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2. Apical four-chamber view during diastole (A) and systole (B) showing attachment of mobile tumor to lateral left atria1 wall furrow). RA, right atrium. Abbreviations as in Fig. 1.
Fig.
tency was removed. The mass was attached to the high lateral left atrial wall between the base of the left atria1 appendage and the entrance to the left superior pulmonary vein. Histologic evaluation confirmed the diagnosis of LAM, and the patient had an uneventful recovery. It is well established that 2DE is the technique of choice in the diagnosis of LAM, thus eliminating the need for invasive investigations.3-5 This report emphasizes the reliability of this technique in providing sufficient preoperative evidence of an uncommon attachment of this type of tumor. It is clear that by far the most common site of attachment of LAM is in the interatrial septum. In a large series’ myxomas arising from sites in the atria other than the septum comprised only 10% of all atria1 myxomas; of these the posterior atria1 wall was the most common site of attachment followed by the anterior atria1 wall and the atria1 appendage. Fyke et aL5 reported one case of LAM arising from the high lateral wall, but its area of attachment was more posterior than in our patient, and no echocardiographic evidence was reported. Certainly LAM seen with this uncommon attachment should raise the suspicion of pathologic masses other than myxoma. Basically a differential diagnosis should include the possibility of metastatic intracavitary cardiac tumors48 6,7 or a pedunculated left atria1 thrombus. However, the absence of both cardiac and extracardiac signs of tumor dissemination favors the diagnosis of LAM. In addition, it is rare for metastatic cardiac tumors to be located in the left atrium. Similarly in our patient the mottled appearance of the mass and the lack of echocardiographic findings indicative of underlying cardiac disease were of value in differentiating between left atria1 thrombus and myxoma. Finally, accurate spatial orientation of 2DE easily permits the exclusion of large mitral vegetations resulting from fungal and other types of microorganisms.g We emphasize that despite an unusual location of a LAM, echocardiographic findings in the
appropriate clinical setting can be sufficient to establish a correct diagnosis of this type of tumor. REFERENCES
1. McAllister Jr HA. Primary tumors of the heart and pericardium. Path01Annu 1979;17:325-55. 2. Bulklev BH. Hutchins GM. Atria1 mvxomas: a fiftv-vear review: AM HEART J 1979;97:639-43. ” 3. Lappe DL, Bulkley BH, WeissJL. Two-dimensional echocardiographic diagnosis of the atria1 myxoma. Chest 1978;74: <
”
55-a.
DePaceNL, Soulen RL, Kotler MN, Mintz GS.Two-dimensional echocardiographicdetection of intraatrial masses.Am J Cardiol 1981;48:954-60. 5. Fyke III FE, Seward JB, Edwards WD, Fletcher Jr AM, Reeder GS,SchattenbergTT, Shub C, Callahan JA, Tajik AJ. Primary cardiac tumors: experiencewith 30 consecutivepatients since the introduction of two-dimensional echocardiography. J Am Co11Cardiol 1985;5:1465-73. 6. Mich RJ, Gillam LD, Weymann AE. Osteogenicsarcomas mimicking left atrial myxomas:clinical and two-dimensional echocardioaranhicfeatures. J Am Co11Cardiol 1985:6:1422-7. 7. Lang RM, Kenneth MB, Neumann A. Metastatic carcinoma involving the left atrium. AM &&ART J 1985;110:884-6. 8. PasternabRC, Cannom DS, Cohen LS. Echocardiographicdiagnosisof large fungal verruca attached to mitral valve. Br Heart J 1976;38:1209-12. 9. Wilson WR, Guliani ER, Danielson GK, Geraci JE. Management of complications of infective endocarditis. Mayo Clin Proc 1982;57:162-70. 4.
Left ventricuhr recurrence of a resected left atria1 myxoma Gregory S. Pavlides, MD, Robert N. Levin, MD, and Andrew M. Hauser, MD. Royal Oak, Mich. From
the Division
of Cardiology,
William
Beaumont
Reprint requests: G. S. Pavlides, MD, Division Beaumont Hospital, 3601 W. 13 Mile Rd., Royal
Hospital.
of Cardiology, William Oak, MI 48072.
Volume 117 Number 6
Intracardiac myxomas are the most common primary tumors of the heart, constituting up to 50% of all primary cardiac tumors. Histologically benign in appearance, myxomas usually present with obstructive, embolic, or constitutional symptoms. Seventy-five percent of myxomas arise in the left atrium, 25% in the right atrium, and 5% arise in the right or left ventricles. Diagnosis is usually established by echocardiographic examination. Since the first resection of left atria1 myxoma in 1954 by Crafoord,’ the definitive treatment has been surgical excision. The recurrence rate of excised myxomas is between 5% and 14 % , with most recurrences at the original tumor site.2 Only a few cases have been reported of remote recurrence of excised left atria1 myxomas in the right atrium, and very rarely in the right ventricle.3 To date, remote tumor recurrence within the left ventricle has not been reported. The interval to recurrence varies from 1 to more than 10 years, averaging 2 years following resection.2a3We report a case of left ventricular recurrence of an excised left atria1 myxoma occurring 10 months following excision. A 22-year-old, autistic, mentally retarded, white man presented with lethargy and right hemiparesis of sudden onset associated with myoclonic movements of the right arm and leg. On examination, right hemiparesis and an early diastolic extra sound followed by a diastolic grade II/VI murmur at the apex were noted. Hematologic and biochemical profiles were normal except for an elevated sedimentation rate and mild hyperbetaglobulinemia. The electrocardiogram and chest roentgenogram showed no abnormality. A two-dimensional echocardiogram revealed a large left atria1 mass attached to the interatrial septum (Fig. 1). The mass had the typical appearance of a myxoma, and virtually occluded the mitral orifice in diastole. No other echocardiographic abnormalities or other masses were evident. A computerized tomographic scan of the head was normal on admission, but repeat examination 4 days later revealed a left cerebral infarct. At operation, the left atrium was opened and a 12 cm tumor attached by a stalk to the interatrial septum was locally resected and the endocardium at the attachment point was cauterized. Inspection of the left atrium and left ventricle revealed no additional tumor. Postoperatively the patient had an uneventful course. Histologically, the tumor had the typical appearance of a myxoma composed of gelatinous-appearing material with few cells and no mitotic activity. A postoperative echocardiogram revealed no residual tumor. The patient remained symptom-free with mild residual right-sided weakness over the subsequent 9 months. A follow-up echocardiogram at 9 months revealed a 4 cm mobile mass within the left ventricle attached to the lateral-apical wall (Fig. 2). Ventricular wall motion was normal. Physical examination and routine laboratory studies were normal. At reoperation, left ventriculotomy was performed and a myxomatousappearing mass attached at the lateral ventricular wall was excised. On inspection of the left atrium, two small recurrent tumors were present at the site of the previous resection. The atria1 septum was excised and recon-
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Fig. 1. Apical four-chamber diastolic view. A large left atria1 tumor attached to the interatrial septum and observed to prolapse into the left ventricle through the mitral valve is visualized. LV, Left ventricle; RV, right ventricle; LA, left atrium; RA, right atrium.
2. Apical four-chamber view. Follow-up echocardiogram shows a left ventricular tumor attached to the apical-lateral left ventricular wall. LV, Left ventricle; RV, right ventricle; LA, left atrium; RA, right atrium.
Fig.
strutted with a Dacron patch. The operation and subsequent recovery were uncomplicated. Histologically both ventricular and atria1 tumors were typical myxomas. The first antemortem diagnosis of left atrial myxoma was made by angiography in 1952 and the first M-mode echocardiogram diagnosis was done in 1959. Since the widespread use of two-dimensional echocardiography in the 1970s myxomas have been diagnosed antemortem with accuracy by this technique. Surgical therapy is generally considered curative, but since the first recurrence reported in 1967,’ the potential for myxomas to recur has been widely recognized. To date, approximately
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30 recurrences have been reported.5 Local or contralateral atria1 tumor recurrences are most common; however, a right ventricular recurrence site has also been described. Less common extracardiac recurrences have been reported in bone and in the central nervous system. Mechanisms of recurrence may include inadequate resection, embolic implantation from the original tumor, unrecognized multicentric origin, and growth of pretumor or reserve ce11s.3 To minimize the likelihood of local recurrence, some authors have recommended wide excision and patch repair of the atria1 septum.6 Intraoperative examination of all four cardiac chambers by a biatrial approach has also been recommended to detect possible additional tumor foci. In this reported case, simple excision of the atria1 myxoma and cauterization of the excision site were performed. Although left ventricular tumor involvement was not observed during intraoperative saline irrigation of the chambers and by preoperative echocardiography, it is possible that at the time of presentation a small undetected tumor focus was present. Left ventricular tumor recurrence following atria1 resection has not been previously reported but is consistent with the hypothesis of a multicentric tumor origin. REFERENCES
Crafoord CL. Discussionon the results of mitral commissurotomy. In: Lam CR, ed. International symposium on cardiovascularsurgery. Philadelphia: W.B. SaundersCo, 1955:20211. 2. Markel ML, Wailer BF, Armstrong WF. Cardiac myxoma. Medicine 1987;66:114-25. 3. Gray IR, Williams WG. Recurring cardiacmyxoma. Br Heart J 1985;53:645-9. 4. Gerbode F, Kerth WJ, Hill JD. Surgical management of tumors of the heart. Surgery 1967;61:94-101. 5. Dein RJ, Frist WI-I, Stinson EB, et al. Primary cardiac neoplasms.J Thorac CardiovascSurg 1987;93:502-11. 6. Marvasti MA, Obeid AI, Potts JL, Parker FB. Approach in the management of atrial myxoma with long term follow up. Ann Thorac Surg 1984;38:53-8. 1.
Doppler achocardiographic diagnosis of aortopulmonary fist& following aortic valve replacement for endocardltis Jayashiri R. Aragam, MD, Mark A. Keroack, MD, and Andrew J. Kemper, MD. West Roxbury and Boston,
Mass.
The first known case of aortopulmonary fistula that was diagnosed by Doppler and color-flow mapping is reported. A &-year-old man with aortic valve endocarditis required From the Cardiology and Infectious Disease Sections, Medicine, Brockton/West Roabury Veterans Administration ter, and Harvard Medical School. Reprint requests: Andrew J. Kemper, MD, Administration Medical Center, 1400 VFW 02132.
Department of Medical Cen-
Cardiology Section, Veterans Parkway, West Roxbury, MA
June 1989 Heart JourneJ
valve replacement; 1 week later, he developed fever and and a new continuous murmur. The diagnosis of aortopulmonary fistula was made by Doppler echocardiography and was later confirmed by right heart catheterization. The patient was successfully treated with antibiotics alone. A 45-year-old black man presented to the Brocktonl West Roxbury Veterans Administration Medical Center with a 2-week history of fevers, chills, and cough. Six months prior to admission, he was treated at another hospital for a viridans group streptococcus endocarditis with 6 weeks of intravenous penicillin. He felt well for the ensuing 4 months until the present illness. The patient denied rheumatic fever, drug abuse, or recent dental procedures. Physical findings included a temperature of 99.7” F, a heart rate of 140/min, and blood pressure of 150/20 mm Hg. There were digital and conjunctival petechiae, and auscultation revealed murmurs of aortic insufficiency and mitral regurgitation. Three sets of blood cultures grew Streptococcus mitis, a viridans group streptococcus. A two-dimensional echocardiogram with Doppler color flow imaging revealed a large aortic valve vegetation, a hyperdynamic, mildly dilated left ventricle, moderate mitral regurgitation, and severe aortic regurgitation. The patient was initially treated with vancomycin and gentamicin. The vancomycin was changed to penicillin G when culture results were available. On the sixth hospital day, he developed progressive congestive heart failure, necessitating aortic valve replacement with a Bjark-Shiley prosthesis 2 days later. Right-sided heart catheterization performed prior to surgery revealed no arteriovenous communication. Large friable aortic vegetations were noted at surgery, with invasion of the valve ring in the region of the right sinus of Valsalva. Gram’s stain of the valve fragments revealed gram-positive cocci, but cultures were sterile. The patient continued taking penicillin and gentamicin through an uneventful early postoperative period. On the fifth postoperative day he developed a fever to 39” C and a new continuous murmur was heard. Echocardiography showed a defect in the aortopulmonary septum above the level of the prosthesis. Color flow imaging showed aortopulmonary shunting (Fig. 1). The prosthesis demonstrated no systolic or diastolic rocking. Right-sided heart catheterization revealed a shunt ratio (Qp:Qs) of 1.4:1. The patient’s fever resolved the following day. Since he was asymptomatic and serial echocardiograms showed no increase in the defect or aortopulmonary jet, he was continued on antibiotics alone for the ensuing 6 weeks without surgery. Prior to discharge, follow-up right heart catheterization revealed no change in the shunt ratio. The patient returned to full employment and moderate exercise, but died suddenly at work 92 days after discharge. Autopsy was performed by the medical examiner, who was unaware of patient’s medical history. No note was made of a residual or healed aortopulmonary fistula. The aortic prosthesis was firmly seated without any sign of residual infection. Cause of death was ascribed to arrhythmia. Aortopulmonary (AP) fistula is a well-recognized con-