Br Heart J: first published as 10.1136/hrt.39.8.847 on 1 August 1977. Downloaded from British Heart Journal, 1977, 39, 847-855 Echocardiography of primitive ventricle CARLOS MORTERA, STEWART HUNTER, GORDON TERRY, AND MICHAEL TYNAN From the Department of Cardiology, Newcastle General Hospital, Westgate Road, Newcastle upon Tyne The angiocardiographic, echocardiographic, and, where available, the necropsy findings were correlated in 32 cases ofprimntive ventricle. Single probe echocardiography was shown to be a reliable and accurate technique for diagnosis ofprimitive ventricle; the ventricular andatrioventricular valve appearances were characteristic, and the outlet chamber was usually recognised when present, though it was not possible to say whether it was right or left sided. Abnormalities of the atrioventricular valves were more accurately shown by echocardio- graphy than by angiocardiography though the two techniques were shown to be complementary in the overall diagnostic process. The introduction of radical surgery for the treat- with the outlet chamber (Anderson and Becker, ment of univentricular hearts makes accurate 1975). anatomical and functional diagnosis essential. The peculiar ability of the echocardiogram to Outlet chamber define intracardiac structures, their relations, and This is a chamber with a trabeculated pouch and an their motion, suggests that the technique should infundibular portion; it communicates with the play an important role in this diagnostic process. main ventricular chamber but contains neither a The definitions and terminology used in this paper complete atrioventricular valve annulus nor the http://heart.bmj.com/ are those proposed by Anderson and his colleagues complete tension apparatus of an atrioventricular (Anderson et al., 1976). Thus the term primitive valve. Echocardiographically its parietal wall moves ventricle is applied to all hearts with absence of the towards the bulboventricular septum during ven- posterior interventricular septum and includes tricular systole and no atrioventricular valve is seen those in which there is atresia of either atrioven- completely within its boundaries. tricular valve (Quero 1970, 1972). By correlating echocardiography and angio- Atrioventricular valve nomenclature cardiography and, where available, necropsy The terms right and left atrioventricular valves on September 24, 2021 by guest. Protected copyright. material, we have set out to define the value of are used to avoid confusion. The anatomical right echocardiography in the assessment of primitive atrioventricular valve is echocardiographically the ventricle. anterior and the left atrioventricular valve the posterior one. The terms right and left atrioven- DEFINITIONS tricular valve are used in this sense throughout. Primitive ventricle This exists when there is failure of development of Connections ofgreat arteries the posterior interventricular septum and both In primitive ventricle with outlet chamber the great atrioventricular valves are in actual or potential arteries are considered to be normally connected communication with the main ventricular chamber when the aorta arises from the main chamber and (Anderson et al., 1976). When an outlet chamber is the pulmonary artery from the outlet chamber. present no complete atrioventricular valve annulus When the converse is true transposition exists is in actual or potential communication with it (Shinebourne et al., 1976). When both great arteries (Macartney et al., 1976). When a single atrioven- arise from one chamber whether an outlet chamber tricular valve is present it communicates with the is present or not, this is a double outlet chamber main ventricular chamber and no other atrioven- and the term malposition is used irrespective of the tricular valve rudiment potentially communicates anteroposterior relation of the great arteries Received for publication 29 December 1976 (Shineboume et al., 1976). The prefixes d and 1 847 Br Heart J: first published as 10.1136/hrt.39.8.847 on 1 August 1977. Downloaded from 848 Mortera, Hunter, Terry, and Tynan indicate only the lateral relation of the great arteries Table 1 Distribution of cases in final diagnosis and have no specific morphogenetic or diagnostic significance (Carr et al., 1968), though knowledge Final diagnosis No. of these relations is of obvious surgical importance. Primitive ventricle with outlet chamber 30 When a single arterial trunk is present the termino- With 2 atrioventricular valves 17 Normal great arteries 1 logy of Shinebourne et al. (1976) is used. Transposition of great arteries (TGA) 16 With 1 atrioventricular valve 13 Right atrioventricular valve atresia with TGA 4 Straddling atrioventricular valves Right atrioventricular valve atresia with normal great In the context of primitive ventricle, straddling arteries 7 refers to an atrioventricular valve, part of whose Left atrioventricular valve atresia with TGA 1 Left atrioventricular valve atresia with single arterial tension apparatus arises in the outlet chamber. trunk (aorta) 1 This atrioventricular valve then straddles the bul- Primitive ventricle without outlet chamber 2 With 2 atrioventricular valves and single arterial trunk boventricular septum (Anderson et al., 1976). A (aorta) 1 straddling valve is assigned to the ventricular With single (common) atrioventricular valve and chamber into which more than 50 per cent of its 1-malposition 1 orifice appears to open. Thus, in primitive ventricle all straddling valves open at least 50 per cent into the main chamber. outlet chamber: in 18 this was to the left and Subjects and methods anterior, in 11 it was to the right and anterior, and in 1 it was directly anterior to the main chamber. Echocardiograms were recorded in 32 patients aged Two patients had no outlet chamber. Eighteen from 1 day to 28 years with an angiocardiographic patients had 2 atrioventricular valves, 11 had right diagnosis of primitive ventricle in 31, necropsy atrioventricular valve atresia, 2 had left atrioven- confirmation of the angiocardiographic diagnosis in tricular valve atresia, and 1 had a common atrioven- 4, and necropsy diagnosis only in 1 case in whom tricular valve. Sixteen of the cases with 2 atrioven- angiocardiography was not performed. In all 31 tricular valves had a left-sided outlet chamber and patients who had angiocardiography main chamber 1 had a right-sided outlet chamber, and 1 had no ventriculograms were recorded using either biplane outlet chamber. Ten of the cases with right atrio- Elema or multiple single plane cine projections. ventricular valve atresia had a right-sided outlet http://heart.bmj.com/ Single probe echocardiograms were obtained using chamber and 1 had a left-sided outlet chamber. In a 2-25 MHz transducer focused at 7-5 cm with an the 2 cases with left atrioventricular valve atresia Echoview X echocardiograph machine and a the outlet chamber was directly anterior in one Honeywell ultraviolet recorder. In all cases the and to the left in the other. In 3 cases with 2 atrio- echocardiographic relations were established using ventricular valves the right atrioventricular valve continuous sweeps. In 20 patients the angiograms straddled the bulboventricular septum. had been performed before echocardiography but Transposition of the great arteries was present in were reviewed by one of us without knowledge of 21 cases, normal arterial relation in 8, single arterial on September 24, 2021 by guest. Protected copyright. the echocardiographic findings. In 12 the echo- trunk (aorta) in 2, 1-malposition of the great cardiographic assessment and diagnosis had been arteries in 1. made before the angiocardiographic studies. Main ventricular chamber size and performance ECHOCARDIOGRAPHY were assessed echocardiographically using end- In 28 of the cases with an outlet chamber its diastolic dimension and the mean rate of internal presence was detected by echocardiography as an dimension shortening (mean VCE, respectively interior echo free chamber (Fig. 1, 3, and 8). (Cooper et al., 1972; Kaye et al., 1975). These However, it was not possible to say with certainty data were compared with values for the normal whether the outlet chamber was to the right or to left ventricle (Hunter et al., 1975). the left. The septal structure separating the outlet chamber from the main chamber was taken to Results represent the bulboventricular septum (Felner et al., 1976) because of its abnormal anterior posi- Table 1 shows the final diagnoses of all patients; tion and because no complete atrioventricular valve these diagaoses are a synthesis of all the available was detected in front of it. In no case were there data obtained from angiocardiograms, echocardio- echoes suggesting the presence of a posterior ven- grams, and necropsies. tricular septum (Assad-Morell et al., 1974; Felner Thirty patients had primitive ventricle with an et al., 1976). Br Heart J: first published as 10.1136/hrt.39.8.847 on 1 August 1977. Downloaded from Echocardiography ofprimitive ventricle 849 w It S X 0 X $ W ; kAy , A Fig. 1 This echocardiogram shows an anteriorly placed outlet chamber, the bulboventricular septum and the main ventricular chamber containing two atrioventricular valves. These valves were visualised completely using slight sideways angulation of the probe. The right atrioventricularnliY*w valve does not show excessive diastolic movement of its posterior cusp. OC, outlet chamber; RA V, right atrioventricular valve; LA V, left atrioventricular valve. http://heart.bmj.com/ In all 18 patients with