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Giant right atrial thrombus causing right ventricular inflow and outflow obstruction
Volume Number
116 5, Part 1
mammary grafts were widely patent. Angiography of the native coronary artery showed no progression of disease (Figs. 1, B and 2, B). Homozygous FH is a rare disorder usually resulting in death in the second or third decade of life due to advanced proximal coronary disease. The extraordinary interventions in this patient have produced gratifying short-term results and provide important insights that may be applicable to patients with coronary artery disease and less severe forms of hypercholesterolemia. It appears that correcting the underlying metabolic abnormality through liver transplantation has diminished the manifestations of severe hypercholesterolemia. Since transplanted hepatic tissue is rich in LDL receptors> 4 this procedure produced a dramatic fall in plasma cholesterol, (76% in this patient), which resulted in a marked reduction of xanthomas and halted the progression of coronary atherosclerosis. In addition, this patient illustrates the utility of IMA bypass grafting.6 After 1 year follow-up, this patient has no apparent narrowing of the IMA grafts and is free of angina. Compared to other patients with homozygous FH who have undergone saphenous vein bypass graft surgery, this patient appears to have a good chance of long-term amelioration of angina and survival. Finally, a significant reduction in LDL cholesterol levels may lead to improved patency of grafts after surgery and may halt the progression of atherosclerosis in patients with less striking hypercholesterolemia. Blankenhorn et al6 demonstrated that reducing LDL cholesterol levels with combined bile acid sequestrant-niacin treatment resulted in significantly less progression of coronary artery disease. Therefore the findings in the present case may have broader implications. In summary, we report the first example of successful combined liver transplantation and IMA bypass grafting as a treatment of angina pectoris in a patient with homozygous FH.
REFERENCES
1. Goldstein JL, Brown MS. Familial hypercholesterolemia. In: Stanbury JB, Wyngaarden JB, Fredrickson DS, Goldstein JL, Brown MS, editors. The metabolic basis of inherited disease. New York: McGraw-Hill Book Co, Inc, 1983:672712. 2. Roberts WC, Ferrans Va. Levy RI, Fredrickson DS. Cardiovascular pathology in hyperlipoproteinemia. Am J Cardiol 1973;31:557-70. 3. Hoeg JM, Starzl TE, Brewer HB Jr. Liver transplantation for the treatment of cardiovascular disease: comparison with medication and plasma exchange in homozygous familial hypercholesterolemia. Am J Cardiol 1987;59:705-7. 4. Bilheimer DW, Goldstein JL, Grundy SM, Starzl TE, Brown MS. Liver transplantation to provide low-density-lipoprotein receptors and lower plasma cholesterol in a child with homozygous familial hypercholesterolemia. N Engl J Med 1984;1658-64. 5. Lytle BW, Loop FD, Cosgrove DM, Ratliff NB, Easley K, Taylor PC. Long-term (5 to 12 years) serial studies of internal mammary artery and saphenous vein coronary bypass grafts. J Thorac Cardiovasc Surg 1985;85:248-58. 6. Blankenhorn DH, Nessim SA. Johnson RL, Sanmarco ME,
Brief
Azen SP, Cashin-Hemphill colestipol-niacin therapy coronary venous bypass
Communications
1367
L. Beneficial effects of combined on coronary atherosclerosis and grafts. JAMA 1987;257:3233-40.
Giant right atriai thrombus causing right ventricular inflow and outflow obstruction Nazmi Gultekin, MD, Hasan Dogar, MD, Cengizhan Turkoglu, MD, Servet Ozturk, MD, Nimet Gokhan, MD, and Cem’i Demiroglu, MD. Istanbul, Turkey
Recent reports1-5 have indicated that two-dimensional echocardiography (2DE) is the investigation of choice for detecting right-sided intracavitary thrombi. We report a patient in whom 2DE and angiography demonstrated an extremely large, right atria1 (RA) mass that proved on operation to be a pedunculated, organized thrombus. A 25-year-old woman presented with a l-month history of palpitation, dizziness, and a syncopal attack. Her past history was unremarkable. On physical examination, she was in no acute distress. Her blood pressure was 110/70 mm Hg, with a pulsus paradox of 15 mm Hg, her pulse was 120 beats/min and regular, and the respiratory rate was lfi/min. Her neck veins were fully distended. Cardiac examination was remarkable for a left parasternal heave, a grade II/VI holosystolic murmur, a short early diastolic murmur varying in intensity with postural changes, an atria1 gallop, and to and fro scratchy sounds, all heard along the left parasternal border. The liver was pulsatile 4 cm below the right costal margin but there was no peripheral edema. Homans’ sign was negative and there was no calf tenderness. Routine laboratory tests, including arterial blood gases, were unremarkable except for low voltage and right axis deviation on electrocardiography. 2DE demonstrated a large, tongue-shaped, noncalcified RA mass, which although fixed to the RA septum, protruded through the tricuspid valve and remained in the RA and right ventricular cavities throughout the cardiac cycle (Fig. 1). The left ventricle was pushed by a markedly dilated right ventricle. The inferior vena cava appeared free of abnormal echoes. Cardiac catheterization was performed with great caution. Mean RA pressure was 19 mm Hg, while right ventricle and pulmonary artery pressures were normal. Right heart cineangiography confirmed the 2DE findings and showed the obstructing mass lodged in the right ventricular outflow tract (Fig. 2). The main pulmonary artery and the proximal portions of its subdivisions were normal. Lung scan was negative for pulmonary embolization (PE). The diagnosis of RA myx-
From the Institute of Cardiology, University of Istanbul. Reprint requests: Hasan Dogar. MD, The Institute of Cardiology, sity of Istanbul, Haseki, Istanbul, Turkey.
Univer-
1368
Brief
Communications
American
Fig. 1. Apical four-chamber view showing a mass (T) with an myocardium during ventricular systole (A) and during diastole that is moving the least-its pedicle. The septum bulges toward right ventricular overload. RVa, right ventricular apex; RA, atrium.
Fig. 2. Right atria1 cineangiographic frame showing the mass (outlined by arrows) during ventricular diastole. RA, right atrium; RV, right ventricle; PA, pulmonary artery. oma was made. At another hospital, the patient underwent surgical resection of a pedunculated, irregular mass from its narrow attachment to the interatrial septum near the fossa ovalis. Tt, measured 8 X 6 X 4 cm. weighed 78 pm. and showed central lysis (Fig. 3, A and B). Histology revealed organized thrombus (Fig. 3, C). Six hours after surgery, the patient became profoundly hypotensive and
November 1988 Heart Journal
acoustic density similar to that of normal (8). Arrow points to the edge of the mass the left ventricle, which is consistent with right atrium; LV, left ventricle; LA, left
suffered a cardiac arrest. Resuscitation was unsuccessful. Autopsy was not permitted. RA thrombi arise primarily from the cavity itself or originate from a peripheral venous source.‘x2 Their antemortem diagnosis has been rare but is increasing as more patients are examined with 2DE.3x4 Recent reviews provide some clues from isolated case reports and are reported e1sewhere.3-5 Primary RA thrombi are seen in lowoutput states, cardiomyopathy, arrhythmias, RA enlargement, following right heart catheterization, or without an underlying disease.le3 In our patient there was no underlying disease, and the thrombus arose near the right side of the fossa ovalis, one of the potential sites for the formation of thrombi.6 Because the sensitivity and specifity of 2DE had not yet been established: a mistaken preoperative diagnosis of myxoma like ours had been the case in several previously reported patients.3 As demonstrated on 2DE and cineangiography, one of the distinctive features of our case was the interference of the thrombus with both the inflow and outflow functions of the right ventricle. The presence of pulsus paradoxus, jugular venous distension, diastolic murmur, atria1 gallop, and elevated RA pressure reflected right ventricular inflow obstruction, while right ventricular dilatation, accompanied by a holosystolic murmur and liver pulsation, might be in response to the subacute right ventricular overload secondary to right ventricular outflow tract obstruction. RA thrombi are often associated with PE,le5 yet our case showed no evidence of PE preoperatively, and the cause of our patient’s death was not understood. Other distinctive features were the central lysis of the thrombus and its production of the peculiar scratchy sounds.
VOlUaw Number
116 5, Part 1
Brief
Fig. 3. A, Pathologic specimen removed thrombus as seen from the inside, showing
at surgery; scale is in centimeters. B, The section its central lysis. C, Histology of the thrombus.
REFERENCES 1. Lam
D, Emilson B, Rapaport E. Septic pulmonary emboli secondary to an infected right atria1 thrombus. AM HEART J 1987;114:178. 2. Lim SP, Hakim SZ, van der Bel-Kahn JM. Two-dimensional echocardiography for detection of primary right atria1 thrombus in pulmonary embolism. AM HEART J 1984;108:1546. 3. Armstrong WF, Feigenbaum H, Dillon JC. Echocardiographic detection of right atria1 thromboembolism. Chest 1985; 4.
87:8dIl. Goldberg SM, Pizzarello
RA, Goldman
VT. Echocardiographic diagnosis of right bolism resulting in massive pulmonary HEART J 1984;108:1371. 5.
MA, Padmanabhan atria1
Communications
thromboem-
embolization.
AM
Kinney EL, Zitrin R, Kohler KR, Cortada X, Varzaly LJ. Sudden appearance of a right atria1 thrombus on two-
dimensional echocardiogram: significance and therapeutic implications. AM HEART J 1985;110:879. 6. Salyer WR, Page DL, Hutchins GM. The development of cardiac myxomas and papillary endocardial lesions from mural thrombi. AM HEART J 1975;89:4.
Heart rate correlates with severity of coronary atherosclerosis in young postinfarction patients Aleksander Perski, PhD, Anders Hamsten, MD, PhD, Kaj Lindvall, MD, PhD, and Tiires Theorell, MD, PhD. Stockholm, Sweden
From the National institute of Psychos&a1 Factors and Health; the King Gustaf V Research Institute; and the Department of Medicine, Karoiinska Hospital and Stiersjukhuset, Karolinska Institute. Reprint requests: Aleksander Perski. PhD, National Institute for Psychosocial Factors and Health, Box 60201, S-104 01 Stockholm, Sweden.
1369
of the
Elevated heart rate alone has been seen to increase the risk of coronary heart disease mortality in epidemiologic studies.1,2 Recently, a number of animal experiments have been conducted in which the relationship between heart rate and progression of coronary artery atherosclerosis has been studied more directly. Kaplan et a1.3 have shown that cynomolgus macaque monkeys fed a diet moderately high in saturated fat and cholesterol for a period of 22 months will develop lesions in the coronary arteries to a different extent depending on their resting heart rate. Monkeys with habitual high resting heart rate develop twice as extensive lesions in the coronary arteries as animals with low resting heart rate. This relationship existed independently of serum lipids and blood pressure. Beere et al.“ obtained similar results in Macaca monkeys by lowering heart rate with surgical ablation of the sinoatrial node. This result was independent of blood pressure, serum lipids, and body weight. Finally, Kaplan et a1.5 observed a retardation of atherosclerosis progression through heart rate reduction by propranolol in socially dominant animals. While the epidemiologic and animal studies strongly it has suggest that heart rate. nr the far:t,ors that influence an effect on the progression of coronary atherosclerosis. no evidence has been presented from controlled human studies. Thus we decided to examine this relationship in a group of unselected, consecutive, young postinfarction patients. During a period of 29 months, 127 male patients under the age of 45 survived a confirmed myocardial infarction in the 11 hospitals with a coronary care unit within Stockholm County and were subsequently referred to the cardiovascular unit at Danderyd Hospital for further metabolic, hemostatic, and cardiologic investigations. A total of 116 patients (92%) were examined 3 to 6 months after the event. Of the remaining 11 patients, two died in
116 5, Part 1
mammary grafts were widely patent. Angiography of the native coronary artery showed no progression of disease (Figs. 1, B and 2, B). Homozygous FH is a rare disorder usually resulting in death in the second or third decade of life due to advanced proximal coronary disease. The extraordinary interventions in this patient have produced gratifying short-term results and provide important insights that may be applicable to patients with coronary artery disease and less severe forms of hypercholesterolemia. It appears that correcting the underlying metabolic abnormality through liver transplantation has diminished the manifestations of severe hypercholesterolemia. Since transplanted hepatic tissue is rich in LDL receptors> 4 this procedure produced a dramatic fall in plasma cholesterol, (76% in this patient), which resulted in a marked reduction of xanthomas and halted the progression of coronary atherosclerosis. In addition, this patient illustrates the utility of IMA bypass grafting.6 After 1 year follow-up, this patient has no apparent narrowing of the IMA grafts and is free of angina. Compared to other patients with homozygous FH who have undergone saphenous vein bypass graft surgery, this patient appears to have a good chance of long-term amelioration of angina and survival. Finally, a significant reduction in LDL cholesterol levels may lead to improved patency of grafts after surgery and may halt the progression of atherosclerosis in patients with less striking hypercholesterolemia. Blankenhorn et al6 demonstrated that reducing LDL cholesterol levels with combined bile acid sequestrant-niacin treatment resulted in significantly less progression of coronary artery disease. Therefore the findings in the present case may have broader implications. In summary, we report the first example of successful combined liver transplantation and IMA bypass grafting as a treatment of angina pectoris in a patient with homozygous FH.
REFERENCES
1. Goldstein JL, Brown MS. Familial hypercholesterolemia. In: Stanbury JB, Wyngaarden JB, Fredrickson DS, Goldstein JL, Brown MS, editors. The metabolic basis of inherited disease. New York: McGraw-Hill Book Co, Inc, 1983:672712. 2. Roberts WC, Ferrans Va. Levy RI, Fredrickson DS. Cardiovascular pathology in hyperlipoproteinemia. Am J Cardiol 1973;31:557-70. 3. Hoeg JM, Starzl TE, Brewer HB Jr. Liver transplantation for the treatment of cardiovascular disease: comparison with medication and plasma exchange in homozygous familial hypercholesterolemia. Am J Cardiol 1987;59:705-7. 4. Bilheimer DW, Goldstein JL, Grundy SM, Starzl TE, Brown MS. Liver transplantation to provide low-density-lipoprotein receptors and lower plasma cholesterol in a child with homozygous familial hypercholesterolemia. N Engl J Med 1984;1658-64. 5. Lytle BW, Loop FD, Cosgrove DM, Ratliff NB, Easley K, Taylor PC. Long-term (5 to 12 years) serial studies of internal mammary artery and saphenous vein coronary bypass grafts. J Thorac Cardiovasc Surg 1985;85:248-58. 6. Blankenhorn DH, Nessim SA. Johnson RL, Sanmarco ME,
Brief
Azen SP, Cashin-Hemphill colestipol-niacin therapy coronary venous bypass
Communications
1367
L. Beneficial effects of combined on coronary atherosclerosis and grafts. JAMA 1987;257:3233-40.
Giant right atriai thrombus causing right ventricular inflow and outflow obstruction Nazmi Gultekin, MD, Hasan Dogar, MD, Cengizhan Turkoglu, MD, Servet Ozturk, MD, Nimet Gokhan, MD, and Cem’i Demiroglu, MD. Istanbul, Turkey
Recent reports1-5 have indicated that two-dimensional echocardiography (2DE) is the investigation of choice for detecting right-sided intracavitary thrombi. We report a patient in whom 2DE and angiography demonstrated an extremely large, right atria1 (RA) mass that proved on operation to be a pedunculated, organized thrombus. A 25-year-old woman presented with a l-month history of palpitation, dizziness, and a syncopal attack. Her past history was unremarkable. On physical examination, she was in no acute distress. Her blood pressure was 110/70 mm Hg, with a pulsus paradox of 15 mm Hg, her pulse was 120 beats/min and regular, and the respiratory rate was lfi/min. Her neck veins were fully distended. Cardiac examination was remarkable for a left parasternal heave, a grade II/VI holosystolic murmur, a short early diastolic murmur varying in intensity with postural changes, an atria1 gallop, and to and fro scratchy sounds, all heard along the left parasternal border. The liver was pulsatile 4 cm below the right costal margin but there was no peripheral edema. Homans’ sign was negative and there was no calf tenderness. Routine laboratory tests, including arterial blood gases, were unremarkable except for low voltage and right axis deviation on electrocardiography. 2DE demonstrated a large, tongue-shaped, noncalcified RA mass, which although fixed to the RA septum, protruded through the tricuspid valve and remained in the RA and right ventricular cavities throughout the cardiac cycle (Fig. 1). The left ventricle was pushed by a markedly dilated right ventricle. The inferior vena cava appeared free of abnormal echoes. Cardiac catheterization was performed with great caution. Mean RA pressure was 19 mm Hg, while right ventricle and pulmonary artery pressures were normal. Right heart cineangiography confirmed the 2DE findings and showed the obstructing mass lodged in the right ventricular outflow tract (Fig. 2). The main pulmonary artery and the proximal portions of its subdivisions were normal. Lung scan was negative for pulmonary embolization (PE). The diagnosis of RA myx-
From the Institute of Cardiology, University of Istanbul. Reprint requests: Hasan Dogar. MD, The Institute of Cardiology, sity of Istanbul, Haseki, Istanbul, Turkey.
Univer-
1368
Brief
Communications
American
Fig. 1. Apical four-chamber view showing a mass (T) with an myocardium during ventricular systole (A) and during diastole that is moving the least-its pedicle. The septum bulges toward right ventricular overload. RVa, right ventricular apex; RA, atrium.
Fig. 2. Right atria1 cineangiographic frame showing the mass (outlined by arrows) during ventricular diastole. RA, right atrium; RV, right ventricle; PA, pulmonary artery. oma was made. At another hospital, the patient underwent surgical resection of a pedunculated, irregular mass from its narrow attachment to the interatrial septum near the fossa ovalis. Tt, measured 8 X 6 X 4 cm. weighed 78 pm. and showed central lysis (Fig. 3, A and B). Histology revealed organized thrombus (Fig. 3, C). Six hours after surgery, the patient became profoundly hypotensive and
November 1988 Heart Journal
acoustic density similar to that of normal (8). Arrow points to the edge of the mass the left ventricle, which is consistent with right atrium; LV, left ventricle; LA, left
suffered a cardiac arrest. Resuscitation was unsuccessful. Autopsy was not permitted. RA thrombi arise primarily from the cavity itself or originate from a peripheral venous source.‘x2 Their antemortem diagnosis has been rare but is increasing as more patients are examined with 2DE.3x4 Recent reviews provide some clues from isolated case reports and are reported e1sewhere.3-5 Primary RA thrombi are seen in lowoutput states, cardiomyopathy, arrhythmias, RA enlargement, following right heart catheterization, or without an underlying disease.le3 In our patient there was no underlying disease, and the thrombus arose near the right side of the fossa ovalis, one of the potential sites for the formation of thrombi.6 Because the sensitivity and specifity of 2DE had not yet been established: a mistaken preoperative diagnosis of myxoma like ours had been the case in several previously reported patients.3 As demonstrated on 2DE and cineangiography, one of the distinctive features of our case was the interference of the thrombus with both the inflow and outflow functions of the right ventricle. The presence of pulsus paradoxus, jugular venous distension, diastolic murmur, atria1 gallop, and elevated RA pressure reflected right ventricular inflow obstruction, while right ventricular dilatation, accompanied by a holosystolic murmur and liver pulsation, might be in response to the subacute right ventricular overload secondary to right ventricular outflow tract obstruction. RA thrombi are often associated with PE,le5 yet our case showed no evidence of PE preoperatively, and the cause of our patient’s death was not understood. Other distinctive features were the central lysis of the thrombus and its production of the peculiar scratchy sounds.
VOlUaw Number
116 5, Part 1
Brief
Fig. 3. A, Pathologic specimen removed thrombus as seen from the inside, showing
at surgery; scale is in centimeters. B, The section its central lysis. C, Histology of the thrombus.
REFERENCES 1. Lam
D, Emilson B, Rapaport E. Septic pulmonary emboli secondary to an infected right atria1 thrombus. AM HEART J 1987;114:178. 2. Lim SP, Hakim SZ, van der Bel-Kahn JM. Two-dimensional echocardiography for detection of primary right atria1 thrombus in pulmonary embolism. AM HEART J 1984;108:1546. 3. Armstrong WF, Feigenbaum H, Dillon JC. Echocardiographic detection of right atria1 thromboembolism. Chest 1985; 4.
87:8dIl. Goldberg SM, Pizzarello
RA, Goldman
VT. Echocardiographic diagnosis of right bolism resulting in massive pulmonary HEART J 1984;108:1371. 5.
MA, Padmanabhan atria1
Communications
thromboem-
embolization.
AM
Kinney EL, Zitrin R, Kohler KR, Cortada X, Varzaly LJ. Sudden appearance of a right atria1 thrombus on two-
dimensional echocardiogram: significance and therapeutic implications. AM HEART J 1985;110:879. 6. Salyer WR, Page DL, Hutchins GM. The development of cardiac myxomas and papillary endocardial lesions from mural thrombi. AM HEART J 1975;89:4.
Heart rate correlates with severity of coronary atherosclerosis in young postinfarction patients Aleksander Perski, PhD, Anders Hamsten, MD, PhD, Kaj Lindvall, MD, PhD, and Tiires Theorell, MD, PhD. Stockholm, Sweden
From the National institute of Psychos&a1 Factors and Health; the King Gustaf V Research Institute; and the Department of Medicine, Karoiinska Hospital and Stiersjukhuset, Karolinska Institute. Reprint requests: Aleksander Perski. PhD, National Institute for Psychosocial Factors and Health, Box 60201, S-104 01 Stockholm, Sweden.
1369
of the
Elevated heart rate alone has been seen to increase the risk of coronary heart disease mortality in epidemiologic studies.1,2 Recently, a number of animal experiments have been conducted in which the relationship between heart rate and progression of coronary artery atherosclerosis has been studied more directly. Kaplan et a1.3 have shown that cynomolgus macaque monkeys fed a diet moderately high in saturated fat and cholesterol for a period of 22 months will develop lesions in the coronary arteries to a different extent depending on their resting heart rate. Monkeys with habitual high resting heart rate develop twice as extensive lesions in the coronary arteries as animals with low resting heart rate. This relationship existed independently of serum lipids and blood pressure. Beere et al.“ obtained similar results in Macaca monkeys by lowering heart rate with surgical ablation of the sinoatrial node. This result was independent of blood pressure, serum lipids, and body weight. Finally, Kaplan et a1.5 observed a retardation of atherosclerosis progression through heart rate reduction by propranolol in socially dominant animals. While the epidemiologic and animal studies strongly it has suggest that heart rate. nr the far:t,ors that influence an effect on the progression of coronary atherosclerosis. no evidence has been presented from controlled human studies. Thus we decided to examine this relationship in a group of unselected, consecutive, young postinfarction patients. During a period of 29 months, 127 male patients under the age of 45 survived a confirmed myocardial infarction in the 11 hospitals with a coronary care unit within Stockholm County and were subsequently referred to the cardiovascular unit at Danderyd Hospital for further metabolic, hemostatic, and cardiologic investigations. A total of 116 patients (92%) were examined 3 to 6 months after the event. Of the remaining 11 patients, two died in