Congenital corrected transposition of the great vessels without cardiac anomalies

Congenital Great ARTHUR

Corrected

Transposition

Vessels Without J. Moss, M.D., AnoLm

Cardiac

JI. HUTTE:K, JR., >r.u.,f

ELLIOT

of the

Anomalies* 0.

LIPCHIK, M.D.

and ROIXX~ E. GALLACHEK, ar.n.1 lhdmtel.,

NCW York

The clinical, angiocardiographic, vectorcardiographic and phonocardiographic findings in a fifty-four year old man with uncomplicated corrected transposition of the great vessels are presented. A soft, grade Z/6 precordial systolic murmur was present, and the second heart sound at the base appeared single on auscultation but was normally and narrowly split on phonocardiogram. The P-R interval was prolonged and paroxysmal nodal tachycardia frequently occurred. The patient never manifested any evidence of heart failure and the inverted ventricles functioned well with their reversed pressure and volume loads. Sixteen well documented cases of uncomplicated corrected transposition have been reported in the American literature, and the available clinical data on seven of these cases are reviewed. The present patient is the only one in the older age group with complete antemortem documentation of all cardiovascular parameters.

T

CASE REPORT This fifty-four year old man (A.K., SMH No. 68-92-80) was the father of seven children. He was admitted to the Strong Memorial Hospital on June 6, 1968, because of palpitations and syncope. He experienced his first syncopal episode in 1960, and during the next six years he had several “fainting spells.” During one of these episodes in 1966 a nodal tachyarrhythmia was documented by electrocardiogram. These symptoms persisted despite treatment with diphenylhydantoin and quinidine sulfate. In the month prior to admission the patient experienced more frequent palpitations which were associated with breathlessness, substernal distress and light-headedness. Exertional dyspnea also developed, but without nocturnal dypsnea, orthopnea or ankle edema. The patient was able to continue working as a heavy laborer until six months prior to admission when the arrhythmic episodes caused him to stop work. The family history was negative for congenital heart disease. Physical examination revealed a healthy-appearing, middle-aged man with a blood pressure of

HE POTENTIAL variations in cardiovascular structure associated with transposition of the great vessels have recently been described and diagrammatically portrayed in a special Circulation supplement [I]. Transposition of the great vessels may be termed “corrected” when ventricular inversion accompanies this lesion, and the blood flow through the heart is functionally normal. In the great majority of cases of corrected transposition, associated congenital defects of the intracardiac structures are present [Z-3]. Corrected transposition without associated intracardiac anomalies is a rare condition. The was oldest patient in whom this diagnosis made during life and who is described in the literature was twenty-five years old at the time of study [4]. In this report we describe the clinical, angiocardiographic, vectorcardiographic and phonocardiographic findings in a fifty-four year old man with uncomplicated corrected transposition of the great vessels.

*From the Departments of Medicine and Radiology, The University of Rochester School of Medicine and Dentistry and The Strong Memorial Hospital, Rochester, New York 14620. This study was supported in part by the Ernest L. Woodward Research Fund. Requests for reprints should be addressed to Dr. Arthur J. Moss, University of Rochester Medical Center, 260 Crittenden Boulevard, Rochester, New York 14620. Manuscript received January 9, 1969. tPresent address: Massachusetts General Hospital, Fruit Street, Boston, Massachusetts. $Present address: Bldg. 10, 2B 51, National Institute of Health, Bethesda, Maryland 20014.

986

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(lorrected

Transposition

of Great

130185 mm. Hg in both arms. The pulse was intermittently rapid and irregular. Marked arcus corneae were present. ‘l‘he jugular venous pulse was accentuated only during runs of the irregular tachyarrhythmia. The thyroid was not enlarged. The lungs were clear. The borders of cardiac dullness extended 2 cm. to the left and 4 to 5 cm. to the right of the mid-sternal line. The apical impulse was not felt, but a diffuse lower sternal im~ndsc was present. During regular rhythm, the first heart sound along the left sternal border was reduced in intensity. At the base, the second heart sound appeared to be a single sound, and the intensity of the second heart sound was accenuated in thr second left intercostal space. A soft grade 2/C ejection tyfx murmur was best heard at the lower left sternal border with poor radiation

Vessels-Moss

ct al.

9Hi

SCALAR

FRON-At

2 m

II HOR’ZON’A. FIG. 2.

F1anh

sv,stem\cctorcartliogratl1

frontal, left sagittal and horizontal planes intervals arc 2.5 msec. SW text for tIerails.

aVR

“4

OVL

“5

CM

“6

acre\\ the precordium. No gallop rhythm or ejection click was audible. Although a mild lumbar scoliosis was present, the thoracic cage was not deformed. The liver edge was felt at the right costal margin; the spleen tip was not felt. The peripheral pulses were intact, and there was no peripheral edema. On the twelve-lead electrocardiogram (Fig. 1) , normal sinus rhythm was present, the P wave was broad, and the I’-R interval was prolonged to 0.26 second. There was no septal q waves in leads I, aVL. V, or V,;. The QRS axis was 0 degrees in VnI.

47,

DI CE\IBFR

1969

I\11h scal31-, l)arh time

the frontal plane, and the R wave in the antterior precordial leads was accentuated. A wide divergence between the QRS and T axes existed in both the frontal and horizontal plane. The Frank system vectorcardiogram (Fig. 2) revealed that the direction of the initial QRS vectors was leftward and anterior in the horizontal plane and clockwise in the frontal plane. In the horizontal and left sagittal planes, the QRS loop wa.5 inscribed in a counterclockwise direction, with a major portion of the loop oriented anterior to the E point. A lead II rhythm strip (Fig. 3) revealed isolated nodal premature beats and ,ihort runs of nodal tachycardia. Phonocardiography revealed an early systolic murmur along the lower left sternal border. 7’lie second heart sound was narrowly split (25 msec.) and varied slightly with respiration. A simultaneous phonocarotid pulse recording demonstrated that the first component of the sccontl Iicarc ~uncl

FIG. 3. .\n electrocardiographic rhythm 11) reveals isolaled nodal premature beats run of nodal tachycardia.


Corrected

988

‘Transposition

of Great

Vessels-Moss

et al.

LLSB

CP ECG with simultaneous carotid pulse (CP) recording FIG. 4. Phonocardiogram and electrocardiogram (ECG). Heart sounds are recorded from the second left intercostal space (2nd LIS) and the lower left sternal border (LLSB) . An early systolic murmur (SM) is evident, and the second heart sound (S,) is narrowly split (25 msec.). The first component of S, precedes the carotid incisuria and this finding confirms a normal aortic and pulmonary valve closul-e sequence. [Paper speed, 75 mm. per second.]

FK. 5. Posteroanterior chest roentgenogram with barium swallow shows the typical cardiac contour of corrected transposition; the aortic shadow forms the upper left convex border, the pulmonary artery segment is hidden medially, and the heart is rotated to the right. The right suprahilar and mediastinal region is bare. The pulmonary vascularity is normal; situs sol&us is present.

originated from aortic valve closure, and the aortic and puhnonic components of the second heart sound exhibited a normal sequence (Fig. 4). Chest roentgenograms with barium swallow (Fig. 5) revealed levoposition of the aorta, relative mesoposition OF the main pulmonary artery, mild rotation of the heart to the right and a normal cardiothoracic ratio. Venous angiocardiography (Fig. 6) revealed a normal right atrium situated to the right of the spine. The venous ventricle leading to the pulmonary artery had no infundibulum, and its muscle trabeculations were smooth, all features of a morphologic left ventricle. The apex of the ovalshaped chamber pointed to the right. The main pulmonary artery arose posteriorly to the aorta,

was positioned to the right of the spine and dilated. Neither pulmonary valvular nor infundibular stenosis was present. The peripheral pulmonary arteries were attenuated but not stenosed. The venous return drained into a normally situated and normally sized left atrium. At the time of retrograde systemic angiocardiography (Fig. 7)) the pressures in the arterial ventricle were 115/10 mm. Hg. The systemic ventricle was situated in the midline and to the left of the venous ventricle. The systemic ventricle resembled a morphologic right ventricle with heavy, irregular trabeculations, a crista supraventricularis and a conus or outflow tract leading to an aorta which arose anteriorly and to the left of the dilated pulmonary artery. No intracardiac shunts were seen, AMERICAN

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Transposition

6.A

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et al.

GR

2. antelopostctiol-. B, iatctal. Sitrgle ItInts ftoru the serial veuous attgiocardiogram show a smooth ~vallctl, o\al-sh,~pc’cl vctrtriclc giving rise artcty xvhich is situated to the to a posteriorly placed, tlilated pulmotlar\ right of the aorta. The apex of this mot-pbologic Iclt \entriclr points to the right. The right atrium was normal. krc.. li.

Frc,. i. A, anter-oposterior. B, lateral. Retrograde angiocardiograms demonstrate a well trabeculated chamber with a distinct outflow tract leading into a left-sided aorta, anterior to the pulmonary artery. both tentriclcs lie in the same frontal plane, i.e., the interventricular septum lies in a tr-ue sagittal ditcction. In the frontal projection the ascending and descending aorta 01 et.lalr. DFoEhIIIt:R

1969

989

990

Corrected

Transposition

of Great Vessels-Moss

et al.

FIG. 8. Single frame reproductions of the selective tine coronary angiograms show a large left-sided (A) coronary artery, which has the morphologic characteristics of a right coronary vessel. The morphologic left coronary artery (B) is situated to the right and arises laterally from the right coronary cusp. The atrioventricular node artery, which usually arises from the right coronary artery, is not identified.

and the systemic atrioventricular

valves

were com-

petent. Selective

coronary angiography (Fig. 8) showed a large “left-sided” vessel arising from the posterior cusp, supplying the posterior aspect of the heart as the posterior descending artery within the atrioventricular groove. A marginal branch was also present, directed somewhat anterolaterally. These features suggested a morphologic right coronary artery. The “right-sided” coronary artery, resembling a morphologic left coronary artery, arose laterally from the right coronary cusp, probably crossed the pulmonary outflow tract anteriorly and ended as the circumflex and anterior descending arteries. This last vessel was not identified with certainty in these studies. The sinus node and atrioventricular node arteries could not be identified. Calcium was present in the walls of the right coronary artery, but significant occlusive disease was not identified in either coronary vessel. After completion of the aforementioned studies, the patient was digitalized with digoxin. Effective control of the paroxysmal nodal tachyarrhythmia was obtained without further prolongation of the P-R interval. The patient has continued to do well and was last seen in September 1968. COMMENTS

Review of the Literature. Corrected transposition without associated structural cardiovascular abnormalities or insufficiency of the systemic atrioventricular valve is an extremely rare condition. We have been able to find only sixteen cases of well documented, uncomplicated corrected transposition which have been previously recorded or referred to in the

American literature (Table I). Prior to the introduction of angiocardiography, the diagnosis was made at autopsy examination in seven patients. Since 1960, the diagnosis has been made during life by angiography in nine additional patients with uncomplicated corrected transposition. From the total group of sixteen patients adequate clinical data are available in seven patients. The information reported in the literature on these seven patients together with our data are presented in Table II. Both sexes were equally affected. The majority of the patients were asymptomatic and were referred for cardiac evaluation either because of an unexplained heart murmur or a A precordial systolic murcardiac arrhythmia. mur was invariably present, and the second heart sound at the base was loud and appeared to be single on auscultation. Chest roentgenograms generally revealed a mesocardiac position and a concave segment along the upper portion of the border on the left The blood pressure was side of the heart. normal. Complete heart block was not congenital, but it developed in childhood in the majority of patients. In the nine year old patient described by Rotem [26], P-R interval prolongation and incomplete atrioventricular conduction preceded the development of complete heart block. In our patient, P-R interval prolongation without complete heart block was present at age fifty-four; the intermittent nodal tachyarrhythmia provided further eviAMERICAN

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Corrected

Transposition

of Great Vessels-Moss TABLE

REPORTED

Age

and Sex

I

33, M 35, M

i 6

7 x !) 10 11

11, M 8, F

12 13

3, F 25, M

15

Autopsy Autopsy Au topsy Autopsy Autopsy Angiocardiography Angiocardiography Angiocardiography Angiocardiography Angiocardiography

of an abnormality within the atriovenjunctional tissue. The QRS portion of the electrocardiogram contained certain information indicative of A prominent Q wave ventricular inversion. was usually present in lead III, and a normal septal q wave was absent in leads I, aVL and Vs. Septal depolarization was reversed, and it proceeded right to left. In addition, the QRS axis in the frontal plane was generally leftward. In the horizontal plane the precordial leads revealed a relatively low amplitude R wave in the lateral precordial leads. To our knowledge, only two vectorcardiograms of uncomplicated [I41 or relatively uncomplicated [3] corrected transposition have appeared in the American literature. In both reports, and in our case, the vectorcardiograms revealed left axis or I-elative left axis deviation in the frontal plane with a clockwise direction to the initial portion of the QRS loop. In addition, the direction of the initial QRS vector was leftwartl anal anterior, an orientation consistent \z‘itll a mirror image arrangement of the ventriculicr conduction pathways [3]. Physiologic Considerations. The precordial systolic murmur in uncomplicated corrected transposition has never been adequately explained. Cummings [I41 believed the systolic ejection murmurs originated in the aorta, but tricular

47.

I)rCE\lnl;n

196” I !W

Angiocardiography Angiocardiography Angiocardiography Angiocardiography Angiocardiography

dence

VOI.

1891 1931 1954 1955 1955 1957 1960 1960 1960 1961 1962

Autopsy

9, F 17, F 54, M

1fi

TRANSPOSITION

Reported

Autopsy

8, M

15

CORRECTElI

Year

7, M ?, ?

14

I

UNCOMPLICATED

Proof

60, M Adnlt 1 26, F 36, M 42, M

‘i 1

OF

(yr4

Case No.

2

CASES

991

et al.

1969

1% t

I9ri5 1965 1969

Graanboom [“I Walmsley [Yj Edwards et al. [Sj Platzer (91 Platzer [Sl Schaefer, Rudolph Copestake [I I] Copestake [I / 1 Taussig I/_‘] Goodman, Kurmat~ Gumming [I I] Gumming [If ] Ellis et Carey, Rotem, Rotem, Present

/ IfJj

[/i]

al. 141 Ruttcn bel-g [I 51 Hultgrcn [16[ Hultgrrn [IhI paper

intracardiac phonocardiogram revealed a loud murmur high in the venous ventricle. Rotem and Hultgren [16] also concluded that the precordial systolic murmur arose in the aorta, possibly because of abnormal torsion on the root of the aorta. In our case, the root of the main pulmonary artgr was dilated, and we believe the precordial systolic ejection murmur arose from the area of the puhnonic valve. The dilated pulmonary root may reflect the forceful ejection of blood into the pulmonary circulation by a morphologic left An ejection click and a systolic ventricle. ejection murmur are frequently present in idiopathic dilatation of the pulmonary artery [IF]. Although an ejection click was not heard in our case, it has been reported in other patients with corrected transposition [f 61. an

The timing relationship of the aortic and pulmonic valvular heart sounds at the base has been controversial. By auscultation, the second heart sound at the base appeared to be single. Gasul demonstrated by phonocardiography a split second heart sound in the second right interspace in a patient with corrected transposition and mitral regurgitation [It?]. In the two cases of uncomplicated corrected transposition without heart block reporte(d by Cummings [14], phonocardiograms revealed a single second heart sound. In the IWO ‘cases

Age

11,M

8,F

10

11

17,F

54,M

16

17

,

2/6 systolic

2/6 systolic 4/6 diastolic 2/6 systolic

2/6 systolic

2/6 systolic

2/6 systolic 2/6 systolic

2/6 systolic

Congestive heart failure None

Fatigue

None

None

None

None

Syncope

Congestive heart failure

Evaluation of heart murmur

Fatigue; evaluation of heart murmur Evaluation of heart murmur

Bradycardia

Bradycardia

Bradycardia

Tachyarrhythmia 2/6 systolic

Heart Murmur

. ..

go/50

115/65

1 lo/75

112/80

[la].

0.26

Complete heart block

Complete heart block Complete heart block

0.16

0.16

0.16

Complete heart block

0

0

80

-30

+60

-50

7

10

13

15

0

17

.

QRS Axis Q. (degrees) (mm.)

CORRECTED TRANSPOSITION

P-R Interval (sec.)

by Rotem

Concave 130/60 pulmonary segment Narrow 140/80 pedicle; globular heart Meso130/85 cardia; concave pulmonary segment

Concave pulmonary segment Mesocardia

Mesocardia

Cardiomegaly; small aorta Globular heart

Blood Pressure (mm. Hg)

the same as those presented

Loud and single

Loud and single; ejection click Loud and single

Loud and single; ejection click Loud and split

Loud and single

Loud and single

Loud and split

at Base

II

UNCOMPLICATED

TABLE WITH

Cardiac X-ray

PATIENTS

SOLUld

Heart

ON EIGHT

Presenting Symptom

Reason for Cardiac Work-up

DATA

The subject headings in this table are essentially

9,F

15

NOTE:

25,M

13

1.2 3,F

36, M

6

Case (yr.) No. Sex

CLINICAL

R 25

QS 15

rS 2/21

....

RS 13/28

RS 15/25

rS 5/20

...

QRSV, (mm.)

R 10

371

R 8

....

R 7

R 7

Rs 17/2

....

QRSV, (mm.)

Paroxysmal nodal tachycardia

....

Mild “mitral” insufficiency

WI

[ICI

PI

1141

VI

El31

PO1

Refer. ence

Present paper

Bradycardia since childhood

Patent foramen ovale

Patent foramen ovale

Moderate coronary atherosclerosis at autopsy Mild pulmonary insufficiency

Comment

(;orrected

Transposition

of Great Vessels-A4oss

; ith heart block reported by Rotem and Hult~lelt l/h], tlle second heart sound was narrowly >ijlir ~0.03 second) and the splitting varied l\ormalIy \Yitll respiration. Such was the findill,? in our case, and, in addition, a concomitant t ,Irotitl pulse recording indicated a normal ~eclliellc:e 01 v;~lve closure with the second ;jor.tic heart sound preceding the second pultrlollicheart 5olmd. These findings suggest I ILLI. the order of valve closure is more del,c~ndent on the pressure relationships in the l,&nonic and aortic vessels than on the con(111~ Lion system since the latter is anatomically I cvcrsed in this condition. l‘he acquired heart block which frequently ;t( companies corrected transposition has never I)een adequately explained. Walmsley [7] noted fibrosis in the region of the bundle of i_Iis irl OIK cast:. In this regard it is interesting rhat. selective coronary arteriogaphy in our I.;IS~ (lid not opacify the artery to the atriovenir-ic ulai- node. Normally, the vessel to this region is supplied by the right coronary artery /9],, and it is usually well opacified by selective coronary studies. In corrected transpositioil the coronary arteries are inverted and transposetl 151. Thus the artery to the atrioventi-icular -node should arise from the leftsided cortonary artery. The failure to deline.Ite the atrioventricular nodal vessel on selecti\c. study of both coronary arteries suggests a possible anatomic or developmental abnorsignificance. Detailed innlit \ of functional po&ortcm studies of the blood supply to the .ttriovcntricular node in correctetl transposiI iota, similar to the types of studies carried out 1)~ James [lS], may be revealing. It is generally believed that a normal life
47,

DECEMBER

1969

et ~1.

llilized by wedging it under the trabecul:le, but this type of electrode placement. would be tlifficult if not impossible to car1.y out in tile smooth venous ventricle of corrected transposition. \$‘e recommend that if :I l~acem~lkc~ is required in the treatment of corrccletl a trimsthoracic epicartli.rl t’lUtransposition, trade system be used. Furthermore, ;I rtlitlsternotomy approach should be employed 50 that the electrodes can be sutured to the ~1)ickwalled morphologic left Tentriclr. i.v., the right-sided venous ventricle. Acknowledgment: We :u-e grateful to Drs. D. Kramer and P. Shah for their constructive comments :md their phonocardiographic assistance. We thank Miss Cynthia Stebbins for her secretarial assistance.

KEFERENCES 1.

S.I.ANc!ZR, l'.,&NASSI, l<. C., KORNS, hI. IX..Jk I, K. L. and EDWARDS, J. E. IXagrarnatic IXII-‘I av.11of

variations in cardiac (supp. 6) : I. 1968.

structure.

Cirruluiio7i.

37

2. SCHIEBLER. G. E., ElWnRlX, J. E., I
f. A. nlltlWOOI,, Ii.. I-1.

Congenital corrcctetl transposition of the great wsscls: a study of 33 cases. z+xEintrifY, 27: 851, 1961. 3. KUTTENULRC, H. I).. Er I.IOT, I.. P., .\NI)TRSON,l< C., .An.z~ls, P. and TuN.~, N. Congenital torwcted transposition of the great vessels. .4,~l. /. Cccxriol., 17: 339, 196G. 4. ELI IS, I<., MoR(:.\N, B. C., UI.URIENI'H.\I , S. and AuDERSEN, D. H. Congenitally rorwclctl rrallsposition of the great vessels. Rnrliology, 79: 35. 19G2. 5. EI.LIOT, 1.. P., AWXATZ, K. and ELIWARDS, J. E. Cororla~y arterial patterns in transposition tolnplexes. Am. I. Cnrdiol., 17: 362, 19GG. 6. GR~ANROOU, J. Ein Fall van Ucxtrocartlizl nlit

Transposition \on allen grossen C;cFiisscn.%tir/~r. Itlin. Med.. 18: 185, 1891. 7. \~‘Ar.x~rEY, T. Trangosition of the \cntticles alrtl arterial stems. 1. An&., 65: 529. 1931. 8. EI)M’ARI)S, J. E., DRY, T. J.,PARKER, R. I,..1%~RCIIELL,

9.

10.

11.

12.

H. B., WOOD, E. H. and B~r.ur!~.~\\. .\.ll. ,\tl,tsof Congenital .\nomalies of the 11~~1r and (:rcat \‘essels, p. 104. Springficltl, Ill., 1053. (:harles C Thomas. PLATZER, W. I. Lwci Fillc \on Tlan~positioncn wit funktioneller Korrcktur. Vircl,ow’\ illrll. /rnllr. Ancct. 7 ~ ?27: 400, 1955. SCHAEFER. J, A. and RUDOWII. i.. :\. (:o~tccrcd transposition of great vessels. .d,rr. Hw,I I.. 54: 610, l9.i7. COIWT~WE, G. Cart-ectetl transposition of the great vessels. (Abstract.) In: Proceedings of the Canadian Heart :!ssociation, Annual Meeting. Toronto, 1960. TAUSSI~, H. B. Congenital Malformations of the Heal-t, vol. 2, p. 941. Cambridge, 1960. Harvard University Press.

994

Corrected Transposition

13. GOODMAN,A. H. and KUZMAN, W. J. Functionally corrected transposition of the great vessels without significant associated defects. Am. Heart I., 61: 811, 1961. 14. CUMMINGS,G. R. Congenital corrected transposition of the great vessels without associated intracardiac anomalies. Am. J. Cardiol., 10: 605, 1962. 15. CAREY, L. S. and RUTTENBERG, H. D. Roentgenographic features of congenital corrected transposition of the great vessels. Am. J. Roentgenol., 92: 623, 1964.

of Great Vessels-Moss et al. 16. ROTEM, C. E. and HULTGREN, H. N. Corrected transposition of the great vessels without associated defects. Am. Heart J., 70: 305, 1965. 17. KARNEGIS, J. N. and WANG, Y. The phonocardiogram in idiopathic dilatation of the pulmonary artery. Am. J. Cardiol., 14: 75, 1964. 18. GASUL, B. M., GRAET~INGER,J. S. and BUCHELEEES, G. Corrected transposition of the great vessels. J. Pediat., 55: 180, 1959. 19. JAMES, T. N. Anatomy of the Coronary Arteries. New York, 1961. Paul B. Hoeber, Inc.

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