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2 years, no expansion of the aneurysm hasbeen noted, and there has been no apparent increasein the volume of the shunt. In consideration of this benign course to date, we plan to perform serial two-dimensional echocardiographic and color Doppler studies, proceedingwith surgery only if the aneurysmenlargessignificantly or signsof left ventricular volume overload appear. The future clinical courseof this patient and the identification and serial noninvasive assessmentof additional similar cases will determine whether conservative managementis an acceptableoption in selectedindividuals with ruptured sinusof Valsalva aneurysms.
227
8. Kerber RE, Ridges JD, Kriss JP, et al. Unruptured aneurysm of the sinus of Valsalva producing right ventricular outflow obstruction. Am J Med 1972;53:775-83.
Constrictive extracardiac
pericarditis tumor
masquerading
as
Robin S. Freedberg, MD: Ira C. Schulman, MD, David Naidich, MD,b Jeffrey Weinreb, MD,b Alfred Culliford, MD,C and Itzhak Kronzon, MD.a New York, N.Y.
REFERENCES
Boutefeu JW, Moret PR, Hahn C, Hauf E. Aneurysms of the sinus of Valsalva. Report of seven cases and review of the literature. Am J Med 1978;65:18-24. 2. Fishbein MC, Obma R, Roberts WC. Unruptured sinus of Valsalva aneurysms. Am J Cardiol 1975;35:918-22. 3. DeMaria AN, Bommer W, Neumann A, Weinert L, Bogren H, Mason DT. Identification and localization of aneurysms of the ascending aorta by cross-sectional echocardiography. Circulation 19’79;59:755-61. 4. Lewis BS, Agathangelow NE. Echocardiographic diagnosis of unruptured sinus of Valsalva aneurysms. AM HEARTJ 1984; 1.
107:1025-7. 5. Shumacker, H. Aneurysms of the sinuses of Valsalva secondary to bacterial endocarditis with special reference to their operative management. J Thorac Cardiovasc Surg 1972;63:896902.
6. Haraoka S, Ueda M, Saito D, Ogino Y, Yoshida H, Kasuhara S. Echocardiographic findings of a case of sinus of Valsalva aneurysm ruptured into the left ventricle. J Cardioar 1978: 8:293--6. 7. Howard RJ, Moller J, Casteneda AR, et al. Surgical correction of sinus of Valsalva aneurysm. J Thorac Cardiovasc Surg 1973;66:420-7.
Echocardiography and magnetic resonance(MR) imaging are valuable tools in the evaluation of paracardiac masses. These noninvasive modalities have also been used in the diagnosisof pericardial constriction resulting from granulomatousdiseaseor in associationwith effusive pericarditis. We report
a patient
with a myeloproliferative
disorder
and clinical evidence of pericardial constriction, whose precordial and transesophagealechocardiogramsand thoracic MR studies demonstrated a mediastinal tumor-like masscompressingthe heart. However, at surgery, mediastinal fibrosis without
a neoplasm
or an infectious
mass was
found. A 62-year-old man with myeloid metaplasia, who had From the Departments of Medicine? Radiology,b and Surgery: University Medical Center. Reprint requests: Itzhak Kronzon, MD, 560 First Ave.-Suite York, NY 10016. 414126463
Fig. 1. Two-dimensional echocardiography, apical four-chamber view. Large echogenic masses (M) compressing the left and the right atrioventricubu junction. LA, Left atrium; LV, left ventricle; curued arrow points to the compressed, slit-like right ventricular cavity.
New York ZE, New
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American
July 1990 Heart Journal
Fig. 2. Magnetic resonanceimaging before (left) and after (right) administration of Gd-DTPA. Note the large masses(M) compressingthe atrioventricular groove, with virtual obliteration of the right ventricular cavity (arrow). There is no enhancementof the massby Cd-DTPA. D, Descendingaorta; other abbreviations as in legend to Fig. 1.
received radiation therapy for suspectedmyeloid infiltration of the pericardium, developed exercise intolerance, peripheral edema, and ascites.The physical examination revealed a blood pressureof lOO/SOmm Hg with a 15 mm paradoxical pulse. There wasjugular venous distention in the upright position. The lungs were clear to percussion and auscultation. Cardiac examination revealed a soft systolic murmur at the lower sternal border. Massive ascites with a tender enlarged liver and marked lower extremity edemawere present. Two-dimensional echocardiography (Fig. 1) revealed a mildly dilated left atrium, paradoxic septal motion, a tiny pericardial effusion, and a large homogenousextracardiac masscompressingthe right atrioventricular canal and right ventricle, and extending around the obtuse margin of the heart to the left atrioventricular sulcus. The inferior vena cava and hepatic veins appeareddilated. The valves weremorphologically normal, but high-velocity diastolic flow and a prolonged pressure half-time in the tricuspid orifice suggestedtricuspid stenosiscausedby compressionof the tricuspid anulus. Transesophagealechocardiography demonstrated an echogenic massencasingthe entire heart. The bulk of the masswas anterior to the heart, compressingthe right atrioventricular canal and right ventricle. The free wall of the right atrium did not contract during atria1systole.The masswas also prominent in the region of the left atrioventricular groove, and extended posteriorly, encasingthe descending thoracic aorta. High-velocity tricuspid flow was again noted, but the appearanceof the valve leaflets wasnormal. MR imaging of the heart (Fig. 2) revealed a large soft tissuemass,which appeared to arise from the pericardium, with extrinsic compression of the right ventricle and extensionaround the atrioventricular groove. Inferior vena caval dilatation wasnoted. The study wasconsideredmost suggestiveof a primary neoplasm.However, becausethe masswas not enhancedby intravenous gadolinium (Gd) DPTA (diethylenetriamine penta-acetic acid), suspicion that the lesionrepresenteda mass-likefibrotic process,resulting from previous radiation, wasraised. Left ventricu-
lar angiographyrevealeda noncontracting inferobasalwall, and right atrial angiography demonstrated external compressionand upward displacement of the right ventricle about the atrioventricular junction. Intracardiac pressures were consistent with pericardial constriction (right atrial, 18 mm Hg; right ventricular, 32/18 mm Hg; pulmonary arterial, 32/18-20mm Hg; pulmonary capillary wedge,18mm Hg; left ventricular, 100/20mm Hg; aortic, 100/50mm Hg). At surgery, a markedly thickened immobile pericardium wasnoted. In addition, approximately 200 cm3of caseouslike material filled the pericardial spacein the region of the right atrium and right ventricle, and appearedto compress the right heart chambers.After an extensive decortication of the abnormal pericardium, marked hemodynamic improvement-manifested by adramatic fall in centralvenous pressureand a rise in cardiac output-was noted. The ascending aorta, which appearedto be encasedbut not compressedby thickened inflammatory material, was left undisturbed. Pathologic examination of the excisedtissuerevealed acute fibrinous and chronic inflammation, fibrosis, and telangiectasia, consistent with radiation-induced and fibrinous pericarditis. No neoplastic cells were seen,and cultures for pyogenic bacteria, mycobacteria, and fungi were negative. Cardiac neoplastic masses,le3 abscesses,4F5 and infiltrative processes61 7 have been well characterized by echocardiography. Enhanced imaging of somecardiac structures (notably the valves, the left atrium and its appendage,and the interatrial septum) as well as the descendingthoracic aorta, has been facilitated by the transesophagealapproach.8This technique has been useful in characterizing the extent of myocardial or intracavitary involvement by paracardiac tumors9 or other infiltrative processes.lO, l1 MR imaginghasenabledclinicians to assess the full extent of paracardiac masses-including intracardiac,i4 intravascular,15and pericardiali’j involvement by neoplasm. The technique has been shown to contribute significant diagnostic information in most cases of paracardiac tumors.i7*ls Tissuecharacterization by MR, including lipo-
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matous infiltration of myocardial structures1g or myocardial abscesses, has been promising. Gd has been used to distinguish neoplasms from nonenhancing infiltrative processes.20 In our patient, the homogenous echogenicity of the extracardiac mass, imaged by both precordial and transesophageal approaches, was considered most characteristic of neoplasm. Its origin in the pericardium was suggested, but not definitively identified, by echocardiography. While non-contrast-enhanced MR imaging demonstrated the pericardial origin of the mediastinal mass, Gd enhancement provided the tissue characterization data that suggested that the mass might be composed of fibrotic material. The lack of involvement of the bronchial bed as assessed by thoracic MR imaging, made fibrosing mediastinitis less likely, and fibrosis in association with the patient’s previous radiation therapy the more probable diagnosis. MR imaging with nonionic contrast enhancement is a valuable technique for assessing unusual paracardiac masses. This noninvasive modality should be used in the evaluation of any bulky intra-or extracardiac mass for which the differential diagnosis includes non-neoplastic pathology. REFERENCES
.
1. Schattenberg TT. Echocardiography diagnosis of left atria1 myxoma. Mayo Clin Proc 1968;43:620-7. 2. Panidis IP, Morganroth J. The role of M-mode and twodimensional echocardiography in the detection of cardiac masses. In: Pohost GM, Higgins CB, Morganroth J, Ritchie JL, Schelbert HR, eds. New concepts in cardiac imaging 1987. Boston: G.K. Hall Medical Publishers, 1987:61. 3. Fyke FE, Seward JB, Edwards WD, Miller FA Jr, Reeder GS, Schattenberg TT, Shub C, Callahan JA, Tajik J. Primary cardiac tumors: experience with 30 consecutive patients since the introduction of two-dimensional echocardiography. J Am Co11 Cardiol 1985;5:1465-73. 4. Scanlon JG, Seward JB, Tajik AJ. Valve ring abscess in infective endocarditis: visualization with wide-angle two-dimensional echocardiography. Am J Cardiol 1982;49:1794-800. 5. Ellis SG, Goldstein J, Popp RH. Detection of endocarditis associated perivalvular abscesses by two-dimensional echocardiography. J Am Co11 Cardiol 1985;5:647-53. 6. Siqueria-Filho AG, Cunha LP, Tajik AJ, Seward JB, Schattenberg TT, Giuliani ER. M-mode and two-dimensional echocardiographic features in cardiac amyloidosis. Circulation 1981;63:188-96. 7. Klein AL, Oh, JK, Miller FA, Seward JB, Tajik AJ. Two-dimensional and Doppler echocardiographic assessment of infiltrative cardiomyopathy. J Am Sot Echo 1989;1:48-59. 8. Seward JB, Khandheria BY, Oh JK, Abel MD, Hughed RW Jr, Edwards WD, Nichols BA, Freeman WK, Tajik AJ. Transesophageal echocardiography: technique, anatomic correlations, implementation, and clinical applications. Mayo Clin Proc 1988;63:649-80. 9. Engberding R, Schulze-Waltrup N, Grosse-Heitmeyer W, Stoll V. Transthoracic and transesophageal 2-D echocardiography in the diagnosis of peri- and paracardial tumors. Dtsch Med Wochenschr 1987;112:49-52. 10. Kindman LA, Wright A, Tye T, Seale W, Appleton C. Lipomatous hypertrophy of the interatrial septum: characterization by transesophageal and transthoracic echocardiography, magnetic resonance imaging, and computed tomography. J Am Sot Echo 1989;1:450-4. 11. Polak PE Gussenhoven WJ, Roelandt JR. Transesophageal cross-sectional echocardiographic recognition of an aortic
Brief Communicatiorzs 229 valve ring abscess and a subannular mycotic aneurysm. Eur Heart J 1987;8:664-6. 12. Amparo EG, Higgins CB, Farmer D, Gamsu G, McNamara MJ, Gated MRI of cardiac and paracardiac masses: initial experiment. AJR 1984;143:1151-6. 13. Gamsu G, Higgins CB. Magnetic resonance imaging of mediastinal masses. Radiology 1986;158:289. 14. Gindea AJ, Steele P, R&nancik WM, Culubret M, Feiner H, Sanger JJ. Biventricular cavity obliteration bv metastatic malignant melanoma: role of magnetic resonanck imaging in the diaznosis. AM HEART J 1987;114:124Q-52 15. GindeaAJ, Gentin B, Naidich DP, Freedberg RS, McCauley D, Kronzon I. Unusual cardiac metastasis in hypernephroma: the complementary role of echocardiography and magnetic resonance imaging. AM HEART J 1988;116:1359-61. 16. Sechtem U, Tsholakoff D, Higgins CB. Pericardial disease. Diagnosis by MRI. AJR 1986;147:245-52. 17. Go RT, O’Donnell JR, Underwood DA, Feiglin DH, Salcedo EE, Pantoja M, MacIntyre WJ, Meaney TF. Comparison of gated cardiac MRI and two-dimensional echocardiography of intracardiac neoplasms. AJR 1985;145:21. 18. Freedberg RS, Kronzon I, Rumancik WM, Liebeskind D. The contribution of magnetic resonance imaging to the evaluation of intracardiac tumors diagnosed by echocardioeranhv. Circulation 1988;77:96-103. 19. Levine RA, Weyman AE, Dinsmore RE, Southern J, Rosen BR, Guyer DE, Brady TJ, Okada RD. Non-invasive tissue characterization: diagnosis of lipomatous hypertrophy of the atrial septum by nuclear magnetic resonance imaging. J Am Co11 Cardial 1986;7:688-92. 20. Barry I, Brant-Zawadski M, Osaki L, Brasch R, Murovic J, Newtown TH. Gd-DPTA in clinical MR of the brain. 2. Extraaxial lesions and normal structures. AJNR 1986;7:78993.
Pulmonary edema with diltiazem in hypertrophic obstructive cardiomyopathy Deepak Natarajan, MBBS, MD, DM, SubhashC. Sharma, MBBS, MD, and Ved P. Sharma, MBBS, MD. New Delhi, India
Limited hemodynamic data are available on diltiazem,* a benzothiazepin derivative, in patients with hypertrophic obstructive cardiomyopathy. This report. describesthe development of pulmonary edemain two patients in a study that examinesthe hemodynamiceffects of oral diltiazem in 10 patients with hypertrophic obstructive cardiomyopathy. Ten patients (9 men and 1 woman); ages16 to 54 years (34 f 14 years) with clinical, echocardiographic, and hemodynamic
findings
that are typical
of hypertrophic
ob-
structive cardiomyopathy were studied. All patients had normal sinusrhythm but were functionally limited at the start of study. They were gradedaccordingto the New York Heart, Association functional classification. The presence From the Department of Cardiology, Dr. Ram Manohar Lohia Hospital, New Delhi, India. Reprint requests: Deepak Natarajan, MBBS, Cardiology, Dr. Ram Manohar Lohia Hospital, 4/4/20464
MD, DM, Department New Delhi, India.
of