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Diagnosis of a false left ventricular aneurysm with magnetic resonance imaging
706
September 1990 American Heart Journal
Brief Communications
3. Endomyocardial biopsy. A, Two fragments of endomyocardium showing excess of lymphomononuclear cellsin interstitium (Hematoxylin & eosin;~44). B, Higher magnification showingepithelioid cell granuloma with Langhan’s original magnification giant cell (arrowi). Hematoxylin & eosin; original magnification X220.) Fig.
restriction to filling of the right ventricle. The diagnosiswas suspectedbased on endomyocardial biopsy findings and was confirmed only at open biopsy. Our patient initially had fever of undetermined origin with features of restrictive cardiomyopathy and varying supraventricular arrhythmias. Resultsof echocardiography showed nodular infiltration of the left atrium and right ventricle, and the diagnosiswasmade by endomyocardial biopsy. Although acid-fast bacillus could not be seen,our diagnosiswasbasedon the history of prolonged fever, calcified right mediastinal lymph node, suggestivehistopathology, and the dramatic response to antitubercular drugs.Tuberculosis of the myocardium, although rare, is a treatable causeof restrictive cardiomyopathy. The diagnosiscan be madein life by endomyocardial biopsy if clinical suspicionis strong and echocardiographicfindings are suggestive. Antitubercular drugsalone may be “curative” and in uncomplicated cases surgical intervention may not be necessary. REFERENCES
1. Anders JM. Tuberculosis of the myocardium. JAMA 1902; 39:1081-6. 2. Kapoor OP, Marcarenhas E, Rananaware MM, Gadgil RK. Tuberculoma of the heart. Report of 9 cases. AM HEART J 1973;86:334-40. 3. Rose AG. Cardiac tuberculosis. A study of 19 patients. Arch Path01 Lab Med 1987;111:422-6. 4. Horn H, Saphir 0. the involvement of the myocardium in tuberculosis. A review of the literature and report of three cases. Am Rev Respir Dis 1935;32:492-506. 5. Behr G, Palm HC, Temperley JM. Myocardial tuberculosis Br Med J 1977;951.
Kinare SG. Interesting facts of cardiovascular tuberculosis. Indian J Surg 1975;37:144-51. Rawls WJ, Shuford WH, Logan WD, Hurst JW, Schlant RC. Right ventricular outflow tract obstruction produced by a myocardial abscess in a patient with tuberculosis. Am J Cardiol 1968; 21:738-45. Halim MA, Mercer EN, Guinn GA. Myocardial tuberculoma with rupture and pseudoaneurysm formation: successful treatment. Br Heart J 1985;54:603-4. Soyer R, Brunet A, Chavalier B, Leroy J, Morere M, Redonnet M. Tuberculous aortic insufficiency. Report of a case with successful surgical treatment. J Thorac Cardiovasc Surg 1981;82:254-6. 10. Krishnaswami H, Cherian G. Right atria1 tuberculoma: report of a case with complete recovery. Thorax 1984;39: 550-l.
Diagnosis aneurysm imaging
of a false left ventricular with magnetic resonance
H. Pollak, MD, H. Mayr, MD, T. Binder, MD, H. Imhof, MD, T. Miihlbauer, MD, and D. Glogar, MD. Vienna,
Austria
From the Ludwig Boltzmann Institut fiir Herzinfarktforschung, the Department of Cardiology, and the MRI Institute, University Clinic Vienna, Austria, Medical School, and the Institute of Pathology, Krankenhaus der Stadt Wien-Lainz. Reprint requests: H. Pollak, forschung, Wolkersbergenstr. 414122051
Ludwig Boltzmann 1, Vienna, Austria
Institut A-1130.
fiir
Herzinfarkt-
Volume
120
Number
3
Brief Communications
Fig. 1. Sagittal Ti weighted MRI showingfalse intramural aneurysm (broken (solid arrow).
Irrespective of their size, pseudoaneurysmsof the heart have a high tendency to rupture.l We present a casethat was diagnosedby magnetic resonanceimaging (MRI). A 57-year-old woman was admitted to an intensive care unit becauseof shock with symptoms and enzyme changes compatible with acute pancreatitis. The ECG was inconclusive for myocardial infarction. Five weeks after this event the patient was seenin our outpatient department becauseof chestpain. Results of two-dimensionalechocardiography showedan echodensemass,approximately 1 cm in diameter, located in the posterolateral wall near the posterior mitral valve leaflet. Ten days later MRI wasperformed on a 1.5 T Magnetom revealing a cystic intramural massin the posterolateral wall, 3 cm in diameter, with thin myocardial layers (Fig. 1) and evidence of blood flow into the structure (signal void on Ti images).On one slide the inner myocardial layer appeared interrupted. In addition, there was a medium-sized pericardial efffusion in some areas showing signal behavior of recent thrombus. Tz weighted imagesalsoshowedhigh signal intensity posterolaterally, consistent with myocardial infarction not older than 6 weeks.Thus basedon results of MRI, a diagnosisof recent myocardial infarction with pseudoaneurysmof the posterolateral wall was made. While awaiting surgery the patient died of acute tamponade 5 days later (6% weeks after the acute event). At autopsy 200 gm of clotted blood wasseenwithin the pericardial sac.Posterolaterally, 2 cm distal to the valvular ring, a myocardial rupture, 8 mm in length, originating from an intramural pseudoaneurysm (3 X 2 cm in diameter), wasseenwith an ostium, 11 mm in length, to the cavity of the left ventricle (Fig. 2). The pseudoaneurysmwas partially filled with thrombotic material and waslocated within a myocardial infarction (8 X 6
arrow)
707
and endocardialtear
cm in size), which was estimated to be approximately 6 weeksold. The circumflex artery was occluded. The pancreasappeared to be normal. Two-dimensional echocardiography may be useful in detecting pseudoaneurysmsand distinguishing them from true aneurysms,2but this diagnosisis madein only 45% of cases3Especially pseudoaneurysmsof the posterolateral wall can easily be missed.3Pulsed Doppler4 and color flow imaging3 are extremely useful in verifying pseudoaneurysms, especially in differentiating them from pericardial cysts or circumscript pericardial effusions. Even with contrast ventriculography the diagnosishas reportedly been missedin asmany as50% of cases2MRI cancontribute not only high-resolution, detailed anatomic slicesof the heart in any possibleplane but adds information on tissue characterization (Ti, Tz, gadolinium-DTPA). With fast gradient sequencesit is alsopossibleto evaluate heart function and blood flow within the heart chambersand great vessels. In our patient MRI clearly showeda recent posterolateral myocardial infarction with consecutivepseudoaneurysmin great detail, a diagnosisthat wasmissedby other noninvasive methods (ECG, echocardiography). MRI indicated impending rupture by demonstrating recent thrombotic masseswithin the pericardial sac. Although echocardiography remains the method of choice in the diagnosisof complications of myocardial infarction, magnetic resonance imaging should be usedas an alternative method in selectedcases. REFERENCES
1. Vlodaver Z, Coe JI, Edwards JE. True and false left ventricular aneurysms. Propensity for the latter to rupture. Circulation 1975;51:567-72.
708
Brief Communications
Fig. 2. Autopsy finding of 3 X 2 cm pseudoaneurysm of posterolateral wall with small endocardial tear shown by probe.
2. Gatewood RP, Nanda NC. Differentiation of left ventricular pseudoaneurysm from true aneurysm with two dimensional echocardiography. Am J Cardiol 1980;46:869-78. 3. Sutherland GR, Smyllie JH, Roelandt JR. Advantages of colour flow imaging in the diagnosis of left ventricular pseudoaneurysm. Br Heart J 1989;61:59-64. 4. Loperfido F, Pennestri F, Mazzari M, Biasucci L, Vigna C, Laurenzi F, Manzoli U. Diagnosis of left ventricular pseudoaneurysm by pulsed Doppler echocardiography. AM HEART J 1985;110:1291-3.
Primary endocardial fibroelastosis associated with hydrops fetalis in a premature infant David J. Wolfson, MD, Samuel H. Pepkowitz, MD, Robert van de Velde, PhD, and Michael C. Fishbein, MD. Los Angeles, Calif. From the Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center. Reprint requests: Michael C. Fishbein, MD, Department of Pathology and Laboratory Medicine, Room 8732, Cedars-Sinai Medical Center, 8700 Beverly Blvd., Los Angeles, CA 90048. 4/4/22060
September 1990 American Heart Journal
Endocardial fihroelastosis (EFE) is a cardiac disorder of uncertain etiology characterized by a proliferation of both elastic and collagenous fibers within the endocardium. Both primary and secondary forms of EFE have been described’ (Table I). EFE typically affects the left ventricle, although the right ventricle and both atria can be affected as well. Classically, the left ventricular papillary muscles have been described as “high-riding”; that is, they are situated more superior on the ventricular wall than usual, with shortened and thickened chordae tendineae associated with a characteristic rolled, free edge of the mitral leaflets.’ Most descriptions of EFE have been in infants during the first year of life. Presentation at birth is rare. We herein review the literature and report a case of “primary” EFE associated with severe nonimmune hydrops fetalis that was present at 28 weeks’ gestation. This is a very rare example of autopsy-proven primary EFE associated with nonimmune hydrops fetalis occurring in a premature infant, and calls attention to this association. A 1130 gm female infant was delivered by cesarean section because of increasing maternal pre-eclampsia to a 20year-old, gravida 1, para 0 woman at 28 weeks’ estimated gestational age. Approximately 2 weeks prior to delivery, an ultrasound examination demonstrated severe fetal hydrops and poor growth. In the hospital’s neonatal intensive care unit a cardiac ultrasound examination demonstrated severe biventricular dysfunction, reduced aortic valve diameter, and retrograde aortic flow suggesting aortic stenosis or aortic atresia. The infant’s condition rapidly deteriorated and she died at 4 hours of age. Both mother’s and infant’s blood types were 0 positive. A cord blood direct antiglobulin test was negative. The immunoglobulin (IgM) level was negligible. Chemical and cytologic analysis of ascitic fluid were unremarkable. Blood and ascitic fluid bacterial, fungal, and viral cultures taken antemortem yielded no growth. The placenta was immature and edematous (790 gm) with no other abnormalities. At autopsy, the body was that of a premature, severely edematous female infant weighing 1160 gm. Internal examination revealed bilateral pleural effusion, pericardial effusion, ascities, and severe tissue edema. The heart weighed 8.9 gm (average approximately 9.5).2 The coronary arteries were unremarkable. Both atria were dilated. The right ventricular wall thickness was 2 to 3 mm (normal right ventricular wall thickness = 0.250 to 0.375 cm).3 The left ventricle was dilated with a wall thickness of 2 to 3 mm (normal left ventricular wall thickness = 0.225 to 0.350 cm.)3 The endocardium of both ventricles was thickened, more on the left (Fig. 1). The anterior leaflet of the mitral valve was slightly “rolled.” The chordae tendineae were short and thickened. The papillary muscles arose from the basal one third of the ventricular wall (“high-riding”) (Fig. 1). Aortic valvular stenosis or atresia were not present. All other valves and the great vessels were unremarkable. No myocardial disease was present. The spleen was not enlarged. Microscopic examination of the thickened endocardium revealed increased collagenous and large, elastic fibers oriented in well-defined layers (Fig. 2). Sections from all organs were without evidence of lipid accumulation. Electron micros-
September 1990 American Heart Journal
Brief Communications
3. Endomyocardial biopsy. A, Two fragments of endomyocardium showing excess of lymphomononuclear cellsin interstitium (Hematoxylin & eosin;~44). B, Higher magnification showingepithelioid cell granuloma with Langhan’s original magnification giant cell (arrowi). Hematoxylin & eosin; original magnification X220.) Fig.
restriction to filling of the right ventricle. The diagnosiswas suspectedbased on endomyocardial biopsy findings and was confirmed only at open biopsy. Our patient initially had fever of undetermined origin with features of restrictive cardiomyopathy and varying supraventricular arrhythmias. Resultsof echocardiography showed nodular infiltration of the left atrium and right ventricle, and the diagnosiswasmade by endomyocardial biopsy. Although acid-fast bacillus could not be seen,our diagnosiswasbasedon the history of prolonged fever, calcified right mediastinal lymph node, suggestivehistopathology, and the dramatic response to antitubercular drugs.Tuberculosis of the myocardium, although rare, is a treatable causeof restrictive cardiomyopathy. The diagnosiscan be madein life by endomyocardial biopsy if clinical suspicionis strong and echocardiographicfindings are suggestive. Antitubercular drugsalone may be “curative” and in uncomplicated cases surgical intervention may not be necessary. REFERENCES
1. Anders JM. Tuberculosis of the myocardium. JAMA 1902; 39:1081-6. 2. Kapoor OP, Marcarenhas E, Rananaware MM, Gadgil RK. Tuberculoma of the heart. Report of 9 cases. AM HEART J 1973;86:334-40. 3. Rose AG. Cardiac tuberculosis. A study of 19 patients. Arch Path01 Lab Med 1987;111:422-6. 4. Horn H, Saphir 0. the involvement of the myocardium in tuberculosis. A review of the literature and report of three cases. Am Rev Respir Dis 1935;32:492-506. 5. Behr G, Palm HC, Temperley JM. Myocardial tuberculosis Br Med J 1977;951.
Kinare SG. Interesting facts of cardiovascular tuberculosis. Indian J Surg 1975;37:144-51. Rawls WJ, Shuford WH, Logan WD, Hurst JW, Schlant RC. Right ventricular outflow tract obstruction produced by a myocardial abscess in a patient with tuberculosis. Am J Cardiol 1968; 21:738-45. Halim MA, Mercer EN, Guinn GA. Myocardial tuberculoma with rupture and pseudoaneurysm formation: successful treatment. Br Heart J 1985;54:603-4. Soyer R, Brunet A, Chavalier B, Leroy J, Morere M, Redonnet M. Tuberculous aortic insufficiency. Report of a case with successful surgical treatment. J Thorac Cardiovasc Surg 1981;82:254-6. 10. Krishnaswami H, Cherian G. Right atria1 tuberculoma: report of a case with complete recovery. Thorax 1984;39: 550-l.
Diagnosis aneurysm imaging
of a false left ventricular with magnetic resonance
H. Pollak, MD, H. Mayr, MD, T. Binder, MD, H. Imhof, MD, T. Miihlbauer, MD, and D. Glogar, MD. Vienna,
Austria
From the Ludwig Boltzmann Institut fiir Herzinfarktforschung, the Department of Cardiology, and the MRI Institute, University Clinic Vienna, Austria, Medical School, and the Institute of Pathology, Krankenhaus der Stadt Wien-Lainz. Reprint requests: H. Pollak, forschung, Wolkersbergenstr. 414122051
Ludwig Boltzmann 1, Vienna, Austria
Institut A-1130.
fiir
Herzinfarkt-
Volume
120
Number
3
Brief Communications
Fig. 1. Sagittal Ti weighted MRI showingfalse intramural aneurysm (broken (solid arrow).
Irrespective of their size, pseudoaneurysmsof the heart have a high tendency to rupture.l We present a casethat was diagnosedby magnetic resonanceimaging (MRI). A 57-year-old woman was admitted to an intensive care unit becauseof shock with symptoms and enzyme changes compatible with acute pancreatitis. The ECG was inconclusive for myocardial infarction. Five weeks after this event the patient was seenin our outpatient department becauseof chestpain. Results of two-dimensionalechocardiography showedan echodensemass,approximately 1 cm in diameter, located in the posterolateral wall near the posterior mitral valve leaflet. Ten days later MRI wasperformed on a 1.5 T Magnetom revealing a cystic intramural massin the posterolateral wall, 3 cm in diameter, with thin myocardial layers (Fig. 1) and evidence of blood flow into the structure (signal void on Ti images).On one slide the inner myocardial layer appeared interrupted. In addition, there was a medium-sized pericardial efffusion in some areas showing signal behavior of recent thrombus. Tz weighted imagesalsoshowedhigh signal intensity posterolaterally, consistent with myocardial infarction not older than 6 weeks.Thus basedon results of MRI, a diagnosisof recent myocardial infarction with pseudoaneurysmof the posterolateral wall was made. While awaiting surgery the patient died of acute tamponade 5 days later (6% weeks after the acute event). At autopsy 200 gm of clotted blood wasseenwithin the pericardial sac.Posterolaterally, 2 cm distal to the valvular ring, a myocardial rupture, 8 mm in length, originating from an intramural pseudoaneurysm (3 X 2 cm in diameter), wasseenwith an ostium, 11 mm in length, to the cavity of the left ventricle (Fig. 2). The pseudoaneurysmwas partially filled with thrombotic material and waslocated within a myocardial infarction (8 X 6
arrow)
707
and endocardialtear
cm in size), which was estimated to be approximately 6 weeksold. The circumflex artery was occluded. The pancreasappeared to be normal. Two-dimensional echocardiography may be useful in detecting pseudoaneurysmsand distinguishing them from true aneurysms,2but this diagnosisis madein only 45% of cases3Especially pseudoaneurysmsof the posterolateral wall can easily be missed.3Pulsed Doppler4 and color flow imaging3 are extremely useful in verifying pseudoaneurysms, especially in differentiating them from pericardial cysts or circumscript pericardial effusions. Even with contrast ventriculography the diagnosishas reportedly been missedin asmany as50% of cases2MRI cancontribute not only high-resolution, detailed anatomic slicesof the heart in any possibleplane but adds information on tissue characterization (Ti, Tz, gadolinium-DTPA). With fast gradient sequencesit is alsopossibleto evaluate heart function and blood flow within the heart chambersand great vessels. In our patient MRI clearly showeda recent posterolateral myocardial infarction with consecutivepseudoaneurysmin great detail, a diagnosisthat wasmissedby other noninvasive methods (ECG, echocardiography). MRI indicated impending rupture by demonstrating recent thrombotic masseswithin the pericardial sac. Although echocardiography remains the method of choice in the diagnosisof complications of myocardial infarction, magnetic resonance imaging should be usedas an alternative method in selectedcases. REFERENCES
1. Vlodaver Z, Coe JI, Edwards JE. True and false left ventricular aneurysms. Propensity for the latter to rupture. Circulation 1975;51:567-72.
708
Brief Communications
Fig. 2. Autopsy finding of 3 X 2 cm pseudoaneurysm of posterolateral wall with small endocardial tear shown by probe.
2. Gatewood RP, Nanda NC. Differentiation of left ventricular pseudoaneurysm from true aneurysm with two dimensional echocardiography. Am J Cardiol 1980;46:869-78. 3. Sutherland GR, Smyllie JH, Roelandt JR. Advantages of colour flow imaging in the diagnosis of left ventricular pseudoaneurysm. Br Heart J 1989;61:59-64. 4. Loperfido F, Pennestri F, Mazzari M, Biasucci L, Vigna C, Laurenzi F, Manzoli U. Diagnosis of left ventricular pseudoaneurysm by pulsed Doppler echocardiography. AM HEART J 1985;110:1291-3.
Primary endocardial fibroelastosis associated with hydrops fetalis in a premature infant David J. Wolfson, MD, Samuel H. Pepkowitz, MD, Robert van de Velde, PhD, and Michael C. Fishbein, MD. Los Angeles, Calif. From the Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center. Reprint requests: Michael C. Fishbein, MD, Department of Pathology and Laboratory Medicine, Room 8732, Cedars-Sinai Medical Center, 8700 Beverly Blvd., Los Angeles, CA 90048. 4/4/22060
September 1990 American Heart Journal
Endocardial fihroelastosis (EFE) is a cardiac disorder of uncertain etiology characterized by a proliferation of both elastic and collagenous fibers within the endocardium. Both primary and secondary forms of EFE have been described’ (Table I). EFE typically affects the left ventricle, although the right ventricle and both atria can be affected as well. Classically, the left ventricular papillary muscles have been described as “high-riding”; that is, they are situated more superior on the ventricular wall than usual, with shortened and thickened chordae tendineae associated with a characteristic rolled, free edge of the mitral leaflets.’ Most descriptions of EFE have been in infants during the first year of life. Presentation at birth is rare. We herein review the literature and report a case of “primary” EFE associated with severe nonimmune hydrops fetalis that was present at 28 weeks’ gestation. This is a very rare example of autopsy-proven primary EFE associated with nonimmune hydrops fetalis occurring in a premature infant, and calls attention to this association. A 1130 gm female infant was delivered by cesarean section because of increasing maternal pre-eclampsia to a 20year-old, gravida 1, para 0 woman at 28 weeks’ estimated gestational age. Approximately 2 weeks prior to delivery, an ultrasound examination demonstrated severe fetal hydrops and poor growth. In the hospital’s neonatal intensive care unit a cardiac ultrasound examination demonstrated severe biventricular dysfunction, reduced aortic valve diameter, and retrograde aortic flow suggesting aortic stenosis or aortic atresia. The infant’s condition rapidly deteriorated and she died at 4 hours of age. Both mother’s and infant’s blood types were 0 positive. A cord blood direct antiglobulin test was negative. The immunoglobulin (IgM) level was negligible. Chemical and cytologic analysis of ascitic fluid were unremarkable. Blood and ascitic fluid bacterial, fungal, and viral cultures taken antemortem yielded no growth. The placenta was immature and edematous (790 gm) with no other abnormalities. At autopsy, the body was that of a premature, severely edematous female infant weighing 1160 gm. Internal examination revealed bilateral pleural effusion, pericardial effusion, ascities, and severe tissue edema. The heart weighed 8.9 gm (average approximately 9.5).2 The coronary arteries were unremarkable. Both atria were dilated. The right ventricular wall thickness was 2 to 3 mm (normal right ventricular wall thickness = 0.250 to 0.375 cm).3 The left ventricle was dilated with a wall thickness of 2 to 3 mm (normal left ventricular wall thickness = 0.225 to 0.350 cm.)3 The endocardium of both ventricles was thickened, more on the left (Fig. 1). The anterior leaflet of the mitral valve was slightly “rolled.” The chordae tendineae were short and thickened. The papillary muscles arose from the basal one third of the ventricular wall (“high-riding”) (Fig. 1). Aortic valvular stenosis or atresia were not present. All other valves and the great vessels were unremarkable. No myocardial disease was present. The spleen was not enlarged. Microscopic examination of the thickened endocardium revealed increased collagenous and large, elastic fibers oriented in well-defined layers (Fig. 2). Sections from all organs were without evidence of lipid accumulation. Electron micros-