7. Van Praagh and Weinberg (41 classified fourteen

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1 CASE REPORT Surgical Treatment of Double-Outlet Left Ventricle in Situs Inversus {I,D,D} Haruo Akagawa, M.D., Fumio Yoshioka, M.D., Tadashi Isomura, M.D., Kiroku Ohishi, M.D., Katsuhiko Hirata, M.D., Hirohisa Kato, M.D., and Michihiro Koga, M.D. ABSTRACT A case of double-outlet left ventricle {I,D,D} with a subaortic ventricular septal defect and pulmonary stenosis is reported. The diagnosis was made at operation and was reconfirmed postoperatively by two-dimensional echocardiography. Repair was achieved by patch closure of the ventricular septal defect and enlargement of the functional right ventricular outflow tract using a valved patch. The postoperative course was uneventful except for a transient complete heart block. To our knowledge, this represents the second instance of surgical treatment of double-outlet left ventricle {I,D,D} reported in the literature. Ever since a successful repair of double-outlet left ventricle (DOLV) was reported by Sakakibara and associates in 1967 [l], the entity has been recognized clinically. In general, the definition of DOLV has been that the whole of one of the great arteries and more than 50% of the other arise above the morphological left ventricle (LV) [2-51. In 1981, Murphy and colleagues [6] stated that published case studies showed 84 patients with DOLV of whom approximately one-quarter underwent operation. We report here a case of surgical treatment of DOLV with situs inversus of viscera and atria and ventricular D-loop and D-malposition of the great arteries {I,D,D}. This is a rare type of DOLV. A girl aged 7 years 9 months was admitted with complaints of mild cyanosis and squatting. A grade 316 systolic-ejection murmur was heard in the third intercostal space on the left sternal border. The chest roentgenogram revealed situs inversus and mesocardia (Fig 1). The cardiothoracic ratio was 49%, and the pulmonary vasculature was normal. The electrocardiogram showed a negative P wave in leads I and avl, with sinus rhythm and mild ST segment depression in leads 11,111, and avf. The first of two cardiac catheterizations was performed when the child was 6 years 5 months old (Table). The systolic pressures in both ventricles were equal. A pullback tracing from the pulmonary trunk to the leftsided ventricle (morphological LV, functional right ventricle [RV]) showed a pattern of valvular pulmonary stenosis, and the systolic pressure gradient was 97 mm Hg. From the Second Department of Surgery and the Department of Pediatrics, School of Medicine, Kurume University, Kurume, Fukuoka, Japan. Accepted for publication June 13, Address reprint requests to Dr. Akagawa, The Second Department of Surgery, Kurume University School of Medicine, 67 Asahi-machi, Kurume-shi, Fukuoka-ken, 830, Japan. An oxygen step-up was recognized in the pulmonary artery, where the oxygen saturation was very close to that in the systemic artery. The second cardiac catheterization was performed when the patient was 6 years 11 months old. The anteroposterior view of the right-sided ventricle showed a coarsely trabecular ventricle, which was the morphological RV (Fig 2A, B). The view also demonstrated clearly the interventricular septum and the contrast medium from the RV going through the subaortic ventricular septal defect (VSD) into both great arteries and the leftsided ventricle. The overriding aorta and the entire pulmonary artery appeared to originate from the left-sided ventricle. The aorta was located beside the right side of the pulmonary artery (see Fig 2A, B). Anteroposterior and lateral views of the left-sided ventricle showed a finely trabecular ventricle, which was the morphological LV (Fig 2C, D). Both great arteries appeared to originate from the LV. The probable diagnosis before operation was DOLV {I,D,D} or corrected transposition of the great arteries (TGA) {I,D,D}. At operation the great arteries arose side by side, with the aorta to the right side of the pulmonary artery. The inferior vena cava and the right atrium were on the left side, and the left atrium was on the right side. A small right superior vena cava remained. Poststenotic dilatation of the pulmonary artery was present. A systolic thrill was palpable in the pulmonary artery (Fig 3). A subaortic VSD was found, measuring 2 cm in diameter, and the aortic valve overrode the interventricular septum by approximately 50%. The aortic and pulmonary valves were both in fibrous continuity with the anterior leaflet of the mitral valve, and there was no conal tissue between the aortic and the pulmonary valves (see Fig 3). Based on these findings, the diagnosis made at operation was DOLV. The pulmonary valve was bicuspid, and pulmonary stenosis was present at the valve ring. The RV was connected to the aorta by closing the subaortic VSD with a Dacron patch. The patch was stitched with interrupted sutures reinforced with spaghettishaped Teflon pledgets along the inferior rim of the VSD, following posteriorly to the mitral valve and superiorly from the inside of the pulmonary valve. The anterior margin of the VSD was stitched with interrupted sutures on Teflon pledgets from the outside of the morphological RV. For pulmonary stenosis, the functional right ventricular outflow tract was enlarged using a valved patch made of porcine pericardium. This patch was partially attached by a Dacron patch from the outside at the ventricular level. After the repair, the residual pressure gradient across the pulmonary valve was 337

2 338 The Annals of Thoracic Surgery Vol 37,No 4 April 1984 Fig 1. Chest roentgenogram. Situs inversus with stomach and aortic arch are on the right. Mesocardia and normal pulmonary msculature are seen. 38 mm Hg. Complete heart block appeared after the operation. However, the heart rhythm became sinus on the ninth postoperative day. The subsequent course was uneventful. Cardiac catheterization performed 49 days postoperatively showed no major shunt and no pressure gradient across the aortic valve (see Table). Subaortic stenosis was absent, according to an angiogram. Contrast medium in the RV opaafied the aorta through the intraventricular conduit, which protruded toward the LV (Fig 4A). The angiogram of the LV through the right 0 atrium showed opadication of the pulmonary artery alone (Fig 4B). A two-dimensional echocardiogram taken six months after the operation reconfirmed the diagnosis of DOLV {I,D,D} (Fig 5). The ultrasonic beam through the third intercostal space at the right sternal border showed the pulmonary artery on the left side and the aorta on the right side. A right parasternal long-axis image with leftward transducer angulation showed the continuity between the pulmonary and the mitral valves (see Fig 5). Medial angulation of the long-axis plane showed the continuity between the aortic and the mitral valves (see Fig 5); both valves were moving like a hinge at their interface. These findings indicated that both of the great arteries were related to the LV. The aortic override and the well-stitched patch also were recognized. Comment Various cardiac types of DOLV have been reported [4,5, 7. Van Praagh and Weinberg (41 classified fourteen anatomical types of DOLV among 36 cases. Their study found that the common form was DOLV in situs solitus andl that DOLV in situs inversus was uncommon. To our knowledge, 5 cases of DOLV in situs inversus have been reported [4, 8, 91 and the patient described here is the second with DOLV {I,D,D} reported in the literature [S]. The generally accepted criterion for DOLV is that more than one and one-half great arteries arise above the morphological LV [2-5). The complete form of DOLV, in which both great arteries emerge completely from the mcrphological LV, is rare [q. Israndt and colleagues [8] stress that it is important to identify the interventricular septum with the radiological projection, usually by the left anterior oblique view, in order to determine the site of origin of the great arteries. In our patient, the interventricular septum happened to be profiled clearly in the anteroposterior view. The aorta overrode the septum by approximately 50%. When there is an aortic ovemde like this, it is difficult to assess the degree of the ovemde [4, 81. Cardiac Catheterization Data Location Superior vena cava... Inferior vena cava... - Right atrium 14 Left-sided ventricleb 132/12- Pulmonary artery , 12 Left atrium 14 Right-sided ventricle' 135/14 Aorta... Femoral artery 150/90, I10.Performed when the patient was 6 years 5 months old. bmorphological left ventricle (functional right ventricle). CMorphological right ventricle (functional left ventricle). Preop." Oxygen Satuia tion Pressure (%) (mm Hg) Days Postop / / l85, % 95.. Oxygen Saturation

3 339 Case Report: Akagawa et al: Double-Outlet Left Ventricle Fig 2. Preoperative angiogmms. (A)Right-sided ventricular cineangiogram shows coarsely trabeculated morphological right ventricle (RV) on the right side. ( 8 ) The ventricular septum is well profiled in the frontal projection. Contrast medium flows from the RV through a subaortic ventricular septa1 defect (VSD)to the aorta (Ao), the pulmonary artery (PA), and the left ventricle (LV). The aorta is to the right of the PA. (C, D).The fine trabecular morphological LV ejects contrast medium to the Ao and the PA, which are side by side.

4 340 The Annals of Thoracic Surgery RSVC Vol 37 No 4 April 1984 w/ LSVC LA Tr Fig 3. Anatomical configuration before repair. (Ao = aorta; PA = pulmonary artery; VSD = ventricular septal defect; RV = right ventricle; LV = left ventricle; RSVC = right superior vena cam; LSVC = left superior vena cam;la = left atrial; RA = right atrial; IVC = inferior vena cava; Des. Ao = descending aorta.) Fig 4. Postoperative angiograms of ( A ) right and (B)left ventricular injections. ( A ) The aorta (Ao) received blood from the right ventricle (RV) through the ventricular septal defect. Arrows indicate the patch that protruded toward the left ventricle (LV). ( B ) The LV ejects into the pulmona ry artery (PA) alone. For the diagnosis of DOLV, absent conus and aorticmitral continuity are not always useful as absolute criteria [2-41. In fact, 1 patient without aortic-mitral continuity has been reported as having DOLV [3], and the cases of 4 patients who had TGA with this type of continuity also have been presented [lo]. However, since aortic-mitral fibrous continuity is regarded as one piece of evidencerelating the aorta to the LV [4], this continuity may have diagnostic value for DOLV when the degree of aortic override is assessed at approximately 50%. Van Praagh and associates [ll]described 2 patients as having a new type of transposition with overriding aorta or almost DOLV. However, they later classified the entities as DOLV [4]. In these patients, in whom the degree of aortic override was similar to that reported he::e, tenuous fibrous continuity of the aortic and the mitral valves was observed [ll].,4 preoperative diagnosis of DOLV was made in approximately half of all case reports in the literature [5]. Various diagnoses were considered, according to the types of cardiac anatomies [2, 4, 5, 7, 8, 101. Corrected TGA {I,D,D}, L-double-outlet right ventricle, and other conditions should be differentiated from DOLV {I,D,D}. Although angiography is important for diagnosing DOLV, identification of the aortic-mitral continuity is difficult. This continuity may be demonstrated by twodimensional echocardiography. In our patient, preoperative demonstration of the continuity was not made by the usual left sternal approach, presumably because of the presence of mesocardia. Postoperative two-dimensional echocardiography by the right sternal approach was successful in clarifying the continuity. Although the accepted operation for DOLV has been repair of the VSD using an intraventricular conduit and repair of the pulmonary stenosis using a valved external conduit, every operation has been executed in accor-

5 341 Case Report: Akagawa et al: Double-Outlet Left Ventricle Mitral valve \ Fig 5. Postoperative two-dimensional echocardiograms obtained from the third intercostal space. (Upper panel) A right parasternal longaxis image with leftward transducer angulation shows the continuity between the pulmonary valve and the mitral valve. (Lower panel) With a medial angulation of the long-axis plane, aortic-mitral valve continuity is seen. These findings indicate that both great arteries are related to the left ventricle (LV). The aortic override and intraventricular patch are also recognized. (RV = right ventricle; PA = pulmonary artery; IVS = intewentricular septum; RA = right atrium; Ao = aorta; A = anterior; P = posterior; I = inferior; S = superior.) dance with the individual pathological anatomical differences [ l, 4, 5, 6, 7, 91. In some types of DOLV, the approach for repair of the VSD and the suturing of a patch should be selected with regard to avoiding possible surgical injury of the conduction system, and to cardiac performance. In DOLV {I,D,D} the conduction system should be preserved, presumably for the same reasons as in corrected TGA {I,D,D}. In the corrected transposition in situs inversus {I,D,D}, the atrioventricular bundle emerges from the posterior atrioventricular node in the left-sided right atrium [12, 131, which is in contrast to the anterior atrioventricular node in corrected TGA in situs solitus {S,L,L} [14]. The bundle proceeds along the posterior and inferior margins of the VSD on the left side of the septum [12, 131. The left and right IVS bundle branches are normally distributed in their appropriate morphological ventricles [13]. Therefore, complete heart block will never occur after left ventricular incision. However, when the stitches are placed from the left side for VSD closure, there will be accompanying surgical injury of the conduction system. In our patient, complete heart block was seen for nine days after operation. Surgical injury of the conduction system was suspected, because the electrocardiogram showed trifascicular bundle-branch block. This suggests that an approach from the RV side would be preferable for patch closure of VSD. However, from the functional point of view, the RV is the functional LV, so the RV incision may be unfavorable. If it is anatomically possible to repair the VSD by the transaortic approach [15], this may be the optimum approach for postoperative cardiac function and the conduction system. Ogawa and colleagues (161 have reported a successful repair of corrected TGA {I,D,D} using the transaortic approach. The identification of coronary arteries is important in deciding the location of ventricular incision. In our patient, the abnormal location of the coronary arteries limited the ventricular incision, and as a result the repair of the pulmonary stenosis remained incomplete. For these reasons, the recommended operative method would be to use a valved external conduit from the distal portion of the LV to the pulmonary artery. It is not yet clear

6 342 The Annals of Thoracic Surgery Vol 37 No 4 April 1984 whether the RV and the tricuspid valve are tolerable as the systemic ventricle and the systemic atrioventricular valve, respectively, in the late period after repair. In our patient, the right ventricular ejection fraction was 62% on the echocardiogram, and tricuspid regurgitation, which is functional mitral regurgitation, was not observed six months after operation. However, long-term follow-up study is essential. References 1. Sakakibara S, Taka0 A, Arai T, et al: Both great vessels arising from the left ventricle. Bull Heart Inst Japan, 1%7, p Kirklii JW, Padfico AD, Bargeron LM Jr, et al: Cardiac repair in anatomically corrected malposition of the great arteries. Circulation 48:153, Paafico AD, Kirklin JW, Bargeron LM Jr, et al: Surgical treatment of double-outlet left ventricle. Circulation 48.Suppl3:19, Van Praagh R, Weinberg PM Double-outlet left ventricle. In Moss AJ, Adams FH, Emmanouilides GC (eds): Heart Disease in Infants, Children and Adolescents. Second edition. Baltimore, Williams & Willcins, 1977, pp Stegmann T, Oster H, Bissenden J, et al: Surgical treatment of double-outlet left ventricle in 2 patients with D-position and L-position of the aorta. Ann Thorac Surg 27121, Murphy DA, Gillis DA, Sridhara KS: Intraventricular repair of double-outlet left ventricle. Ann Thorac Surg 31:364, Bharati S, Lev M, Stewart R, et al: The morphologic spectrum of double-outlet left ventricle and its surgical significance. Circulation 58:558, Brandt PWT, Calder AL, Barratt-Boyes BG, et al: Doubleoutlet left ventricle. Am J Cardiol38:897, Urban AE, Anderson RH, Stark J: Double-outlet left ventricle associated with situs inversus and atrioventricular concordance. Am Heart J 9491, Van Praagh R, P6rez-Trevii10 C, L6pez-Cuellar M, et al: Transposition of the great arteries with posterior aorta, anterior pulmonary artery, subpulmonary conus and fibrous continuity between aortic and atrioventricular valves. Am J Cardiol28:621, Van hagh R, Calder AL, Delisle G, et al: Transposition of the great arteries with overriding aorta and pulmonary stenosis: new entity and its surgical management. Circulation 46:Suppl2%, Dick M II, Van Praagh R, Rudd M, et ak Electrophysiologic delineation of the specialized atrioventricular conduction system in two patients with corrected transposition of the great arteries in situs inversus {I,D,D}. Circulation 55:8%, Ih S, Fukuda K, Abe H, et al: An autopsy case of mixed levocardia with atrial inversion with complete (corrected) transposition, pulmonary stenosis and septal defects. Heart , Anderson RH, Bedcer AE, Arnold R, et a1 The conducting tissues in congenitally corrected transposition. Circulation 50911, Cooley DA, Hallman GL, Wukasch DC, Sandiford FM: Transaortic repair of ventridar septal defect. Ann Thorac Surg 1699, Ogawa K, Hoshino S, Harada M, et al: A successful Rastelli operation for corrected transposition of the great arteries in situs inversus {I,D,D} associated with pulmonary atresia. Heart 11:1378, 1979