valve, moderate aortic regurgitation or a gradient of > 10 mm Hg. CONCLUSIONS: Our results suggest that although early subaortic resection may not reduce the rate of recurrence of fixed subaortic stenosis, it is likely to reduce acquired damage to the aortic valve.

Balloon angioplasty of recurrent coarctation: a 12-year review.

OBJECTIVES: This study was undertaken to investigate the long-term outcome of balloon angioplasty for recurrent coarctation of the aorta in a large series of patients. BACKGROUND: Balloon angioplasty has become the standard treatment for residual or recurrent aortic coarctation. Despite the widespread use of this treatment modality, there are few data outlining the long-term outcome of a large patient cohort. METHODS: Clinical, echocardiographic, hemodynamic and angiographic data on 90 consecutive patients who underwent balloon angioplasty between January 1984 and January 1996 were reviewed. RESULTS: Mean systolic pressure gradients were reduced from 31 +/- 21 to 8 +/- 9 mm Hg after dilation (p = 0.0001). The mean diameter of the stenotic site, measured in the frontal and lateral views, increased by 38% and 35%, respectively (p = 0.001). Neurologic events occurred in two patients, with one death. An aortic tear occurred in one patient, requiring surgical intervention. Optimal results were defined as a postprocedure gradient < 20 mm Hg and were obtained acutely in 88% of patients. At long-term follow-up (12 years), 53 (72%) of 74 patients with an early optimal result remained free from reintervention. Transverse arch hypoplasia, defined as an arch dimension < 2 SD below the mean for age, was the primary predictor of the need for reintervention. CONCLUSIONS: Although the majority of patients undergoing percutaneous balloon angioplasty for recoarctation of the aorta will achieve long-term benefit, the need for further surgical intervention in those with transverse arch hypoplasia remains high.

Systemic venous collateral development after the bidirectional cavopulmonary anastomosis. Prevalence and predictors.

OBJECTIVES: To determine the prevalence of systemic venous collaterals after the bidirectional cavopulmonary anastomosis and the factors associated with their development. BACKGROUND: Systemic venous collaterals have been found after cavopulmonary anastomosis. Methods. Cardiac catheterization was performed in 103 patients before and after a bidirectional cavopulmonary anastomosis. RESULTS: After surgery, 51 venous collaterals were identified in 32 patients (31%). Collateral development was associated with an abnormal superior vena caval connection (56% incidence vs. 26% with a single right superior vena cava, p = 0.01) and postoperative factors including pulmonary artery distortion (53% incidence vs. 22% without distortion, p = 0.002); increased superior vena caval mean pressure (14 +/- 5 mm Hg versus 11 +/- 4 mm Hg with no collaterals, p = 0.0002); increased pulmonary artery mean pressure (13 +/- 4 mm Hg vs. 11 +/- 4 mm Hg with no collaterals, p = 0.02); lower right atrial mean pressure (5 +/- 2 mm Hg vs. 6 +/- 3 mm Hg with no collaterals, p = 0.04); and increased mean gradient between superior vena cava and right atrium (8 +/- 3 mm Hg vs. 5 +/- 4 mm Hg with no collaterals, p = 0.0002). Using multiple logistic regression, only this last factor was independently associated with collateral development with an odds ratio per 1 mm Hg of 1.33 (95% CI 1.12-1.58, p = 0.001) for their presence. CONCLUSIONS: Systemic venous collaterals occur frequently after a bidirectional cavopulmonary anastomosis and are found postoperatively when a significant pressure gradient occurs between cava and right atrium.

Complications associated with pediatric cardiac catheterization.

OBJECTIVES: The aim of this study was to determine the relative risks of pediatric diagnostic, interventional and electrophysiologic catheterizations. BACKGROUND: The role of the pediatric catheterization laboratory has evolved in the last decade as a therapeutic modality, although remaining an important tool for anatomic and hemodynamic diagnosis. METHODS: A study of 4,952 consecutive pediatric catheterization procedures was undertaken. RESULTS: Patient ages ranged from 1 day to 20 years (median 2.9 years). One or more complications occurred in 436 studies (8.8%) and were classified as major in 102 and minor in 458, with vascular complications (n=189; 3.8% of procedures) the most common adverse event. Arrhythmic complications (n=24) were the most common major complication. Death occurred in seven cases (0.14%) as a direct complication of the procedure and was more common in infants (n=5). Independent risk factors for complications included a young patient age and undergoing an interventional procedure. CONCLUSIONS: Complications continue to be associated with pediatric cardiac catheterization. Efforts should be directed to improving equipment for flexibility and size, and finding alternative methods for vascular access. Patient age and interventional studies are risk factors for morbidity and mortality.

Outcomes in critically ill neonates with pulmonary stenosis and intact ventricular septum: a multiinstitutional study. Congenital Heart Surgeons Society.

OBJECTIVE: This study attempted to determine the optimal therapeutic interventions by risk-adjusted comparisons of early and intermediate-term outcomes. BACKGROUND: The variety of interventions and the small case load at individual institutions have delayed the generation of reliable information concerning therapy for this condition. METHODS: In this prospective 27-institution study, 101 neonates were consecutively enrolled (between January 1, 1987 and January 1, 1991). Treatment was determined by the physicians. Demographic and morphologic details were tabulated. Dimensions of the pulmonary "anulus" and tricuspid anulus were measured on echocardiograms, and right ventricular cavity size was estimated. Right ventricular-pulmonary trunk pressure gradients were tabulated. Numerous analyses were made. RESULTS: Severe pulmonary valve stenosis and an intact ventricular septum were present in all patients. The right ventricular-pulmonary trunk junction ("anulus") was severely narrowed in 15%. Right ventricular cavity size was severely reduced in 4%. The tricuspid valve was small in 15% of patients; its diameter was poorly correlated with right ventricular cavity size. Eighty-nine percent and 81% of patients survived > or = 1 month and 4 years, respectively, after the initial procedure. Multivariable analysis identified no patient-specific risk factors for death. Only open pulmonary valvotomy without a support technique was uniformly a procedural risk factor; under some circumstances, transannular patching without a shunt was a risk factor. The right ventricular-pulmonary trunk gradient immediately after valvotomy was < 30 mm Hg in 81% of patients and was similar after surgical and balloon valvotomy. In 74% of patients, no intervention was required after the first accomplished intervention. CONCLUSIONS: Marked variation in morphology is uncommon in critical pulmonary stenosis in neonates. Percutaneous balloon valvotomy and certain types of surgical valvotomy are optimal initial procedures. The unusual situation of a small pulmonary "anulus" may initially require a transannular patch and a systemic-pulmonary artery shunt.

Management and outcomes of right atrial isomerism: a 26-year experience.

OBJECTIVES: We sought to determine, in a large series of patients with right atrial isomerism, factors associated with mortality. BACKGROUND: Right atrial isomerism is associated with complex congenital heart disease and high morbidity and mortality. METHOD: All data from patients diagnosed with right atrial isomerism between January 1970 and March 1996 were reviewed. RESULTS: A total of 91 consecutive patients (54 male) were identified. Most patients (89%) presented within the first month of life, 62% at birth. Cardiac abnormalities included common atrioventricular (AV) valve (81%), ventricular hypoplasia or single ventricle (73%), abnormal ventriculoarterial connections (96%), pulmonary outflow tract obstruction (84%), anomalous pulmonary venous drainage (87%) and pulmonary vein obstruction (30%). The overall mortality rate was 69%. No interventions were planned or performed in 24%, 95% of whom died. The mortality rate for patients requiring their first cardiovascular operation in the neonatal period was 75% versus 51% for those with later first operations (p < 0.05). The surgical mortality rate for patients undergoing pulmonary vein repair was 95%. Overall survival estimates were 71% at 1 month, 49% at 1 year and 35% at 5 years. Independent risk factors for decreased time to death included the absence of pulmonary outflow obstruction (relative risk [RR] 2.23, p < 0.03), presence of major AV valve anomaly (RR 5.23, p < 0.03) and obstructed pulmonary veins (RR 5.43, p < 0.0001). CONCLUSIONS: Right atrial isomerism continues to have an associated high mortality despite surgical innovations. Management of pulmonary vein obstruction remains a serious problem and is associated with high mortality.