The Syndrome of Dextroversion of the Heartcirc.ahajournals.org/content/circulationaha/18/1/25.full.pdfThe Syndrome of Dextroversion of the Heart ... A, the normal heart, B, classical

The Syndrome of Dextroversion of the HeartBy ROBERT P. GRANT, M.D.

On the basis of 3 personal cases and 119 additional cases collected from the literaturethe difference between dextroversion and mirror-image dextrocardia is outlined. It isconcluded that dextroversion is a part of an exceedingly primitive arrest in developmentthat frequently includes other thoracic and abdominal organs as well as intracardiacstructures. The defect is altogether different in embryogenesis and anatomic findingsfrom mirror-image dextrocardia.

T HERE are 3 conditions that are charac-terized by dextrocardia, or right-lying

heart. The commonest and most familiar ismirror-image dextrocardia, in which the an-terior-posterior relationships of the variousparts of the heart are normal but their right-to-left orientation is reversed. It is usuallyeasily recognized because it is practically al-ways associated with some degree of abdom-inal situs inversus, rarely are there any othercardiac abnormalities, and the electrocardio-graphic changes it produces are diagnostic,with inverted P, QRS, and T waves in lead I.The second cause of dextrocardia is dextropo-sition, in which an otherwise normal heart isshifted to the right by some extracardiacfactor such as eventration of the left dia-phragm, fibrosis of the right lung, etc., andtherefore is also usually easily recognized.The third type is dextroversion. It is theleast familiar, but perhaps the most impor-tant of the 3 from the clinical point of viewbecause it is frequently accompanied by otherintracardiac abnormalities, often of a seriousnature. Furthermore, the embryogenesis ofdextroversion is closely related to the em-bryogenesis of the cono-truncal region of theheart, and understanding the mechanism ofdextroversion sheds light on other congenitalmalformations.

Dextroversion has been recognized underseveral different names in the past: "iso-lated" dextrocardia, incomplete rotation ofthe heart, dextrotorsion, etc. However, the

From the Clinic of General Medicine and Experi-mental Therapeutics, National Heart Institute, Na-tioiial Institutes of Health, Bethesda, Md.

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recent comprehensive studies by Korth andSchmidt' 2 have made it clear that it is adistinctive syndrome with broader clinicaland embryologic implications than thesenames imply, justifying the special term dex-troversion. In general, dextroversion con-sists of a rotation of the ventricular part ofthe heart to the right, as in turning the pageof a book, with the atria remaining in normalposition. Usually there are transposition ofthe great vessels and a ventricular septal de-fect. Since the atria are in normal position,the direction of spread of atrial depolariza-tion is the same as in the normal subject andtherefore the P wave in lead I is upright,differentiating it from mirror-image dextro-cardia. The QRS and T waves in dextrover-sion depend upon the type and degree ofassociated intracardiac malformation. Ifthere is no significant associated abnormality,the mean T vector is often rightward, pro-ducing a negative T1 aiid the QRS loop iscounterclockwise in the frontal plane, pro-ducing a Q,. In the past the negative T1 hasoften been attributed to ischemia; but, asshown in figure 1, it is likelier that it is dueto rotation of the ventricular electric field olnits long axis, for the QRS-T angle is usually anarrow one, as in the normal heart. A moredetailed description of the certain aspects ofthe syndrome will follow the presentation of3 selected cases, 2 of which were studied bybiplane angiocardiography and 1 at postmor-temn examination.

Case 1. D.D. (Clinical Center #016355): a2-year-old girl had been cyanotic with episodes oflabored breathing since birth. There were no signsof congestive heart failure. The heart was not

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A B

RA

H I\/ I / \M& \ / v/ I /

/ \QRS ,\ /

maFIG. 1. A, the normal heart, B, classical mirror-image dextrocardia, C, classical dextro-

version. The position of the 4 cardiac chambers, the great vessels, and the gastric gas

bubble are shown for each. Below, standard limb leads and the frontal plane projectionsof mean cardiac vectors in the uncomplicated case are shown. Note the difference in cardiacsilhouette between mirror-image dextrocardia and dextroversion. In the latter, the apex isblunter, there is a prominent shadow high on the right side due to the right atrium, and thetransposed aorta forms a prominent slow curve o1i the upper left side of the silhouettein the typical case.

enlarged, lay to the right, and a high pitched gradeIII systolic murmur was noted over the entire pre-cordium, maximal in the second interspace to theright of the sternum. The abdominal organs ap-peared to be normally placed. The hematocritvalue was 83 per cent (Wintrobe). In the elec-trocardiogram the P waves were upright in all 3limb leads, the mean QRS vector was directedsuperiorly and to the right with QS deflectionsin the 3 standard limb leads and conventional V3to Ve. The mean T vector was directed inferiorly.On cardiac catheterization the catheter was passedthrough the venous right-lying atrium into bothan arterial and a venous ventricle. The pressurein the venous ventricle was slightly higher thanin the arterial ventricle; neither the aorta norpulmonary artery could be entered. On selectiveangiocardiography with the catheter in one of theventricles (fig. 2) there was immediate filling ofboth ventricles, with a small jet of radiopaquemedium extending into the pulmonary artery (firstfilm). At 3/4 second the pulmonary artery waswell filled, showing narrowing proximally, and thecontrast material could be seen regurgitatingslightly into both atria. At 11/4 seconds ventricu-lar diastole had occurred, with marked reductionin the degree of opacification of the right ventricleand a central area of translucency in the left ven-tricular shadow due to the entrance of nonopaci-

fled blood from the atria. The difference in opaci-fieation between the right and left ventricles atthis stage demarcated the ventricular septum,which was more or less parallel with the horizon-tal or transverse plane of the body. At 134 sec-onds the ventricles had contracted and the aortawas opacified for the first time, showing it to lieanteriorly to the pulmonary artery. In later films(not shown) the pulmonary veins were opacifiedand could be seen draining to the lower left regionof the cardiac silhouette, indicating that the leftatrium was normally placed. The gastric bubbleappeared normally placed. The diagnosis wasdextroversion, with atrial and ventricular septaldefects and transposition of the great vessels.

Case 2. J.W. (Clinical Center #017046), a 5-month-old girl, had been eyanotic and slightlyshort of breath since birth. There were no mur-murs or signs of decompensation. Hemoglobinwas 15 Gm. per cent, with many Howell-Jollybodies end a few spherocytes in the peripheralblood smear. Her electrocardiogram showed up-right P waves in the 3 standard limb leads; themean QRS axis was leftward and inferior in direc-tion with qR deflections on leads I and II andprincipally upright QRS complexes of qR typefrom V2 to V4. During her episodes of dyspneathe mean QRS vector gradually but transientlyrotated to marked left axis deviation without pro-

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27SYNDROME OF DEXTROVERSION OF THE HEART

FIG. 2. Simultaneous lateral (top) and anterior-posterior (bottom) biplane angiocardio-gram in case 1. The opacification for each film is diagrammed below it, and the time of thefilm after injection of the contrast material is indicated at the bottom of the figure. Theposition of the catheter through which the contrast material was injected is shown. Bothventricles fill in the first film, demonstrating the presence of a ventricular septal defect. Inthe second and third anterior-posterior films, the plane separating the right and left ventriclesis well defined, indicating that the ventricular septum lay more or less perpendicular tothe frontal plane of the body, with the right ventricle superior to the left ventricle. Inthe fourth lateral film it can be seen that the aorta is anterior to the pulmonary artery,demonstrating the presence of transposition of the great vessels; usually the aorta has amore leftward position than in this case. In still later films (not shown) the passage ofcontrast material from the pulmonary veins into a chamber to the left of the left ventriclecould be seen, indicating that the left atrium was normally located.



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FIG. 3. The heart in case 2, viewed frontally as it lay in the chest. Right, schema of theinternal structure of the heart from the same view. The interventricular septum, representedby only a shelf, is perpendicular to the frontal plane of the body; the right ventricularportion. of the heart lies superiorly to the left ventricle. The muscular ridge at the proximalend of the tissues separating aorta and pulmonary artery is interpreted to be the cristasupraventricularis; it is more or less parallel with the frontal plane of the body, which is itsnormal anterior-posterior position, and in this heart it is at right angles to the position ofthe ventricular septum.

longation of the QRS interval. The mean T vec-tor was directed rightward, superiorly, andslightly anteriorly. Shortly after admission shebegan to have episodes of sudden limpness withgrunting respirations and intensified cyanosis. Shedied during one of these episodes before diagnos-tic studies could be undertaken.At autopsy (performed by Dr. Louis Thomas)

abnormalities of thoracic and abdominal organswere found. The heart lay principally rightwardwith the posterior sulcus (which normally facesthe diaphragm and contains the descending branchof the right coronary artery) facing anteriorly,indicating that the right ventricle was superior tothe left (fig. 3). The heart was somewhat en-larged, measuring 5.5 em. in its long axis, withright and left ventricular walls 5 mm. thick. Therewere 4 venae cavae. The 2 superior venae cavaelay on each side of the nmediastinum, one enteringthe right, the other the left side of a single largeatrial chamber, which had only a few strands ofatrial septal tissue. These strands indicated thatthe septum was more or less parallel with thefrontal plane of the body, its normal position.The identification of right versus left atrial struc-tures was not possible, but in view of the normallydirected P vector in the electrocardiogram, thesinus node lay rightward, indicating that the atriawere normally placed. One inferior vena cavadrained solely the left lobe of the liver and enteredthe left side of the atrial chamber. The other

inferior vena cava entered into the right side ofthe common atrium, draining the right lobe of theliver, the kidneys, adrenal glands, pancreas, andlower extremities with only minor variations fromthe normal in its architecture. The ventricularportion of the heart consisted of essentially asingle chamber, with a rudimentary ledge at itslateral and inferior border suggesting a ventricu-lar septum. A single A-V orifice connected thecommon atrium and common ventricle. It had 3leaflets that were incompletely developed and sep-arated. There was a prominent muscular ridgein the outflow region of the common ventricle thatseparated the aortic and pulmonary orifices. Bothorifices had 3 well-formed cusps. The coronaryartery orifices were normally placed, and the coro-nary arteries had a normal distribution over theventricular myocardium. The pulmonary arterywas anterior and to the left of the root of theaorta, and was 1.4 em. in diameter while the aortawas 1.0 em. A completely obliterated ligamentumarteriosus connected the 2 vessels. The branch-ings and distributions of the pulmonary arteryand aorta were normal. The pulmonary veinsfrom both lungs joined to form a common pul-nionarv vein that entered the right superior venacava. The right lung had 3 lobes, and a promi-nent demarcation of the lingular part of the leftlung gave it 3 lobes also. In the abdomen, theleft lobe of the liver was larger than the rightlobe and the gallbladder was attached to the left-

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SYNDROME OF DEXTROVERSION OF THE HEART

FIG. 4. Angiocardiogram of case 3. The anterior-posterior view prior to injection ofcontrast material is shown on the left, illustrating the rightward position of the heart. Middle,a film selected from the angiocardiographic series to show the positions of the right ventricleand right atrium. Right, diagram of the opacification in this film. Later filmns outlined theaorta and pulmonary artery, shown by dotted lines in the diagram. The plane separatingright from left ventricle has a more sagittal position in this case than in case 1. There wasno transposition of the great vessels. Note the gastric gas bubble under the left leaf of thediaphragm. The catheter through which the contrast material was injected can also be seen.

lying lobe. The spleen was absent. The stomachlay in the left upper quadrant, its antral regiondeviated by a torsion in the position of the duode-num that was folded by its attachment to the left-lying gallbladder. The tail of the pancreas ex-tended leftward but was not retroperitoneal andlay free in the abdomen. Both the lesser andgreater omenta were incompletely developed, aswere also the mesenteries of the small and largebowels. Counterclockwise rotation of the bowelhad failed to take place, and the ileocecal valve,cecum and vermiform appendix lay in the leftupper quadrant. The large bowel from the sig-noid to the rectum was normal in location. Theportal venous system was normal. The diagnoseswere dextroversion of the heart with atrial andventricular septal defects, common pulmonaryvein, single atrioventricular orifice, persistentright superior and inferior venae cavae, asplenia,partial situs inversus of the liver and gallbladderand incomplete development of other abdominaland thoracic organs.

Case 3. S.C. (Clinical Center #019515), an 8-year-old girl, was first noted to have a murmurat 3 months of age. Cyanosis had not been noted,but she complained of fatigability on exertion.On examination the arterial blood pressure was90/60; there was no cyanosis, clubbing, or evi-dence of congestive heart failure. The maximalcardiac impulse was noted to be in the right fifthinterspace, but abdominal organs appeared to benormally placed. An extremely loud, high-pitcheddiastolic murmur was noted along the left sternalborder. Hemoglobin was 16 Gm. per cent. Theelectrocardiogram disclosed upright P waves inthe standard limb leads, the mean QRS axis was

B

cC D

FIG. 5. Topologic schemata of the surfaces sepa-rating arterial from venous parts of the heart; A,the normal heart, B, transposition of the great vesselsin a normally placed heart, C, dextroversion withouttransposition (drawn from case 2; the atrial andventricular septa were not completely developed inthis heart, the undeveloped portions are representedlby dotted lines), D, dextroversion with transpositionof the great vessels. T. the plane tangential to boththe pulmonary artery and the aorta; V, ventricularseptum; A, atrial septum; C.S., crista supraventricu-laris.

directed rightward and inferiorly, with a qrScontour in lead I; the mean T vector was directedhorizontally rightward, and the conventional pre-

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cordial leads all showed RS contours with in-verted T waves as far as Vo. On right heart cath-eterization the pulmonary artery wedge pressurewas normal, pulmonary artery pressure was110/60, right ventricular pressure was 110/5, andright atrial pressure was normal. The arterialblood was 81 per cent saturated and both right-to-left and left-to-right shunts were detected atthe level of the pulmonary artery. On angiocar-diography (fig. 4) the pulmonary artery and aortafilled simultaneously with the proximal part ofthe aorta poorly visualized and the pulmonaryartery extremely large in size. There was no evi-dence of transposition of the great vessels, andthe aortic arch was left-sided. The atria werenormally placed, but the right ventricle lay to theright of the left ventricle with the interventricularseptum parallel with the sagittal plane of thebody (fig. 5). The gastric bubble was normal inlocation. The diagnosis was mesoversion of theheart with patent ductus arteriosus. In view ofthe pulmonary hypertension and evidence of re-versed flow through the ductus, surgery was post-poned.

DISCUSSIONOne hundred and nineteen cases of dextro-

version have been reported in the literature,proved either by electrocardiogram or autop-sy.* The electrocardiogram is diagnostic ofthis syndrome, for in the presence of a con-genitally right-lying heart an upright P wavein lead I is, with rare exceptions,15 19, 22 in-dicative of dextroversion. An analysis of the119 cases discloses a number of features ofthis syndrome that have not been widely ap-preciated in the past.

It is often believed that a basic differencebetween mirror-image dextrocardia and dex-troversion is that the former is associatedwith situs inversus while the latter is not. Infact, it is because of this notion that dextro-version has frequently been called "isolateddextrocardia" in the past. However, whilethe first part of this statement is relativelyaccurate (among more than a thousand casesof mirror-image dextrocardia in the litera-ture, Korth and Schmidt could find only 12that did not have situs inversus, and only 1of these was studied at autopsy,1' 23-25) the

*Korth and Schmidt" 2 listed 97; an additional 19were found in the literature,3'21 and 3 cases are inthe present report.

second part is not. A number of cases of well-authenticated dextroversion have been re-ported in which other thoracic and abdominalorgans were abnormal either in structure orlocation, often taking a form that superficiallyresembled situs inversus. Among the 69 casesof dextroversion in which autopsy data areadequate, at least 16 had some degree of ab-dominal heterotaxy, including case 2 of thepresent report. However, there tends to be abasic difference between situs inversus andthe heterotaxy seen with dextroversion. Insitus inversus the viscera are inverted but,with few exceptions,24 2628 they are otherwisenormal and fully developed. On the otherhand in dextroversion with abdominal hetero-taxy, the involved abdominal organs are near-ly always abnormal in form or structure. Theabnormality takes a form that is perhaps bestdescribed as an embryologic arrest prior tothe development of body asymmetry, for theviscera tend to be abnormally symmetric inform and primitive in structure. For exam-ple, in the typical case of dextroversion withadvanced abdominal heterotaxy, the liver issymmetric with both lobes equal in size, thegallbladder is often central, the duodenummay be either central or nonrotated, the stom-ach may be central and embedded in the sub-stance of the liver or it may have primitivemesenteric attachments permitting it to lieanywhere in the abdomen, the mesentery ofthe bowel is often underdeveloped with manydifferent bowel arrangements possible, thepancreas may be a central, unorganized tissuemass, both lungs will be tri-lobed, etc. Theheart in dextroversion often best illustratesthis arrest at a stage when the body has asimple, symmetric form, for it may showmany of the features of the "single hearttube." For example, of the 16 cases of dex-troversion with abdominal heterotaxy re-ported in the literature, 8 had cor biloculareand 5 of these had the combination of a singlepulmonary vein, single atrium, single atrio-ventricular ring, single ventricle, and singleoutflow organ (truncus arteriosus).2935A curious anomaly often associated with

dextroversion, and one which further empha-

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sizes the difference between dextroversion andmirror-image dextrocardia with situs inversus,is congenital absence of the spleen, a subjectthat has recently been reviewed by Ivemark36and Putscher and Manion.37 Ten of the 69autopsied cases of dextroversion (includingcase 2 of the present report) had asplenia,while among hundreds of cases of situs in-versus totalis reported in the literature, only1 had asplenia and in this case the diagnosiswas made during surgical exploration andnot at autopsy.38 Nearly half of the 107cases of asplenia reported in the literaturehad right-lying hearts, and although the dataare incomplete in many of them, most if notall were instances of dextroversion. Further-more, among all cases of asplenia 90 per centhad associated intracardiac anomalies, usu-ally cono-truncal abnormalities; this is thesame incidence of cono-truncal deformityseen in dextroversion, while in mirror-imagedextrocardia additional intracardiac anoma-lies are exceedingly uncommon. Finally, theabdominal heterotaxy that usually accompa-nies asplenia more closely resembles that seenwith dextroversion (i.e., symmetric hetero-taxy) than it does situs inversus. Ivemarkexplained the association of asplenia, abdom-inal heterotaxy, and cono-truncal malforma-tion by ascribing it to an arrest of develop-ment at the stage when the body first searchesout its normal asymmetry, and he points outthat the splenic bud appears and cono-truncaldifferentiation takes place at the same fetaldate. Putscher and Manion suggested thatasplenia and heterotaxy may be due to sup-pression or inhibition of structures that areasymmetric by their left-sidedness; howeverthis would not account for the high incidenceof cono-truncal abnormalities in these cases.The degree of developmental arrest in the

dextroversion syndrome varies from case tocase and from organ to organ in individualcases. In the heart, for example, certain car-diac metameres may be arrested while adja-cent ones develop normally. This is illus-trated by case 2 of this report where, withmost of the heart arrested at the "singleheart tube" stage (bilateral superior and in-

ferior venae cavae, single atrium, single atri-oventricular ring, single ventricle, singlecommon pulmonary vein), the great vesselswere completely differentiated without eventransposition. And there are cases at theother end of the spectrum, with only a singlefeature of the dextroversion syndrome pres-ent. For example, Korth and Schmidt foundin the literature 6 cases of dextroversion inwhich the rightward rotation of the ventricu-lar part of the heart was the only abnormalityfound at autopsy, with no septal defects,transposition, or abdominal heterotaxy.'When the abnormality is limited in its exten-siveness, as in these cases, it is clinically be-nign. Of the 119 cases of proved dextrocar-dia in the literature, 50 were adults and liv-ing at the time they were reported. Althoughother abnormalities were identified in manyof these cases by clinical or x-ray examination,the abnormalities were not marked enough tointerfere seriously with cardiac function.One of the commonest associated intracar-

diac abnormalities in dextroversion is transpo-sition of the great vessels. Of 59 cases inwhich the position of the great vessels was

described either from autopsy or angiocardi-ography, 5 had truncus arteriosus, and 44 hadtransposition of the great vessels. Thus 80per cent had some form of outflow abnormal-ity. The transposition is somewhat differentfrom that seen when the heart is normallyplaced, for in dextroversion the aorta lies tothe left of the pulmonary artery and onlyslightly anterior to it, while in transpositionwith a normally placed heart the aorta is usu-ally directly anterior to the pulmonary ar-tery. Thus, the roentgenogram of the heart indextroversion shows a wide base if transpo-sition is present, with the aorta often forminga prominent, long left margin of the cardiacsilhouette as shown in figure 1. Since thegreat vessels lie side by side in this form oftransposition, one might wonder if they arenot simply "uncoiled" by the abnormalrightward position of the heart. However,turning the normally placed heart to the rightwould tighten the coil of the great vessels, notuncoil them. To uncoil the great vessels, the

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ventricular part of the heart must rotatemarkedly leftward, a phenomenon that Sha-ner has reported in the pig embryo and pos-sibly in 1 human fetus.39 The transpositionof dextroversion is nearly always accompa-nied by a ventricular septal defect. Of 535cases of dextroversion with adequate autopsydata, 44 had ventricular septal defects. Thereis only a single case of dextroversion in theliterature with transposition but no ventric-ular septal defect.4"

This means that a high percentage of casesof dextroversion have one or more associatedintracardiac anomalies, 90 per cent of autop-sied cases). This is in striking contrast withmirror-iniage dextrocardia. Amongy morethan a thousand cases of mirror-image dex-trocardia with situs inversus in the literatureonly 5 cases have been found in which addi-tional intracardiae anomalies were or mayhave been presenlt,22' 41-43 an incidence notgreatly different from that of the populationat large. This difTerence between the 2 synl-(Ironies emphasizes the fact that they must bedue to quite different einbryologic defects. Italso leads to the useful clinical aphorism thatif, ini a patient with a right-lying heart, thereis also cyanosis or an abnormal cardiac mur-niur, the diagnosis is probably dextroversiolland not mnirror-image dextrocardia regardlessof the position of the abdominal organs. Thevariety of intracard'iac and extracardiac ab-normalities that may be associated with dex-troversion of the heart warrant treatinog it asa 'syndrome"' of which the right-lying(, heartis only a part and perhaps not always px eneiit.For example, amiong Ivemnark 's 69 cases ofaspleinia, abdominal hleterotaxy, and cono-

truncal deformity, 46 had no dextroversion.Although precise data are not availal)le, cliin-ical experielnce also indicates that dextrover-sion is iuneomnnion amiong all cases of cono-truncal deformnity.The most striking positional anomnaly iii

dextroversion is the rightward location of theventricular part of the heart. There are 2reasons for believing that this is due to an

arrest at the stage of cardiac developmentwhen the ventri(ulilar 1l0) normally hlas' a

riglhtward p051t101144' 4 and is not due toeither abnormal inversion or torsion of theheart. In the first place the ventricles arethe onlly part of the heart to have the abnor-imial J)ositioll. If it were due to inversion onews-ould expect the atria or the great vesselswould also occasionally be inverted. How-ever, there are no cases of dextroversion sofar reported in whom the atrial bodies werealso abnormal ill position; and the arch of theaot.ta is left-sided in the maajority of cases ofdextroversion, whether or ilot transpositioiiis )resent.' (Most students do Iiot considertralslosition of the great vessels to be dueto inversion, if l)v inversionl one meamis atranslovation resultinr from a disturbance offactors that determine body laterality, as inisitti iniversus; genierally, transposition isconisidered due to a local disturbance in eitherthe rate or mnagiiituide of the spiralling of thetruneal septuin as it descenids.44 43> ) In1 tilesecond place, there are in the literature 13(ases in which b)oth atrial appendages lay tothe left of the aorta hut with lnormally placedatrial bodies, all(l at least 8 of these had some(legree of dextroversioni.'2 18, 46 32) Clearly,iii these cases tlme anomalous location of theright atrial appenidage must have been dueto (delay or failure of the bulbus; to migrate tothe left, and ini the (ases with dextroversionthis failure evideimtly- included the entire vemn-tricular loo1).The particular position of the ventricles ini

lextroversion requires coillmmelt. W\llile itmay 1)e graphie to describe the ventricles as'turned to the right as ill turningi- the p)ageof a book, '' this is ilot comi)letely accurate.Ass can be seeni fromn the schemea in figure 5,the anatomic drawsing in figure 3, and theallnriocardiogramii ill figure 2, thie right veli-triele lies superiorly to the left ventriele, an(1the interventricular septum is at right ainglesto the frontal pulanie of the bo(dy. while nor-

immally it is parallel with the fronital plane.'I'lTus the ventricular part of tIme heart is not

onilv rotated to the right but is also rotatedoni its long axis. For a normially placed heartto take this position it may- he srwuiig right-ward in eithler the frontal l)lalle (like a pen-

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dulum) or in the horizontal plane (like turn-ing the page of a book). The difference is ofsome importance because it is also necessaryto rotate the ventricular heart on its long axisto simulate dextroversion completely, and forthe former the supplemental rotation of theventricles is 90 degrees in a counterclockwisedirection while for the latter it is 90 degreesin a clockwise direction as viewed from theapex. This is, of course, an artificial way tolook at dextroversion. Rather one should askwhat is the embryologic positional shift thatfails to take place, is it a leftward swing inthe frontal plane (pendular) or a leftwardswing in the horizontal plane (page turning) ?Perhaps this is answered by the angiocardio-gram of case 3 (fig. 4), for this is an exampleof mesoversion, with the heart less markedlyrightward than in dextroversion. It can beseen that the ventricular septum lies in thesagittal plane of the body, with the right ven-tricle to the right and the left ventricle to theleft. If this heart is representative of themid-position between dextroversion and thenormally placed heart, then the ventricularloop comes to lie in the left chest by swingingabout 120 degrees as a pendulum in the fron-tal plane, and the long axis rotation thatbrings the right ventricle to its normal posi-tion anterior to the left ventricle is a clock-wise rotation of 90 degrees. Evidently thislatter type of rotation occurs late in the courseof the leftward swing of the ventricular heartfor, in case 3 at least, it has not yet takenplace.

It was pointed out earlier that when theaorta and pulmonary artery are transposedin dextroversion they have a somewhat dif-ferent location than when they are transposedin the normally placed heart. Doerr53 ex-plained this difference by suggesting that indextroversion there is an inversion of the bul-bus cordis in addition to transposition. How-ever, when the heart of dextroversion isstudied topologically it is found not necessaryto postulate the additional anomaly. In fig-ure 5 are shown the positional relationshipsbetween the various parts of the normal heartand the dextroverted heart, with and without

transposition. The positions have been sche-matized by plotting 3-dimensionally the sur-faces that separate the right and left sides ofthe heart. The surface between the atria isdefined by the atrial septum, that between theventricles by the ventricular septum, and thatbetween the aorta and the pulmonary arteryby the plane tangential to both. It can beseen from the diagrams that the peculiarityof the transposition seen in dextroversion isdue to the fact that the rightward position ofthe ventricles is simply communicated to thetransposed vessels. For example, if the trunc-al surface in D is swung as a pendulum to theleft it will have the same spatial position asin transposition in the normally placed heart,B, and there is no evidence of inversion of thebulbar region. In contrast with this, in C isshown the schema of the cardiac surfaces incase 2. When the truncal surface in this caseis swung leftward as a pendulum, it will havethe same spatial position as in the normalheart, A. In other words there is no transpo-sition in this case, and the normally relatedgreat vessels are simply deviated to the rightby the rightward position of the ventricles.Note that in C while the crista supraventricu-laris has a normal spatial location, as a resultof the ventricular dextroversion it is at rightangles to the ventricular septum. This re-sulted in a much severer degree of deformityfrom a functional point of view than if trans-position had taken place. Perhaps, then, thehigh incidence of transposition in cases ofdextroversion is simply due to the fact thatnormal descent of, the truncal septum in aheart with dextroversion often results in adeformity that is incompatible with survivalbeyond earliest fetal stages and thereforerarely comes to the attention of either theclinician or the pathologist. In any case, it islikely that case 2 survived as long as she didonly because of the presence of large ventricu-lar and atrial septal defects and a single atri-oventricular orifice, permitting oxygenatedblood to reach the systemic circuit.

SUMMARY AND CONCLUSIONSThree cases of dextroversion and an anal-

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ysis of an additional 116 cases collected fromthe literature are presented. There are 3major differences between dextroversion andmirror-image dextrocardia. 1. In dextrover-sion the atria have a normal position but theventricular heart lies rightward, as if swunglike a pendulum through an arc of 120 de-grees in the frontal plane from its normalposition; there is also a 90 degree counter-clockwise rotation of the ventricular heart onits long axis, with the right ventricle lyingsuperiorly to the left ventricle. In contrastwith this, in mirror-image dextrocardia allcardiac structures are rightward mirror-im-ages of the normal heart. 2. The vast majorityof cases of dextroversion (90 per cent of au-topsied cases) have additional intracardiacmalformations, usually of the cono-truncalregion, while in mirror-image dextrocardiaadditional intracardiac anomalies are prob-ably no more frequent than in the populationat large. 3. An abdominal heterotaxy is oftenpresent in dextroversion, which is differentfrom the situs inversus that accompanies mir-ror-image dextrocardia: instead of inversionof abdominal organs there is a tendency forabdominal structures to be primitive and bi-laterally symmetric, frequently with congen-ital absence of the spleen.

It is suggested that dextroversion is a partof a potentially multiorgan developmental de-fect that takes place at a very early fetal stagewhen the body first begins to search out itsnormal asymmetry, when cardiac septationand cono-truncal differentiation start, whenthe splenic bud first appears, and when theventricular loop is normally predominantlyrightward in location. Depending upon theintensity and distribution of the abnormalitya wide variety of intracardiac and extracar-diac anomalies and combination of anomaliesmay result. Dextroversion of the heart isperhaps only one part of this syndrome.

Transposition of the great vessels is pres-ent in over 80 per cent of cases of dextrover-sion. Indirect evidence is offered which sug-gests that the high incidence of transpositionmay partly be due to the fact that, in thepresence of dextroversion, a normal descentof the truncal septum often produces a sever-

er functional abnormality than does transpo-sition and these cases do not usually surviveearliest fetal stages.

ACKNOWLEDGMENT

I wish to acknowledge the cooperation andassistance of Dr. Andrew G. Morrow, Chief of theClinic of Surgery of the National Heart Institute,on whose service the 3 patients were studied andwhose staff performed the catheterizations andangiocardiograms, the Diagnostic X-Ray Depart-ment of the Clinical Center for assistance in per-forming the angiocardiograms, and Dr. Lewis B.Thomas, Chief of the Post-Mortem PathologicService of the Clinical Center, who performed theautopsy on case 2.

SUMMARIO IN INTERLINGUA

Es presentate 3 casos de dextroversion delcorde e un analyse de 116 casos additionalcolligite ab le litteratura. Ii ha 3 differentiasprincipal inter dextroversion del corde e dex-trocardia specular. (1) In dextroversion leatrios occupa un position normal, durante quele corde ventricular es displaciate verso ledextera, como si-in le maniera de un pendulo-illo habeva essite movite a transverso unarco de 120 grados in le plano frontal forasde su position normal. Ii ha etiam un rota-tion sinistrorse de 90 grados circa le axe longedel corde ventricular, e le ventriculo dextereoccupa un sito superior al ventriculo sinistre.Per contrasto con isto, in dextrocardia specu-lar, omne le structuras cardiac es dextrorsecorrespondentias specular del corde normal.(2) In le grande majoritate del casos de dex-troversion del corde (90 pro cento secundo lenecropsias reportate), il ha additionalmentemalformationes intracardiac, usualmente in leregion cono-truncal, durante que anomaliasintracardiac in subjectos con dextrocardiaspecular es probabilemente non plus frequenteque in le population general. (3) Un hetero-taxia abdominal es frequentemente incontratein casos de dextroversion del corde, que differeab le sito inverse associate con dextrocardiaspecular. In loco de inversion del organosabdominal, il ha le tendentia que le structurasabdominal remane primitive e es bilateral-mente symmetric, frequentemente con absen-tia congenite del splen.

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Es presentate le these que dextroversion es

possibilemente un parte de un potentialmenteextense defecto disveloppamental que occurre

multo precocemente in le vita fetal, quando leorganismo comencia su "tentativas de asym-

metria," quando le septation cardiac e ledifferentiation cono-truncal se initia, quandole button splenic se maniifesta, e quando leansa ventricular occupa normalmente un locopredominantemente dextrorse. In dependen-tia del intensitate e del distribution del de-fectos disveloppamental, un grande varietatede anomalias intra- e extra-cardiac e de com-binationes dq tal anomalias pote resultar.Dextroversion del corde es forsan solmenteun parte de iste syndrome.

Transposition del vasos major es presentein plus que 80 pro cento del casos de dextro-version. Es presentate indicios indirecte que

supporta le these que le alte incidentia detransposition del vasos majores in parte cau-sate per le facto que in le presentia de dex-troversion un descendita normal del septotruncal produce frequentemente un anormali-tate functional que es plus sever que illo pro-

ducite per transposition, e in caso de un

descendita normal del septo truncal le fetonon supervive usualmente al prim stadios desu disveloppamento.

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9.For true Philosophers, who are perfectly in love with truth and wisdom, never find

themselves so wise, or full of wisdom, or so abundantly satisfied in their own knowledge,but that they give place to truth whensoever, or from whosoever it comes.-WILLIAMHARVEY. De Motu Cordis, 1628.

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ROBERT P. GRANTThe Syndrome of Dextroversion of the Heart

Print ISSN: 0009-7322. Online ISSN: 1524-4539 Copyright © 1958 American Heart Association, Inc. All rights reserved.

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The Syndrome of Dextroversion of the Heartcirc.ahajournals.org/content/circulationaha/18/1/25.full.pdfThe Syndrome of Dextroversion of the Heart ... A, the normal heart, B, classical