taussig-bing malformation, coarctation of the aorta, and...

Taussig-Bing Malformation, Coarctation of the

Aorta, and Reversed Patent Ductus Arteriosus

Operative Correction in an Infant

By ANNE LIr-LE WEDEMEYER, M.D., RUSSELL V. LUCAS, JR., M.D.,AND ALDO R. CASTANEDA, M.D.

SUMMARYOperative correction of a Taussig-Bing malformation was successfully accomplished

in a 23-mo-old boy, weighing 6.8 kg. Associated coarctation of the aorta and reversedpatent ductus arteriosus were corrected in a preliminary operation. The patient's man-

agement was complicated by severe preoperative thrombocytopenia which respondedto multiple phlebotomies. Surgical repair of the Taussig-Bing malformation consistedin patching the ventricular septal defect to create physiologically complete transposi-tion of great vessels. Then a Mustard procedure was done.

Additional Indexing Words:Phlebotomy Thrombocytopenia Platelet counts Coagulation data

T HE Taussig-Bing malformation,' one ofseveral forms of origin of both great

vessels from the right ventricle2 3 has eludedsuccessfuJ operative correction until recently.4The subject of this report is a 6.8-kg infant

with the Taussig-Bing malformation who hassurvived successful surgical correction. Thecoexistence in this infant of coarction of theaorta and reversed patent ductus arteriosusadded to the diagnostic and surgical problems.Significant hematologic abnormalities furthercomplicated his care.The purpose of this communication is to

define the anatomic features of his defect, torelate these to the other varieties of origin of

From the Departments of Pediatrics and Surgery,University of Minnesota, Minneapolis, Minnesota.

This work was supported in part by the DwanFamily Fund and Minnesota Heart Association Grant-in-Aid and was completed during Dr. Wedemeyer'stenure of a Cardiovascular Clinical Research TrainingGrant HE 05222-13 from the U. S. Public HealthService.

Address for reprints: Dr. Aldo Castaneda, Univer-sity of Minnesota Medical Science Center, Box 91,Minneapolis, Minnesota 55455.

Received April 14, 1970; revision accepted forpublication August 6, 1970.

Ci,cudation, Volume XLII, December 1970

both great vessels from the right ventricle, andto discuss methods of operative correction.

Report of CaseThis male infant was the product of a full-term

pregnancy, uncomplicated by infections or drugs.He weighed 3.4 kg at birth. Cyanosis occurredshortly after birth, and a systolic heart murmurwas heard at 2 weeks of age. At age 4 mocongestive heart failure was recognized, and heresponded to digitalis.At age 13 mo (January 1968), when he was

referred to the University of Minnesota MedicalCenter, the mother reported increasing cyanosis,mild tachypnea, and delayed growth and devel-opment.

His weight of 6.4 kg and length of 68 cm wereless than the third percentile for his age. Flushblood pressures were 90 mm Hg in the right armand 80 mm Hg in the left arm and leg; allperipheral pulses were strong. Reversed differen-tial cyanosis was noted, with moderate cyanosis ofhis lips and upper extremities and mild cyanosisof the lower extremities. All fingers and toes wereclubbed. A precordial bulge approached acarinate deformity. S, was loud, and the secondsound was very loud and thought to be single. Asystolic ejection click and a grade III/VI harshholosystolic murmur were heard maximally at theleft midsternal border. The liver was palpable 3cm below the right costal margin.

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WEDEMEYER ET AL.

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Preoperative electrocardiogram. The QRS axis is 1100, an unusual finding in origin of bothgreat vessels in the right ventricle. Note the evidence of biventricular hypertrophy.

The hemoglobin value was 14.1 g/ 100 ml, andthe hematocrit was 51%. The electrocardiogramrevealed a mean QRS frontal plane axis of+1100, mild right atrial enlargement, andbiventricular hypertrophy (fig. 1). Thoracicroentgenograms revealed cardiomegaly, massivelyincreased pulmonary vascular markings, a promi-nent left atrium, and an enlarged pulmonaryartery segment (fig. 2).The clinical impression was either a variant of

transposition of the great vessels and reversedpatent ductus arteriosus or truncus arteriosus.

Angiocardiography and cardiac catheterizationwere performed. The right ventriculogram (fig.3) demonstrated a hypertrophied right ventricle.The dilated pulmonary artery arose from the rightventricular infundibulum in its usual location.The aorta opacified more densely than thepulmonary artery and arose via a subaortic conus

from the right ventricle immediately to the rightof the pulmonary artery. The semilunar valveswere at the same plane and the great vessels were

side by side. There was no continuity betweensemilunar and atrioventricular valves. In addition,mild tubular hypoplasia of the distal aortic archand isolated severe coarctation of the aorta distalto the left subclavian artery were present. Bloodflow through a large patent ductus was predomi-nantly right to left. A pulmonary arteriogramrevealed regurgitation of contrast material intoboth ventricles. The descending aorta opacifiedvia the reversed patent ductus arteriosus.The hemodynamic features (table 1) further

defined the defect. The high (82%) O. saturationin the main pulmonary artery and low (47%) O.0saturation in the right brachial artery (obtainedvia cannulation though not simultaneously) were

compatible with origin of both great vessels fromthe right ventricle (type IIA Taussig-Bingmalformation).The equal pressures proximal and distal to the

coarctation and the equal (and high) O.0saturation in main pulmonary artery anid descenld-

Circulation, Volume XLII, December 1970

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TAUSSIG-BING MALFORMATION

Figure 2Preoperative thoracic roenitgenograms. (A) Posteroanterior view. Pulmonary vascular markingsare increased. There is generalized cardiomegaly and prominence of pulmonary artery. Theleft atrial appendage is apparent at the left cardiac border. (B) Left lateral view. The posteriordeviation of the esophagus confirms the presence of left atrial enlargement.

Figure 3

Selective right ventriculograms. (A) The right ventricle is dilated and heavily trabeculaed. Theaorta and pulmonary artery fill simultaneously. The aorta is more densely opacified than thepulmonary artery. The aortic and pulmonary valve planes are at the same level and the aortadoes not take its usual leftward swing in this view.

(B) Lateral view at the same time as A. The aorta and pulmonary artery lie in the sameplane and the valves are at the same level. The aorta clearly rises anterior to the ventricularseptum. In both views the descending aorta is poorly opacified. Coarctation of the aorta andreversing ductus arteriosus were demonstrated by aortography.


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1WEDEMEYER ET AL.

Table 1Hemodynamic Data

02 saturationSite (%) Pressure (mm Hg)

Superior vena cava 54 -

Right atrium 55 a = 6v =5m= 5.5

Right ventricle 59 94/0, m = 12Main pulmonary artery 82 100/43, m = 68Descending aorta 80 95/40, m = 73Right brachial artery 47

100

0 4 8 12 16 20

indic,tes phfebofo..y DAYS

Figure 4

ing aorta defined the reversedarteriosus.

patent ductus

As a prelude to corrective intracardiac surgery,the child underwent division and suture of thepatent ductus arteriosus and repair of thecoarctation of the aorta in February 1968.Following the procedure, cyanosis appeared to beequal in his head and upper and lowerextremities. He was discharged but was tocontinue on digoxin 8 days after surgery.

In May 1968, a relative anemia was treatedwith iron given orally. A month later hemoglobinwas 18.9 g/ 100 ml, hematocrit was 75%, andplatelet count, 6,000/cu mm. Hematologic find-ings (table 2) were not consistent with typicalintravascular coagulation.

Heparin therapy failed to correct the thrombo-cytopenia. Peripheral platelet destruction wasruled out by normal platelet survival after platelettransfusion.

Continued iron therapy over the next 2 weeksresulted in an increase in hematocrit (81%) andfurther decrease in platelets (4,000/cu mm).

Table 2Coagulation Data

Test Patient's values Normal values

Prothrombin time (sec) 10.3 11-13Kaolin partial thrombo-

plastin time (sec) 56.4 3545Thrombin time (sec) 18.0 14-16Venous platelet count 17,000 200,000-400,000Fibrinogen (mg/100 ml) 210 200-400Bleeding time (min) 5 3-6Coagulation factorsV(%) 100 70-130VIII (%) 115 70-130IX (%) 50 70-130XI (%) 29 70-130

Relationship of platelet count (solid dots) to hema-tocrit (open dots).

Because of the reciprocal relationship betweenhematocrit and platelets, the infant was subjectedto multiple small phlebotomies. During a 3-weekperiod, approximately half his total blood volumewas withdrawn without replacement. He toler-ated these procedures well. The platelet count(80,000 on the day of definitive surgery) hadincreased after phlebotomy as the hematocrit fell(fig. 4).On October 10, 1968, he underwent total

physiologic correction of his cardiac defect withthe aid of a pump oxygenator. * The heart wasexposed via a median sternotomy. On totalcardiopulmonary bypass a transverse right ven-triculotomy was performed. A large ventricularseptal defect was found above the cristasupraventricularis, its upper border being incontinuity with pulmonary valvar tissue. Theaortic valve was to the right of this defectseparated from it by a prominent muscle mass.The left lateral margin of the ventricular septaldefect was formed by the parietal band of thecrista and the inferior margin by the muscularseptum. Interrupted 4-0 Tefdack stitches wereplaced along the free margin of the ventricularseptum, the parietal band, and the right lateralmargin. These stitches were then passed througha 1/16-inch thick Teflon felt patch and tied. Theremaining free edge of the prosthetic patch wassutured to the upper lip of the transverseventriculotomy incision. Left ventricular-pulmo-nary artery continuity or a functional transposi-tion of the great vessels was thereby created (fig.5). The final physiologic correction of this defectwas accomplished by the Mustard transatrial

*Olson Medical Products, Inc., Ashland, Massa-chusetts, and Bentley Laboratories, Santa Ana,California.


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(

Figure 5Method of surgical correction. The patent ductus arteriosus and coarctation of the aorta wererepaired at a previous operation. (Left) This illustrates the preoperative anatomy and hemo-dynamic features. (Right) Operative management. First, a Teflon patch (lower patch) waspositioned in such a way as to create a tunnel from the ventricular sepatal defect to the baseof the pulmonary artery. This maneuver resulted in the hemodynamic pattern of completetransposition of the great vessels and intact ventricular septum. Second, the atrial septumwas excised and a pericardial baffle positioned within the atria in the fashion described byMustard. As a consequence systemic venous blood passed through the pericardial tunnel intothe anatomic left ventricle, then through the ventricular septal defect and Teflon tunnel intothe pulmonary artery. Pulmonary venous blood was channeled into the anatomic right ventricleand thence out through the aorta.

baffle, technic.5 This channeled the pulmonaryvenous blood into the anterior (right) ventricleand aorta, and all vena caval blood into theposterior (left) ventricle and pulmonary artery.

Following surgery the child was given oneplatelet concentrate, as his platelet count oncoming off bypass was 47,000. One week aftersurgery the platelet count was 254,000. He wasdischarged from the hospital on November 7,1968. Two weeks later when seen at a clinic visithe was acyanotic, had an excellent appetite, andhad increased activity. A grade II/VI systolicejection murmur was present at the upper leftsternal border, and the pulmonary component ofthe second heart sound was loud. The liver wasnot palpable.On September 8, 1969, this child was acyanotic


and active. His weight was 11.1 kg. The plateletcount was 228,000, the hematocrit was 39%, andhemoglobin 12.1 g/ 100 ml. The electrocardio-gram revealed complete right bundle-branchblock and normal P waves; the thoracic roentgen-ograms demonstrated mild cardiomegaly andnormal pulmonary vascular markings. On July 20,1970, the child weighed 14 kg and was totallyasymptomatic.

DiscussionOrigin of both great vessels from the right

ventricle is a relatively uncommon cardiacmalformation. Neufeld and associates3 empha-sized that the location of the ventricular septaldefect in this anomaly is largely responsible

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WEDEMEYER ET AL.

for the physiologic features. In type I, theventricular septal defect is located subjacentto the crista supraventricularis; this locationallows blood from the left ventricle to flowpreferentially into the aorta. In type IIATaussig-Bing malformation, on the otherhand, the ventricular septal defect lies abovethe crista and is closely related to thepulmonary valve. Left ventricular blood thuspreferentially enters the pulmonary artery andsystemic venous blood is directed into theaorta.There are, however, a number of anatomic

variations among patients with origin of bothgreat vessels from the right ventricle. Theventricular septal defect may be above thecrista supraventricularis and confluent withthe pulmonary valve (our case, Neufeld andassociates3). The defect may be supracristalbut separated from the pulmonary valve bythe subpulmonary conal free wall (the origi-nal Taussig-Bing heart,6 the cases of High-tower and associates4). The defect may besupracristal, underlying the pulmonary valve,and extending under the aortic valve as well(type IIB of Neufeld and associates3). Finallythe defect may be subcristal and immediatelyopposite the subaortic conus.3The location of the ventricular septal defect

in origin of both great vessels from the rightventricle is of critical importance in theoperative correction. When the defect islocated subjacent to the crista supraventricu-laris, a patch can be sewn to establishcontinuity between left ventricle and thesubaortic conus of the right ventricle, therebycompletely correcting the defects.7When the ventricular septal defect is

supracristal, this simple internal rerouting isnot possible. In our patient the high subpul-monary position of the ventricular septaldefect and the large muscle mass separatingthe defect from the aortic valve prevented theinternal routing of left ventricular blood intothe aorta. On the other hand, it was a simplematter to establish continuity between the leftventricle and the pulmonary artery with apatch without compromising the outflow toeither pulmonary artery or aorta. This physio-

logic pattern of complete transposition of thegreat vessels with intact ventricular septumwas then amenable to correction utilizing thetechnic pioneered by Mustard.

This same technic was employed by Kirk-lin's group.4 Their patients differed from ourpatient in that the aorta was anterior, and theventricular septal defect was located lower,and was separated from the pulmonary valveby infundibular muscle. They suggested that atunnel from ventricular septal defect to aortacould have been made and an aortic homo-graft utilized to connect the right ventricle tothe pulmonary artery. This procedure couldnot have been employed in our patient.

In those patients in whom the supracristaldefect underlies both pulmonary artery andaorta, a review of the anatomy suggests thatthe subaortic portion of the ventricular septaldefect could be enlarged and then anintraventricular tunnel constructed withoutobstructing aortic pulmonary outflow.Thus the surgeon, preparing to correct

origin of both great vessels from the rightventricle with a supracristal defect, must havean accurate preoperative anatomic and physi-ologic assessment and be prepared at opera-tion to utilize a variety of operative technics,depending on the precise anatomic features heencounters.The association of coarctation of the aorta

and patent ductus arteriosus is common in theTaussig-Bing malformation.3 When these arepresent, technical considerations of cardiopul-monary bypass necessitate preliminary surgi-cal elimination of the coarctation of the aortaand patent ductus arteriosus.

Utilization of multiple phlebotomies to treatthrombocytopenia was based on observationsreported by Hartmann.8 The dramatic re-sponse of the hematologic abnormalities in ourpatient confirms the usefulness of multiplephlebotomies as preoperative preparation inthis clinical setting.The majority of patients with the Taussig-

Bing malformation die in infancy. Further, thepresence of high pressure and high flowwithin the pulmonary circuit may result in


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early pulmonary vascular disease. Thus, cor-rective operation in infancy would be highlydesirable. Our patient weighed only 6.8 kg.Since we are accumulating encouraging experi-ence with open heart surgery in infantsweighing 3 to 4 kg, we propose that the infantwith the Taussig-Bing malformation havecorrective operation performed as soon as thediagnosis can be established.

References1. TAUSSIG HB, BING RJ: Complete transposition of

the aorta and a levoposition of the pulmonaryartery. Amer Heart J 37: 551, 1949

2. NEUFELD HN, DUSHANE JW, WOOD EH, ET AL:Origin of both great vessels from the rightventricle: I. Without pulmonary stenosis.Circulation 23: 399, 1961

3. NEUFELD HN, LUCAS RV JR, LESTER RG, ET AL:

Origin of both great vessels from the rightventricle without pulmonary stenosis. BritHeart J 24: 393, 1962

4. HiGrrowER BM, BARcIA A, BARGERON LM, ETAL: Double-outlet right ventricle with trans-posed great arteries and subpulmonary ventric-ular septal defect. Circulation 39 (suppl I): I-207, 1969

5. MUSTARD WT, KEiTH JD, TRUSLER GA, ET AL:Surgical management of transposition of thegreat vessels. J Thorac Cardiovasc Surg 48:953, 1964

6. VAN PRAAGH R: What is the Taussig-Bingmalformation? Circulation 38: 445, 1968

7. LucAs RV JR, ADAMS P JR, WINCHELL P, ET AL:Origin of both great vessels from the right ven-tricle, without pulmonary stenosis. Amer HeartJ 62: 415, 1961

8. HARTMAN RC: A hemorrhagic disorder occur-ring in patients with cyanotic congenital heartdisease. Bull Hopkins Hosp 91: 49, 1952

The factor in human life provocative of a noble discontent is the gradual emergenceinto prominence of a sense of criticism, founded upon appreciations of beauty, and ofintellectual distinction, and of duty.-ALFRED NORTH WHITEHEAD: Adventures of Ideas.New York, The Macmillan Co., 1933, p. 12.


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CASTANEDAANNE LITTLE WEDEMEYER, RUSSELL V. LUCAS, JR. and ALDO R.

Ductus Arteriosus: Operative Correction in an InfantTaussig-Bing Malformation, Coarctation of the Aorta, and Reversed Patent

Print ISSN: 0009-7322. Online ISSN: 1524-4539 Copyright © 1970 American Heart Association, Inc. All rights reserved.

is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231Circulation doi: 10.1161/01.CIR.42.6.1021

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