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Double outlet right ventricle with pulmonic stenosis and anteriorly positioned aorta (Taussig-Bing variant)
CASE REPORTS
Double Outlet Right Ventricle with Pulmonic Stenosis and Anteriorly Positioned Aorta (Taussig-Bing Variant) Report of a Case and Surgical Correction
BROJAN AGARWALA, MD EUGENIE F. DOYLE, MD, FACC DELORES DANILOWICZ, MD, FACC FRANK C. SPENCER, MD NOEL M. MILLS, MD
New York, New York
From the Departments of Pediatrics and Surgery, New York University Medical Center, New York, N. Y. Manuscript accepted May 9, 1973. Address for reprints: Eugenie F. Doyle, MD, Department of Pediatrics, New York University Medical Center, 550 First Ave., New York, N. Y. 10016.
850
An 11 year old girl, markedly cyanotic and symptomatic with double outlet right ventricle, infundibular pulmonic stenosis and anteriorly positioned aorta (Taussig-Bing variant) underwent successful surgical correction. The procedure involved construction of a Dacron tunnel from the ventricular septal defect to the aortic orifice and infundibular resection. To our knowledge an identical case with a similar type of surgical correction has not previously been reported. Diagnostic features of double outlet right ventricle, including the TaussigBing anomaly, are discussed. Angiocardiography differentiated our case from complete transposition of the great arteries with pulmonic stenosis and from tetralogy of Fallot.
Double outlet right ventricle indicates the anatomic situation in which both great arteries arise wholly from the right ventricle. The term was introduced by Witham 1 but the anatomic features were described earlier by several other authors. 2-5 The ventricular septal defect was the only outlet from the left ventricle in all reported cases with the exception of the case of McMahon and Lipa, 6 in which the only septal defect was at the atrial level. Neufeld et al. 7-9 proposed a p.athologic classification of double outlet right ventricle according to the location of the ventricular septal defect and the presence or absence of pulmonic stenosis. Of lesions without pulmonic stenosis, type I double outlet right ventricle is characterized by an infracristal ventricular septal defect, close to the aortic outlet so that blood from the left ventricle can be ejected directly into the aorta; hence, no cyanosis is apparent. In type II, there is a supracristal ventricular septal defect under the pulmonic valve making direct streaming of blood from the left ventricle to the aorta impossible so that admixed venous and oxygenated blood is ejected into the aorta and some cyanosis results. Neufeld et al. 1° also described the group of patients who have pulmonic stenosis in addition to double outlet right ventricle which limits pulmonary blood flow, causing severe cyanosis. In the case of double outlet right ventricle presented here, there were a large subpulmonic ventricular septal defect and severe infundibular pulmonic stenosis, but the additional unique feature was the position of the aorta directly anterior to the pulmonary artery. This alignment of the great arteries and their relation to the ventricular septal defect resembles that seen with the anatomic features of Taussig-Bing malformation. 11
November1973 The American Journal of CARDIOLOGY
Volume 32
DOUBLE OUTLET RIGHT VENTRICLE--AGARWALA ET AL.
Case
TABLE I
Report
The patient, a Puerto Rican girl born on August 19, 1958, was the product of a normal full-term pregnancy; birth weight was 7 1/2 lb. Cyanosis was noted at birth and became more intense during infancy. Growth and development were near normal. Exercise tolerance was limited; squatting began at age 2 years. There were no "spells" or symptoms of congestive failure. At age 2 years, 5 months she underwent her first cardiac _'atheterization, which demonstrated systemic pressure in the right ventricle and severe pulmonic stenosis by cineangiocardiography. P r e m a t u r e filling of the aorta from ~he right ventricle was noted. The data were judged con~istent with tetralogy of Fallot, although transposition of the great arteries with pulmonic stenosis was also considered. One month later (February 1962) a left subclavian!eft pulmonary artery anastomosis was performed. Her general condition improved, the cyanosis decreased and there was a slight increase in exercise tolerance. During the next few years severe cyanosis returned. A second cardiac catheterization at age 7 years (July 1965) demonstrated double outlet right ventricle with severe in[undibular and mild valvular pulmonic stenois (Table I). A perplexing feature was the markedly anterior position of the aorta.
Findings at Second Cardiac Catheterization, July 1965" Oxygen Saturation (%) Superior vena cava 67 Inferior vena cava 59 High right atrium 66 Mid right atrium 65.20 Low right atrium 65.30 Right ventricle, body 72.80 Right ventricular 90.80 infundibulum Main pulmonary artery 91 Left ventricle 93 Femoral artery 78 Ascending aorta ... Hematocrit 70%
Pressures (ram Hg) . .. ... a = 6; v = 4 ... ... 75/5t 33/10 22/4 68/10 ... 64/38t
* Course of catheter: right ventricle to left ventricle; right ventricle to aorta; right ventricle to main pulmonary artery. 1 These pressures were not recorded simultaneously.
FIGURE 1. Cineangiograms. A, anteroposterior view. Catheter is shown in the right ventricle (RV) from the inferior vena cava through the tricuspid valve (TCV). The injection shows filling of both the aorta (Ao) and pulmonary artery (PA). Valve levels are indicated by the arrows. The right coronary artery (rca) is seen filling from the aorta. B, left anterior oblique view. The catheter is in the right ventricle and the injection outlines the entire chamber. Arrows show the ventricular septum; both the aorta and pulmonary artery arise from the right ventricle. The left ventricle (LV) is posterior to the area of the septum. In addition to the double outlet right ventricle, the aorta arises anterior to the pulmonary artery, indicating a transposition of the great arteries.
November 1973
The American Journal of CARDIOLOGY
Volume 32
851
DOUBLE OUTLET RIGHT VENTRICLE--AGARWALA
-
~Tlm2il~ltll,~
~
,0 I
t ~ T i C S Oral I ,
i I
h.I
Clil$
1 t 1 ~ 1 ) 11 I l l l l , l t 5 0 I
ET AL.
"
0,1 I I ~ i ~ .
FIGURE 2. Electrocardiogram. The tracings show right axis deviation (mean QRS axis + 1 3 5 ) , a tall peaked P wave in lead II, tall R in V1 and dominant S waves in the left precordial leads.
A third cardiac study to delineate the a n a t o m y was performed at age 11 years (1969) when her deteriorating clinic a l condition required an a t t e m p t at surgical intervention. This study {Fig. 1) suggested t h a t total correction might be feasible by directing left ventricular flow through a prosthesis to the aorta and by resecting the infundibulum. Physical examination at the time of operation showed a weight of 67 lb and a height of 55 inches. Vital signs were normal. The child was a fairly well developed, well nourished but severely cyanotic 11 year old girl in no acute distress. The chest was symmetrical, and there was a thoracotomy scar on the left side. E x a m i n a t i o n of the heart showed the left ventricular impulse to be in the fifth intercostal space just outside the midclavicular line. A parasternal impulse and a systolic thrill were felt along the lower left sternal border. The first sound was normal, the second single and loud; a grade 4/6 harsh holosystolic m u r m u r was heard along the lower left sternal border, and a grade 2/6 continuous m u r m u r was audible at the left
base. The hematocrit was 73 percent. The electrocardiogram (Fig. 2) showed regular sinus rhythm, right axis deviation and right atrial and right ventricular hypertrophy. The chest roentgenogram (Fig. 3) revealed a slightly increased cardiothoracic ratio with downward displacement of the rounded apex; p u l m o n a r y vascularity was within normal limits. Open heart surgery was performed on October 21, 1969. The anterior location of, the aorta arising from the right ventricle was identical to t h a t seen with complete transposition (Fig. 4). The p u l m o n a r y artery was to the left and partly posterior; it was of moderate size, about two thirds the diameter of the aorta. An anomalous anterior descending coronary artery, 3 m m in diameter, crossed the surface of the right ventricle near the pulmonic annulus. After cardiopulmonary bypass was established, the previous left Blalock-Taussig anastomosis was ligated. The interior of the right ventricle was unusual. There was a huge ventricular septal defect, almost 4 cm in diameter, in the upper portion of the septum, and the septal leaflet of the tricuspid valve a t t a c h e d to its posterior rim. The mitral valve was widely visible in the left ventricle. Both atria and ventricles and the mitral and tricuspid valves were normally formed. The opening to the pulmonary artery was severely stenosed and a small posterior infundibular chamber lay adjacent to the ventricular septal defect. The upper margin of the ventricular septal defect w a s i m m e d i a t e l y adjacent to the stenosed pulmonary ostium. The aortic ostium was anterior. Initially the infundibular chamber was resected, exposing the pulmonic valve which could be seen only with difficulty. No significant valvular obstruction was encountered. After resection of the infundibular obstruction, ready entry could be m a d e through the pulmonic valve to both. the right and left pulmonary arteries. A tunnel of knitted Dacron ® was t h e n constructed a p p r o x i m a t e l y 5 cm in circumference from the ventricular septal defect to the aortic
FIGURE 3. (left) Chest roentgenogram. The apex is displaced inferiorly and to the left. The ascending aorta is dilated. The pulmonary arterial branches and the peripheral pulmonary vascular markings are near normal. FIGURE 4. (right). Operative photograph demonstrating the difated aorta arising anteriorly from the right ventricle. The pulmonary artery is located posteriorly, partially hidden by the aorta. PA = pulmonary artery; RV = right ventricle.
852
November 1973
The American Journal of CARDIOLOGY
Volume 32
DOUBLE OUTLET RIGHT V E N T R I C L E - - A G A R W A L A
ostium. Initially sutures were placed along the posterior rim of the septal defect extending through the base of the septal leaflet of the tricuspid valve to avoid the conduction tissue. At the point where the margins of the ventricular septal defect and the pulmonary outflow tract were adjacent, the patch was in apposition so that the final construction was not a cylinder but a tube narrowed at its midportion (Fig. 5). Proximally the patch was attached around the margins of the ventricular septal defect. Distally it was attached around the aortic annulus. Great care was taken to widely resect the anterior wall of the right ventricle so that flow through the outflow tract anterior to the tunnel into the pulmonary artery would not be obstructed. After bypass, the following pressures (in millimeters of mercury) were recorded: left ventricle 90/5; aorta 90/60; right ventricle 35/5; pulmonary artery 25/10. These values indicated adequacy of the tunnel lumen and only minimal residual right outflow tract obstruction. At the completion of the repair transient complete heart block was noted. The patient's early postoperative course was complicated by congestive heart failure requiring vigorous digitalis and diuretic therapy. Intermittent atrioventricular A-V dissociation, 2:1 A-V block and first degree block developed but normal sinus rhythm returned on the 9th postoperative day. However, on the llth postoperative day complete heart block returned and persisted. Six weeks later a permanent transthoracic epicardial pacemaker was inserted. During the next few months the patient showed progressive clinical improvement and 6 months after operation, she returned to school. Three years postoperatively she is doing well and has good exercise tolerance. Her color is excellent, and the hematocrit is 43 percent. She attends regular class in school. • Discussion
Taussig and Bing 11 originally described a case of "complete transposition of the aorta and a levoposition of the pulmonary artery." A large subpulmonic ventricular septal defect was present. Van Praagh 12 subsequently redescribed their original specimen as one of the types of double outlet right ventricle with both subaortic and subpulmonic conus, the aortic and pulmonic valves lying side by side at approximately the same level, and the dilated pulmonary artery overriding the ventricular septal defect. However, the pulmonary valve did not override the left ventricular cavity; the ventricular septal defect was not confluent with the pulmonary valve because the subpulmonary conal free wall musculature formed the roof of the ventricular septal defect. The pulmonary-mitral valve fibrous continuity observed in complete transposition of the great arteries was not present. In the Taussig-Bing malformation the ventricular septal defect is high and located just below the pulmonic valve. The left ventricle ejects blood directly through the ventricular septal defect into the main pulmonary artery, and the aorta receives blood from the right ventricle. These features physiologically resemble the circulation in transposition of the great arteries. Cyanosis due to arterial desaturation is always present, and the pulmonary arterial blood contains a higher oxygen content than the aorta. The
ET AL.
FIGURE 5. Diagram of the transventricular tunnel constructed at operation to transmit blood from the left ventricle through the ventricular septal defect to the aorta, without obstructing the right ventricular outflow tract. Because of the anatomic abnormality, the prosthesis was narrowed to form a tube in its midportion, constituting a transventricular graft, one end of which was connected to the ventricular septal defect and the other to the annulus of the aortic valve. L.A. = left atrium; L.V. = left ventricle; Pulm. a. = pulmonary artery; R.A. = right atrium; R.V. = right ventricle.
right ventricular pressure is always at systemic levels. The pulmonary vascular disease is usually severe in this malformation possibly because of high oxygen content in the pulmonary artery and direct systemic pressure from the left ventricle. Pulmonic stenosis is a rarely associated lesion. 13 The clinical findings associated with the usual Taussig-Bing anomaly include cyanosis of various degrees of severity, clubbing, early congestive heart failure and markedly reduced exercise tolerance. Squatting is infrequent, and "spells" do not occur. A loud holosystolic murmur sometimes with a thrill and an accentuated narrowly split second heart sound are audible. The electrocardiogram is of little help in differential diagnosis, usually showing right axis deviation and right ventricular hypertrophy; additional left ventricular hypertrophy may be present. The chest roentgenogram usually shows increased heart size with a dilated main pulmonary artery and pulmonary vascular plethora. Angiographic studies are crucial in differentiating double outlet right ventricle with subpulmonic ventricular septal defect (Taussig-Bing anomaly) from other malformations. Contrast material injected into the right ventricle fills both great arteries simultaneously, the aorta opacifying more than the pulmo-
November 1973
The American Journal of C A R D I O L O G Y
Volume 32
853
DOUBLE OUTLET RIGHT VENTRICLE--AGARWALA ET AL.
nary artery. The left ventricular injection demonstrates the ventricular septal defect and filling of both great arteries, the main pulmonary artery being more opacified than the aorta. In the posteroanterior view both great arteries lie side by side, the aorta lying to the right of the main pulmonary artery; in the lateral view they are superimposed. The pulmonary and aortic valves lie at the same level, and the aortic valve is anterior to the ventricular septum. The diagnosis of Taussig-Bing anomaly is usually difficult in. living patients. The conditions from which this malformation must be differentiated include all cyanotic congenital cardiac abnormalities with increased pulmonary blood flow, such as complete transposition of the great arteries with ventricular septal defect, Type I truncus arteriosus, single ventricle with or without transposition, large ventricular septal defect with Eisenmenger complex, total anomalous pulmonary venous drainage, common atrium and, rarely, tricuspid atresia with a large ventricular septal defect. When the TaussigBing malformation is further complicated by pulmonic stenosis, the clinical features resemble those
of tetralogy of Fallot or complete transposition of the great arteries with pulmonic stenosis. This was true in our case. Angiocardiography is of crucial importance in differentiating these conditions.
Surgical treatment of the Taussig-Bing malformation is complicated. Kirklin et al. 14 and Hightower et al. la reported that in this situation they found it feasible to repair the ventricular septal defect so t h a t the left ventricular blood is ejected into the pulmonary artery and to provide for intraatrial transposition of venous return. They had success in three such cases. In our case, complicated by infundibular obstruction, a different approach was used. After wide infundibular resection, direct exit of blood from the left ventricle to the aorta was achieved. This appears to be an alternate method of repair. The occurrence of complete heart block was an unfortunate complication. Its transient occurrence in the operating room had led us to believe t h a t it was not due to suture placement. Replacement of critical sutures with greater care to prevent tension of the patch around the margins of the ventricular septal defect might have prevented this problem.
References 1. Witham AC: Double outlet right ventricle. Am Heart J 53:928-939, 1957 2. Peacock TB: Malformations of the Human Heart, second edition. London, Churchill & Sons, 1866, p 204 3. Saphir O, Lev M: The tetralogy of Eisenmenger. Am Heart J 21:31-46, 1941 4. Braun K, DeVries A, Feingold DS, et ah Complete dextroposition of the aorta, pulmonary stenosis, interventricular septal defect and patent foramen ovale. Am Heart J 43:773-780, 1952 5. Abbott ME" Atlas of Congenital Heart Disease. New York, American Heart Association, 1936, p 44 6. McMahon HE, Lipa M: Double outlet right ventricle with intact ventricular septum. Circulation 30:745-748, 1964 7. Neufeld HN, DuShane JW, Wood EH, et ah Origin of both great vessels from the right ventricle. I. Without pulmonic stenosis. Circulation 23:399-412, 1961 8. Neufeld HN: Origin of both great vessels from the right ventricle, chap 27. In, Heart Disease in infants, Children and Adolescents (Moss AJ, Adams F, ed). Baltimore, Williams & Wilkins, 1968, p 577-588
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November 1973
The American Journal of CARDIOLOGY
9. Neufeld HN, Lucas RV Jr, Lester RG, et al: Origin of both great vessels from the right ventricle without pulmonic stenosis. Brit Heart J 24:393-408, 1962 10. Neufeld HN, DuShane JW, Edwards JE: Origin of both great vessels from the right ventricle. II. With pulmonic stenosis. Circulation 23:603-612, 1961 11. Taussig HB, Bing RJ: Complete transposition of the aorta and a levoposition of the pulmonary artery. Am Heart J 37:551-559, 1949 12. Van Praagh R: What is the Taussig-Bing malformation? Circulation 38:445-449, 1968 13. Lev M: Double outlet right ventricle. Scientific exhibit, American College of Cardiology meeting, Chicago, March 1972 14. Kirklin JW, Barcia A, Deverall PB, et ah The surgical treatment of complex forms of transposition. Br Heart J 33: supph73-80, 1971 15. Hightower BM, Barcia A, Bargeron LM Jr, et ah Double outlet right ventricle with transposed great arteries and subpulmonary ventricular septal defect: the Taussig-Bing malformation. Circulation 39: suppl 1:207-213, 1969
Volume 32
Double Outlet Right Ventricle with Pulmonic Stenosis and Anteriorly Positioned Aorta (Taussig-Bing Variant) Report of a Case and Surgical Correction
BROJAN AGARWALA, MD EUGENIE F. DOYLE, MD, FACC DELORES DANILOWICZ, MD, FACC FRANK C. SPENCER, MD NOEL M. MILLS, MD
New York, New York
From the Departments of Pediatrics and Surgery, New York University Medical Center, New York, N. Y. Manuscript accepted May 9, 1973. Address for reprints: Eugenie F. Doyle, MD, Department of Pediatrics, New York University Medical Center, 550 First Ave., New York, N. Y. 10016.
850
An 11 year old girl, markedly cyanotic and symptomatic with double outlet right ventricle, infundibular pulmonic stenosis and anteriorly positioned aorta (Taussig-Bing variant) underwent successful surgical correction. The procedure involved construction of a Dacron tunnel from the ventricular septal defect to the aortic orifice and infundibular resection. To our knowledge an identical case with a similar type of surgical correction has not previously been reported. Diagnostic features of double outlet right ventricle, including the TaussigBing anomaly, are discussed. Angiocardiography differentiated our case from complete transposition of the great arteries with pulmonic stenosis and from tetralogy of Fallot.
Double outlet right ventricle indicates the anatomic situation in which both great arteries arise wholly from the right ventricle. The term was introduced by Witham 1 but the anatomic features were described earlier by several other authors. 2-5 The ventricular septal defect was the only outlet from the left ventricle in all reported cases with the exception of the case of McMahon and Lipa, 6 in which the only septal defect was at the atrial level. Neufeld et al. 7-9 proposed a p.athologic classification of double outlet right ventricle according to the location of the ventricular septal defect and the presence or absence of pulmonic stenosis. Of lesions without pulmonic stenosis, type I double outlet right ventricle is characterized by an infracristal ventricular septal defect, close to the aortic outlet so that blood from the left ventricle can be ejected directly into the aorta; hence, no cyanosis is apparent. In type II, there is a supracristal ventricular septal defect under the pulmonic valve making direct streaming of blood from the left ventricle to the aorta impossible so that admixed venous and oxygenated blood is ejected into the aorta and some cyanosis results. Neufeld et al. 1° also described the group of patients who have pulmonic stenosis in addition to double outlet right ventricle which limits pulmonary blood flow, causing severe cyanosis. In the case of double outlet right ventricle presented here, there were a large subpulmonic ventricular septal defect and severe infundibular pulmonic stenosis, but the additional unique feature was the position of the aorta directly anterior to the pulmonary artery. This alignment of the great arteries and their relation to the ventricular septal defect resembles that seen with the anatomic features of Taussig-Bing malformation. 11
November1973 The American Journal of CARDIOLOGY
Volume 32
DOUBLE OUTLET RIGHT VENTRICLE--AGARWALA ET AL.
Case
TABLE I
Report
The patient, a Puerto Rican girl born on August 19, 1958, was the product of a normal full-term pregnancy; birth weight was 7 1/2 lb. Cyanosis was noted at birth and became more intense during infancy. Growth and development were near normal. Exercise tolerance was limited; squatting began at age 2 years. There were no "spells" or symptoms of congestive failure. At age 2 years, 5 months she underwent her first cardiac _'atheterization, which demonstrated systemic pressure in the right ventricle and severe pulmonic stenosis by cineangiocardiography. P r e m a t u r e filling of the aorta from ~he right ventricle was noted. The data were judged con~istent with tetralogy of Fallot, although transposition of the great arteries with pulmonic stenosis was also considered. One month later (February 1962) a left subclavian!eft pulmonary artery anastomosis was performed. Her general condition improved, the cyanosis decreased and there was a slight increase in exercise tolerance. During the next few years severe cyanosis returned. A second cardiac catheterization at age 7 years (July 1965) demonstrated double outlet right ventricle with severe in[undibular and mild valvular pulmonic stenois (Table I). A perplexing feature was the markedly anterior position of the aorta.
Findings at Second Cardiac Catheterization, July 1965" Oxygen Saturation (%) Superior vena cava 67 Inferior vena cava 59 High right atrium 66 Mid right atrium 65.20 Low right atrium 65.30 Right ventricle, body 72.80 Right ventricular 90.80 infundibulum Main pulmonary artery 91 Left ventricle 93 Femoral artery 78 Ascending aorta ... Hematocrit 70%
Pressures (ram Hg) . .. ... a = 6; v = 4 ... ... 75/5t 33/10 22/4 68/10 ... 64/38t
* Course of catheter: right ventricle to left ventricle; right ventricle to aorta; right ventricle to main pulmonary artery. 1 These pressures were not recorded simultaneously.
FIGURE 1. Cineangiograms. A, anteroposterior view. Catheter is shown in the right ventricle (RV) from the inferior vena cava through the tricuspid valve (TCV). The injection shows filling of both the aorta (Ao) and pulmonary artery (PA). Valve levels are indicated by the arrows. The right coronary artery (rca) is seen filling from the aorta. B, left anterior oblique view. The catheter is in the right ventricle and the injection outlines the entire chamber. Arrows show the ventricular septum; both the aorta and pulmonary artery arise from the right ventricle. The left ventricle (LV) is posterior to the area of the septum. In addition to the double outlet right ventricle, the aorta arises anterior to the pulmonary artery, indicating a transposition of the great arteries.
November 1973
The American Journal of CARDIOLOGY
Volume 32
851
DOUBLE OUTLET RIGHT VENTRICLE--AGARWALA
-
~Tlm2il~ltll,~
~
,0 I
t ~ T i C S Oral I ,
i I
h.I
Clil$
1 t 1 ~ 1 ) 11 I l l l l , l t 5 0 I
ET AL.
"
0,1 I I ~ i ~ .
FIGURE 2. Electrocardiogram. The tracings show right axis deviation (mean QRS axis + 1 3 5 ) , a tall peaked P wave in lead II, tall R in V1 and dominant S waves in the left precordial leads.
A third cardiac study to delineate the a n a t o m y was performed at age 11 years (1969) when her deteriorating clinic a l condition required an a t t e m p t at surgical intervention. This study {Fig. 1) suggested t h a t total correction might be feasible by directing left ventricular flow through a prosthesis to the aorta and by resecting the infundibulum. Physical examination at the time of operation showed a weight of 67 lb and a height of 55 inches. Vital signs were normal. The child was a fairly well developed, well nourished but severely cyanotic 11 year old girl in no acute distress. The chest was symmetrical, and there was a thoracotomy scar on the left side. E x a m i n a t i o n of the heart showed the left ventricular impulse to be in the fifth intercostal space just outside the midclavicular line. A parasternal impulse and a systolic thrill were felt along the lower left sternal border. The first sound was normal, the second single and loud; a grade 4/6 harsh holosystolic m u r m u r was heard along the lower left sternal border, and a grade 2/6 continuous m u r m u r was audible at the left
base. The hematocrit was 73 percent. The electrocardiogram (Fig. 2) showed regular sinus rhythm, right axis deviation and right atrial and right ventricular hypertrophy. The chest roentgenogram (Fig. 3) revealed a slightly increased cardiothoracic ratio with downward displacement of the rounded apex; p u l m o n a r y vascularity was within normal limits. Open heart surgery was performed on October 21, 1969. The anterior location of, the aorta arising from the right ventricle was identical to t h a t seen with complete transposition (Fig. 4). The p u l m o n a r y artery was to the left and partly posterior; it was of moderate size, about two thirds the diameter of the aorta. An anomalous anterior descending coronary artery, 3 m m in diameter, crossed the surface of the right ventricle near the pulmonic annulus. After cardiopulmonary bypass was established, the previous left Blalock-Taussig anastomosis was ligated. The interior of the right ventricle was unusual. There was a huge ventricular septal defect, almost 4 cm in diameter, in the upper portion of the septum, and the septal leaflet of the tricuspid valve a t t a c h e d to its posterior rim. The mitral valve was widely visible in the left ventricle. Both atria and ventricles and the mitral and tricuspid valves were normally formed. The opening to the pulmonary artery was severely stenosed and a small posterior infundibular chamber lay adjacent to the ventricular septal defect. The upper margin of the ventricular septal defect w a s i m m e d i a t e l y adjacent to the stenosed pulmonary ostium. The aortic ostium was anterior. Initially the infundibular chamber was resected, exposing the pulmonic valve which could be seen only with difficulty. No significant valvular obstruction was encountered. After resection of the infundibular obstruction, ready entry could be m a d e through the pulmonic valve to both. the right and left pulmonary arteries. A tunnel of knitted Dacron ® was t h e n constructed a p p r o x i m a t e l y 5 cm in circumference from the ventricular septal defect to the aortic
FIGURE 3. (left) Chest roentgenogram. The apex is displaced inferiorly and to the left. The ascending aorta is dilated. The pulmonary arterial branches and the peripheral pulmonary vascular markings are near normal. FIGURE 4. (right). Operative photograph demonstrating the difated aorta arising anteriorly from the right ventricle. The pulmonary artery is located posteriorly, partially hidden by the aorta. PA = pulmonary artery; RV = right ventricle.
852
November 1973
The American Journal of CARDIOLOGY
Volume 32
DOUBLE OUTLET RIGHT V E N T R I C L E - - A G A R W A L A
ostium. Initially sutures were placed along the posterior rim of the septal defect extending through the base of the septal leaflet of the tricuspid valve to avoid the conduction tissue. At the point where the margins of the ventricular septal defect and the pulmonary outflow tract were adjacent, the patch was in apposition so that the final construction was not a cylinder but a tube narrowed at its midportion (Fig. 5). Proximally the patch was attached around the margins of the ventricular septal defect. Distally it was attached around the aortic annulus. Great care was taken to widely resect the anterior wall of the right ventricle so that flow through the outflow tract anterior to the tunnel into the pulmonary artery would not be obstructed. After bypass, the following pressures (in millimeters of mercury) were recorded: left ventricle 90/5; aorta 90/60; right ventricle 35/5; pulmonary artery 25/10. These values indicated adequacy of the tunnel lumen and only minimal residual right outflow tract obstruction. At the completion of the repair transient complete heart block was noted. The patient's early postoperative course was complicated by congestive heart failure requiring vigorous digitalis and diuretic therapy. Intermittent atrioventricular A-V dissociation, 2:1 A-V block and first degree block developed but normal sinus rhythm returned on the 9th postoperative day. However, on the llth postoperative day complete heart block returned and persisted. Six weeks later a permanent transthoracic epicardial pacemaker was inserted. During the next few months the patient showed progressive clinical improvement and 6 months after operation, she returned to school. Three years postoperatively she is doing well and has good exercise tolerance. Her color is excellent, and the hematocrit is 43 percent. She attends regular class in school. • Discussion
Taussig and Bing 11 originally described a case of "complete transposition of the aorta and a levoposition of the pulmonary artery." A large subpulmonic ventricular septal defect was present. Van Praagh 12 subsequently redescribed their original specimen as one of the types of double outlet right ventricle with both subaortic and subpulmonic conus, the aortic and pulmonic valves lying side by side at approximately the same level, and the dilated pulmonary artery overriding the ventricular septal defect. However, the pulmonary valve did not override the left ventricular cavity; the ventricular septal defect was not confluent with the pulmonary valve because the subpulmonary conal free wall musculature formed the roof of the ventricular septal defect. The pulmonary-mitral valve fibrous continuity observed in complete transposition of the great arteries was not present. In the Taussig-Bing malformation the ventricular septal defect is high and located just below the pulmonic valve. The left ventricle ejects blood directly through the ventricular septal defect into the main pulmonary artery, and the aorta receives blood from the right ventricle. These features physiologically resemble the circulation in transposition of the great arteries. Cyanosis due to arterial desaturation is always present, and the pulmonary arterial blood contains a higher oxygen content than the aorta. The
ET AL.
FIGURE 5. Diagram of the transventricular tunnel constructed at operation to transmit blood from the left ventricle through the ventricular septal defect to the aorta, without obstructing the right ventricular outflow tract. Because of the anatomic abnormality, the prosthesis was narrowed to form a tube in its midportion, constituting a transventricular graft, one end of which was connected to the ventricular septal defect and the other to the annulus of the aortic valve. L.A. = left atrium; L.V. = left ventricle; Pulm. a. = pulmonary artery; R.A. = right atrium; R.V. = right ventricle.
right ventricular pressure is always at systemic levels. The pulmonary vascular disease is usually severe in this malformation possibly because of high oxygen content in the pulmonary artery and direct systemic pressure from the left ventricle. Pulmonic stenosis is a rarely associated lesion. 13 The clinical findings associated with the usual Taussig-Bing anomaly include cyanosis of various degrees of severity, clubbing, early congestive heart failure and markedly reduced exercise tolerance. Squatting is infrequent, and "spells" do not occur. A loud holosystolic murmur sometimes with a thrill and an accentuated narrowly split second heart sound are audible. The electrocardiogram is of little help in differential diagnosis, usually showing right axis deviation and right ventricular hypertrophy; additional left ventricular hypertrophy may be present. The chest roentgenogram usually shows increased heart size with a dilated main pulmonary artery and pulmonary vascular plethora. Angiographic studies are crucial in differentiating double outlet right ventricle with subpulmonic ventricular septal defect (Taussig-Bing anomaly) from other malformations. Contrast material injected into the right ventricle fills both great arteries simultaneously, the aorta opacifying more than the pulmo-
November 1973
The American Journal of C A R D I O L O G Y
Volume 32
853
DOUBLE OUTLET RIGHT VENTRICLE--AGARWALA ET AL.
nary artery. The left ventricular injection demonstrates the ventricular septal defect and filling of both great arteries, the main pulmonary artery being more opacified than the aorta. In the posteroanterior view both great arteries lie side by side, the aorta lying to the right of the main pulmonary artery; in the lateral view they are superimposed. The pulmonary and aortic valves lie at the same level, and the aortic valve is anterior to the ventricular septum. The diagnosis of Taussig-Bing anomaly is usually difficult in. living patients. The conditions from which this malformation must be differentiated include all cyanotic congenital cardiac abnormalities with increased pulmonary blood flow, such as complete transposition of the great arteries with ventricular septal defect, Type I truncus arteriosus, single ventricle with or without transposition, large ventricular septal defect with Eisenmenger complex, total anomalous pulmonary venous drainage, common atrium and, rarely, tricuspid atresia with a large ventricular septal defect. When the TaussigBing malformation is further complicated by pulmonic stenosis, the clinical features resemble those
of tetralogy of Fallot or complete transposition of the great arteries with pulmonic stenosis. This was true in our case. Angiocardiography is of crucial importance in differentiating these conditions.
Surgical treatment of the Taussig-Bing malformation is complicated. Kirklin et al. 14 and Hightower et al. la reported that in this situation they found it feasible to repair the ventricular septal defect so t h a t the left ventricular blood is ejected into the pulmonary artery and to provide for intraatrial transposition of venous return. They had success in three such cases. In our case, complicated by infundibular obstruction, a different approach was used. After wide infundibular resection, direct exit of blood from the left ventricle to the aorta was achieved. This appears to be an alternate method of repair. The occurrence of complete heart block was an unfortunate complication. Its transient occurrence in the operating room had led us to believe t h a t it was not due to suture placement. Replacement of critical sutures with greater care to prevent tension of the patch around the margins of the ventricular septal defect might have prevented this problem.
References 1. Witham AC: Double outlet right ventricle. Am Heart J 53:928-939, 1957 2. Peacock TB: Malformations of the Human Heart, second edition. London, Churchill & Sons, 1866, p 204 3. Saphir O, Lev M: The tetralogy of Eisenmenger. Am Heart J 21:31-46, 1941 4. Braun K, DeVries A, Feingold DS, et ah Complete dextroposition of the aorta, pulmonary stenosis, interventricular septal defect and patent foramen ovale. Am Heart J 43:773-780, 1952 5. Abbott ME" Atlas of Congenital Heart Disease. New York, American Heart Association, 1936, p 44 6. McMahon HE, Lipa M: Double outlet right ventricle with intact ventricular septum. Circulation 30:745-748, 1964 7. Neufeld HN, DuShane JW, Wood EH, et ah Origin of both great vessels from the right ventricle. I. Without pulmonic stenosis. Circulation 23:399-412, 1961 8. Neufeld HN: Origin of both great vessels from the right ventricle, chap 27. In, Heart Disease in infants, Children and Adolescents (Moss AJ, Adams F, ed). Baltimore, Williams & Wilkins, 1968, p 577-588
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9. Neufeld HN, Lucas RV Jr, Lester RG, et al: Origin of both great vessels from the right ventricle without pulmonic stenosis. Brit Heart J 24:393-408, 1962 10. Neufeld HN, DuShane JW, Edwards JE: Origin of both great vessels from the right ventricle. II. With pulmonic stenosis. Circulation 23:603-612, 1961 11. Taussig HB, Bing RJ: Complete transposition of the aorta and a levoposition of the pulmonary artery. Am Heart J 37:551-559, 1949 12. Van Praagh R: What is the Taussig-Bing malformation? Circulation 38:445-449, 1968 13. Lev M: Double outlet right ventricle. Scientific exhibit, American College of Cardiology meeting, Chicago, March 1972 14. Kirklin JW, Barcia A, Deverall PB, et ah The surgical treatment of complex forms of transposition. Br Heart J 33: supph73-80, 1971 15. Hightower BM, Barcia A, Bargeron LM Jr, et ah Double outlet right ventricle with transposed great arteries and subpulmonary ventricular septal defect: the Taussig-Bing malformation. Circulation 39: suppl 1:207-213, 1969
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