Intraventricular Repair of Double-Outlet ceft Ventricle David A. Murphy, M.D., D. Alex Gillis, M.D., a n d K. S. Sridhara, M.D. ABSTRACT A 3V.-year-old child with doubleoutlet left ventricle and subaortic ventricular septal defect underwent successful surgical intervention with an intraventricular repair and a boomerang-shaped piece of Dacron patch. While an intraventricular repair for double-outlet left ventricle has been reported, we discuss here its application to this particular type of double-outlet left ventricle. Surgical repair of double-outlet left ventricle has been described previously [l, 21. However, there appears to be only one report of successful, total intraventricular repair of this condition [2]. The usual surgical technique for correcting double-outlet left ventricle is to close the ventricular septal defect and the pulmonary artery origin, and connect the right ventricle to the distal pulmonary artery with some form of extracardiac conduit. The following report describes an entirely intraventricular repair in a child whose great vessel anatomy and surgical repair differ from those previously reported. The patient was first seen when she was 10 days old for congestive heart failure. She was tentatively diagnosed as having aortic stenosis because of low-volume peripheral pulses and left ventricular overloading demonstrated on electrocardiogram. She responded well to digitalis and diuretics. She was not catheterized at that time. An echocardiogram done when she was 10 weeks old did not show evidence of severe aortic stenosis. From the Maritime Heart Center at the Izaak Walton Killam Hospital for Children, and the Department of Surgery, Dalhousie University, Halifax, Nova Scotia, Canada. Supported by the Nova Scotia Heart Foundation. We thank the following physicians who were involved with the care of this patient: Drs. D. Roy, M. Nanton, and C. Belcourt. Accepted for publication Mar 24, 1980. Address reprint requests to Dr. Murphy, 5850 University Ave, Halifax, Nova Scotia, Canada 83J3G9.
She was seen again when she was 7 months old because of diarrhea iind vomiting. Physical examination revealed central cyanosis and a grade 3/6 systolic murmur heard maximally at the left sternal border. The electrocardiogram showed terminal right intraventricular conduction delay and right axis deviation. The results of cardiac catheterization (Table) and angiography (Fig 1) were interpreted as severe tetralogy of Fallot. The lateral view of the right ventricular injection (see Fig 1B) demonstrated dye passing from the right ventricle across the ventricular septal defect and into the aorta. The conotruncus was considered to be of a tetralogy type, and was crossed by a prominent septal band. The precise connection of the right ventricle to the pulmonary artery was not well visualized. Following the injection of dye into the right ventricle, the pulmonary artery filled after the aorta. The pulmonary artery valve was considered to be at the same level as the aortic valve, but the latter was slightly posterior. There was mild aortic regurgitation. The patient subsequently underwent an elective right Blalock shunt. Apart from cyanosis with crying, the patient did well until she was 3% years old. Due to increasing cyanosis without syncope or squatting, she was readmitted for further study. On physical examination, clubbing and cyanosis were present. The heart was hyperkinetic. The second heart sound was accentuated and single. There was a grade 316 full-length ejection murmur maximal over the pulmonary area, but radiating down the left sternal border. A continuous shunt murmur was present. The hemoglobin was 15.8 gm. Chest roentgenogram revealed a heart size on the upper limits of normal, and lung fields and pulmonaiy vasculature again on the upper limits of normal. Repeat cardiac catheterization demonstrated essentially the same anatomy as previously described (see Table). Pullback tracings from the main pulmonary artery to the "infundibulum" and to the right
364 0003-4975/81/040364-06$01.25 @ 1980 by The Society of Thoracic Surgeons
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Case Report: Murphy, Gillis, and Sridhara: Repair of Double-Outlet Left Ventricle
Cardiac Catheterization 7 Months Old
Oxygen Saturation (YO)
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Pressure (mm Hg)
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3% Years Old (Preop)
Oxygen Saturation (YO)
Pressure (mm Hg)
Oxygen Saturation (%)
Pressure (mm Hg)
60.5 70.5 53.5 65.5 79 86 86
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76 87 76 76.5 80
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3 11010, ED 5 18/10 10ZO ED 7 107137
98
0 3210 2913 10010 100150
SVC = superior vena cava; IVC = inferior vena cava; RA = right atrium; RV = right ventricle; ED = end-diastolicpressure; PA = pulmonary artery; LV = left ventricle; Ao = aorta.
A
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Fig 1 . (A)Anteroposterior and (B)lateral views of right ventricular injection. The diagnosis of severe tetralogy of Fallot was made from these angiograms since it appears that the pulmonary artery (PA)arises near the top of the right ventricular infundibulum (RVInf). ( A 0 = aorta; VSD = ventricular septa1 defect.)
Surgical Procedure
ventricle were recorded as 15/7,3910, and 110/0 mm Hg, respectively. In retrospect, the etiology of the pressure gradient across the pulmonary valve is really unclear, since it was not anatomically confirmed at operation.
The heart was exposed through a median sternotomy. The great vessels arose side by side, with the aorta to the right and slightly posterior to the pulmonary artery. The coronary anatomy was normal except for the prominent and more rightward position of the right coronary artery origin. There were no major conal branches, and the left coronary artery passed behind the main pulmonary artery. It was difficult from the external examination of the heart to be sure whether the infundibular area of the right ventricle was in continuity with the pulmonary
366 The Annals of Thoracic Surgery Vol 31 No 4 April 1981
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Fig 2. The internal anatomy demonstrated through a right ventriculotomy. For illustrative purposes a vertical ventriculotomy is made, although a transverse one was employed at operation. The retractor on the anterior edge of the ventricular septal defect (VSD)erposes the aorta and the pulmonary artery arising from the left ventricle, Both great vessels were in continuity with the mitral valve annulus.
artery. A systolic thrill was palpable over the main pulmonary artery. The right Blalock anastomosis was isolated and subsequently ligated. The internal anatomy of the heart was inspected (Fig 2) through a right ventriculotomy. Since the pulmonary artery could not be entered by probing the right ventricular infundibulum, it was opened distal to the valve. When a Hegar dilator was inserted through the valve and into the heart, it became apparent that the pulmonary artery arose completely to the left of, and slightly anterior to, the aortic valve. It was in direct continuity with the mitral valve. The ventricular septal defect measured approximately 1.5 cm, and was located directly below the aortic annulus. The aortic annulus was separated from the tricuspid valve by fibromuscular interventricular septal muscle. The aortic valve was in continuity in part with the anteromedial aspect of the mitral valve, and there was no conal tissue between the aortic and pulmonary valves.
The ventricular septal defect was enlarged by excising part of the anterior and cephalic edges until it measured 2.5 cm in diameter. An intraventricular repair was done with a Dacron patch, which was tailored to a boomerang shape (Fig 3A) and utilized to redirect the blood to the appropriate artery, The patch was sutured into the defect with interrupted 3-0 Ticron suture on Teflon pledgets (Fig 3B), first along the anterior rim of the aortic annulus, then posteriorly into the pulmonary artery annulus, along the roof of the left ventricle to reach the anterocephalic edge of the enlarged ventricular septal defect. The remainder of the ventricular septal defect closure was done with a continuous stitch (Fig 3C). Before the patch was placed, the main pulmonary artery had accepted a No. 14 Hegar dilator, indicating that rigid fibrous stenosis of the pulmonary ring was not present. However, after the baffle was inserted, this artery would accept only a No. 8 Hegar dilator. For this reason, the pulmonary annulus was divided anteriorly into the left ventricle and widened with a diamond-shaped piece of pericardium. The patient came off bypass uneventfully and remained in sinus rhythm. Postoperatively, oliguria persisted for forty-eight hours but responded to conservative measures. Postoperative cardiac catheterization (Fig 4; see Table) demonstrated no gradient across the
367 Case Report: Murphy, Gillis, and Sridhara: Repair of Double-Outlet Left Ventricle
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left ventricular outflow tract. There was a residual left-to-right shunt ( 4 . 5 :1) across the ventricular septal defect. Additionally, there was some mild aortic regurgitation. The patient remains well three years following repair. She is no longer on a regimen of digoxin.
Comment This unusual cardiac defect has been well reviewed by Van Praagh and Weinberg [3], Bharati and co-workers [4], and Stegmann and colleagues [5].Including the 2 patients reported by Stegmann and associates [51, there appear to be published case studies of 84 patients with this condition. Van Praagh and Weinberg [3] described fourteen distinct anatomical types of
Fig 3 . A boomerang patch of single velour Dacron i s sutured in front of the aortic annulus and behind the pulmonary annulus. The ventricular septal defect (VSD)has been enlarged anteriorly. The hatched lines indicate proposed suture lines.
double-outlet left ventricle. The combination of double-outlet left ventricle, {S,D,D},* subaortic ventricular septal defect, and pulmonary stenosis seems to be the most common form (10 out of 35 patients) in their series. Our patient was likely in this category since she had a demonstrated gradient across the pulmonary outflow tract, although the cause for this was not identified at operation. Approximately one-quarter of the reported patients underwent operation. Mistakes in the preoperative diagnosis are common, and our patient was no exception; she was diagnosed as Situs solitus of viscera and atria, and a ventricular D-loop with D-malposition of aorta.
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The Annals of Thoracic Surgery Vol 31
N o 4 April 1981
A
B
Fig 4 . (A) Anteroposterior and ( B ) lateral projections of right ventricular (RV) injection done three months postoperatively. (PA = pulmonary artery.)
view, with a left ventricular injection, we still were unable to be certain of the origin of the pulmonary artery. Surgical repair usually has consisted of closure of the ventricular septal defect in such a way that the aorta and pulmonary artery are partitioned into the left ventricle. The pulmonary artery is then connected to the right ventricle with an external valved conduit [13 or, by unroofing the right ventricular infundibulum, it is connected to the pulmonary artery, with a roof of Dacron over the infundibulum, and into the pulmonary artery [3, 5, 7, 91. The pulmonary artery is then closed with sutures. A totally intraventricular repair has
having tetralogy of Fallot. Brandt and coworkers [ 6 ] stressed the importance of using a radiological projection that profiles the ventricular septum in order to establish the origins of the great arteries. Despite a hepatoclavicular Fig 5 . Differences in left ventricle-great vessel anatomy between our patient (left) and the patient reported b y Sokakibara and co-workers [2]. ( A 0 = aorta; PA = pulmonary artery; TV = tricuspid valve; M v = mitral valve; VSD = ventricular septal defect; RV = right ventricle; LV = left ventricle.)
369
Case Report: Murphy, Gillis, and Sridhara: Repair of Double-Outlet Left Ventricle
been used only once-Sokakibara a n d associates [2]. The location of the ventricular septal defect in their patient was situated directly below the pulmonary artery orifice, the latter, of course, situated i n the left ventricle. An oval patch was used to divert blood through the ventricular septal defect to the pulmonary artery, much a s one does for the aorta i n tetralogy of Fallot. Their patient differs from ours in that the pulmonary artery was adjacent to the ventricular septal defect a n d to the right of the aorta. The aortic valve was to the left of a n d posterior to the pulmonary valve. In our patient, the reverse was true: the ventricular septal defect was related to the aorta, a n d the pulmonary valve was to the left (Fig 5). The principles of intraventricular repair for transposition of the great arteries and ventricular septal defect are well described by McGoon [8]. They were readily adaptable for our patient, and accomplished the same hemodynamic result a s reported by Sokakibara a n d colleagues [2].
vessels arising from the left ventricle. Bull Heart Inst Jpn 11:66, 1967 3. 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 & Wilkins, 1977, pp 367-380 4. Bharati S, Lev M, Stewart R, et al: The morphologic spectrum of double outlet left ventricle and its surgical significance. Circulation 58:558, 1978 5. 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 27:121, 1979 6. Brandt PWT, Calder AL, Barratt-Boyes BG, Neutze JM: Double outlet left ventricle. Am J Cardiol 283397, 1976 7. Villani M, Lipscombe S, Ross DN: Double outlet left ventricle. J Cardiovasc Surg (Torino) 20:413, 1979 8. McGoon DC: Intraventricular repair of transposition of the great arteries. J Thorac Cardiovasc Surg 64:430, 1972 9. Kerr AR, Barcia A, Bargeron LM, Kirklin JW: Double outlet left ventricle with ventricular septal defect and pulmonary stenosis: report of surgical repair. Am Heart J 81:688,1971
Addendum References 1. Pacific0 AD, Kirklin JW, Bargeron LM, Soto 8 : Surgical treatment of double-outlet left ventricle. Circulation 48:Suppl 3:111-19,1973 2. Sokakibara S, Takao A, Arai T, et al: Both great
Since this article was accepted for publication, R. Rivera, C. Infantes, and M. Gil De La Pena have reported the case of a patient similar to ours except that enlargement of the ventricular septal defect was not required (J Cardiovasc Surg 21:361,1980).