Prolapsing right atrial myxoma evaluated by transesophageal echocardiography

Volum* Number

122 3, Part

revealed a high-grade angiosarcoma. The patient died 2 months later. Pathologically two types of angiosarcoma have been described.4 In one type, the tumor is large and protrudes into the right atrial cavity; it can obstruct the orifice of the tricuspid valve or either vena cava. In the other type, there is extensive invasion of the tumor into the epicardium and pericardium overlying the right atrium. The tumor is smaller and sessile. At times the tumor may extend to encase the entire heart in a thick layer of tumor. The tumor may impair right ventricular filling by compressing the right heart chambers or by producing a hemorrhagic pericardial effusion with tamponade. The antemortem diagnosis of this cardiac tumor is difficult. In a review of 41 cases by Glancy et al.,l only seven patients had accurate antemortem diagnosis. Noninvasive cardiac imaging techniques, especially transthoracic echocardiography, have proven useful in the early detection of right atrial angiosarcoma.5s 6 A large intracavitary tumor protruding into the right atrium may be recognized by transthoracic echocardiography. However, when it primarily involves the pericardium, a small sessile tumor may not be detected, or it may be difficult to differentiate the tumor from fibrin strands and thrombus commonly visualized in benign inflammatory effusiona.’ Transesophageal echocardiography may overcome many limitations of transthoracic echocardiography in detecting pericardial angiosarcoma by providing high-quality images of the pericardial and epicardial surfaces. In our patient, transesophageal echocardiography was able to recognize tumor involving the right atrial and right ventricular walls,, easily differentiating it from a benign pericardial process, In any patient who presents with an unexplained hemorrhagic pericardial effusion, a perieardial angiosarcoma, although uncommon, must be suspected. Although the prognosis is poor, early detection may improve survival6 If transthoracic echocardiography is nondiagnostic, transesophageal echocardiography may allow early, preoperative detection. REFERENCES

1. Glancy DL, Morales JB, Roberts WC. Angiosarcoma of the heart. Am J Cardiol 196&21:413-g. 2. Lin TK, Stech JM, Bckert WG, Lin JJ, Farha SJ, Hagan CT.

Pericardialan&sarcomasimulatingpericardialeffusionby 3.

4. 5. 6.

7.

Brief

1

echocardiography. Chest 1978;73:881-3. Schiavone WA, Calafiore PA, Currie PJ, Lytle BW. Doppler echocardiograpbic demonstration of pulmonary venous flow velocity in three patients with constrictive pericarditis before and after pericardiectomy. Am J Cardiol 1989;63:145-7. Janigan DT, Husain A, Robinson NA. Cardiac angiosarcomas. A review and a case report. Cancer 1986;57:852-9. Sorlie D, Myhre ESP, Stalsberg H. Angiosarcoma of the heart. Unusual presentation and survival after treatment. Br Heart J 1984;51:94-7. Freedberg RS, Kronzon I, Rumancik WM, Liebeskind D. The contribution of magnetic resonance imaging to the evaluation of intracardiac tumors diagnosed by echocardiography. Circulation 1988;77:96-103. Cheesman MG, Webster J, Rowlands D. Pericardial obliteration by angiosarcoma. Br Heart J 1988;60:262-3.

Communications

875

Manuel Rey, MD, JoseT&on, MD, Humberto Compres,MD, RosaRabago, MD, Julian Fraile, MD,B and Pedro Rabago, MD. Madrid, Spain

Right atria1myxomasare rare, beingthree to four times less frequent than those occurring in the left atrium.‘? 2Recent reports indicate that transesophagealechocardiography (TEE) has been demonstrated to be superior to tranethoracic two-dimensional echocardiography (2DE) in the diagnosisand assessmentof atria1 myxamas.3*4 Herein we present the first TEE findings of a typical prolapsingright atrial myxoma and both transthoracic and transesophageal 2DE features are compared. A 46year-old woman was referred to our hospital becauseof a 2-month history of asthenia, and short self-limited episodesof palpitations. On examination the patient was obese.Blood pressurewas 130/85 mm Hg. A slightly prominent “a” wave waspresent in the neck without jugular venous distension. A 2/6 diastolic rumble increasing with inspiration and a protodiastolic extra sound were heard at the tricuspid area. Electrocardiographic (ECG) findings were unremarkable. The chest x-ray examination demonstrated mild enlargement of the heart with normal pulmonary vasculature. Transthoracic 2DE usingaToshiba SSH-16OA ultrasound system with a 2.5 MHz transducer (model PSF-25DT, Toshiba Corp., Tokyo, Japan) disclosed an echogenicand fairly mobile right atrial mass, which entered the right ventricle through the tricuspid orifice during diastole and returned to the right atrium during systole. This masshad a jelly-like appearanceand its maximal diastolic dimensionson apical four-chamber view were6.7 X 5.3 cm. However, due to a poor acoustic window, the images were not completely satisfactory and the attachment of the tumor could not be domonstrated. TEE was subsequently performed using a Hewlett-Packard 7702OCV ultrasound system with a 5 MHz transducer (model 21362A, Hewlett-Packard Co., Medical Products Group, Andover, Mass.), permitting a very superior definition of the tumor (Figs. 1 and 2); the maximal tumor dimensionson TEE were 11.2 X 6 cm. Additionally, a short and echogenictumor stalk attached to the interatrial septum was clearly visualized

(Fig. 2). Surgical

and pathologic

examinationsboth showeda 12.2 X 7 cm (80 gm) gelatinous and pedunculated right atrial myxoma, arising from the rim of the fossaovalis in the atria1 septum. Transthoracic

2DE has been considered

From the Departments of Cardiology ci6n Jim&vsz Diaz. Reprint JimBnez 414130434

and %ardiovasruiar

requests: Dr. M. Rey, Departamento Diaz. Avda. Reyes Cat6licos 2, 28040

as the procedure Surgery.

de Cardiologia, Madrid. Spain.

FundaFundacibn

876

Brief Communications

Fig. 1. Transesophagealfour-chamber view in systole (A) and diastole (B) showing a large right atria1 myxoma prolapsing through the tricuspid valve. The massis highly deformed from systoleto diastole (jelly-like appearance)and the absenceof additional myxomas is well demonstrated. The attachment of the tumor to the interatrial septum in the region of the fossaovalis is also imaged, although no clear stalk is visualized. LA, Left atrium; LV, left ventricle; RA, right atrium, RV, right ventricle.

of choice in the diagnosis and assessmentof atria1 myxomas.5v6 With the advent of TEE, someauthors have noted the advantages of this technique with respect to conventional 2DE in this fie1d.b4 However, to our knowledge, only a small nonprolapsing right atrial myxoma, which wasassociatedwith a left prolapsingatria1 myxoma, hasbeen described.4In our patient, becauseof a nonsatisfactory acoustic window, transthoracic 2DE imaging of the right atrial myxoma was poor and the attachment of the tumor to the interatrial septum by a short stalk was not clearly defined. By contrast, TEE was very superior to

Amwlcan

September 1991 Heart Journal

Fig. 2. Transesophagealexamination of right side of heart in early diastole (A), midsystole (B), and late systole (C). The right atria1 myxoma is visualized almost completely occupying the right atrium in systole (6 and C) and initially entering the right ventricle through the tricuspid valve in early diastole (A). In this view the echogenicand short stalk (arrows) is clearly seenand its attachment to the interatrial septum (arrowheads) is partially demonstrated (C). Abbreviations as in Fig. 1.

transthoracic 2DE in the demonstration of the tumor and the exact morphologic details, size, and prolapsing behavior, and the attachment to the interatrial septum by a pediclecould all be very preciselydemonstrated.Moreover,

Volume122 Number 3, Pmt

Brief Communications 877

1

TEE permitted us to exclude the presence of associated myxomas in other locations that could have remained undetected by transthoracic 2DE.’ Thus the additional advantages of TEE with respect to transthoracic 2DE described in left atria1 myxomas are confirmed in the first case of a prolapsing right atrial myxoma studied by TEE reported in the literature. REFERENCES 1.

2. 3. 4. 5. 6.

Imperio J, Summers D, Krasnow N, Piccone VA Jr. The distribution patterns of biatrial myxomas. Ann Thorac Surg 1979;29:469-73. Fallon JT, Dee GW. Cardiac tumors. In: Eagle KA, ed. The practice of cardiology. Boston: Little, Brown & Company, 1989:1013-29. Obeid A, Marvasti M, Parker F, Rosenberg J. Comparison of transthoracic and transeaophageal echocardiography in diagnosis of left atria1 m\rxoma. Am J Cardiol 1989:63:1006-S. Dittmann H, Voelke; W, Karsch K, Seipel L. &lateral atrial myxomas detected by transesophageal two-dimensional echocardiography. AM HEART J 1989;118:172-3. DePace NL, Soulen RL, Kotler MN, Mintz GS. Two-dimensional echocardiographic detection of intraatrial masses. Am J Cardiol 1981;48:954-60. Fyke FE III, Seward JB, Edwards WD, Fletcher AM Jr, Reeder GS, Schattenberg TT, Schub C, Callahan JA, Tajik AJ. Primary cardiac tumors: experience with 30 consecutive patients since the introduction of two-dimensional echocardiography. J Am Co11 Cardiol 1985;5:1465-73.

Cholesterol tampon&e Edward Richard

~icarditis

causing cardiac

J. Ford, MD, Philip A. Bear, DO, and W. Adams, MD. Des Moines, Iowa

A 73-year-old

man was referred

for evaluation

of refractory

congestiveheart failure. The patient had been well until 3 years previously, when he began to experience increasing shortnessof breath on exertion, which precipitously worsened 3 months prior to admission.He wastreated with diuretics

with

no improvement.

There

was no history

of

endocrine disease,arthritis, tuberculosis, or cancer. The patient had sustainedno recent chest trauma. Medications included furosemide,80 mg twice daily; potassium,20 r&q three times daily; and aspirin, one tablet every day. On admission,the patient wasorthopneic and in mild respiratory distressat rest. Blood pressurewas 120/88mm Hg with 12 mm of pulsus paradoxus. Neck veins were distended with prominent X descent and decreased Y descent. No Kussmaul’s sign was seen. Auscultation of the lungs revealed

decreasedbreath sounds, especially underneath the left From the Departments of Medicine and Pathology, Iowa Methodist Medical Center. Reprint requests: Dr. Philip Bear, Iowa Heart Center, 1215 Pleasant St., Suite 618, Des Moines, IA 50309. 414130600

scapula,with no ralea.The heart soundswere distant, and a two-component, pericardial friction rub wasnoted. The liver edgewaepalpable 3 cm below the right costal margin and wassmoothand nontender. The spleenwasnot palpable. There was l+ pretibial pitting edema.Electrocardiogram showed low voltage and nonspecific ST-T wave changes.Chest x-ray film showeda large cardiac silhouette and normal pulmonary vasculature with clear lung fields. Thyroid function tests were normal. Complete blood count (CBC) and screeningchemistry panel were within normal limits for age.Two-dimensional echocardiogramshoweda massivepericardial effusion that wascircumferential (Fig. 1). Diastolic collapseof the right atrium, left atrium, and right ventricle wasseen.Ultrasound-guided pericardiocentesiswas performed, with placement of a pericardial catheter and initial removal of 1600 cm3 of opaque greenish fluid; subsequentlya total of 2700cm3of Auid wasremoved over 72 hours. The cholesterol content in the pericardial effusion was 60 mg/dl, and plate-like cholesterol crystals were seenon microscopicexamination (Fig. 2). Computed tomography (CT) of the chestshowedno thoracic tumor or mediastinal adenopathy. Multiple cultures of the pericardial fluid, including bacterial, fungal, and mycobacterial, were negative. Despite repeated pericardiocentesis, fluid reaccumulated.A pericardial window wascreated through a left thoracotomy approach. A 10 X 10 cm window was openedanterior to the phrenic nerve, allowing drainage of exudate into the left pleural space.At surgery, the appearanceof the pericardium and epicardium surface wasthickenedand cloudy, with depositsof yellow-grey material embeddedwithin the epicardium and pericardium. A section of thickened pericardium was removed and was sent for pathologic study. Examination showedforeign body granulomatous inilammation surrounding cholesterol clefts. Follow-up echocardiogramat 3 months postdischargedemonstrated no reaccumulation of fluid. The first caseof cholesterol pericarditis is credited to Alexander,’ who described the “scintillating gold paint” appearanceof cholesterol crystals in a patient with myxedemawith a pericardial effusion.’ When present, pericardial effusionscontaining cholesterol are usually associated with systemic diseasesuch asrheumatoid arthritis, tuberculosis,or myxedema.2t3Eleven casesof idiopathic cholesterol pericarditis have been reported in the English literature since 1964.Cholesterol pericarditis in the euthyroid patient is thought to be initiated by recurrent attacks of acute pericarditis, subclinical or symptomatic, resulting in generation of an inflammatory effusion, pericardial thickening, and impairment of the absorptive capacity of the pericardium.3 The resultant increased concentration of cholesterol is thought to result in precipitation of cholesterol crystals that may becomeencasedin the pericardial membrane, resulting in further granulomatous foreign body reaction and regeneration of the cycle of inflammation and exudation of fluid. Acute episodesof pericarditis often go unrecognized by the clinician, especiallywhen they are associatedwith collagenvascular disorders.2Patients may present with resistant heart failure and may often reIate a history of refrac-