Patterns of anomalous pulmonary venous connection/drainage in hypoplastic left heart syndrome: diagnostic role of Doppler color flow mapping and surgical implications.

Differentiation between anomalous connection and anomalous drainage of the pulmonary veins in hypoplastic left heart syndrome is important before either the Norwood procedure or heart transplantation is performed. To determine the prevalence of echocardiographically detected anomalous connection or drainage, or both, of pulmonary veins in patients with this syndrome, preoperative two-dimensional echocardiographic and Doppler color flow mapping studies of 317 patients who underwent the stage I Norwood procedure were reviewed. The term "connection" was used to describe the precise anatomic attachment of the pulmonary veins and the term "drainage" to describe the physiologic end point of pulmonary venous flow. Twenty patients (6.3%) had anomalous connection or drainage, or both, of the pulmonary veins by preoperative echocardiographic and Doppler examination. The subcostal and suprasternal scans best showed the anatomic details of the pulmonary veins. All these patterns were confirmed intraoperatively and could be grouped as follows: 1) partial anomalous connection and drainage (two patients); 2) total anomalous connection and drainage (eight patients); 3) normal connection with total anomalous drainage (eight patients); and 4) normal connection with partial anomalous drainage (two patients). The advantage of adding Doppler color flow mapping to two-dimensional echocardiography and conventional Doppler study was clearly demonstrated in the detection of small accessory vertical veins, their course and the presence or absence of obstruction. Doppler color flow mapping was especially helpful in detecting anomalous drainage of the right pulmonary veins to the right of the superior attachment of the septum primum.

Is routine preoperative cardiac catheterization necessary before repair of secundum and sinus venosus atrial septal defects?

Between January 1976 and July 1983, 217 patients with atrial septal defect underwent surgical repair at Children's Hospital. Thirty with a primum atrial septal defect and 26 who underwent cardiac catheterization elsewhere before being seen were excluded from analysis. Of the 161 remaining patients, 52 (31%) underwent preoperative cardiac catheterization, 38 because the physical examination was considered atypical for a secundum atrial septal defect and 14 because of a preexisting routine indication. One hundred nine (69%) underwent surgery without catheterization, with the attending cardiologist relying on clinical examination alone in 5, additional technetium radionuclide angiocardiography in 5, M-mode echocardiography in 13 and two-dimensional echocardiography in 43; both M-mode echocardiography and radionuclide angiography were performed in 24 and two-dimensional echocardiography and radionuclide angiography in 19. Since 1976, there has been a trend toward a reduction in the use of catheterization and use of one rather than two noninvasive or semiinvasive techniques for the detection of atrial defects. Of the 52 patients who underwent catheterization, the correct anatomic diagnosis was made before catheterization in 47 (90%). Two patients with a sinus venosus defect and one each with a sinus venosus defect plus partial anomalous pulmonary venous connection, partial anomalous pulmonary venous connection without an atrial septal defect and a sinoseptal defect were missed. Of 109 patients without catheterization, a correct morphologic diagnosis was made before surgery in 92 (84%). Nine patients with a sinus venosus defect, three with sinus venous defect and partial anomolous pulmonary venous connection, four with partial anomalous pulmonary venous return without an atrial septal defect and one with a secundum defect were incorrectly diagnosed.(ABSTRACT TRUNCATED AT 250 WORDS)

Early changes in ventricular septal defect size and ventricular geometry in the single left ventricle after volume-unloading surgery.

OBJECTIVES: This study investigated the phenomenon of, and the relation between, alterations in ventricular geometry after acute surgical volume unloading of the ventricle and the development of subaortic stenosis in patients with a single ventricle and ventricular septal defect-dependent systemic flow. BACKGROUND: Subaortic outflow obstruction has been observed to occur in patients with a single left ventricle after placement of a pulmonary artery band. The timing and etiology of this phenomenon are not well defined. METHODS: The preoperative and postoperative echocardiograms of 18 patients 14.9 +/- 22.8 months old (mean +/- SD) with a diagnosis of single left ventricle who underwent pulmonary artery banding or cavopulmonary connection were reviewed. Postoperative studies were performed a mean of 7.0 +/- 6.5 days after operation. The ventricular septal defect diameter was measured in two orthogonal views and the area calculated using the formula for an ellipse. Interventricular septal and posterior wall thickness and left ventricular diameter and length were also measured. RESULTS: Mean ventricular septal defect area indexed to body surface area diminished by 36 +/- 23% (3.1 +/- 2.7 to 2.0 +/- 1.8 cm2/m2, p < 0.01). Mean interventricular septal and posterior wall thickness increased significantly, and left ventricular diameter and length decreased significantly. A greater diminution in ventricular septal defect area was noted after cavopulmonary connection (41 +/- 19%, p < 0.01) than after pulmonary artery banding (25 +/- 28%, p = 0.22). CONCLUSIONS: In the single left ventricle, diminution in ventricular septal defect size occurs early and is related to an acute alteration in ventricular geometry that accompanies the decrease in ventricular volume. Ventricular septal defect diminution was greater after volume unloading of the ventricle after cavopulmonary connection than after pulmonary artery banding.

Surgical management of severe aortic outflow obstruction in lesions other than the hypoplastic left heart syndrome: use of a pulmonary artery to aorta anastomosis.

Between December 1985 and April 1990, 50 infants with a variety of congenital cardiac lesions other than the hypoplastic left heart syndrome underwent surgical relief of aortic outflow obstruction by creation of a pulmonary artery to aorta anastomosis. The patients were grouped anatomically by ventriculoarterial alignment. Nineteen had normally aligned great arteries (group I); 25 had transposition of the great arteries, all with a univentricular heart of left ventricular morphology (group II); and 6 had a double-outlet right ventricle (group III). All patients had either aortic stenosis with atresia, subaortic stenosis or a restrictive ventricular septal defect. Sixteen had normal arch anatomy; 34 had arch anomalies consisting of arch hypoplasia (n = 17), coarctation (n = 11), interruption of the arch (n = 4) and complex arch anomalies (n = 2). Surgery was performed at a median age of 10 days (range 2 to 184). Of the 50 infants, 33 survived. No significant difference in early survival (30 days) was noted among the groups of varying ventriculoarterial alignment (68% group I, 72% group II, 83% group III) (p greater than 0.05). Overall actuarial survival was 63% at 18 months. Analysis of actuarial survival by arch anatomy, although not statistically significant, revealed a trend toward better survival at 18 months postoperatively in infants with normal arch anatomy (81%) than in infants with arch anomalies (54%). Of the 33 survivors, 26 have proceeded to the next surgical stage, including the Fontan procedure in 8, superior cavopulmonary anastomosis in 13 and biventricular repair in 5.(ABSTRACT TRUNCATED AT 250 WORDS)

Primary surgical closure of ventricular septal defect in the first year of life: results in 128 infants.

Between January 1973 and July 1981, 128 patients less than 1 year of age with failure to thrive, congestive heart failure or pulmonary artery hypertension underwent primary repair of a ventricular septal defect. The hospital mortality rate was 7.8% (10 of 128), and the late mortality rate was 2.3% (3 of 128). Mortality was highest among younger infants with preexisting respiratory problems or a hemodynamically significant residual lesion postoperatively. Complications included a large residual shunt in eight (6.2%), transient neurologic problems in five (3.9%) and persistent complete heart block in three (2.3%). Lung biopsy specimens obtained from 49 patients showed pulmonary vascular abnormalities in all. Complete right bundle branch block developed in 74 (64%) and bifascicular block appeared in 11 (9%). Recatheterization in 70 patients (55%) showed normal pulmonary artery pressures in all but 2 patients with a large residual shunt. Complete closure of the defect had been achieved in 49 (70%), and a hemodynamically insignificant shunt remained in 19 (27%). Patients without significant hemodynamic residua were asymptomatic and tended to accelerate in growth after surgery.

Coronary artery abnormalities and right ventricular histology in hypoplastic left heart syndrome.

OBJECTIVES: To determine whether right ventricular preservation is compromised in any anatomic subgroup of hypoplastic left heart syndrome, we assessed the coronary artery anatomy and myocardial histologic features of 151 postmortem specimens with hypoplastic left heart syndrome. BACKGROUND: Although previous studies have shown that coronary artery abnormalities are more prominent in the subgroup of patients who have a patent mitral valve and obstructed aortic valve, it has not been established that these abnormalities compromise right ventricular perfusion. METHODS: Eighty-nine specimens with a patent mitral valve and aortic atresia, 52 with mitral and aortic atresia and 10 with patent mitral and aortic valves were examined. Histologic sections of the right ventricle, left ventricle and coronary arteries were obtained from 64 study hearts and 5 control hearts. RESULTS: Gross coronary anomalies included coronary-cameral communications (n = 29), single left coronary artery (n = 2), single right coronary artery (n = 1) and tortuosity (n = 19). Coronary-cameral communications and tortuosity were significantly associated with the subgroup that had mitral hypoplasia and aortic atresia. Coronary artery wall thickness relative to lumen diameter was not different among the subgroups. No areas of coronary artery stenosis or interruption were discovered. Although endocardial fibroelastosis of the left ventricle was strongly associated with the mitral hypoplasia and aortic atresia group, the right ventricular histologic findings were similar in all subgroups and were not differentially affected by older age. CONCLUSIONS: Although the incidence of coronary abnormalities is greater in patients with mitral hypoplasia and aortic atresia, in this study there was no apparent difference in perfusion of the right ventricle among the anatomic subgroups of hearts with hypoplastic left heart syndrome.

Valve replacement in children less than 5 years of age.

Between 1966 and 1984, there were 63 children less than 5 years of age who underwent 70 valve replacements (49 mitral [5 repeat], 6 aortic, 11 tricuspid [systemic ventricle; 2 repeat]), 2 tricuspid [pulmonary ventricle] and 1 multiple [mitral-aortic]) at Children's Hospital. Tissue valves were used in 20%. Since 1980, only Björk-Shiley and St. Jude valves have been used. The most common indication for valve replacement was mitral regurgitation after repair of atrioventricular (AV) canal (34%). Mortality dropped considerably over time: 76% before 1979, 33% from 1979 to 1982 and 22% since 1982. More than two-thirds of fatalities were operative deaths, usually within 3 days of surgery. Actuarial survival curves for those operated on since 1980 predict 1 and 5 year survival of 73 and 51%, respectively. For the 46 operative survivors 1 and 5 year valve survival was 97 and 70%, respectively. Postoperative hemodynamics were significantly improved on elective postoperative catheterizations. All but one patient with non-tissue valves received anticoagulant therapy. Postoperative complications included thromboembolism (1.6/100 patients-years) and hemorrhage (0.8/100 patient-years). The frequency of intravascular hemolysis and endocarditis was 1.6%, comparable with adult experience. Complete heart block requiring a pacemaker developed in nine patients (14.1%), in all after AV valve replacement. Valve replacement in young children generally results in considerable hemodynamic improvement. The mortality rate remains above that observed in adults but has declined considerably for those operated on after 1980.

Two-dimensional and Doppler echocardiographic assessment of neonatal arterial repair for transposition of the great arteries.

The arterial switch procedure has become an accepted reparative technique for transposition of the great arteries with or without ventricular septal defect. In this study the accuracy of prospective noninvasive imaging in detecting arterial tract obstruction and the prevalence and severity of arterial valvular regurgitation (as assessed by Doppler ultrasound) were evaluated in survivors of arterial repair. All 53 study patients underwent two-dimensional echocardiographic examination 2 days to 20 months (median 7 months) postoperatively; 43 patients also had pulsed and continuous wave Doppler studies. The accuracy of the noninvasive evaluation of arterial tract obstruction was determined by comparison of Doppler maximal instantaneous gradients with peak to peak gradients at nonsimultaneous catheterization in 26 patients. Twenty-one (81%) of the 26 patients underwent catheterization and successful pulsed and continuous wave Doppler examination of the right heart; 17 (81%) of these 21 had a maximal pressure gradient within 20 mm Hg of the peak to peak gradient obtained at catheterization. Echocardiographic identification of the stenotic site was correct in all eight of the patients in this group requiring reoperation. Twenty-three (88%) of the 26 patients who underwent catheterization had successful Doppler interrogation of the aortic tract; 22 (96%) of these 23 had a maximal instantaneous gradient within 20 mm Hg of the peak to peak catheterization gradient. Fourteen (32%) of 43 patients had mild or moderate pulmonary regurgitation by Doppler study. Three (7%) of the 43 had mild aortic regurgitation.

Hypoplastic left heart syndrome: hemodynamic and angiographic assessment after initial reconstructive surgery and relevance to modified Fontan procedure.

After undergoing initial reconstructive surgery for hypoplastic left heart syndrome performed between August 1985 and March 1989, 59 patients (age range 3 to 27 months, mean 13.8 +/- 4.5) underwent elective cardiac catheterization in anticipation of a modified Fontan procedure. Five important hemodynamic and anatomic features considered to be components of successful reconstructive surgery were specifically addressed. 1) Interatrial communication: Only two patients had a measured pressure difference of greater than 4 mm Hg across the atrial septum. 2) Tricuspid valve function: Angiography demonstrated significant tricuspid valve regurgitation in only five patients (moderate in two and severe in three). 3) Aortic arch: Pressure tracings from the right ventricle to the descending aorta revealed a gradient greater than 25 mm Hg in only two patients. 4) Pulmonary vasculature: Ten patients had a calculated pulmonary vascular resistance greater than 4 U.m2; 51 (86%) of the 59 patients had no evidence of distortion (stenosis or hypoplasia) of either the left or the right pulmonary artery. 5) Right ventricular function: Five patients had an end-diastolic pressure in the right ventricle greater than 12 mm Hg and two patients had qualitative assessment of decreased ventricular function. Comparison of catheterization data between survivors and nonsurvivors of the subsequent modified Fontan procedure showed that only significant tricuspid regurgitation is a possible predictor of poor outcome. After first stage reconstructive surgery for hypoplastic left heart syndrome, most survivors have favorable anatomy and hemodynamics at follow-up cardiac catheterization for a subsequent Fontan procedure.

Prospective detection by Doppler color flow imaging of additional defects in infants with a large ventricular septal defect.

The use of Doppler color flow imaging and axial contrast angiography in the preoperative detection of additional ventricular septal defects (in the setting of a known large defect) were compared in a prospective fashion. One hundred seventy-nine infants with two ventricles (each of at least normal size) and a large, nonrestrictive ventricular septal defect underwent reparative surgery before 2 years of age. The reference standard for the presence of additional defects was intraoperative verification or (in cases in which the surgeon did not visualize any additional defect) subsequent identification at postoperative angiography, postoperative color Doppler examination or reoperation. Only six patients (3%) had additional ventricular septal defects confirmed at the time of repair; an additional five (3%) had defects found only postoperatively. The negative predictive value of Doppler color flow imaging and angiography was 0.95 (168 of 176) and 0.97 (168 of 174), respectively. The sensitivity was 0.27 (3 of 11) and 0.45 (5 of 11), respectively. For certain malformations with a very low prevalence of additional muscular defects (such as perimembranous ventricular septal defect with normally aligned great arteries), a clinical trial of reparative surgery without prior invasive study appears reasonable.

Hemodynamic response to high-frequency ventilation in infants following cardiac surgery.

The hemodynamic response to high-frequency ventilation was compared with conventional ventilation in six infants following cardiac surgery. While undergoing high-frequency ventilation, adequate gas exchange was maintained in all infants. High frequency ventilation allowed a reduction of peak ventilatory pressure at the airway opening by 19%, and peak tracheal pressure by 42%. No clinically important changes in heart rate, systemic and pulmonary arterial pressure, cardiac index, or systemic and pulmonary vascular resistance were noted when high-frequency ventilation was compared with conventional ventilation.

The pharmacokinetics of injectable allopurinol in newborns with the hypoplastic left heart syndrome.

OBJECTIVE: The purpose of this investigation was to determine the pharmacokinetic disposition of intravenous allopurinol and its metabolite oxypurinol in neonates with the hypoplastic left heart syndrome (HLHS) and to evaluate the subsequent degree of xanthine oxidase inhibition using serum uric acid as a marker. METHODS: Pharmacokinetic data were evaluated in 12 stable preoperative neonates with HLHS after a single intravenous allopurinol administration of 5 mg/kg or 10 mg/kg. Pharmacokinetic parameters were determined for elimination half-life, clearance, volume of distribution, and mean residence time. Xanthine oxidase inhibition, measured by serum uric acid reduction, was also measured. RESULTS: Pharmacokinetic parameters revealed no statistically significant differences between a 5-mg/kg and 10-mg/kg dose of intravenous allopurinol on elimination half-life, clearance, volume of distribution, and mean residence time. Mean serum uric acid levels were significantly reduced from baseline by 39.99 and 42.94%, respectively, in the 5- and 10-mg/kg treatment groups. DISCUSSION: The enzyme xanthine oxidase plays a key biochemical role in the generation of toxic oxygen-derived free radicals during ischemia-reperfusion conditions. Allopurinol and its active metabolite oxypurinol inhibit xanthine oxidase, and significantly reduce the conversion of hypoxanthine to xanthine and xanthine to uric acid. Cell injury may be caused by toxic oxygen free radicals produced by ischemia-reperfusion injury such as could occur during the repair of HLHS under hypothermic total circulatory arrest. We hypothesize that allopurinol may provide protection from cellular injury in this clinical context.

Intraoperative balloon angioplasty of aortic coarctation in infants with hypoplastic left heart syndrome.

During a 1-year period, 9 children, aged 3 to 18 months, underwent intraoperative transluminal balloon angioplasty of coarctation of the aorta. Each patient had previously undergone palliative surgery for hypoplastic left heart syndrome. In 1 patient the angioplasty catheter was introduced into the ascending aorta during surgery to correct intracardiac defects. In the other 8 infants the catheters were inserted directly into the descending thoracic aorta via thoracotomy because the catheter size precluded percutaneous insertion into the femoral artery. In each case, after measuring the systolic pressure gradient across the coarctation, the angioplasty catheters were advanced over the guidewire across the coarctation site. The balloons were then inflated 2 to 3 times with an internal pressure of 3 to 6 atm. After the dilation sequence the angioplasty catheters were removed and the pressures were again measured above and below the coarctation. Preoperative gradients across the coarctation site ranged from 26 to 85 mm Hg (mean 45). Immediately after the procedure the gradient decreased in each case, ranging from 0 to 12 mm Hg (mean 4.3). There were no signs of disruption of the aortic wall. Relief of the pressure gradient persisted in the 7 patients who underwent follow-up cardiac catheterization after the procedure. The patients have been followed for as long as 18 months after the procedure and none has shown physical or echocardiographic evidence of recurrent aortic obstruction.(ABSTRACT TRUNCATED AT 250 WORDS)

Reoperation for isthmic coarctation of the aorta: follow-up of 26 patients.

Twenty-six patients underwent reoperation for coarctation of the aorta (C of A) between 1972 and 1980. Most patients (73%) had undergone primary repair in infancy. The interval to reoperation was 5 months to 18 years (mean 8.2 years). Indications for reoperation included symptoms in 4, exercise systemic hypertension in 1, electrocardiographic changes of left ventricular strain in 1, arm hypertension in 21, and a C of A gradient at rest greater than 30 mm Hg in all. Surgical procedures included patch aortoplasty (16 patients), bypass graft (6 patients), left subclavian angioplasty (3 patients), and end-to-end anastomosis (1 patient). There were no operative complications or mortality. There was 1 late death from aortic valve disease. Duration of follow-up in the remaining 25 patients was 2 weeks to 7 years (mean 2.5 years). All patients are asymptomatic. C of A gradients were significantly reduced from 30 to 132 mm Hg (mean 56) preoperatively to 0 to 48 mm Hg (mean 15) postoperatively. Right arm systolic blood pressure also was significantly improved from 120 to 237 mm Hg (mean 153) preoperatively to 100 to 160 mm Hg (mean 124) postoperatively. Systemic systolic hypertension persisted in 5 of 25 (20%). It is concluded that C of A reoperation is a low risk procedure that improves symptoms and reduces C of A gradient and arm blood pressure. However, systolic hypertension does not always resolve postoperatively. Patch aortoplasty appears to be a safe and effective surgical approach.

Hypoplastic left heart syndrome: experience with palliative surgery.

Aortic atresia is a form of hypoplastic left heart syndrome always complicated by associated severe hypoplasia of the ascending aorta and various degrees of mitral valve and left ventricular hypoplasia. At present it is a universally fatal lesion in early infancy. This is a report of a new palliative procedure for hypoplastic left heart syndrome that has resul;ed in early ongoing survival of two infants with aortic atresia. On the basis of experience with a third patient, an operation for future physiologic correction is proposed.

Early assessment of hemodynamic status after repair of tetralogy of Fallot: a comparison of 24 hour (intensive care unit) and 1 year postoperative data in 98 patients.

Hemodynamic data obtained in the intensive care unit, immediately after repair of tetralogy of Fallot, were compared with measurements obtained at 1 year postoperative catheterization in 98 infants and children. Eight of 12 patients who had pulmonary artery oxygen saturation of 80% or greater in the intensive care unit had a pulmonary to systemic flow ratio greater than 1.5 at catheterization; all 79 patients who had a pulmonary artery oxygen saturation of ess than 80% in the intensive care unit had a pulmonary to systemic flow ratio of 1.5 or less at catheterization. Five of six patients who had a right ventricular outflow tract pressure gradient greater than 40 mm Hg in the intensive care unit had a gradient greater than 40 mm Hg at catheterization; 7 of 61 patients who had a right ventricular outflow tract gradient of 40 mm Hg or less in the intensive care unit had a gradient greater than 40 mm Hg at catheterization. The addition of measurements of right ventricular pressure and the right ventricular to systemic arterial pressure ratio in the intensive care unit did not improve sensitivity in identifying patients with a right ventricular outflow tract gradient greater than 40 mm Hg at catheterization. Intensive care unit measurement of pulmonary artery oxygen saturation is valuable for determining the presence or absence of a significant left to right shunt after repair of tetralogy of Fallot and should be considered an adjunct to patient management. Intensive care unit measurement of the right ventricular outflow tract gradient identifies patients with a significant right ventricular outflow tract gradient at catheterization but is not highly sensitive.

Use of prostaglandin E1 in infants with d-transposition of the great arteries and intact ventricular septum.

Prostaglandin E1 was used to treat five infants with d-transposition of the great arteries and intact ventricular septum who had persistent severe hypoxemia after the creation of an interatrial communication. Three infants had a dramatic improvement in systemic arterial oxygen saturation associated with dilation of the ductus arteriosus; in two of the three cases urgent surgery was avoided. Two infants had no clinical evidence of increased ductal shunting and no improvement in oxygen saturation. A trial of prostaglandin E1 is recommended for treatment of severe hypoxemia in infants with d-transposition of the great arteries with intact ventricular septum if the presence of a large atrial septal defect is established.

Subxiphoid two-dimensional echocardiographic identification of tricuspid valve abnormalities in transposition of the great arteries with ventricular septal defect.

Tricuspid valve morphology was examined using subxiphoid 2-dimensional echocardiography (2-D echo) in 39 infants aged 2 years or younger who had transposition of the great arteries (TGA) and ventricular septal defect (VSD) (group I). Age-matched control groups were 21 patients with simple TGA (group II), 30 patients with VSD and normally related great arteries (group III), and 15 normal patients (group IV). Valve abnormalities, consisting of chordal attachments to the infundibular septum or ventricular septal crest, straddling, overriding or some combination of these, were identified in 25 of 39 patients (64%) in group I, no patients in groups II or IV and 6 of 30 patients (20%) in group III. Intraatrial baffle repair was performed in 27 patients in group I (median age at surgery 3.5 months) and 19 patients in group II (median age 4 months). Preoperative right ventricular angiography, performed in all patients with TGA, demonstrated tricuspid regurgitation (TR) with biventricular dysfunction in 1 patient in group I. After surgery, TR was present in 9 of 17 group I patients and none of the 8 group II patients who underwent catheterization. All patients in whom TR was not present preoperatively had abnormal chordal attachments; 3 required valve replacement. These results demonstrate that tricuspid valve abnormalities are common in patients with TGA and VSD and may be identified preoperatively using 2-D echo. Patients with abnormal chordal attachments are at increased risk for TR after intraatrial baffle repair and should be considered for arterial switch repair.

Postmortem echocardiography and tomographic anatomy of hypoplastic left heart syndrome after palliative surgery.

A study was undertaken to improve the understanding of the 3-dimensional (3-D) topology of a complex surgical reconstruction. The pathologic anatomy was investigated by first fixing postmortem heart specimens in such a way as to preserve the 3-D relations. Next, a technique for postmortem 2-D echocardiography was developed to aid in selection of tomographic planes for sectioning the specimens. Subsequent adjustment of planes of section was made to better show particular facets of the anatomy. The material for this investigation was drawn from cases of hypoplastic left heart syndrome after surgical palliation by the Norwood procedure. Three potential hemodynamic sequelae--restrictive inter atrial communication, aortic obstruction at any level and distortion of the pulmonary artery confluence--served as the anatomic focus for this study. Careful preservation of 3-D topology and postmortem 2-D echocardiographic imaging coupled with tomographic sectioning of specimens led to development of new, clinically relevant echocardiographic views for imaging specific atrial septal and aortic arch anomalies. These techniques offer insight into the spectrum of anatomic sequelae of this type of surgery and may be applied to echocardiographic imaging of patients and refinement of surgical technique for other forms of complex congenital heart disease.

Fate of the pulmonic valve after proximal pulmonary artery-to-ascending aorta anastomosis for aortic outflow obstruction.

Transection of the main pulmonary artery and end-to-side anastomosis of the proximal pulmonary artery to the ascending aorta has been increasingly used in palliative surgery for cardiac malformations such as single ventricle with small outlet foramen (bulboventricular foramen) and hypoplastic left-heart syndrome. To evaluate pulmonary valve competence after this operation, we used color Doppler flow mapping to examine 45 survivors of pulmonary artery-to-ascending aorta anastomosis a median of 202 days postoperatively. Of 37 patients with hypoplastic left heart syndrome, mild regurgitation was detected in 9 (24%) and moderate regurgitation in 1 (3%). Of 8 with other lesions, mild regurgitation was observed in 2 and moderate regurgitation in 1. Seven of 11 patients imaged greater than or equal to 12 months postoperatively had regurgitation. In summary, one-fourth of survivors developed mild pulmonary regurgitation. Its presence should not be considered a contraindication to eventual application of Fontan's principle, although further follow-up appears warranted because the long-term fate of pulmonary valve function is not yet known.