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Unusual presentation and echocardiographic features of surgically proven nonbacterial thrombotic endocarditis
February
404
Brief Communications
1990
American Heart Journal
M a n a g e m e n t of patients with M P T is still debatable. Heparin and vitamin K antagonists are probably the first choice of t h e r a p y in most centers, 2, 3 but open-heart surgery may be considered in selected cases. 6 Persistence of a protruding t h r o m b u s with considerable mobility, despite anticoagulant t r e a t m e n t , may be an indication of impending embolism. 3 In this setting, limited myocardial injury with good overall left ventricular function and a low surgical risk may favor the decision of early thrombectomy after healing of the AMI. T h e present report illustrates that clinical and echocardiographic resemblance between M P T and myxoma m a y make surgical intervention m a n d a t o r y in rare cases. REFERENCES
1. Visser CA, Kan G, Lie KI, Denver D. Left ventricular thrombus following acute myocardial infarction: a prospective serial echocardiographic study of 96 patients. Eur Heart J 1983;4: 333-7. 2. Egeblad H. Intracardiac thrombus--systemic arterial embolism. Contribution of echocardiography. Acta Med Scand 1988;(suppl. 730):1-112. 3. Johannesen K-A, Nordrehaug JE, von der Lippe G, Vollset SE. Risk factors for embolisation in patients with left ventricular thrombus and acute myocardial infarction. Br Heart J 1988;60:104-10: 4. Domenicucci R, Bellotti P, Chiarella F, Lupa G, Vecchio C. Spontaneous morphologic changes in left ventricular thrombi: a prospective two-dimensional echocardiographic study. Circulation 1987;75:737-43. 5. Lehrmann KL, Prozan GB, Ullyot D. Atrial myxoma presenting as acute myocardial infarction. AM HEART J 1985;110: 1293-5. 6. Lewin RF, Vidne B, Sclarowsky S, Agmon J. Two-dimensional real-time echocardiographic detection of left ventricular aneurysm associated with mobile pedunculated thrombi. Chest 1980;77:704-6.
Unusual presentation and echocardiographic features of surgically proven nonbacterial thrombotic endocardiUs
Fig. 1. Two-dimensi0nal echocardiograms in the fourchamber view in systole (A) and in diastole (B) demonstrating the large vegetation (v) arising from the tricuspid valve. LA, Left atrium; L V, left ventricle; RA, right atrium; RV, right ventricle.
M o h ' d A. Habbab, MD, M u a y e d A. Al-Zaibag, MD, Akram M. A1-Hilali, MD, and Mohamed R. A1-Fagih, MD. Riyadh, Saudi Arabia
Nonbacterial thrombotic endocarditis (NBTE) is often diagnosed only at p o s t m o r t e m examination. Few reports have described the a n t e m o r t e m two-dimensional echocardi0graphic features of this condition in autopsy-proven casesfl 2 We report an unusual presentation and describe t h e echocardiographic features of a surgically proven case of N B T E in a p a t i e n t with acute leukemia. From the Departments of Cardiology" and Hematology, Riyadh Armed Forces Hospital. RePrint requests: Moh!d A. Habbab, MD, SUNY Health Science Center at Brooklyn, Cardiology Division, 450 Clarkson Ave.--Box 1199, Brooklyn, NY 11203. 4/4/17530
Fig, 1C. Two-dimensional echocardiogram in the shortaxis View at the mitral valve level demonstrates the large vegetation arising:from the tricuspid valve. LV, Left ventricle; R V, right ventricle; V, vegetation.
Volume 119 Number 2, Part 1
Brief Communications 405
Fig. 2. A and B, Macroscopic appearance of two fragments of the large vegetation attached to the tricuspid valve (arrows) and the subvalvular apparatus (arrowheads). C, Microscopic appearance showing the vegetation (V) covering both surfaces of the tricuspid valve leaflet (L) with connective tissue proliferation and fibrosis (F) at the site of attachment. D, Microscopic appearance with higher magnification of the vegetation, which is composed primarily of blood components with the absence of inflammatory cells.
A 14-year-old patient presented with a 3-week history of fever, temperature of 40 ~ C, and shortness of breath on minimal exertion. He was well-developed but had a toxic appearance with a heart rate of 100 beats/min and a blood pressure of 120/80 mm Hg. Cervical lymph nodes were enlarged and there was 8 cm jugular venous distension at the 45-degree position. A 3/6 diastolic murmur was heard at the lower left sternal border that increased with inspiration. The liver was enlarged 3 cm below the costal margin, and the spleen was just palpable. The two-dimensional echocardiogram revealed a 4 • 2 • 2 cm ~ mass attached to the tricuspid valve (Fig. 1). Doppler studies showed 3+ tricuspid regurgitation. Initial laboratory data showed a hemoglobin of 11.1 gm/dl, platelet count of 14,000/mm 3, and white blood cell count of 98,000/mm 3 with 67 % blasts, 17 % neutrophils, and 8% lymphocytes. Bone marrow aspiration confirmed the diagnosis of acute lymphoblastic leukemia. Ventilation-perfusion (V/Q) scan of the lungs revealed multiple mismatched defects consistent with pulmonary emboli. Five blood cultures were negative for bacterial and fungal growth, and the cerebrospinal fluid was free of leukemic cells. The patient was started on intravenous netilmicin and cefotaxime as well as chemotherapy, which initially included vincristine, daunorubicin, asparaginase, and prednisolone, and later on, cytosine arabinoside and teniposide were added. Six weeks later, the following repeated transfusions with packed red blood cells (RBCs) and platelets, the hemoglobin was 9.6 gm/dl, platelet count was 104,000/mm 3, and white blood cell count was 5900/mm 3 with 89 % neutrophils and no blasts. At that time, tricuspid valve replacement
was performed. At operation, a fungating friable 4 • 2 • 2 cm3 mass was found attached to both sides of the tricuspid valve leaflets and extending to the subvalvular apparatus (Fig. 2, A and B). Microscopic examination confirmed the diagnosis of NBTE, as it showed a thrombus of fibrin, platelets, and constituents of blood adherent to the tricuspid valve, no organisms or inflammatory reaction, and fibrosis of the valve with neovascularization (Fig. 2, C and D). Postoperative recovery was uncomplicated. Chemotherapy guided by repeated bone marrow aspiration was continued 10 days following the surgery. One month later the patient was discharged on maintenance chemotherapy. The antemortem diagnosis of NBTE has gained importance, since NBTE may cause significant morbidity and mortality and a diagnosis will aid attempts to prevent its potential consequences. Echocardiography is a very useful tool that is able to assist the antemortem diagnosis of this condition. There are only three reports of the antemortem echocardiographic detection of pathologically proven NBTE: one by M-mode echocardiography in a patient in whom the diagnosis was confirmed at surgery3 and two by two-dimensional echocardiography in autopsy-proven cases.l, 2 Our case is unique in that it is the first report of two-dimensional echocardiographically determined features of NBTE with direct surgical validation in a living patient. Also, the unusually large valvular lesion (vegetation) that caused significant mechanical obstruction and regurgitation of the tricuspid valve, and the involvement of both surfaces of the valve as well as the subvalvular apparatus, make the clinical and pathologic presentation of this case different from those commonly reportedJ
406
--'--l~ne] Communications
February 1990 American Heart Journal
REFERENCES
1. Lopez JA, Fishbein MC, Siegel RJ. Echocardiographic features of non-bacterial thrombotic endocarditis. Am J Cardiol 1987;59:478-80. 2. Lim SP, Hakim SZ, SchoenhoffDD. Non-bacterial thrombotic endocarditis. Two-dimensional echocardiographic features in an autopsy proven case. Chest 1987;92:176-7. 3. Estevez CM, Corva BC. Serial echocardiographic abnormalities in non-bacterial thrombotic endocarditis of the mitral valve. Chest 1976;69:801-4. 4. Lopez JA, Ross RS, Fishbein MC, Siegel RJ. Non-bacterial thrombotic endocarditis: a review. AMHEARTJ 1987;113:77384.
Lipomatous hypertrophy of the interatrial septum: Diagnosis by percutaneous transvenous biopsy Gregg W. Stone, MD, Richard T. O'Kell, MD, Thomas H. Good, MD, and Geoffrey 0. Hartzler, MD. Kansas
City, Mo.
Lipomatous hypertrophy of the interatrial septum is a disorder characterized by excessive accumulation of adipose tissue that may result in marked thickening of the atrial septum. 1 While this condition has been associated with a variety of atrial arrhythmias and sudden death, 2 precise diagnosis may be essential to exclude other causes of septal lesions, including myxoma and infiltrating tumor. Unfortunately, given the relative inaccessibility of atrial tissue, definitive diagnosis is rarely made during life. 2 Though percutaneous transvenous bipsy of the right ventricular septum enjoys widespread use, s this technique has rarely been applied to obtain atrial septal tissue. We herein describe a patient with marked atrial thickening in whom lipomatous hypertrophy of the interatrial septum was diagnosed by percutaneous transvenous biopsy. The patient, a 63-year-old man with long-standing steroid-dependent asthma, presented in January 1989 with a 7-year history of multifocal atrial tachycardia refractory to digoxin, quinidine, procainamide, disopyramide, and verapamil. Mild to moderate pedal edema, exertional dyspnea, and chest discomfort had been present since 1985, all of which had recently progressed. Two-dimensional echocardiography demonstrated marked atrial septal thickening with increased echogenicity (Fig. 1). Right and left heart catheterization confirmed the clinical impression of pulmonary hypertension and predominant right ventricular failure. The left ventricle was moderately hypertrophied and the anterior wall was dyskinetic. The ejection fraction was 47 %. Severe triple-vessel coronary artery disease was From the Mid America Heart Institute, St. Luke's Hospital. Reprint requests: Dr. Geoffrey 0. Hartzler, Cardiovascular Consultants, Inc., Medical Plaza II-20, 4320 Wornall Road, Kansas City, M0 64111. 4/4/17533
Fig. 1. Apical four-chamber two-dimensional echocardiogram demonstrating marked thickening of the interatrial septum (arrows).
present. A 9F introducer sheath was placed in the right internal jugular vein, and three biopsies of the right ventricular endomyocardium were obtained with a Scholten bioptome (Scholten Surgical Instruments, Redwood City, Calif) using a standard technique. The bioptome was then withdrawn and a marked right angle curve was imparted to the distal 10 cm of the device. The bioptome was reinserted through the right internal jugular vein sheath, the anulus of the tricuspid valve identified through catheter manipulation, and the bioptome jaws were rotated posteriorly from the tricuspid anulus while being manipulated superiorly to avoid the ostium of the coronary sinus. With this approach, three specimens were obtained from adjacent but different sites of the interatrial septum. Two of the atrial specimens showed a grossly fatty appearance. Microscopic examination disclosed moderate hypertrophy of the ventricular and atrial myocytes. There was minimal interstitial fibrosis and no inflammation. Mature adipose tissue replaced portions of the atrium (Figs. 2 and 3). The fatty tissue was composed of mature fat cells interlaced with occasional blood vessels and wisps of collagen. There was no demonstrable capsule. These findings are consistent with lipomatous hypertrophy of the interatrial septum. 4 The patient's dyspnea improved following vigorous diuresis, inhaled bronchodilator agents, and increased steroid dosage. The multifocal atrial tachycardia was controlled, though not eliminated, with encainide and verapamil. Exertional angina was managed with nitrates and verapamil. Two months following discharge, the patient was less dyspneic, though he still noted palpitations. Lipomatous hypertrophy of the interatrial septum has been recognized as a distinct clinical entity since 1964, although to date the diagnosis is not usually established until autopsy. 2 While recent reports suggest a role for
404
Brief Communications
1990
American Heart Journal
M a n a g e m e n t of patients with M P T is still debatable. Heparin and vitamin K antagonists are probably the first choice of t h e r a p y in most centers, 2, 3 but open-heart surgery may be considered in selected cases. 6 Persistence of a protruding t h r o m b u s with considerable mobility, despite anticoagulant t r e a t m e n t , may be an indication of impending embolism. 3 In this setting, limited myocardial injury with good overall left ventricular function and a low surgical risk may favor the decision of early thrombectomy after healing of the AMI. T h e present report illustrates that clinical and echocardiographic resemblance between M P T and myxoma m a y make surgical intervention m a n d a t o r y in rare cases. REFERENCES
1. Visser CA, Kan G, Lie KI, Denver D. Left ventricular thrombus following acute myocardial infarction: a prospective serial echocardiographic study of 96 patients. Eur Heart J 1983;4: 333-7. 2. Egeblad H. Intracardiac thrombus--systemic arterial embolism. Contribution of echocardiography. Acta Med Scand 1988;(suppl. 730):1-112. 3. Johannesen K-A, Nordrehaug JE, von der Lippe G, Vollset SE. Risk factors for embolisation in patients with left ventricular thrombus and acute myocardial infarction. Br Heart J 1988;60:104-10: 4. Domenicucci R, Bellotti P, Chiarella F, Lupa G, Vecchio C. Spontaneous morphologic changes in left ventricular thrombi: a prospective two-dimensional echocardiographic study. Circulation 1987;75:737-43. 5. Lehrmann KL, Prozan GB, Ullyot D. Atrial myxoma presenting as acute myocardial infarction. AM HEART J 1985;110: 1293-5. 6. Lewin RF, Vidne B, Sclarowsky S, Agmon J. Two-dimensional real-time echocardiographic detection of left ventricular aneurysm associated with mobile pedunculated thrombi. Chest 1980;77:704-6.
Unusual presentation and echocardiographic features of surgically proven nonbacterial thrombotic endocardiUs
Fig. 1. Two-dimensi0nal echocardiograms in the fourchamber view in systole (A) and in diastole (B) demonstrating the large vegetation (v) arising from the tricuspid valve. LA, Left atrium; L V, left ventricle; RA, right atrium; RV, right ventricle.
M o h ' d A. Habbab, MD, M u a y e d A. Al-Zaibag, MD, Akram M. A1-Hilali, MD, and Mohamed R. A1-Fagih, MD. Riyadh, Saudi Arabia
Nonbacterial thrombotic endocarditis (NBTE) is often diagnosed only at p o s t m o r t e m examination. Few reports have described the a n t e m o r t e m two-dimensional echocardi0graphic features of this condition in autopsy-proven casesfl 2 We report an unusual presentation and describe t h e echocardiographic features of a surgically proven case of N B T E in a p a t i e n t with acute leukemia. From the Departments of Cardiology" and Hematology, Riyadh Armed Forces Hospital. RePrint requests: Moh!d A. Habbab, MD, SUNY Health Science Center at Brooklyn, Cardiology Division, 450 Clarkson Ave.--Box 1199, Brooklyn, NY 11203. 4/4/17530
Fig, 1C. Two-dimensional echocardiogram in the shortaxis View at the mitral valve level demonstrates the large vegetation arising:from the tricuspid valve. LV, Left ventricle; R V, right ventricle; V, vegetation.
Volume 119 Number 2, Part 1
Brief Communications 405
Fig. 2. A and B, Macroscopic appearance of two fragments of the large vegetation attached to the tricuspid valve (arrows) and the subvalvular apparatus (arrowheads). C, Microscopic appearance showing the vegetation (V) covering both surfaces of the tricuspid valve leaflet (L) with connective tissue proliferation and fibrosis (F) at the site of attachment. D, Microscopic appearance with higher magnification of the vegetation, which is composed primarily of blood components with the absence of inflammatory cells.
A 14-year-old patient presented with a 3-week history of fever, temperature of 40 ~ C, and shortness of breath on minimal exertion. He was well-developed but had a toxic appearance with a heart rate of 100 beats/min and a blood pressure of 120/80 mm Hg. Cervical lymph nodes were enlarged and there was 8 cm jugular venous distension at the 45-degree position. A 3/6 diastolic murmur was heard at the lower left sternal border that increased with inspiration. The liver was enlarged 3 cm below the costal margin, and the spleen was just palpable. The two-dimensional echocardiogram revealed a 4 • 2 • 2 cm ~ mass attached to the tricuspid valve (Fig. 1). Doppler studies showed 3+ tricuspid regurgitation. Initial laboratory data showed a hemoglobin of 11.1 gm/dl, platelet count of 14,000/mm 3, and white blood cell count of 98,000/mm 3 with 67 % blasts, 17 % neutrophils, and 8% lymphocytes. Bone marrow aspiration confirmed the diagnosis of acute lymphoblastic leukemia. Ventilation-perfusion (V/Q) scan of the lungs revealed multiple mismatched defects consistent with pulmonary emboli. Five blood cultures were negative for bacterial and fungal growth, and the cerebrospinal fluid was free of leukemic cells. The patient was started on intravenous netilmicin and cefotaxime as well as chemotherapy, which initially included vincristine, daunorubicin, asparaginase, and prednisolone, and later on, cytosine arabinoside and teniposide were added. Six weeks later, the following repeated transfusions with packed red blood cells (RBCs) and platelets, the hemoglobin was 9.6 gm/dl, platelet count was 104,000/mm 3, and white blood cell count was 5900/mm 3 with 89 % neutrophils and no blasts. At that time, tricuspid valve replacement
was performed. At operation, a fungating friable 4 • 2 • 2 cm3 mass was found attached to both sides of the tricuspid valve leaflets and extending to the subvalvular apparatus (Fig. 2, A and B). Microscopic examination confirmed the diagnosis of NBTE, as it showed a thrombus of fibrin, platelets, and constituents of blood adherent to the tricuspid valve, no organisms or inflammatory reaction, and fibrosis of the valve with neovascularization (Fig. 2, C and D). Postoperative recovery was uncomplicated. Chemotherapy guided by repeated bone marrow aspiration was continued 10 days following the surgery. One month later the patient was discharged on maintenance chemotherapy. The antemortem diagnosis of NBTE has gained importance, since NBTE may cause significant morbidity and mortality and a diagnosis will aid attempts to prevent its potential consequences. Echocardiography is a very useful tool that is able to assist the antemortem diagnosis of this condition. There are only three reports of the antemortem echocardiographic detection of pathologically proven NBTE: one by M-mode echocardiography in a patient in whom the diagnosis was confirmed at surgery3 and two by two-dimensional echocardiography in autopsy-proven cases.l, 2 Our case is unique in that it is the first report of two-dimensional echocardiographically determined features of NBTE with direct surgical validation in a living patient. Also, the unusually large valvular lesion (vegetation) that caused significant mechanical obstruction and regurgitation of the tricuspid valve, and the involvement of both surfaces of the valve as well as the subvalvular apparatus, make the clinical and pathologic presentation of this case different from those commonly reportedJ
406
--'--l~ne] Communications
February 1990 American Heart Journal
REFERENCES
1. Lopez JA, Fishbein MC, Siegel RJ. Echocardiographic features of non-bacterial thrombotic endocarditis. Am J Cardiol 1987;59:478-80. 2. Lim SP, Hakim SZ, SchoenhoffDD. Non-bacterial thrombotic endocarditis. Two-dimensional echocardiographic features in an autopsy proven case. Chest 1987;92:176-7. 3. Estevez CM, Corva BC. Serial echocardiographic abnormalities in non-bacterial thrombotic endocarditis of the mitral valve. Chest 1976;69:801-4. 4. Lopez JA, Ross RS, Fishbein MC, Siegel RJ. Non-bacterial thrombotic endocarditis: a review. AMHEARTJ 1987;113:77384.
Lipomatous hypertrophy of the interatrial septum: Diagnosis by percutaneous transvenous biopsy Gregg W. Stone, MD, Richard T. O'Kell, MD, Thomas H. Good, MD, and Geoffrey 0. Hartzler, MD. Kansas
City, Mo.
Lipomatous hypertrophy of the interatrial septum is a disorder characterized by excessive accumulation of adipose tissue that may result in marked thickening of the atrial septum. 1 While this condition has been associated with a variety of atrial arrhythmias and sudden death, 2 precise diagnosis may be essential to exclude other causes of septal lesions, including myxoma and infiltrating tumor. Unfortunately, given the relative inaccessibility of atrial tissue, definitive diagnosis is rarely made during life. 2 Though percutaneous transvenous bipsy of the right ventricular septum enjoys widespread use, s this technique has rarely been applied to obtain atrial septal tissue. We herein describe a patient with marked atrial thickening in whom lipomatous hypertrophy of the interatrial septum was diagnosed by percutaneous transvenous biopsy. The patient, a 63-year-old man with long-standing steroid-dependent asthma, presented in January 1989 with a 7-year history of multifocal atrial tachycardia refractory to digoxin, quinidine, procainamide, disopyramide, and verapamil. Mild to moderate pedal edema, exertional dyspnea, and chest discomfort had been present since 1985, all of which had recently progressed. Two-dimensional echocardiography demonstrated marked atrial septal thickening with increased echogenicity (Fig. 1). Right and left heart catheterization confirmed the clinical impression of pulmonary hypertension and predominant right ventricular failure. The left ventricle was moderately hypertrophied and the anterior wall was dyskinetic. The ejection fraction was 47 %. Severe triple-vessel coronary artery disease was From the Mid America Heart Institute, St. Luke's Hospital. Reprint requests: Dr. Geoffrey 0. Hartzler, Cardiovascular Consultants, Inc., Medical Plaza II-20, 4320 Wornall Road, Kansas City, M0 64111. 4/4/17533
Fig. 1. Apical four-chamber two-dimensional echocardiogram demonstrating marked thickening of the interatrial septum (arrows).
present. A 9F introducer sheath was placed in the right internal jugular vein, and three biopsies of the right ventricular endomyocardium were obtained with a Scholten bioptome (Scholten Surgical Instruments, Redwood City, Calif) using a standard technique. The bioptome was then withdrawn and a marked right angle curve was imparted to the distal 10 cm of the device. The bioptome was reinserted through the right internal jugular vein sheath, the anulus of the tricuspid valve identified through catheter manipulation, and the bioptome jaws were rotated posteriorly from the tricuspid anulus while being manipulated superiorly to avoid the ostium of the coronary sinus. With this approach, three specimens were obtained from adjacent but different sites of the interatrial septum. Two of the atrial specimens showed a grossly fatty appearance. Microscopic examination disclosed moderate hypertrophy of the ventricular and atrial myocytes. There was minimal interstitial fibrosis and no inflammation. Mature adipose tissue replaced portions of the atrium (Figs. 2 and 3). The fatty tissue was composed of mature fat cells interlaced with occasional blood vessels and wisps of collagen. There was no demonstrable capsule. These findings are consistent with lipomatous hypertrophy of the interatrial septum. 4 The patient's dyspnea improved following vigorous diuresis, inhaled bronchodilator agents, and increased steroid dosage. The multifocal atrial tachycardia was controlled, though not eliminated, with encainide and verapamil. Exertional angina was managed with nitrates and verapamil. Two months following discharge, the patient was less dyspneic, though he still noted palpitations. Lipomatous hypertrophy of the interatrial septum has been recognized as a distinct clinical entity since 1964, although to date the diagnosis is not usually established until autopsy. 2 While recent reports suggest a role for