Cloning and characterization of vascular endothelial growth factor (VEGF) from zebrafish, Danio rerio.

We have cloned and sequenced a zebrafish (Danio rerio) Vascular Endothelial Growth Factor (vegf) cDNA. It encodes a precursor protein of 188 amino acids with a putative 23 amino acids signal peptide. Sequence comparison analysis indicates that the zebrafish vegf cDNA corresponds to the human VEGF165 isoform and shows about 52% identity to human VEGF165 at the amino acid level. A 2.8 kb vegf message RNA was detected in adult zebrafish by Northern blot analysis. Expression of vegf165 is also detected by RT-PCR in adult fish and throughout the zebrafish embryonic development. Whole mount in situ hybridization of zebrafish embryos indicates strong expression in four areas of the 18-19 h post-fertilization (hpf) embryo: within the anterior central nervous system in the prospective optic stalk, in mesoderm overlapping the bilaterally located merging heart fields, in mesoderm underlying and flanking the hindbrain posterior to rhombomere 4, and in medial regions of the somites. The study of vegf function in zebrafish embryonic vascular development will contribute to our understanding of the mechanisms of vertebrate endothelial cell differentiation and vasculature formation.

Imaging of pulmonary venous pathway obstruction in patients after the modified Fontan procedure.

To evaluate the efficacy of chest roentgenograms and echocardiograms in identifying pulmonary venous pathway obstruction in patients after the modified Fontan procedure, the records of 297 patients who underwent 307 Fontan procedures between 1984 and 1990 were reviewed. Twelve cases of pulmonary venous pathway obstruction documented by autopsy (3 cases) or cardiac catheterization (9 cases with an A wave gradient greater than or equal to 4 mm Hg) were found in 10 patients (mean age 43 +/- 28 months). The mechanisms of obstruction included narrow pulmonary vein ostia in six cases, narrow left atrial outlet in four and atrial baffle obstruction in three. Two causes of obstruction were present in one case. No patient had pulmonary venous congestion on chest roentgenograms. Pathway diameters indexed to 3 square root of body surface area with two-dimensional echocardiography were found to be 6.8 +/- 1.2 mm/3 square root of m2 in the subcostal frontal view, 7.9 +/- 0.6 mm/3 square root of m2 in the subcostal sagittal view and 6.5 +/- 1.7 mm/3 square root of m2 in the apical "four-chamber" view. These values differed significantly from those in 11 age-matched patients undergoing the Fontan procedure without pulmonary venous pathway obstruction documented by catheterization (p = 0.001). With pulsed Doppler ultrasound, there was a relatively narrow range of velocities distal to the obstruction (1.3 to 2.5 m/s). In five of the seven cases with pulsed Doppler measurements, flow was continuous and the Doppler spectral recordings were not phasic. Thus, in patients who have undergone the Fontan procedure and have pulmonary venous pathway obstruction 1) chest roentgenography cannot be used as a screening tool; 2) distal velocities as low as 1.3 m/s occur, usually with nonphasic, continuous forward flow; and 3) pathway diameters indexed to 3 square root of body surface area may be used as an output-independent parameter to cross-check Doppler data.

Subcostal two-dimensional echocardiographic identification of anomalous attachment of septum primum in patients with left atrioventricular valve underdevelopment.

Five variations of atrial septal morphology occur in hypoplastic left heart syndrome. One variety, termed anomalous attachment of septum primum, has been described only in necropsy series. Two-dimensional echocardiography was utilized to determine the incidence of this anomaly in patients with left atrioventricular (AV) valve underdevelopment, including those with other ventriculoarterial alignments, such as transposition of the great arteries. Forty-eight (37%) of 129 patients with normally aligned great arteries (and two ventricles) had anomalous attachment of septum primum. Ten (34%) of 29 patients with double outlet right ventricle and left AV valve underdevelopment had this anomaly. Four (50%) of eight patients with single ventricle exhibited this atrial septal variant. The most reliable view to identify anomalous attachment was the subcostal left oblique-equivalent cut. Recognition of atrial septal morphology has implications for preoperative and intraoperative management of patients with left AV valve underdevelopment. The similar prevalence of this atrial septal variant in patients with normally aligned great arteries, double outlet right ventricle and transposed great arteries suggests that there may be a common mechanism for left AV valve underdevelopment that is independent of the development of the arterial portion of the heart.

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.

Ultrasonographic display of complex vascular rings.

Ultrasonographic display of vascular ring anatomy has been limited to single-plane views. This does not readily allow for a three-dimensional interpretation of structural relations. A method that included a sweep consisting of multiple contiguous frontal planes was used in 12 patients with a vascular ring before repair for the evaluation of the arch sidedness and number, brachiocephalic vessel pattern, upper descending thoracic aorta sidedness, ductus arteriosus site or sites and proximal pulmonary arteries; 7 patients had Doppler color flow imaging. Complete imaging of the luminal vascular components was possible in all but one patient. In four other patients, atretic segments of the vascular ring could not be displayed. The addition of Doppler color flow imaging especially aided in the tracing of multiple vascular structures in complex cases and in assessing ductus arteriosus and arch patency. The use of a suprasternal frontal sweep with posterior angulation could display encirclement of the air-filled trachea. Vascular ring segments without lumens could not be displayed.

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)

Two-dimensional echocardiographic diagnosis of pulmonary artery sling in infancy.

The vascular anomaly in which the left pulmonary artery arises from the right pulmonary artery and passes posteriorly and leftward between the trachea and the esophagus is termed a pulmonary artery sling. Two-dimensional echocardiograms were performed in five infants with this anomaly and successfully identified it in four, including one patient with truncus arteriosus communis. The subxiphoid long-axis sweep was useful in identifying the origin and initial course of the left pulmonary artery, and short-axis subxiphoid views showed both its origin from the right pulmonary artery and its initial posterior course. Angulation toward the cardiac apex displayed the right pulmonary artery in cross section anteriorly and the left pulmonary artery in cross section posteriorly. A transducer orientation midway between the subxiphoid long- and short-axis positions was helpful in distinguishing a large right upper lobe branch of the right pulmonary artery from a pulmonary artery sling. The precordial short-axis plane displayed the origin and initial posterior and leftward course of the left pulmonary artery, while the bifurcation of the main pulmonary artery, usually easily seen in this view, could not be demonstrated. Two-dimensional echocardiography offers a rapid, noninvasive diagnosis of pulmonary artery sling in infants.

Late ventricular geometry and performance changes of functional single ventricle throughout staged Fontan reconstruction assessed by magnetic resonance imaging.

OBJECTIVES: We sought to test the hypothesis that late ventricular geometry and performance changes occur in functional single ventricles as they progress through staged Fontan reconstruction. BACKGROUND: Indexes of ventricular geometry and performance are important in evaluating the functional state of the heart. Magnetic resonance imaging determines these indexes in complex ventricular shapes with minimal geometric assumptions. Previous studies have shown that 1 week after hemiFontan, the mass/volume ratio markedly increases. METHODS: Multiphase, multislice, spin echo (n = 5) and cine (n = 30) magnetic resonance imaging was performed in 35 patients with a functional single ventricle (1 week to 12 years old) at various stages of Fontan reconstruction (15 in the pre hemiFontan stage, 11 after [6 to 9 months] the hemiFontan procedure and 9 after [1 to 2 years] the Fontan procedure). Volume and mass were calculated at end-systole and end-diastole. Ventricular output was then obtained. Ventricular centroid motion was also calculated. RESULTS: No difference was noted (power > 72%) from the pre hemiFontan stage to 6 to 9 months after the hemiFontan procedure in (mean +/- SD) end-diastolic volume (104 +/- 24 vs. 123 +/- 40 cc/m2), mass (171 +/- 46 vs. 202 +/- 61 g/m2), ventricular output (7.9 +/- 2.2 vs. 6.6 +/- 2.4 liters/min per m2) or centroid motion (6.9 +/- 2.8 vs. 6.7 +/- 2. mm/m2). Patients in the Fontan group demonstrated a marked decrease in all indexes, indicating significant volume unloading and decrease in mass and ventricular performance. Mass/volume ratio was not significantly different among all three groups. CONCLUSIONS: No geometric and performance changes from the volume-loaded stage are noted 6 to 9 months after the hemiFontan procedure; however, major changes occur 1 to 2 years after the Fontan procedure. The dramatic changes in the mass/volume ratio seen early after the hemiFontan procedure were not detected at 6 to 9 months. Furthermore diminution of mass, volume and ventricular performance are present at least 2 years after the Fontan procedure.

Subaortic obstruction in complex congenital heart disease: management by proximal pulmonary artery to ascending aorta end to side anastomosis.

Six patients with univentricular heart and one patient with d-transposition of the great arteries had transection of the main pulmonary artery with an end to side anastomosis of the main pulmonary artery to the ascending aorta to relieve subaortic obstruction. Two operations were performed as a palliative procedure within the first 6 months of life and five were performed as part of a definitive repair (four modified Fontan procedures and one repair of transposition of the great arteries with ventricular septal defect). There was one surgical death (14%) occurring 1 day postoperatively from low cardiac output. The remaining six patients are doing well 1 to 19 months postoperatively (mean 11.4 months). The proximal pulmonary artery to ascending aorta end to side anastomosis is an effective means of bypassing subaortic obstruction associated with complex congenital heart disease.

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.

Accuracy of prospective two-dimensional/Doppler echocardiography in the assessment of reparative surgery.

Between January 1987 and January 1989, all 129 patients (aged 11 days to 25 years, median 39 months) undergoing both an echocardiographic examination and cardiac catheterization after reparative surgery were prospectively included in a study to assess the accuracy of combined two-dimensional and Doppler color flow imaging. The patient diagnoses were transposition of the great arteries (n = 20), tetralogy of Fallot (n = 38), coarctation of the aorta (n = 24), complete atrioventricular (AV) canal (n = 15), atrial septal defect (n = 8), ventricular septal defects (n = 3), pulmonary stenosis (n = 4), aortic stenosis (n = 8) and subaortic stenosis (n = 9). In arterial tract stenosis, there was high correlation between Doppler estimates and catheterization-derived measurements of residual right ventricular outflow tract obstruction in patients after the arterial switch operation for transposition of the great arteries (r = 0.95) as well as in patients after corrective repair of tetralogy of Fallot (r = 0.84). In semilunar/AV valve regurgitation, graded as none, mild, moderate or severe, echocardiographic estimates correlated exactly with angiographic grading in 84% and differed by one angiographic grade in the other 16%. In residual left to right shunting, no hemodynamically significant shunt was missed by echocardiography. For residual shunts at the ventricular level (n = 32), addition of Doppler color flow imaging improved the sensitivity (from 63% to 94%) and the negative predictive value (from 88% to 98%). In elevated right ventricular pressure, Doppler-derived right ventricular-right atrial pressure estimates in 24 patients correlated well with catheterization measurements (r = 0.93). Combined two-dimensional and Doppler color flow echocardiography was highly accurate in the prospective evaluation of these four types of postoperative residual.

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.

The role of vascular endothelial growth factor (VEGF) in vasculogenesis, angiogenesis, and hematopoiesis in zebrafish development.

Vascular endothelial growth factor (VEGF, VEGF-A), a selective mitogen for endothelial cells is a critical factor for vascular development. Two isoforms that differ in the presence of exons 6 and 7, Vegf(165) and Vegf(121), are the dominant forms expressed in zebrafish embryo. Simultaneous overexpression of both isoforms in the embryo results in increased production of flk1, tie1, scl, and gata1 transcripts, indicating a stimulation of both endothelial and hematopoietic lineages. We also demonstrate that vegf can stimulate hematopoiesis in zebrafish by promoting the formation of terminally differentiated red blood cells. Simultaneous overexpression of both isoforms also causes ectopic vasculature and blood cells in many of the injected embryos as well as pericardial edema in later stage embryos. Overexpression of vegf also resulted in earlier onset of flk1, tie1, scl, and gata1 expression in the embryo, indicating a possible role of vegf in stimulating the differentiation of both vascular and hematopoietic lineages. Co-injection of RNAs for both isoforms results in increased expression of three of these markers over and above that observed when either RNA is singly injected and analysis of vegf expression in the notochord mutants no tail and floating head suggests that the notochord patterns the formation of the dorsal aorta by stimulating adjacent somite cells to express vegf, which in turn functions as a signal in dorsal aorta patterning. Finally, studies of vegf expression in cloche mutant indicate that vegf expression is generally independent of cloche function. These results show that in the zebrafish embryo, vegf can not only stimulate endothelial cell differentiation but also hematopoiesis. Moreover, these effects are most dramatic when both vegf isoforms are co-expressed, indicating a synergistic effect of the expression of the two forms of the VEGF protein.

Bone morphogenetic protein-4 expression characterizes inductive boundaries in organs of developing zebrafish.

We have cloned and examined the expression pattern of zebrafish bone morphogenetic protein-4 (BMP4) as a start to evaluating signals which might participate in the fashioning of organ systems in this genetically tractable species. The predicted sequence of the mature zebrafish protein is more than 75% identical to that of other vertebrates and 66% identical to Drosophila decapentaplegic (Dpp). As in other species, BMP4 is expressed ventrally during gastrulation, but the zebrafish is unusual in having an additional dorsal domain of expression. Subsequent BMP4 expression is especially prominent in sensory organs, fin buds, and in the gut, kidney, and heart. In all these sites, it becomes particularly enriched in regions of inductive demarcations. For example, expression initially extends through the entire heart tube but then becomes limited to the boundaries between cardiac chambers (sinus venosus-atrial junction, atrio-ventricular junction, and aortic root) prior to cushion formation. In early pectoral fin development, BMP4 is at first expressed uniformly but then becomes restricted to the mesenchyme subjacent to the apical ectodermal ridge. This suggests that among its roles in development, BMP4 serves as a signal in primordial outgrowth and also as a signal demarcating the borders within organs or structures where subspecializations occur.

Design and baseline characteristics for the ACE Inhibitor After Anthracycline (AAA) study of cardiac dysfunction in long-term pediatric cancer survivors.

PURPOSE: The ACE Inhibitor After Anthracycline (AAA) study is a randomized, double-blind, controlled clinical trial comparing enalapril with placebo to determine whether treatment can slow the progression of cardiac decline in patients who screen positive for anthracycline cardiotoxicity. METHODS: The primary outcome measure is the rate of decline, over time, in maximal cardiac index (in liters per minute per meters squared) at peak exercise; the secondary outcome measure is the rate of increase in left ventricular end systolic wall stress (in grams per centimeters squared). Patients >2 years off therapy and <4 years from diagnosis, aged 8 years and older, were eligible if they had received anthracyclines and had at least one cardiac abnormality identified at any time after anthracycline exposure. RESULTS: A total of 135 patients were randomized to enalapril or placebo. Baseline characteristics were similar across treatment groups. CONCLUSIONS: The AAA study will provide important information concerning the efficacy of using angiotensin-converting enzyme inhibitors to offset the effects of late anthracycline cardiotoxicity.

Semiquantitative assessment of mitral regurgitation by Doppler color flow imaging in patients aged < 20 years.

Doppler color flow imaging is a rapid, noninvasive method to estimate the severity of mitral regurgitation (MR) in adult patients; theoretically, it should be possible to use the technique at least as successfully in infants and children, because problems with beam penetration should be diminished. Doppler color flow studies in 27 pediatric patients (age range 12 days to 17 years, median 7 months) with various degrees of MR (but without coexistent aortic regurgitation or intracardiac shunt) were analyzed. All patients underwent left ventriculography within 2 weeks of their color Doppler examination. Three sets of parameters were investigated, including: (1) maximal length of regurgitant jet: by itself, and indexed to cube root of body surface area (BSA) and to left atrial depth; (2) proximal width of jet; by itself, and indexed to cube root of BSA and to diameter of mitral annulus; and (3) regurgitant jet area (RJA): by itself, and indexed to BSA and left atrial area (LAA). Of these parameters, RJA indexed to BSA, and the ratio of RJA/LAA had the best correlation with angiographic grading (r = 0.84 and 0.92, respectively). With our instrumentation, the "cutoff" values in infants and children separating mild, moderate and severe MR appear to be 4 and 10 cm2/m2 for RJA/BSA, and 30 and 50% for RJA/LAA, respectively. Both inter- and intraobserver correlations were good for RJA (r = 0.95 and 0.94, respectively), and the ratio of RJA/LAA (r = 0.92 and 0.94, respectively). Semiquantitative assessment of MR appears to be possible in pediatric patients, using 1 instrument and a standardized technique.

Prevalence, relation to spontaneous closure, and association of muscular ventricular septal defects with other cardiac defects.

Previous studies on muscular ventricular septal defect (VSD) have not taken into account the specific defect location in the septum. We retrospectively reviewed all patients with a muscular VSD, with and without associated malformations, diagnosed over 32 months to determine the prevalence and rate of spontaneous closure of single defects in relation to location in the muscular septum. Defects were classified into 4 groups: midmuscular, apical, anterior, and posterior. Two hundred seven patients were identified, of whom 125 had a single defect. The relative prevalence of single muscular VSD was: midmuscular 55 (44%), apical 31 (25%), anterior 33 (26%), and posterior 6 (5%). Thirty patients had signs of spontaneous closure and only 1 underwent surgery. There was no difference in rate of closure with respect to anatomic locations. Patients with multiple muscular VSD were either referred for surgery in the first year of life or had a course similar to patients with a single VSD. Muscular VSD associated with other cardiac malformations was more often encountered in patients with conoventricular VSD and coarctation of the aorta. The distribution of anatomic groups of muscular VSD in association with malformations was similar to the single VSD.

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.

2-dimensional echocardiographic appearance of complete left-sided juxtaposition of the atrial appendages.

This report describes the 2-dimensional (2-D) echocardiographic appearance of left-sided juxtaposition of the atrial appendages (JAA). From January 1, 1978, to June 30, 1979, 2 infants had the diagnosis of left-sided JAA at autopsy. Both patients had previously been examined by subxiphoid 2-D echocardiography. On review of these studies, the septum secundum was found to be oriented posteriorly in the transverse view of the atria. From July 1979 to June 1981, prospective evaluation of all infants for the presence of this finding revealed 2 more patients. Left-sided JAA was confirmed at surgery in 1 case and by selective right atrial angiography in the other. The diagnosis of left-sided JAA has important implications for both atrial baffle operations and for the Fontan procedure.

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.