7I4

Brief Communications

American

regurgitation severeright ventricular overloading and cardiac decompensationensue.The increasedright ventricular stroke volume aggravatesdilatation of the pulmonary arteries and tracheobronchial compression.These hemodynamic derangementscould be corrected by closing the ductus arteriosusand taking measuresto ensuremaximum pulmonary vasodilatation until regressionof the pulmonary hypertension. Surgical correction of the tracheobronchial obstruction and pulmonary valve incompetence by pulmonary arterioplasty and insertion of a pulmonary valve substitute requires extracorporeal circulation, which can be hazardous in a sick newborn infant. Also insertion of a pulmonary substitute is not without long-term complications. The clinical courseof our patient hasshownthat our approach is effective and is probably a preferred method of managementof a symptomatic newborn infant with absentpulmonary valve, patent ductus arteriosus,and intact interventricular septum. REFERENCES

1. Smith RD, Dushane JW, Edwards JE. Congenital insufficiency of the pulmonary valve; including a case of fetal cardiac failure. Circulation 1959;20:554-60. 2. Ito T, Engle MA, Holswade GR. Congenital insufficiency of the pulmonary: a rare cause of neonatal heart failure. Pediatrics 1961;28:712-8. 3. Alpert BS, Moore HV. “Absent” pulmonary valve with atria1 septal defect and patent ductus arteriosus. Pediatr Cardiol 1985;6:107-11. 4. Thanopoulos D, Fisher EA, Hastreiter AR. Large ductus arteriosus and intact ventricular septum associated with congenital absence of the pulmonary valve. Br Heart J 1986;55: 602-4. 5. Sethia B, Jamieson MPG, Houston AS. “Absent” pulmonary valve with ASD and PDA [Letter]. Pediatr Cardiol1986;7:119. 6. Kron IL, Johnson AM, Carpenter MA, Gutgesell Jr HP, Overholt ED. Rheuban KS. Treatment of absent nulmonarv valve syndrome with homograft. Ann Thorac Suri 1988;46:;79-81. 7. Stellin G, Jonas RA, Goh TH, Brawn WJ, Venables AW, Mee RBB. Surgical treatment of absent pulmonary valve syndrome in infants: relief of bronchial obstruction. Ann Thorac Surg 1983;3&468-75. 8. Cloez JL, Isaaz K, Pernot C. Pulsed Doppler flow characteristic of ductus arteriosus in infants with associated congenital anomalies of the heart and great arteries. Am J Cardiol 1986;57:845-51.

Large atrial septal aneurysm cystic right atrial mass

mimicking

a

Andrew J. Smith, MD, Ioannis P. Panidis, MD, Stanley Berger, MD, and Ray Gonzales.Philadelphia, Pa.

From the Division of Cardiology, Department of Medicine, sity

Temple

Univer-

Hospital.

Reprint University 414122054

requests: Ioannis Hospital, 3401

P. Panidis, N. Broad

MD, Section St., Philadelphia,

of Cardiology, PA 19140.

Temple

September 1990 Heart Journal

Aneurysm of the atria1 septum is an uncommon anomaly that is usually diagnosedas an incidental finding by twodimensional echocardiography (2D-echo).1-3This defect may be associatedwith other congenital cardiac malformations or with acquired diseasesthat result in elevated pressuresin one atrial chamberwith bulging of the septum to the opposite site.l In this report we describeda patient with a large atria1 septal aneurysm appearing as a cystic right atrial mass by 2D-echo and magnetic resonanceimaging (MRI). A 45-year-old man was admitted to the hospital with acute aortic valve endocarditis secondaryto Streptococcus pneumoniae. He had no previous history of cardiacdisease. 2D-echo and color Doppler study revealed a large vegetation on the aortic valve, severeaortic regurgitation, and a normal-sized left ventricle with hyperdynamic contractility. A large cystic masswith well circumscribed, thin borders but no phasic motion was noted in the right atrium (Fig. 1). On color Doppler imaging there was no evidence of flow within the cavity of the massor shunting acrossthe atria. Gated MRI was performed to further evaluate the atria1abnormality. A 4.3 cm sphericalmasswasdetected in the right atrium, which appeared to originate from the atria1 septum (Fig. 2). The signal intensity from the inner aspectsof the masswasincreasedin relation to free-flowing blood in the cardiac chambersand waslessthan that of the myocardial signal intensity. During open-heart surgery visualization of the right atrium revealed no evidence of a massor thrombus. A large aneurysm involving the entire atria1septumwasnoted to be bulging into the right atrium; two small atria1 septal defects were detected within the aneurysm.The aneurysm wasresectedand oversewnwithout evidence of residual defects. This wasfollowed by aortic valve replacement and the patient subsequentlyhad an uneventful recovery. Atria1 septal aneurysmshave been associatedwith septal defects,atria1 arrhythmias, systolic clicks, atrioventricular valve prolapse, and systemic and pulmonary embolism, and they usually occur at the fossaovalis.lp4 Aneurysms of the entire atria1 septum are rare and invariably coexist with complex congenital malformations.’ This case is unique becausea large atrial aneurysmwasincidentally discovered in the absence of other congenital cardiac anomalies.The PD-echostudy wassuggestiveof a smoothwalled cystic massin the right atrium. Color Doppler imaging demonstrated a blood flow pattern around this structure, with no evidence of flow within the inner cavity, suggestinga space-occupyingintracavity massin the right atrium. MRI was also suggestiveof an atria1 masswith a signal intensity from the inner aspectsof the masssimilar to that of surrounding cardiac tissuesand distinctly different from that of free-flowing blood within the cardiac chambers.Thesefindings werelikely the result of stagnant blood flow within the cavity of the aneurysm.5Basedon the echocardiographicand MRI findings, the differential diagnosisof the right atria1structure included myxoma, thrombus, hydatid cyst, and aneurysmof the atria1 septum. The

Volume Number

120 3

Fig.

Brief Communications

1.

7 15

Two-dimensional echocardiographic right ventricular inflow view demonstrating a cystic mass in the right atrium with an echo-free cavity. RV, Right ventricle; TV, tricuspid valve.

(arrowheads)

Fig. 2. Magnetic resonanceimaging showingmarked bulging of the entire atria1septum (arrowheads) ward the right atrium. LA, Left atrium; LV, left ventricle; R, right; RV, right ventricle.

smooth contour and the echo-free cavity of the masswere not characteristic of a myxoma.6 Thrombus and hydatid cyst were not likely, basedon the clinical presentation and the other echocardiograhicfindings.7The persistent bulging of the aneurysmtoward the right atrium and its lack of phasicmotion may be related to high left atria1pressurein a patient with severe and acute aortic regurgitation. This report illustrates that large aneurysmsof the entire atria1 septum may appear as cystic echo-free cavitary massesin

to-

the right atrium and may not be associatedwith other congenital cardiac anomalies. REFERENCES

1. Hanley Hagler septal port of 2. Hauser

PC, Tajik AJ, Hynes JK, Edwards WD, Reeder GS, DJ, Seward JB. Diagnosis and classification of atria1 aneurysm by two-dimensional echocardiography: re80 consecutive cases. J Am Co11 Cardiol1985;6:1370-82. AM Timmis GC, Stewart JR, Ramos RG, Gangadha-

September 19QO American Heart Journal

7 16 Brief Communications

3.

4. 5.

6. 7.

ran V, Westveer DC, Gordon S. Aneurysm of the atria1 septum as diagnosed by echocardiography: analysis of 11 patients. Am J Cardiol 1984;53:1401-2. Longhini C, Brunaxzi MC, Musacci G, Caneva M, Bandello A, Bolomini L, Barbiero M, Toselli T, Barbaresi F. Atria1 septal aneurysm: echopolycardiographic study. Am J Cardiol 1985;56:653-6. Gallet B, Malergue MC, Adams C, Saudemont JP, Collot AMC, Druon MC, Hiltgen M. Atria1 septal aneurysm-a potential cause of systemic embolism. Br Heart J 1985;53:292-7. Higgins CB, Byrd BF, McNamara MT, Lanzo P, Lipten MJ, Botvinick E, Schiller NB, Cooks LE, Kaufman L. Magnetic resonance imaging of the heart: a review of the experience in 172 subjects. Radiology 1985;155:671-9. Panidis IP, Kotler MN, Mintz GS, Ross J. Clinical and echocardiographic features of right atrial masses. AM HEART J 1984;107:745-58. Alfonso F, Rey M, Balaguer J, Artiz V, Rabago G. Hydatid cyst of the right atrium diagnosed by echocardiography. Am J Cardiol 1987;60:931-2.

The patient wasadmitted to the hospital and underwent combinedtransthoracic and transesophagealstudies,using a 5.0 MHz probe No. 77020 AC, Hewlett-Packard Co., Medical Products Group, Andover, Mass.). This revealed an easily identifiable intimal flap with an entry size approximately 2 to 3 cm above the aortic valve cusps.The dilated aortic root was 8 cm in diameter (Fig. 1). Color Doppler studies demonstrated bidirectional flow in both the true and false lumens. Color Doppler analysis of the descendingaorta (Fig. 1) in this region showedmore than one exit site. The patient underwent aortic valve replacement with a Medtronic Hall aortic valved conduit (the Bentall procedure) (Medtronic Inc., Minneapolis, Minn.). The patient madea good recovery and on the first postoperative day underwent another transesophagealexamination. aortic

he had undergone an aortic valve commissurotomy for congenital aortic stenosis. Physical examination revealed a healthy white man with a blood pressure in the right arm

of 130/78mm Hg and a pressurein the left arm of 120/80 mm Hg. Precordial examination revealed normal heart sounds,a 216systolic ejection murmur at the right sternal border, and a 214 early diastolic murmur at the cardiac apex. Pulses were equal and there were no focal neurologic abnormalities. His laboratory examination was normal. A chest x-ray film showed cardiomegaly with a dilated ascending aorta. An electrocardiogram recorded for left ven-

tricular hypertrophy. A transthoracic color flow Doppler echocardiogramrevealed a massively dilated aortic root, severe aortic sion.

regurgitation,

and a small pericardial

valve prosthesis

and

(Fig.

2). In both

the transverse

and the

section in young men may alsobe secondaryto trauma, or

A 2%year-old white man presented with a 7-day history of intermittent retrosternal chest pain. Eight years previously

criteria

lumen

associated

J. V. Nixon, MD. Richmond, Va.

and voltage

a stable aortic

tient relates to his history of a congenitally biscuspid aortic valve despite his having a commissurotomy,due to the accompanying abnormal blood flow velocities. Aortic dis-

Thomas Porter, MD, Michael Lenhart, MD, James Arrowood, MD, William Moskowitz, MD, Walter Paulsen,MD, Gary Lofland, MD, and

sinus rhythm

revealed

descendingaorta, a persistent false lumen with systolic flow was recorded. The probable etiology of the aortic dissectionin this pa-

Dissecting aortic aneurysm eight years after aortic commissurotomy for congenital aortic stenosis: Detailed identification by transesophageal echocardiography

normal

This

conduit surrounded by thrombus in the previous native

effu-

with Marfan’s

syndrome

or Ehlers-Danlos

syn-

drome. Although aortic dissectionhas been describedfollowing aortic cross clamping or aortic cannulation, it is usually seenin the early postoperative period. Proximal aortic dissections have been described late after aortic valve replacement,i with the most likely reasonbeing abnormal turbulent motion in the ascendingaorta. However, aortic dissection late after aortic valvotomy has not been described. Transesophagealechocardiography is a sensitive and specific diagnostic technique for the diagnosisof aortic dissection.2In addition, it has been useful in the serial evaluation of dissectionrepair, which hasbeencomplicated by a small incidence of aneurysmsat the site of anastomosis, and aneurysms occurring distal to the previous anastomosis.4The transthoracic Doppler echocardiogram complemented the examination by documenting the severity of aortic regurgitation and identifying the upper ascending aorta, which was not easily identified by transesophageal examination. In conclusion, transesophageal echocardiography wasthe definitive diagnostic procedure in this unique caseof type I aortic dissection.Transesophageal echocardiography

has become the procedure

of choice

for the diagnosisof aortic dissectionand the serial followup of its repair.

REFERENCES

From the Division ogy at the Medical Reprint requests: of Virginia, MCV 4/4/22066

of Cardiology, Cardiac College of Virginia.

J. V.

Nixon, Station-Box

Surgery,

and

MD, Division of Cardiology, 128, Richmond, VA 23298.

Pediatric Medical

CardiolCollege

1. Muna WF, Spray TL, Morrow AG. Aortic dissection after aortic valve replacement in patients with valvular aortic stenosis. J Thorac Cardiovasc Surg 1977;74:65. 2. Erbel R, Engberding R, Daniel W, Rowlandt J, Visser C, Rennollet H, and the European Cooperative Study Group for

Large atrial septal aneurysm mimicking a cystic right atrial mass.

7I4 Brief Communications American regurgitation severeright ventricular overloading and cardiac decompensationensue.The increasedright ventricular...
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