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Juxtaposition of the Atrial AppendagesA Report of Six Necropsied Cases

By HENRY R. WAGNER, M.D., Luis E. ALDAY, M.D., AND PETER VLAD, M.D.

SUMMARYSix cases of juxtaposition of the atrial appendages are documented by necropsied

specimens and analyzed. A high incidence of conotruncal anomalies, especially trans-position of the great arteries, further A-V valve abnormalities, and pulmonary valveatresia, is found. The recognition of juxtaposition of the atrial appendages duringcardiac catheterization should alert the investigator to the presence of a complex cardiacanomaly. A case with a rare type of right juxtaposition of the atrial appendages andbizarre spatial orientation of the atria, ventricles, and great arteries is presented.

Additional Indexing Words:Conotruncal malformation Transposition of great arteries Pulmonary valve atresiaMalrotation of cardiac loop

IN THE frontal view of the heart the rightand left atrial appendages are normally

separated by the great arteries. As the name

indicates, juxtaposition of the atrial appen-

dages may be defined as their side-by-sidelocation, not separated by the great arteries(juxta [Latin] = close by, near to).There are two types of juxtaposition, left

and right, depending on whether the side-by-side atrial appendages both lie to the left or to

the right of the great arteries (fig. 1A and B).Juxtaposition of the atrial appendages has

not been reported as an isolated congenitalcardiac malformation but is accompanied byother almost predictable structural anomaliesof the heart, usually of a severe nature andresulting in clinical cyanosis. This coexistence

From the Children's Hospital of Buffalo, Division ofCardiology, and the Department of Pediatrics, StateUniversity of New York at Buffalo, School ofMedicine, Buffalo, New York.

This study was supported in part by ResearchGrant HE 09474-04 from the National Heart andLung Institute, National Institutes of Health, Bethes-da, Maryland.

Address for reprints: Henry R. Wagner, M.D.,Children's Hospital, 219 Bryant Street, Buffalo, NewYork 14222.

Received March 3, 1970; revision accepted forpublication March 20, 1970.Circulation, Volume XLII, July 1970

of a specific group of anomalies with juxtapo-sition of the atrial appendages constitutes acharacteristic syndrome comparable to theasplenia syndrome. Since juxtaposition iseasily demonstrated by angiocardiography(fig. 2), its presence suggests a complexcardiac malformation with frequently predict-able components. Melhuish and Van Praaghlrecently reviewed 21 cases from the literatureand added 21 new observations. The presentreport analyzes six further patients, one ofthem being the fourth hitherto reported casewith the rare type of right juxtaposition.

MethodsSix specimens with juxtaposition of the atrial

appendages were found among 800 heartsobtained at necropsy collected during the years1955 to 1968. All patients died at the Children'sHospital of Buffalo, New York. The segmentalapproach and terminology as proposed by VanPraagh and associatesl-3 were used to describeand classify the anatomic characteristics of thespecimens.

ResultsThe clinical and anatomic data are pre-

sented in tables and photographs. Table 1gives the clinical data in our six patients.Table 2 presents the anatomic findings. Case 6has unusual and hitherto undescribed anatom-ic features which are illustrated in figure 3.

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WAGNER ET AL.

Figure 1

(A) Left-sided juxtaposition of the atrial appendages (case 4). (B) Right-sided juxtaposition ofthe atrial appendages (case 6). Abbreviations: R = right atrial appendage; L = left atrialappendage; PA= pulmonary artery; Ao _aorta; LV= left ventricle.

Clinical Data

There were three males and three females.The patients survived from 1 day to 12 yearsof age (mean 434 years).

All patients had signs and symptoms ofsevere cyanotic heart disease since early lifesuch as cyanosis, polycythemia, congestiveheart failure, heart murmur, and abnormalelectrocardiogram and chest x-ray. The per-tinent data are listed in table 1. Figure 2shows the typical angiographic appearance ofleft juxtaposition (case 3).

Anatomic Data

There were five cases with left juxtaposition(cases I to 5) and one case (case 6) withright juxtaposition. The anatomic features ofthe case with right juxtaposition will bedescribed in detail while the ones with leftjuxtaposition will be summarized.Abnormal position of the heart within the

chest was present in two cases, one withdextrocardia and one with mesocardia.The most frequently found segmental type

of cardiac structure, found five times in ourseries, is S-D-D (situs solitus of viscera andatria, D-loop of the ventricles or non-invertedventricles, and D-transposition of great arte-

ries). Case 6 exhibits a malrotated D-loop,with situs solitus of the atria and abnormallyrelated great arteries.An anomaly of the conotruncus producing

absence of mitral-aortic fibrous continuity ispresent in all six cases. In five cases (cases 1 to5) this conotruncal malformation effectedwhat is known as D-transposition of the great

Figure 2

Angiographic appearance of left-sided juxtaposition ofatrial appendages (case 3).

Circulation, Volume XLII, July 1970

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WAGNER ET AL.

Table 2

Anatomic Findings in Six Patients with Juxtaposition of the Atrial Appendages

Case Age at Heart Cardiac Atrial R. A-V L. A-V Morph. Morph.no. Sex death position segments septum valve valve R. vent. L. vent.

Left juxtaposition of atrial appendages1 M 3 mo L S-D-D ASD II N N H + E H + E

3 X 5mm

2 F 7 wk L S-D-D PFO N N H + E TW + SC

3 F 12 yr L S-D-D PFO N N H + E H

4 M 2 yr L S-D-D ASD II TS N H + E TW + SC6 X 8 mm

5 M 12 yr D S-D-D ASD II TAT N Hypoplastic H + E10 X 12 mm RV situs

Right juxtaposition of atrial appendages6 F 1 day M Malrotated ASD II N MV Abnormal Small H + E

D-loop 8 X 8 mm TV (L) (R)Abbreviations: O = absent; + = present; H - hypertrophy; E = enlargement; N = normal for age; L = levocardia;

D =dextrocardia; M =mesocardia; S- = situs solitus of atria; -D- = D-loop (non-inverted ventricles); -D =D-trans-position of great arteries; ASD II = secundum type of atrial septal defect; PFO =patent foramen ovale; N - normal;TS -tricuspid stenosis; TAT = tricuspid atresia; Morph. = morphologically; TW + SC thick-walled and smallchambered; (R) = right sided; (L) = left sided; VSD = ventricular septal defect; A = subaortic; B = bilateral; SLV-AVV continuity _ semilunar valve-atrioventricular valve continuity; PV = pulmonary valve; MV = mitral valve; PAT =pulmonary valve atresia; PS = pulmonary stenosis; inf.= infundibular; L = left; R - right.

arteries (aortic valve anterior and to the rightof the pulmonary artery). One of these five(case 1) has a subaortic conus only (permit-ting pulmonary valve-mitral valve continuity),while four specimens are characterized by abilateral conus (no semilunar valve-atrioven-tricular valve continuity). Case 6 also exhibitsa conotruncal malformation but here transpo-sition of the great arteries is not produced. Abilateral conus (subpulmonary and subaortic)is present. The aorta is related to the anatomicleft ventricle, and the pulmonary artery takesoff from the right ventricle.

In four specimens an atrial septal defect ofthe secundum type is found; two have apatent foramen ovale-type opening. A highventricular septal defect, usually of moderatesize, is present in all six hearts.

Tricuspid valve atresia is present in one case(case 5). Case 6 also presents a tricuspidvalve anomaly, as described separately.

In four hearts the pulmonary valve isatretic, and one other has infundibular pul-monic obstruction (case 6). Case 1 shows a

narrowed subpulmonic fibrous ring and abicuspid pulmonic valve.

Case 4 exhibits a right aortic arch and aright-sided patent ductus arteriosus. Thespleen was present in all cases.

Anatomic Description of Case 6 (Fig. 3)The heart is located in the mid chest but the

apex points toward the left. Situs solitus of theviscera including lungs and atria is present.There is right-sided juxtaposition of the atrialappendages (fig. 3A). The right atrial appen-dage is extremely to the right and somewhatposterior to the left atrial appendage. Theright-sided morphologically right atrium re-ceives the superior vena cava, the inferiorvena cava, and the coronary sinus normally.The septal surface displays a secundum typeatrial septal defect. The right atrium opensinto a morphologically right ventricle which isleft-sided and lies posterior to the otherventricle. The tricuspid valve ring is relativelysmall, and multiple wartlike polyps along thefree margin of the valve leaflets are present.


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Vent. Type of SLV-AVV Outflow tract Aortic Preductile Patent ductus Additional anatomicseptum conus continuity obstruction arch coarctation arteriosus data

Left juxtaposition of atrial appendagesVSD A PV-MV Subpulmonic L 0 + Hypoplastic-proximal

6 X 12 mm fibrous ring left pulmonary arteryBicuspid PV

VSD B 0 PAT L 0 06 X 5mmVSD B 0 PAT L 0 0

15 X 10 mmVSD B 0 PAT R 0 Right-sided Mirror-image branching

10 X 10 mm cephalic vesselsVSD B 0 PAT L 0 +

18 X 8 mmRight juxtaposition of atrial appendages

VSD B 0 PS, inf. L + + Hypoplastic ascending8 X 8 mm aorta and arch

All chordae tendineae insert directly into thewall of the right ventricle with no evidence oftrue papillary muscles (fig. 3B). The pulmo-nary artery originates above the morphologi-cally right ventricle (fig. 3C). The subpulmo-nary conus is well developed, resulting infibrous discontinuity between the pulmonaryvalve and both atrioventricular valves below.The morphologically left atrium which is leftsided and anterior to the right atrium butcompletely to the right of the great arteriesreceives the pulmonary veins (fig. 3A). Itopens into the morphologically left ventricle viaa structurally normal mitral valve (atrioventric-ular concordance). The left ventricle, how-ever, is anterior and to the right. The aorta isrelated to the left ventricle, but the presenceof a subaortic conus separates the aortic valveabove from the mitral valve below.The semilunar valves are approximately side

by side, the aortic valve is to the right, and thepulmonary valve is to the left. The pulmonaryvalve is somewhat more superior than theaortic valve. Both semilunar valves are tricus-pid. The aortic valve is distinctly smaller thanthe pulmonary valve. Viewed from below, asubaortic conus is present due to the subaorticinfundibulum. The ascending aorta and theleft-sided aortic arch are hypoplastic. A largepatent ductus arteriosus with a considerablyCirculation, Volume XLII, July 1970

wrinkled intima leadsaorta.

to the descending

DiscussionMelhuish and Van Praaghl noted the rarity

of right-sided juxtaposition as compared toleft (less than 10% of all juxtapositions, or onlythree cases reported so far), and this is relatedto the fact that inverted ventricles (L-loops)are much less frequent than non-invertedventricles (D-loops), since all three had L-loops. In our case with right juxtaposition,however, ventricular inversion is simulated bymalrotation of a D-loop. This case clearly doesnot have a discordant ventricular loop becauseof the presence of atrioventricular concor-dance. The anatomic left ventricle, however, isanterior to the anatomic right ventricle, whichis posterior. The rule of coexistence ofjuxtaposition of the atrial appendages onlywith L-loops is broken by our case 6.

All cases have conotruncal abnormalities.The combined juxtaposition-conotruncal mal-formation appears almost uniform, since onlythree previously reported patients had normal-ly related great arteries.1' 5, 6 Our case 6 has adistinct conotruncal anomaly but no transposi-tion. The aortic valve and ascending aorta areabnormally related to the mitral valve, al-though they take off from the left ventricle.

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WAGNER ET AL.

Figure 3(A) AP view of case 6. The morphologically left atrium, including the appendage, is to the rightof the great arteries and connects to the anteriorly located left ventricle. The aorta takes offfront this ventricle. (B) Specimen of case 6 seen from right side. The enlarged right atriumempties via an abnormal tricuspid valve into the posteriorly located anatomic right ven-tricle. (C) Case 6 seen from left side. The anatomic right ventricle is posteriorly placed andgives off the pulmonary artery. The pulmonary valve is to the left of the aortic valve.

Abbreviations: R-=right atrial appendage; L=left atrial appendage; PA= pulmonaryartery; Ao aorta; RV= right ventricle; LV= left ventricle; HA=right atrium; LAleft atrium; VSD = ventricular septal defect.

This case illustrates that not all conotruncalanomalies (such as a subaortic conus) lead totransposition of the great arteries.

D-transposition in the S-D-D segmentalcombination is found most frequently in ourcollection as well as in the review by Melhuishand Van Praagh.1

Other salient features in our series such asatrial and ventricular septal defects, A-V valveabnormalities, outflow tract obstruction, pul-monary valve atresia, right aortic arch, andpatent ductus arteriosus occur in about assimilar a frequency as in the recent reviewfrom the literature.1 We have not, however,


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encountered double outlet right ventricle inour small series. Five specimens (cases 1 to 5)have similar anatomy and correspond to themost frequently described segmental combi-nation and concommitant anomalies. Case 6with right juxtaposition, situs solitus of theatria, a malrotated D-loop, and a subaorticconus but no transposition of the great arteriesis distinctly different from any previouslyreported specimen with juxtaposition.

Morphogenetic considerations raised byMelhuish and Van Praaghl merit mention. Intheir opinion, juxtaposition of the atrialappendages appears to be a secondary effectproduced by one or more associated anoma-lies, not a primary cardiac malformation perse. Our series shows that in left juxtapositiononly the right atrial appendage is placed tothe left of the great arteries while the rest ofthe right atrium remains to the right. In ourcase with right juxtaposition, however, theentire left atrium is placed to the right side ofthe great arteries. Here, the concommitantmalrotation of the primitive cardiac loop mayhave contributed to this unusual spatialarrangement. This suggests that several fac-

tors may be responsible for the pathogenesisof juxtaposition of atrial appendages.

AcknowledgmentWe are indebted to Dr. R. Van Praagh for his

critical review of the manuscript and his help inanalyzing case 6.

References1. MELHUISH BPP, VAN PRAAGH R: Juxtaposition of

the atrial appendages. Brit Heart J 30: 269,1968

2. VAN PRAAGH R, VAN PRAAGH S, VLAD P, ET AL:Anatomic types of congenital dextrocardia:Diagnostic and embryologic implications. AmerJ Cardiol 13: 510, 1964

3. VAN PRAAGH R, VLAD P, KEITH JD: Completetransposition of the great arteries. In HeartDisease in Infancy and Childhood, ed. 2, editedby JD Keith, RD Rowe, P Vlad. New York,The Macmillan Co., 1967, p 682

4. TAUSSIG HB: Congenital malformations of theHeart. Cambridge, Harvard University Press,1947, p 510

5. FRAGOYANNIS SG, NICERSON D: An unusualcongenital heart anomaly. Amer J Cardiol 6:678, 1960

6. RUEBSCHMANN P: Zwei Falle von seltenerHerzmissbildung (sogenannter Trikuspidalver-schluss). Verh Deutsch Ges Path 18: 174,1921


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HENRY R. WAGNER, LUIS E. ALDAY and PETER VLADJuxtaposition of the Atrial Appendages: A Report of Six Necropsied Cases

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.42.1.157

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