Brief Communications


October 1990 Heart Journal

Fig. 2. Preoperative radionuclide ventriculogram in the lateral projection (A) demonstratesthe characteristic hourglassconfiguration of a large inferoposterior false aneurysm (FA) with narrow communication with the left ventricle (LV). Seven weeksafter surgical repair, the saccularaccessorychamber is no longer visualized (B).

vealed a residual bidirectional flow disturbance acrossone site of the repair, with subsequent resolution over a 2-month period. The clinical importance of such a residual flow abnormality rests on the differentiation between inadequate or incomplete surgical repair and a transient postoperative shunting acrosssuture lines that will eventually obliterate or endothelialize. In that respect,this residual flow abnormality appearsanalogousto flow disturbances described after ventricular septal defect patch repair, where it hasbeen postulated to occur along the suture line and not through the patch material itself.grlo This study illustrates the usefulnessof serial postoperative echocardiographicand color Doppler evaluation in ascertaining the true nature of post-repair residualshunting. We conclude that the diagnosisof a pseudoaneurysmand the detection of shunting flow through its communication with the left ventricle can be significantly facilitated by colorflow Doppler imaging. Early residual shunting after surgical repair may indicate a transient flow disturbance along suture lines, with subsequentobliteration or endothelialization. REFERENCES

I. Roberts cle. Am 2. Gueron aneurysm Thor& 3. Davidson Fishbein

WC, Morrow AG. Pseudoaneurysm of the left ventriJ Cardiol 1967;43:639-44. M. Wanderman KL. Hirsch M, Borman J. Pseudoof the left ventricle after mybcardial infarction. J Cardiovasc Surg 1975;69:736-42. KH. Parisi AF. Harrineton JJ. Bareamian EM. MC. ‘Pseudoaneurysm of-the left ve&icle: an un: usual echocardiographic presentation. Ann Intern Med 1977;86:430-3. 4. Roelandt J, Sutherland GR, Yoshida K, Yoshikawa J. Improved diagnosis and characterization of left ventricular pseudoaneurysm by Doppler color flow imaging. J Am Co11 Cardiol 1988;12:807-11. 5. Catherwood E, Mintz GS, Kotler MN, Parry WR, Segal BL.

Two-dimensional echocardiographic recognition of left ventricular pseudoaneurysm. Circulation 1980;62:294-303. 6. Wang R, DeSantola JR, Reichek N, Edie R. An unusual case of postoperative pseudoaneurysm of the left ventricle: Doppler echocardiographic findings. J Am Co11 Cardiol1986;8:699102. I. Sorensen SG, Crawford MH, Richards KL, Chaudhuri TK, O’Rourke RA. Noninvasive detection of ventricular aneurysm by combined two-dimensional echocardiography and equilibAM HEART J 1982;104z145-52. rium radionuclide angiography. 8. Loperfido F, Pennestri F, Mazzari M, Biasucci LM, Vigna C, Laurenzi F, Manzoli U. Diagnosis of left ventricular pseudoaneurysm by pulsed Doppler echocardiography. AM HEART J

1985;110:1291-3. L, Pieroni D, Roland J, Shemalek J. Recognition 9. Valdez-Cruz of residual post-operative shunts by contrast echocardiographic techniques. Circulation 1977;55:148-52. 10. Stevenson JG. Kawabori I. Stamm SJ. Bailev WW. Hall DG. Mansfield PB: Rittenhousk EA. Pulsed Doppler echocardio: graphic evaluation of ventricular septal defect patches. Circulation 1984;7O(suppl 1):38-46.

Congenital left atrial appendage aneurysm with intact pericardium: Diagnosis by transesophageal echocardiography Keith Allen Comess,MD,a Dennis Peter Labate, RCDS, CRT,a Jerrold A. Winter, MD,a Arthur C. Hill, MD,b and D. Craig Miller, MD.b Stanford, Calif. From the Departments of%ternal Medicine (Cardiology) and hCardiovascular Surgery, Santa Clara Valley Medical Center and Stanford University School of Medicine. Reprint requests:Keith Comess,MD, Cardiology, Room 522, SCVMC, 751 South Bascom Ave., San Jose, CA 95128. 4/4122682

Volume Number

120 4

Brief Communications



1. Preoperative electrocardiogram with left atria1 abnormality and -30 degree axis.

Isolated congenital aneurysm of the left atria1 appendage with an intact pericardium is an unusual anomaly, with 36 cases so far reported worldwide.’ Patients are often asymptomatic and are diagnosed incidentally,2 but may present with embolization or supraventricular arrhythmias.3-6We report a caseof a left atria1 appendageaneurysm diagnosedby transesophagealechocardiography and confirmed by catheterization and surgery. An 1%year-old healthy Hispanic man had a positive tuberculin skin test on a routine physical examination. A chestradiograph wasobtained and the patient wasreferred for cardiac evaluation, due to the unusual cardiac silhouette. The patient denied any medical problems, past or present. Specifically, there were no symptoms suggesting thromboembolism and there was no history of heart rhythm disturbances.The initial blood pressurewas 120/ 80 mm Hg. The pulse was80 and regular. The patient appeared healthy. Physical examination wasentirely normal, including the first and secondheart sounds,jugular and carotid pulsations, and lung fields. The electrocardiogram (Fig. 1) showedsinus rhythm, axis -30 degrees,and a left atria1 abnormality. The chest radiograph (Fig. 2) showed an abnormal opacity in the left atria1 area. An echocardiogram was ordered to further evaluate the apparent left atria1 enlargement. The echocardiogram was done using standard technique with a 3.5 MHz transducer. Image quality waspoor, but a sonolucentspacewasseenadjacent to the left atrium in an off-axis apical four-chamber view (Fig. 3). This could not be replicated in other views, and a

Fig. 2. Preoperative anteroposterior chest film showing unusualcontour of the left atria1border (arrows), produced by the atria1 appendageaneurysm.

definite point of attachment to the heart was not clearly seen. A transesophagealstudy was ordered. The transesophagealechocardiogramdemonstrated an aneurysmof the left atria1 appendage(Fig. 4). This appearedas a saccular, narrow-necked extension from the appendage,with

3. Transthoracic echocardiogram in a modified apical four-chamber view showing a sonolucent space Fig. adjaIcent to the left atrium, lateral to the left ventricle, representing the appendage aneurysm (AA). Color Dop lpler indicates flow within the space. LV, Left ventricle; RV, right ventricle.

4. Transesophageal echocardiogram, short-axis view at the level of the left atrium (LA, ). The krates endage aneurysm (arrows) is seenasan echolucentextension of the atrium. Color Doppler demons low, -velocity inflow and continuity of the aneurysm with the atrium. AO, Aorta; PA, pulmonary aItery. Fig. am





Brief Communications


5. Anteroposterior projection of left atria1 appendageaneurysm (AA) visualized by transseptal cardiac catheterization (A). Characteristic indentation of the lateral wall of the left ventricle by the atria1 appendageaneurysm in an anteroposterior view is shown (arrows) in B.


free communication demonstrated by Doppler color flow mapping in both the short-axis and four-chamber views. Flow within the aneurysm was low velocity, with swirling echoesin the aneurysmcavity. The walls were well defined and thrombus was not identified. The pericardium appeared to be intact. Cardiac catheterization was done to complete the preoperative evaluation. Injection of the atria1 appendage aneurysm was done by the transseptal technique (Fig. 5), confirming the transesophagealechocardiographic diagnosisof a congenital left atria1 appendage aneurysm. Hemodynamicswere normal, aswere the coronary arteries and valves. The left ventriculogram showeda characteristic indentation in the high lateral wall, under the appendageaneurysm (Fig. 5). At surgery, a 6 X 6 cm thin-walled left atria1 appendage aneurysm was found, which indented the anterolateral left ventricular wall at the base (Fig. 6). The overlying pericardium was entirely intact. The diagnosisof an isolated congenital left atria1 appendageaneurysm was confirmed. Other cardiac anomalies were not found. This is the first report of transesophogealechocardiographic diagnosisof a congenital aneurysmof the left atria1 appendage.Though first described in 1953,7this entity is one of the rarest of congenital cardiac abnormalities, with only 36 casespreviously described.l The aneurysm is postulated to arisefrom a developmental weaknessin the atria1 wa11.4While supraventricular arrhythmias and systemic thromboembolism have been described in the literature, many casesare discoveredincidentally. Surgical excision at the time of diagnosisis recommendedto avert these complications.1-6~ 8,g The differential diagnosisincludes partial absenceof the pericardium with atria1 herniation through the defect, paracardiac tumors or cysts, enlarged coronary sinus, and aneurysmaldilation of the left atrium due to valvular heart disease.Radionuclide angiographylO and cardiac cathe-

terization* have also beenused to establishthe diagnosis, asthe electrocardiogramand standard chest radiographic findings are not specific’s3and the physical examination is not diagnostic.l,4,l1 Theoretically, a false negative angiogram could occur if the aneurysm is filled with thrombus. Definitive diagnosisof a left atria1 appendageaneurysm should be possible using standard two-dimensional echocardiography in many cases.12 Foale et a1.12proposed that theseaneurysmswerecharacterized by: (1) origin from an otherwise normal left atrium; (2) a well-defined communication with the atrium; (3) a position within the pericardium and; and (4) distortion of the left ventricular free wall by the aneurysm. The echocardiogram should also help exclude the other rare associatedcongenital cardiac abnormalities (atria1 septal defects and persistent left superior vena cava,13 anomalous pulmonary venous drainage,2),which may be overlooked at catheterization. The causeof the left ventricular wall deformity (indentation, asshownin Fig. 5, B) is unknown. We speculatethat the low-pressureleft atrium indents the left ventricle in utero, when pressuresin both chambersare low. In this case, two-dimensional echocardiography was suggestive,but not diagnostic, due to poor imagingquality. However, the diagnosiswasconfirmed by the transesophogeal study, meeting the four criteria specified by Foale et a1.12Diagnostic transesophagealstudiescan frequently be obtained in caseswhere the transthoracic echo is technically unacceptable.Additionally, transesophagealechocardiography has a high sensitivity and specificity for atria1 appendagethrombi,14 and should be helpful in detecting thesewithin the aneurysm, aswell asexcluding associated abnormalities. In conclusion,transesophagealechocardiography is not only the diagnostic instrument of choice for the evaluation of the atria1 appendagefor the presenceof *References

1, 2, 4. 6, 9. and



Brief Communicatkms


urn: value of diagnostic therapy. Zeit Kardiol

2. LaBarre



for recognition,

1983;72:548-52. Stamato NJ, Hwang

Stephanides L, Scanlon PJ. Left atria1 with associated anomalous pulmonary

October 1990 Heart Journal




Jacobs WR,

appendage venous

aneurysm return. AM

HEART J 1987;114:1243-5. 3. Krueger SK, Ferlic RM, Mooring

PK. Left atria1 appendage aneurysm: correlation of noninvasive with clinical and surgical findings; report of case. Circulation 1975$2:732-S. 4. Coselli JS. Beall AC. Ziaddi GM. Congenital intraoericardial



of the left airial



Thorac Surg 1985;39:466-8. Bramlet DA, Edwards JE. Congenital aneurysm of the left atria1 appendage. Br Heart J 1981;45:97-100. Eie H, Semb G, Muller 0, Holm HA. Aneurysms of the left atri-

al appendage. Stand J Thorac Cardiovasc Surg 1972;6:149-53. Fry W. Herniation of the left auricle. Am J Surg 1953;86: 736-8. Grinfeld R, Trainini JC, Roncoroni A, Fabrykant F, Cacheda H, Tripodi G. Congenital aneurysm of the left atrium. Ann Thorac Surg 1985;39:469-71. 9. Aytac A, Oram A, Olga R, Demircioglu F, Saylam A. Intrapericardial aneurysm of the left atria1 appendix. Cardiovasc Surg 1980;21:509-12. PJ, Zienkowicz BS, Heidendal GAK, Majid PA, 10. DeFeyter Roos JP. Radionuclide angiography in the diagnosis of congenital intrapericardial aneurysm of the left atria1 appendage.

Thorax 1980;35:154-5. Godwin TF, Auger P, Key JA, Wigle ED. lntrapericardial aneurysmal dilatation of the left atria1 appendage. Circulation 1968;37:397-401. 12. Foale RA, Gibson TC, Guyer DE, Gillam L, King ME, Weyman AE. Congenital aneurysms of the left atrium: recognition by cross-sectional echocardiography. Circulation 1982;66: 1065-g. LF. Congenital atriomegaly. Circulation 1962;25: 13. Parmley 553-8. 14. Aschenberg W, Schluter M, Kremer P, Schroder E, Siglow V, Bleifeld W. Transesophageal two-dimensional echocardiography for the detection of left atria1 appendage thrombus. J Am Co11 Cardiol 1986;7:163-6. 11.

Cardiac manifestations of Churg-Strauss syndrome: Report of a case and review the literature Fig. 6. Atria1 appendage aneurysm posterolateral thoracotomy before (panel 2) excision. The left ventricle anterior and right of the aneurysm; (line of arrowheads, panel 1) is seen by sutures. Note the indentation of ventricle produced by the aneurysm.

viewed from a left (panel 1) and after (LV) is immediately the pericardial edge superiorly, suspended the surface of the left

thrombi, but allows thorough evaluation of this frequently difficult-to-image area for other pathologic conditions that may be overlooked by standard diagnositic methods, including transthoracic echocardiography. REFERENCES

1. Hentrich F, Schramm Congenital aneurysms

G, Gala1 0, Stoermer J, Reidmeister JC. of the left atrium with intact pericardi-

Peggy B. Hasley, MD, MHS, William P. Follansbee, MD, and John L. Coulehan, MD, MPH. Pittsburgh,



Allergic granulomatosis and angiitis (Churg-Strauss syndrome) is classically characterized by hypereosinophilia and systemic necrotizing vasculitis in patients with previous allergic rhinitis or bronchial asthma. Typically there are three phases to the disease. The initial prodromal phase Frmn the Divisions Clinical Epidemiology School of Medicine.

of General Medicine and Cardiology, and Preventive Medicine, University

Reprint requests: William P. Follansbee, ogy, Presbyterian University Hospital. Pittsburgh. PA 15213. 414122914

Department of Pittsburgh


MD, Division of Nuclear CardiolRoom 376, DeSoto at O’Hara St..

Congenital left atrial appendage aneurysm with intact pericardium: diagnosis by transesophageal echocardiography.

992 Brief Communications American October 1990 Heart Journal Fig. 2. Preoperative radionuclide ventriculogram in the lateral projection (A) demons...
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