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rxcc 531 Vol. 2. No 3 September J983 .531-7 PEDIATRIC CARDIOLOGY Atrioventricular Valve Abnormalities in Infancy: Two-Dimensional Echocardiographic and Angiocardiographic Comparison HOWARD P. GUTGESELL, MD, FACC , JOHN CHEATHAM, MD, LARRY A. LATSON, MD, MICHAEL R. NIHILL , MD, FACC, CHARLES E. MULLINS, MD, FACC Houston . Texas The results of two-dimensional echocardiography and biplane angiocardiography from 47 infants with congenital atrioventricular (AV) valve abnormalities were compared. Eleven patients had atresia of the right AV valve, 10 had atresia of the left AV valve, 4 had hypoplasia of the right AV valve and 5 had hypoplasia of the left AV valve. Twelve patients had endocardial cushion defect, three had single ventricle and two had straddling of the left AV valve. There was agreement between the two techniques as to the number of AV valves present in each patient. The echocardiographic estimate of valveanular diameter was below normal in seven of the eight patients thought to have a hypoplastic anulus by angiocardiography. In 10 of the 12 patients with endocardial cushion defect, there was agreement between the two techniques as to the presence or absence of atrial and ventricular septal defect. The chordal attachments of straddling valves were better visualized by echocardiography; flow patterns and effective orifice size were better demonstrated by angiocardiography. The subcostal four chamber echocardiographic views and cranially angulated oblique angiocardiographic views were comparable and provided the best images for determination of the size and number of AV valves and their relation to the atrial and ventricular septa. The development of intracardiac operations for lesions formerly thought to be uncorrectable has heightened the need for accurate preoperative diagnoses in infants with congenital heart disease. In addition, nonoperative techniques such as balloon and blade atrial septostomy provide palliation in infants with appropriate intracardiac anatomy. Therefore, precise determination of the number, size and chordal attachments of the atrioventricular (AV) valves has become increasingly important in the management of infants with congenital heart disease. Axial angiocardiography (1-3) and two-dimensional echocardiography are diagnostic techniques that provide detailed anatomic information in children with congenital heart disease. Axial angiocardiography, utilizing cranially angulated views in either the straight anteroposterior or oblique projections, has been used primarily in assessment of the atrial and ventricular septa , the left ventricular outflow tract and the pulmonary arteries. Our initial experience suggested that these views were also useful in assessment of AV valve anatomy . Two-dimensional echocardiography gives improved spatial orientation compared with that available by M-mode echocardiography. The four chamber echocardiographic views obtained from either the cardiac apex (4) or the subcostal area (5,6) provide a frontal section of the heart that is similar to that of the cranially angulated left anterior oblique (hepatoclavicular) angiocardiogram . The present study was undertaken to compare and contrast selective angiocardiography and two-dimensional echocardiograph y in the assessment of AV valve abnormalities in infants . From the Lillie Frank Abercrombie Section of Cardiology . Department of Pediatrics, Baylor College of Medicine and Texas Children 's Hospital , Houston, Texas . This study was supported in part by Grant HL-07190 from the National Institutes of Health , U. S. Public Health Service. Bethesda . Maryland , and by Grant RR-OOI88 from the General Clinical Research Branch of the National Institutes of Health . Manuscnpt received January 12, 1983; revised manuscript received April 5, 1983, accepted April 6. 1983. Address for repnnts: Howard P. Gutgesell, MD, Pediatric Cardiology. 6621 Fannin . Houston, Texas 77030 Methods © 1983 by the American College of Cardiol ogy Subjects. The study group consisted of 47 infants under 2 years of age , 33 of whom were less than 6 months of age at the time of initial evaluation . Their weights ranged from 2.5 to 10.0 kg. Eleven patient s had atresia of the right atrioventricular (AV) valve, 10 had atresia of the left AV valve , 4 had a patent but hypoplastic right AV valve (each 0735-1097/83/$3 00 532 GUTGESELL ET AL. ATRIOVENTRICULAR VALVE ABNORMALITIES associated with pulmonary valve atresia), 5 had a hypoplastic left AV valve, 12 had endocardial cushion defect (partial AV canal in 3, complete AV canal in 9), 3 had single ventricle with a common AV valve and 2 had AV discordance, large ventricular septal defect and a straddling left AV valve. In addition to their AV valve abnormalities, 17 of the patients had transposition of the great arteries, 3 had tetralogy of Fallot and 1 had total anomalous pulmonary venous connection. Echocardiography. Two-dimensional echocardiograms were performed with mechanical sector scanners utilizing 5 MHz transducers. Images were obtained in the parasternal long-axis, parasternal short-axis, apical and subcostal projections. All studies were recorded on '12 inch (1.27 em) videotape for subsequent analysis. Echocardiograms were analyzed with particular attention to the following: 1) number of AV valves, 2) relative and absolute size of each AV valve anulus, 3) chordal attachments, and 4) the relation of the AV valves to the atrial septum. Anular size was estimated by measurement of the largest diameter of each valve ring at the proximal attachment of the leaflets from the apical or subcostal four chamber image (Fig. 1). For comparison, similar measurements were made from the echocardiograms of 30 normal infants between 1 day and 12 months of age. Figure 1. Subcostal four chamber two-dimensional echocardiogram (A) and selective right atrial angiocardiogram (B) in a 2 week old infant with right atrioventricular AV valve atresia. In the echocardiogram, the atretic right AV valve produces a dense curvilinear band of echoes at the inferior border of the right atrium (RA). The dashed line illustrates the points used for estimation of the lateral diameter of the left AV valve anulus. On the angiocardiogram, obtained in a cranially angulated left anterior oblique projection, all contrast material leaves the right atrium (RA) by way of the foramen ovale into the left atrium (LA). LV = left ventricle. lACC Vol. 2, No 3 September 1983531-7 Angiocardiography. Angiocardiograms were performed as part of cardiac catheterization in each patient. Selective left or right atrial injections (0.8 to 1.2 cc/kg of contrast medium) were made in 30 of the patients. These angiograms were imaged in the cranially angulated, left anterior oblique projection (hepatoclavicular projection) to achieve maximal separation of the cardiac chambers. Angiocardiograms were analyzed with respect to: 1) the number of valves, 2) the size of the anulus of each valve, and 3) the "commitment" of the valve with respect to the ventricles, based on the flow pattern through the valve. The angiocardiographic assessment of whether the anulus was hypoplastic was subjective, based on visual comparison of the anulus with the other AV valve, the ventricular chamber or chambers and the overall cardiac size. Results Number of Atrioventricular Valves There was complete agreement as to the number of atrioventricular (AV) valves in each patient as determined by the two techniques. In the patients with atresia of the right AV valve, a dense, curvilinear and relatively immobile band of echoes was present at the inferior aspect of the right atrium (Fig. l A). The left AV valve anulus appeared large with mobile leaflets. Selective right atrial angiocardiograms were performed in eight of these patients (Fig. lB). In each patient, all contrast material left the atrium by way of the foramen ovale. The inferior border of the atrium was smooth except for the density produced by contrast material refluxing into the coronary sinus. In the patients with atresia of the left AV valve, a dense mass of echoes separated the left atrium from the ventricular chambers (Fig. 2A). The echocardiographic features of this tissue did not appreciably differ from those present in the lACC Vol 2. No 3 September 1983:531-7 patients with atresia of the right AV valve. Left atrial angiocardiograms were performed in each of these patients, and all contrast material passed through an atrial septal defect or foramen ovale into the right atrium (Fig. 2B). One patient with atresia of the left AV valve was initially thought to have common atrium and a common AV valve by echocardiography . Catheterization and angiocardiography demonstrated a small left atrium with a restrictive atrial septal defect and atresia of the left AV valve. Review of the echocardiograms confirmed these findings and empha sized the need for a careful search for atrial septal tissue in all patients with evidence of only one AV valve. Anular Diameter By angiocardiography, five patients were diagnosed as having a hypoplastic left AV valve anulus (Fig. 3) and three as having a hypoplastic right AV valve anulus. The echo- GUTGESELL ET AL ATRIOVENTRICULAR VALVE ABNORMALITIES 533 Figure 2. Subcostal four chamber two-dimensional echocardiogram (A) and selective left atrial angiocardiogram (B) in a 3 week old infant with atresia of the left atrioventricular (AV) valve. There was AV discordance, the right atrium (RA) connecting to the anatomic left ventricle (LV). The atretic left AV valve produces a relatively thick, immobile band of echoes at the inferior surface of the left atrium (LA). On the left atrial angiocardiogram, the sole egress of contrast material is through the atrial septal defect into the right atrium. Figure 3. Subcostal four chamber echocardiogram (A) and selective left atrial angiocardiograrn (B) in a 6 week old infant with a hypoplastic but patent left atrioventricular(AV) valve. The anulus is 8 mm in diameter by echocardiography and the leaflets appear thick. The angiocardiogram, obtained in a cranially angulated left anterior oblique projection, demonstrates relative hypoplasia of the left atrium (LA), left AV valve and left ventricle (LV). RA = right atrium; RV = right ventricle. A l ACC Vol 2. No 3 September 1983:531-7 GUTGESELL ET AL. ATRIOVENTRICULAR VALVE ABNORMALITIES 534 20 B 111 2 23 22 18 21 17 ... •• HI 15 20 111 18 • .... ANULUS 17 DIAMETER18 (mm) 15 ANULUS14 DIAMETER (mm) 13 12 14 o 13 11 10 .. 0- Il 8 3 . .. . •• .. ... • • • 0 1 • o 0 2 12 11 o •• 5 8 7 8 BODY WEIGHT (Kg) 8 II 10 Figure 4. Graph s correlating the diameter of the left (A) and right (B) atriove ntricular (AV) valve anulus, as determined by echocardiography, with body weig ht. The solid dots are from normal infants, the open circles from eight infant s believed to have a hypoplastic anulus by angioc ardiography . The echographically detennined anular diameter is distinctl y less than normal in each of the three patients with a hypopl astic right AV valve (B) and in four of the five patients with hypopla stic left AV valve (A). Figure 5. Subcostal four chamber two-dim ensional echocardiogram (A) and selective left atrial angiocardiogram (B) in a 7 month old infant with atrio ventr icular (AV) discordance (ventricular inversion ) and a straddling left AV valve . In the echocardiogram , the medial leaflet of the left AV valve (a r r ows) passes through the ventricular septal defect and inserts on the contralateral side of the interventricular septum (l VS). The angiocardiograrn, performed in a cranially angulated right anterior oblique projection , shows the stream of contrast material passing through the left AV anulus , then splitting into separate streams into the left (LV) and right ventricles (RV). LA = left atrium ; RA = right atrium. 0 2 3 4 5 7 8 BODY WEIGHT (Kg) 8 II 10 cardiographic estimates of anular diameter in these patients and the values from the 30 normal ch ildre n are shown in Figure 4 . In th e five patients judged to have a small left A V valve anulus by an g iocardiography , the ec hocard iograph ic estimate of anular diamet er was clearly below th e normal range in four a nd at th e lower limit of normal in one (F ig. 4 A) . The echographic estimate of anular diameter was below the normal rang e in e ac h of the three patients with an an g ioc ard iogra phic appearance of right A V valve hypoplasia (Fig.4B) . In general, the most useful angiocardiographic techniqu e for demonstration of anular diameter was se lective atrial inj ec tion with the heart imaged in th e hepatoclavicular projection. The exceptio ns to this were the patients with pulmonary atre si a a nd intact ventricular se ptum , in who m right atria l injections produced poor opacifi c ation of the right ve ntricle and the anulus was more clearly visualized during the late stages o f the right ventricular angiocardiogram . lACC Vol 2, No.3 September 1983:531-7 535 GUTGESELL ET AL. ATRIOVENTRICULAR VALVE ABNORMALITIES Table 1. Two-Dimensional Echocardiographic and Angiocardiographic Findings in 12 Infants With Endocardial Cushion Defect Two-Dimensional Echocardiography Pattent Ostium Primum ASD I 2 3 4 5 6 7 8 9 10 II 12 + + + + + + + + + + + + Angiocardiography VSD Chordal Attachments Ostium Pnmum ASD VSD + + + + + + + + LV,RV LV,RV LV,RV Crest of septum Right side of septum Right side of septum Right side of septum Right side of septum Right side of septum Right side of septum LV,RV LV,RV + + + + + + + + + + + + + (small) + (small) + + + + + + + + ASD = atrial septal defect; LV = left ventricle; RV = right ventricle; VSD = ventncular septal defect; defect not present. Valve Commitment Two patients had a "straddling" left AV valve with chordal attachments on each side of the ventricular septum. The abnormal chordal attachments were best demonstrated by echocardiography in the subcostal and apical four chamber views (Fig. 5A). The abnormal flow pattern through the valve was well demonstrated by angiocardiography. Both of these patients had AV discordance (ventricular inversion) with an abnormally oriented ventricular septum. As a result, the lesion was best demonstrated by selective left atrial injection in a cranially angulated, right anterior oblique projection (Fig. 5B). Two additional patients had a straddling right AV valve in association with atresia ofthe left AV valve. The abnormal flow patterns through these valves were best demonstrated by cranially angulated, left anterior oblique projections. Endocardial Cushion Defect Echocardiography. The echocardiographic appearance of the AV valves in patients with the various forms of endocardial cushion defect was distinctive, and these lesions were easily distinguished from the other conditions described in this study. Table I summarizes the echocardiographic and angiocardiographic findings in the patients with endocardial cushion defect. In each of the 12 patients, a large ostium primum atrial septal defect was present. The medial portion of the AV valve apparatus was displaced inferiorly toward the apex. In eight patients, a discrete gap was present between the anterior bridging leaflet of the AV valve and the crest of the ventricular septum (Fig. 6A). In six of these patients, chordae from the anterior leaflet appeared to insert on the right side of the ventricular septum, + denotes defect present; - denotes suggestive of Rastelli type B lesions (7). In two, the anterior leaflet had no apparent attachments to the septum, the chordae connecting to papillary muscles in the respective ventricular chambers (Rastelli type C). Each of these eight patients was thus believed to have complete AV canal with a definitive ventricular septal defect by echocardiography (Table 1). In two patients, the anterior leaflet appeared to be firmly adherent to the ventricular septum, and in another two patients, there appeared to be a dense network of chordae between the anterior leaflet and the ventricular septum (Fig. 6A). These four patients were diagnosed as having partial or intermediate forms of AV canal without distinct ventricular septal defect. Angiocardiography. By angiocardiography, the ostium primum atrial septal defect was clearly demonstrated by left atrial or right upper pulmonary vein injection in the hepatoclavicular projection. The degree of attachment of the valve leaflets to the ventricular septum was best demonstrated by left ventricular injection in this projection. In each of the eight patients whose echocardiogram had demonstrated a distinct gap between the anterior leaflet and the septum, the left ventricular angiocardiogram demonstrated prompt opacification of the right ventricle, with contrast medium passing over the crest of the ventricular septum in systole (Fig. 6B). No early opacification of the right ventricle occurred in the two patients whose echocardiogram showed attachment of the leaflets directly to the septum. In the two patients with multiple chordae between the anterior leaflet and the septum, the left ventricular angiocardiogram demonstrated a trace of shunt from left ventricle to right ventricle. One of these patients died and at autopsy was found to have a dense but probe-patent network of chordae 3 to 4 mm long between the anterior bridging leaflet and the crest of the septum. 536 GUTGESELL ET AL. ATRIOVENTRICULAR VALVE ABNORMALITIES Figure 6. Subcostal four chamber two-dimensional echocardiogram (A) and left ventricular angiocardiogram (B) in a 4 month old infant with complete form of atrioventricular(AV) canal. Note the large gap (arrow) between the anterior bridging and leaflet of the common AV valve and the crest of the interventricular septum (lVS). There was almost complete absence of atrial septal tissue. The angiocardiogram performed in a cranially angulated left anterior oblique projection demonstrates the ventricularseptal defect with shunting of dye from left (LV) to right (RV) ventricle. CA = common atrium. Discussion The two imaging techniques employed in these infants gave complementary information about the anatomy of the atrioventricular (AV) valves and the surrounding structures. There was complete agreement as to the number of AV valves present. In our initial experience with echocardiography, we occasionally demonstrated two AV valves in patients previously thought to have tricuspid or mitral atresia on the basis of cardiac catheterization and angiocardiography. In retrospect, this diagnosis had generally been based on inability to pass a catheter through the valve orifice and visualization of only one valve orifice by negative "washout" during ventricular injections. Conversely, we were occasionally able to demonstrate only one AV valve by Mmode echocardiography in patients subsequently found to have two valves . However, as demonstrated in this study, improved angiographic and echocardiographic techniques allow an accurate assessment of the number of AV valves in virtually all patients. The hepatoclavicular angiocardiographic view and the subcostal echocardiographic projections display cardiac anatomy in a similar orientation, allowing easy comparison of images obtained by the two methods . lACC Vol 2. No 3 September 1983.531-7 AV anulus diameter: hypoplastic or atretic valves. Knowledge of AV anulus diameter is important in planning surgical therapy for many congenital cardiac anomalies, especially if valve replacement is a consideration. Autopsy data are available relating AV anulus circumference to body size in normal children (8) . We are unaware of similar data obtained by angiocardiography . Thus. the angiocardiographic diagnosis of a "hypoplastic" anulus is generally subjective, based on a comparison of the anulus to the overall heart size, the other AV valves and the catheter size . In the present study, valves diagnosed as hypoplastic by angiocardiography consistently had a diameter smaller than normal (based on body size) when measured by echocardiography. We did not consider it justified to define the precise limits of normal anular diameter on the basis of the relatively small number of normal subjects, and chose to simply present the data in graphic form (Fig . 4). We are currently measuring anular diameter in a large number of normal children, ranging from newborn through adolescence, to prepare growth charts relating anular diameter to body size . We have found a good correlation between anular size estimated by echocardiography and that measured at autopsy (9). The atretic right and left ventricular AV valves in this study had a similar appearance by echocardiography. They produced a relatively thick (3 to 5 mm) immobile band of echoes separating the atrium from the appropriate ventricle. Thus, we assume that these patients have so-called absent AV connection rather than an imperforate valve (10) ; however, only two patients have had autopsy confirmation . Endocardial cushion defect: partial versus complete AV canal. There was good agreement between echocardiography and angiocardiography in the assessment of patients with various forms of endocardial cushion defect. The single lACC Vol. 2, No.3 September 1983 531-7 most reliable echocardiographic feature allowing differentiation of complete AV canal from the partial or immediate forms was the presence of a gap between the anterior bridging leaflet and the crest of the ventricular septum in the four chamber projection, In each patient with this finding , angiocardiography demonstrated direct shunting from the left ventricle to the right ventricle, consistent with the observations of others (11,12) . The most difficult distinction to make by either technique was between large ostium primum defect with intact ventricular septum and the Rastelli type A form of AV canal with multiple chordae between the anterior leaflet and the ventricular septum . Although no distinct ventricular septal defect was apparent by echocardiography in these patients, a small amount of left to right shunting was present by angiocardiography , Previous studies have also reported occasional error s in distingu ishing partial from complete forms of AV canal (13-15) . Straddling AV valves. The recognition of straddling AV valves is of importance because their presence com plicates or precludes intracardiac repair of the associated ventricular septal defect. The M-mode echocardiographic features have been described (16,17), but recognition of straddling valves has been greatl y facilitated by two-di mensional echocardiography (18,19). Of the available angiocardiographic techniques , we prefer ventricular injections to determine the plane of the ventricular septum, followed by selective atrial injections with cranial angulation to separate the atria from the ventricles and then oblique angulation as required to place the ventricular septum parallel to the X-ray beam. Contrast echocardiography with selective atrial injections is an alternative method for studying flow patterns through straddling AV valves . Implications. The techniques used in this study are complementary. Echocardiography can be performed quickly and safely , and is the first procedure performed. It provides an outline of the intracardiac anatomy and allows cardiac catheterization to be performed more expediently. In particular , the echocardiogram can be used to determine which angiocardiographic projections will best delineate the intracardiac anatomy. In some instances, echocardiography may provide sufficient information for appropriate medical or surgical therapy . In many infants , however, cardiac catheterization is indicated as a therapeutic as well as diagnostic procedure , Of the 47 infants in our study , 22 had balloon or blade atrial septostomy performed at the time of initial cardiac catheterization. If AV valve abnormalities are suspected , they are best demon strated by angiocardiography in cranially angulated oblique projections with selective atrial injections. GUTGESELL ET AL ATRIOVENTRICULAR VALVE ABNORMALITIES 537 References I. Bargeron LM, Ethotr LP, Soto B, Bream PR, Curry Gc. Axial cineangiography in congenital heart disease. Section I. Concept , technical and anatomic considerations. Circulation 1977;56:1075-83. 2. Elliott LP, Bargeron LM, Bream PR, Soto B, Curry oc. Axial Cineangiography in congenital heart disease. Section II. Specific lesions. Circulation 1977;56:1084-93. 3. Fellows KE, Keane JF , Freed MD. 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