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Aortic valve thrombus first seen as inferior myocardial infarction in a patient with polycythemia
Volume 127, Number 5 American Heart Journal
Warner
3. Westbrook RI. Primary dissecting aneurysm of the coronary artery. Nebr State Med J 1975;60:8-10. 4. Virmani R, Forman MB. Coronary artery dissections. Nonatherosclerotic ischemic heart disease. New York: Raven Press, 1989:325-54. 5. Tsinouras P. Del Mastro R. Sarfarazi M. Lee B. VitaIe E. Child A, Godfrey h, Devereux fi, Hewett D,‘Steindann B, qiljoen D, Sykes B, Kilpatrick M, Ramirez F, the International Marfan Syndrome Collaborative Study. Genetic linkage of the Marfan syndrome, ectopia lentis, and congenital contractural arachnodactyly to the fibrillin genes on chromosomes 15 and 5. N Engl J Med 1992;236:905-9. 6. Virmani R, Forman MB, Robinowitz M, McAllister HA. Coronary artery dissections. Cardiol Clin 1984;2:633-46. 7. Virmani R, Robinowitz M, McAllister HA. Marfan’s syndrome: a consistent high incidence of cardiovascular changes with and without characteristic eye changes. Circulation 1981;64(suppl):IV-312. 8. McKeown F. Dissecting aneurysm of the coronary artery in arachnodactvlv. Br Heart J 1960:22:434-6. 9. Lee G de J,‘katthews MB, Shhpey-Schafer EP. Effect of Valsalva manoeuvre on systemic and pulmonary arterial pressure in man. Br Heart J 1954;16:311-6.
Aortic valve thrombus first seen as inferior myocardial infarction in a patient with polycythemia James G. Warner, Jr., MD, EdD, Leslie L. Rupard, MD, Gregory J. Davis, MD, Patrick E. Lantz, MD, and Abdel-Mohsen Nomeir, MD Winston-Salem, N. C.
Thrombotic complications of both primary and secondary forms of polycythemia are well described and probably result from increased blood viscosity.‘, 2 Thrombosis of prosthetic heart valves is also a well-known complication, but it rarely occurs on native heart valves. To our knowledge, there are no reports of native aortic valve thrombosis in patients with polycythemia. We describe the case of a patient who presented with an inferior myocardial infarction caused by a large aortic valve thrombus. A 44-year-old man with a history of polycythemia (hematocrit 56% in 1986), a non-Q wave myocardial infarction at age 37, and a stroke at age 38 arrived at the emergency department with substernal chest pain radiating to both arms and into his back. The pain was associated with shortness of breath, nausea, and diaphoresis. His cardiac risk factors included smoking and hypertension. He had no history of collagen vascular diseases,venous throm-
From the Section of Cardiology Gray School Reprint
requests:
The Bowman ston-Salem,
and Department
James
G. Warner,
Jr., MD, EdD,
Gray School of Medicine,
Medical
NC 27157.1045.
AM HEART J 1994;127:1407-11. Copyright
of Pathology,
The Bowman
of Medicine.
@ 1994 by Mosby-Year
000%8703/94/$3.00+
0
4/4/53460
Book,
Inc.
Section of Cardiology, Center Boulevard, Win-
et al.
1407
boembolic disease, or family history of these disorders. On examination he was a normal-appearing male in moderate distress; he did not have a fever. His systolic blood pressure was 70 to 80 mm Hg, the pulse was 110 beats/min, and the respiratory rate was 22/min. Jugular venous distension was present to the angle of the jaw. His lungs were clear. Cardiac examination revealed a grade I/VI systolic murmur at the apex and a grade I/VI diastolic murmur at the left sternal border. Abdominal examination was unremarkable. Pulses were normal and symmetric. Laboratory data showed a complete white blood cell count of 10,100/mm3, hematocrit of 62 % , and a platelet count of 105,000/mm3. Prothrombin time was 25.2 seconds, activated partial thromboplastin time was 62.7 seconds, and the dimer test was positive at 4000 to 8000 rig/ml. Arterial blood gas on 4 L of oxygen by nasal cannula showed a pH of 7.24, Pacoz of 39 mm Hg, Pa@ of 89 mm Hg, and oxygen saturation of 95 % . Total creatine kinase (CK) peaked at 2327 U/L, with 736 U/L of CK-MB fraction 11 hours after admission. The initial electrocardiogram revealed atria1 fibrillation, 1 mm of ST segment elevation in the inferior leads, and nonspecific ST-T wave changes laterally. The chest x-ray film demonstrated a possible widened mediastinum, but was otherwise unremarkable. An emergency transesophageal echocardiogram was performed to assessa possible aortic dissection. This scan showed an akinetic left ventricular inferior wall and a hypokinetic right ventricle. There was a large mass involving the right and posterior cusps of the aortic valve, interpreted as thrombus, tumor, or less likely, as a vegetation (Fig. 1, A). There was mild aortic regurgitation by color-flow Doppler, but no evidence of aortic dissection. The patient was taken to the cardiac catheterization laboratory where an aortic root injection revealed a large filling defect involving the right coronary cusp, with 2+ aortic regurgitation (Fig. 1, B). A left ventriculogram was not performed because of the aortic valve mass. Coronary angiography showed a normal left system. The right coronary artery had a long filling defect throughout most of the vessel, interpreted as possible thrombus or dissection. The patient was transferred to the coronary care unit, where his blood pressure became stable with intravenous fluids and his chest pain resolved after the administration of heparin. The Cardiothoracic Surgery Division was consulted and surgery was planned for 3 days later unless he again became unstable. Two days after admission, he became acutely short of breath and developed pulmonary edema. The blood pressure was 200/100 mm Hg and the rectal temperature was 105’ F. He was treated with intravenous furosemide and nitroprusside as well as broad spectrum antibiotics. He later went into cardiac arrest, with his rhythm showing electromechanical dissociation; a prolonged resuscitation effort was unsuccessful. At autopsy, a 2.3 X 1.5 X 2.0 cm firm, dark brown thrombus was affixed to the right and posterior cusps of the aortic valve and to the aortic wall (Fig. 2). Upon sectioning, the right cusp was yellow-white, thickened to 0.3 cm, and was fixed superiorly with the posterior cusp at their junction (Fig. 3, A). Beneath the junction of the two cusps was a firm, tan-white, 0.8 X 0.5 X 1.0 cm nodule with a smooth base.
1408
Warner et al.
American
May 1994 Heart Journal
echocardiogram showing a large thrombus occupying the right an Id nc corFig. 1 Top, Transesophageal onary Cllsps of the aortic valve (arrows). Bottom, Aortogram showing a large defect involving 1the r igh coronary Clusp (arrows). LA, Left atrium; RA, right atrium.
Volume 127, Number 5 American Heart Journal
Warner
et al.
1409
2. Superior view of the aortic root demonstrating a thrombus (T) affixed to the right and posterior cusps, the right coronary artery (RCA) with dark thrombus visible through the arterial wall, and the patent left main (LM) artery with proximal branches.
Fig.
The cut surface of the mass was variegated brown-tan. The ostium of the right coronary artery was occluded by firm, white-tan material (Fig. 3, B). The remainder of the right coronary artery was patent, except for focal mild to moderate stenosing atherosclerosis. The left coronary artery only displayed mild atherosclerotic changes throughout its length. Within the posterior left ventricle was a 3.6 x 0.9 cm area of pale tan myocardium containing a focal area of dark red hemorrhage, with similar hemorrhagic areas in the anterior superior septum and right ventricular wall (Fig. 3, A). Microscopically, the thrombus was composed of fibrin and inflammatory cells and exhibited an ingrowth of capillaries and fibroblasts at the junction of the cusps and thrombus, indicating organization (Fig. 4). To our knowledge, aortic valve thrombus first seen as an acute myocardial infarction has only once been reported in the literature, and this case involved a mechanical valve.3 Nonbacterial thrombotic endocarditis (NBTE) can also
present as a myocardial infarction and can affect all of the heart valves, although the mitral and aortic valves are most commonly involved.4 These lesions are verrucous thrombotic vegetations that adhere to the closure site of the valve leaflets and are usually located on the atria1 side of mitral valves and on the ventricular surface of aortic valves5 NBTE is usually associated with malignancies and disseminated intravascular coagulation (DIC), raising the possibility of a hypercoagulable state in affected patients.4, 5 Primary and secondary forms of polycythemia can be associated with thrombotic complications, including coronary thrombosis.6 There are no reports, however, of aortic valve thrombosis in patients with polycythemia. Our patient was first seen with a myocardial infarction, erythrocytosis, DIC, and an aortic valve thrombus. The remainder of the gross and histologic examination did not reveal evidence of polycythemia vera or other malignancies. The right aortic valve cusp was affected by an
1410
Warner
et al.
American
my 1994 Heart Journal
Fig. 3. A, Long-axis view of the aortic outflow region showing thrombus (T) attached to the posterior and right aortic cusps. Areas of hemorrhage are apparent in the posterior wall (PW) and septum (S) of the left ventricle. 6, Close-up of the aortic valve showing the thrombus (T) and ostium of the right coronary artery, which is occluded by thrombotic material (arrow).
Fig. 4. Microscopic view of the thrombus (T) revealing organization with hematopoietic elements, fibrin, inflammatory cells, fibroblastic ingrowth, and neovascularization. Extension of the thrombus into the right coronary artery is shown in the right-hand corner (arrow). (Hematoxylin and eosin stain; original magnification X10, inset X100.)
Volume 127, Number 5 American Heart Journal
Adubofour,
unknown valvulitis that could have served as a nidus for NBTE. However, the attached thrombus was not consistent with NBTE by location, gross appearance, or histology. The thrombus was located on the aortic side of the valve, while most cases of NBTE affecting the aortic valve are located on the ventricular surface. The thrombus was also much larger than is commonly seen with NBTE, where most vegetations are less than 10 mm in diameter. Finally, there were no hematoxylin bodies of Libman-Sacks vegetations noted on histologic examination. Mild to moderate pulmonary emphysema was noted on lung examination, which could explain the secondary polycythemia. Hyperviscosity as a result of the elevated hematocrit may have contributed to the aortic valve thrombus, with previous coronary and cerebral events perhaps also being implicated. This patient’s hematocrit was known to have been elevated as far back as 1986. Other undiagnosed causes of a hypercoagulable state cannot be ruled out, since these were not evaluated before the patient’s death. The cause of the DIC is unclear, as all blood cultures showed no growth. We speculate that the valvulitis affecting the right aortic cusp, in combination with polycythemia, accounted for the thrombus formation. To our knowledge, this is the first case report of a large native aortic valve thrombus complicating polycythemia. As this report illustrates, aortic valve thrombosis may be an unusual complication in patients with polycythemia. REFERENCES
1. Chievitz E, Thiede T. Complications and causes of death in uolvcvthemia Vera. Acta Med Stand 1962:172:513-23. 2. Hocking WG, Golde DW. Polycythemia: evaluation and management. Blood Rev 1989;3:59-65. 3. Quintanilla MA, Haque AK. Thrombotic obstruction of prosthetic aortic valve presenting as acute myocardial infarction. AM HEART
4. Kuramoto thrombotic infarction. 5. Lopez JA, thrombotic
J 1989;117:1378-9.
K, Matsushita S, Yamanouchi H. Nonbacterial endocarditis as a cause of cerebral and myocardial Jpn Circ J 1964;48:1000-6. Ross RS, Fishbein MC, Siegel RJ. Nonbacterial endocarditis: a review. AM HEART J 1987;113:773-
84.
6. Gosalakkal JA, Neligan MC. Coronary artery bypass grafting for coronary thrombosis complicating polycythemia rubra Vera. Stand J Thorac Cardiovasc Surg 1991;25:159-60.
Coronary artery aneurysms in association with adult polycystic kidney disease Kwabena Adubofour, MD, Larry Sidaway, DO, and Thomas Glatter, MD Phoenix, Ark
From the Cardiovascular Division, Department of Internal Medicine, Maricopa Medical Center, Phoenix. Reprint requests: Thomas Glatter, MD, Department of Medicine, Maricopa Medical Center, P.O. Box 5099, Phoenix, AZ 85010. AM HEART J 1994;127:1411-3.
Copyright G 1994 by Mosby-Year Book, OOOZ-8703/94/$3.00 +0 4/4/53519
Inc.
Sidaway,
and Glatter
1411
The pathognomonic hallmark of polycystic kidney disease (PKD) is the presence of renal cysts. The most prominent extrarenal manifestations of this condition include hepatic cysts, intracranial berry aneurysms, aortic aneurysms, and gastrointestinal involvement in the form of diverticulosis and annuloaortic ectasia.l PKD has been associated with cardiac and aortic lesions,2 and Hossack et a1.,3in a recent review, comment on the echocardiographic findings in autosomal dominant PKD. Aneurysmal dilatation of the coronary arteries may be congenital or acquired and occurs in about 1.5% of patients studied by autopsy or coronary angiography.4 In reported series these localized areas of coronary dilatation are multiple and seem to have a predilection for the right coronary artery.5 This case report demonstrates the association of aneurysmal dilatation of the coronary arteries and adult PKD in the same patient. Cardisc catheterization and angiography were carried out as a prelude to renal transplantation. The angiographic study revealed the presence of multiple aneurysmal dilatations of the coronary vessels. A MEDLINE search (1966 to 1992) revealed that this association had not been previously reported. A 36-year-old man was referred to the cardiology clinic for evaluation before renal transplantation for treatment of adult PKD complicated by renal failure and hypertension. Five years before presentation he had been diagnosed at another hospital with PKD and dilated cardiomyopathy. A renal sonogram showed that he had multiple large and small cysts, which occupied the entirety of the renal parenchyma bilaterally. An endomyocardial biopsy, done to help define the cause of the dilated cardiomyopathy, _ _ demonstrated no significant abnormality. Two years before this admission, the patient had undergone bilateral nephrectomy for treatment of rapidly enlarging kidneys with worsening renal function and marked abdominal pain. Coronary angiography was performed in expectation of renal transplantation, With the right femoral artery, angiography was carried out with 7F Judkins catheters. The left ventricle showed marked dilatation with globally decreased left ventricular ejection fraction calculated at 30 % . The patient had a dominant left circulation. The small right coronary artery exhibited several fusiform dilatations, which were aneurysmal in type and not associated with atherosclerotic disease. These measured 3 to 5 mm in diameter. The left coronary artery showed normal left main and left anterior descending arteries. However, the circumflex artery demonstrated a long fusiform dilatation in the distal segment measuring 5 to 6 mm in width. Before this aneurysmal dilatation, there did not appear to be any significant atherosclerotic lesion, although in some views streaming of the dye created an apparent lesion. The vessels, aside from these dilatations, generally appeared completely smooth and free of atherosclerotic disease (Figs. 1 and 2). Although many extrarenal manifestations of PKD have been noted, the combination of PKD and coronary artery aneurysms has not been reported. Aneurysmal dilatation of intracranial vessels has been well noted, and hemorrhage from these cerebral aneurysms is one of the leading causes of death in patients with PKD. Intracranial aneurysms occur in 10% to 40?; of patients with PKD.’ One of the hy-
Warner
3. Westbrook RI. Primary dissecting aneurysm of the coronary artery. Nebr State Med J 1975;60:8-10. 4. Virmani R, Forman MB. Coronary artery dissections. Nonatherosclerotic ischemic heart disease. New York: Raven Press, 1989:325-54. 5. Tsinouras P. Del Mastro R. Sarfarazi M. Lee B. VitaIe E. Child A, Godfrey h, Devereux fi, Hewett D,‘Steindann B, qiljoen D, Sykes B, Kilpatrick M, Ramirez F, the International Marfan Syndrome Collaborative Study. Genetic linkage of the Marfan syndrome, ectopia lentis, and congenital contractural arachnodactyly to the fibrillin genes on chromosomes 15 and 5. N Engl J Med 1992;236:905-9. 6. Virmani R, Forman MB, Robinowitz M, McAllister HA. Coronary artery dissections. Cardiol Clin 1984;2:633-46. 7. Virmani R, Robinowitz M, McAllister HA. Marfan’s syndrome: a consistent high incidence of cardiovascular changes with and without characteristic eye changes. Circulation 1981;64(suppl):IV-312. 8. McKeown F. Dissecting aneurysm of the coronary artery in arachnodactvlv. Br Heart J 1960:22:434-6. 9. Lee G de J,‘katthews MB, Shhpey-Schafer EP. Effect of Valsalva manoeuvre on systemic and pulmonary arterial pressure in man. Br Heart J 1954;16:311-6.
Aortic valve thrombus first seen as inferior myocardial infarction in a patient with polycythemia James G. Warner, Jr., MD, EdD, Leslie L. Rupard, MD, Gregory J. Davis, MD, Patrick E. Lantz, MD, and Abdel-Mohsen Nomeir, MD Winston-Salem, N. C.
Thrombotic complications of both primary and secondary forms of polycythemia are well described and probably result from increased blood viscosity.‘, 2 Thrombosis of prosthetic heart valves is also a well-known complication, but it rarely occurs on native heart valves. To our knowledge, there are no reports of native aortic valve thrombosis in patients with polycythemia. We describe the case of a patient who presented with an inferior myocardial infarction caused by a large aortic valve thrombus. A 44-year-old man with a history of polycythemia (hematocrit 56% in 1986), a non-Q wave myocardial infarction at age 37, and a stroke at age 38 arrived at the emergency department with substernal chest pain radiating to both arms and into his back. The pain was associated with shortness of breath, nausea, and diaphoresis. His cardiac risk factors included smoking and hypertension. He had no history of collagen vascular diseases,venous throm-
From the Section of Cardiology Gray School Reprint
requests:
The Bowman ston-Salem,
and Department
James
G. Warner,
Jr., MD, EdD,
Gray School of Medicine,
Medical
NC 27157.1045.
AM HEART J 1994;127:1407-11. Copyright
of Pathology,
The Bowman
of Medicine.
@ 1994 by Mosby-Year
000%8703/94/$3.00+
0
4/4/53460
Book,
Inc.
Section of Cardiology, Center Boulevard, Win-
et al.
1407
boembolic disease, or family history of these disorders. On examination he was a normal-appearing male in moderate distress; he did not have a fever. His systolic blood pressure was 70 to 80 mm Hg, the pulse was 110 beats/min, and the respiratory rate was 22/min. Jugular venous distension was present to the angle of the jaw. His lungs were clear. Cardiac examination revealed a grade I/VI systolic murmur at the apex and a grade I/VI diastolic murmur at the left sternal border. Abdominal examination was unremarkable. Pulses were normal and symmetric. Laboratory data showed a complete white blood cell count of 10,100/mm3, hematocrit of 62 % , and a platelet count of 105,000/mm3. Prothrombin time was 25.2 seconds, activated partial thromboplastin time was 62.7 seconds, and the dimer test was positive at 4000 to 8000 rig/ml. Arterial blood gas on 4 L of oxygen by nasal cannula showed a pH of 7.24, Pacoz of 39 mm Hg, Pa@ of 89 mm Hg, and oxygen saturation of 95 % . Total creatine kinase (CK) peaked at 2327 U/L, with 736 U/L of CK-MB fraction 11 hours after admission. The initial electrocardiogram revealed atria1 fibrillation, 1 mm of ST segment elevation in the inferior leads, and nonspecific ST-T wave changes laterally. The chest x-ray film demonstrated a possible widened mediastinum, but was otherwise unremarkable. An emergency transesophageal echocardiogram was performed to assessa possible aortic dissection. This scan showed an akinetic left ventricular inferior wall and a hypokinetic right ventricle. There was a large mass involving the right and posterior cusps of the aortic valve, interpreted as thrombus, tumor, or less likely, as a vegetation (Fig. 1, A). There was mild aortic regurgitation by color-flow Doppler, but no evidence of aortic dissection. The patient was taken to the cardiac catheterization laboratory where an aortic root injection revealed a large filling defect involving the right coronary cusp, with 2+ aortic regurgitation (Fig. 1, B). A left ventriculogram was not performed because of the aortic valve mass. Coronary angiography showed a normal left system. The right coronary artery had a long filling defect throughout most of the vessel, interpreted as possible thrombus or dissection. The patient was transferred to the coronary care unit, where his blood pressure became stable with intravenous fluids and his chest pain resolved after the administration of heparin. The Cardiothoracic Surgery Division was consulted and surgery was planned for 3 days later unless he again became unstable. Two days after admission, he became acutely short of breath and developed pulmonary edema. The blood pressure was 200/100 mm Hg and the rectal temperature was 105’ F. He was treated with intravenous furosemide and nitroprusside as well as broad spectrum antibiotics. He later went into cardiac arrest, with his rhythm showing electromechanical dissociation; a prolonged resuscitation effort was unsuccessful. At autopsy, a 2.3 X 1.5 X 2.0 cm firm, dark brown thrombus was affixed to the right and posterior cusps of the aortic valve and to the aortic wall (Fig. 2). Upon sectioning, the right cusp was yellow-white, thickened to 0.3 cm, and was fixed superiorly with the posterior cusp at their junction (Fig. 3, A). Beneath the junction of the two cusps was a firm, tan-white, 0.8 X 0.5 X 1.0 cm nodule with a smooth base.
1408
Warner et al.
American
May 1994 Heart Journal
echocardiogram showing a large thrombus occupying the right an Id nc corFig. 1 Top, Transesophageal onary Cllsps of the aortic valve (arrows). Bottom, Aortogram showing a large defect involving 1the r igh coronary Clusp (arrows). LA, Left atrium; RA, right atrium.
Volume 127, Number 5 American Heart Journal
Warner
et al.
1409
2. Superior view of the aortic root demonstrating a thrombus (T) affixed to the right and posterior cusps, the right coronary artery (RCA) with dark thrombus visible through the arterial wall, and the patent left main (LM) artery with proximal branches.
Fig.
The cut surface of the mass was variegated brown-tan. The ostium of the right coronary artery was occluded by firm, white-tan material (Fig. 3, B). The remainder of the right coronary artery was patent, except for focal mild to moderate stenosing atherosclerosis. The left coronary artery only displayed mild atherosclerotic changes throughout its length. Within the posterior left ventricle was a 3.6 x 0.9 cm area of pale tan myocardium containing a focal area of dark red hemorrhage, with similar hemorrhagic areas in the anterior superior septum and right ventricular wall (Fig. 3, A). Microscopically, the thrombus was composed of fibrin and inflammatory cells and exhibited an ingrowth of capillaries and fibroblasts at the junction of the cusps and thrombus, indicating organization (Fig. 4). To our knowledge, aortic valve thrombus first seen as an acute myocardial infarction has only once been reported in the literature, and this case involved a mechanical valve.3 Nonbacterial thrombotic endocarditis (NBTE) can also
present as a myocardial infarction and can affect all of the heart valves, although the mitral and aortic valves are most commonly involved.4 These lesions are verrucous thrombotic vegetations that adhere to the closure site of the valve leaflets and are usually located on the atria1 side of mitral valves and on the ventricular surface of aortic valves5 NBTE is usually associated with malignancies and disseminated intravascular coagulation (DIC), raising the possibility of a hypercoagulable state in affected patients.4, 5 Primary and secondary forms of polycythemia can be associated with thrombotic complications, including coronary thrombosis.6 There are no reports, however, of aortic valve thrombosis in patients with polycythemia. Our patient was first seen with a myocardial infarction, erythrocytosis, DIC, and an aortic valve thrombus. The remainder of the gross and histologic examination did not reveal evidence of polycythemia vera or other malignancies. The right aortic valve cusp was affected by an
1410
Warner
et al.
American
my 1994 Heart Journal
Fig. 3. A, Long-axis view of the aortic outflow region showing thrombus (T) attached to the posterior and right aortic cusps. Areas of hemorrhage are apparent in the posterior wall (PW) and septum (S) of the left ventricle. 6, Close-up of the aortic valve showing the thrombus (T) and ostium of the right coronary artery, which is occluded by thrombotic material (arrow).
Fig. 4. Microscopic view of the thrombus (T) revealing organization with hematopoietic elements, fibrin, inflammatory cells, fibroblastic ingrowth, and neovascularization. Extension of the thrombus into the right coronary artery is shown in the right-hand corner (arrow). (Hematoxylin and eosin stain; original magnification X10, inset X100.)
Volume 127, Number 5 American Heart Journal
Adubofour,
unknown valvulitis that could have served as a nidus for NBTE. However, the attached thrombus was not consistent with NBTE by location, gross appearance, or histology. The thrombus was located on the aortic side of the valve, while most cases of NBTE affecting the aortic valve are located on the ventricular surface. The thrombus was also much larger than is commonly seen with NBTE, where most vegetations are less than 10 mm in diameter. Finally, there were no hematoxylin bodies of Libman-Sacks vegetations noted on histologic examination. Mild to moderate pulmonary emphysema was noted on lung examination, which could explain the secondary polycythemia. Hyperviscosity as a result of the elevated hematocrit may have contributed to the aortic valve thrombus, with previous coronary and cerebral events perhaps also being implicated. This patient’s hematocrit was known to have been elevated as far back as 1986. Other undiagnosed causes of a hypercoagulable state cannot be ruled out, since these were not evaluated before the patient’s death. The cause of the DIC is unclear, as all blood cultures showed no growth. We speculate that the valvulitis affecting the right aortic cusp, in combination with polycythemia, accounted for the thrombus formation. To our knowledge, this is the first case report of a large native aortic valve thrombus complicating polycythemia. As this report illustrates, aortic valve thrombosis may be an unusual complication in patients with polycythemia. REFERENCES
1. Chievitz E, Thiede T. Complications and causes of death in uolvcvthemia Vera. Acta Med Stand 1962:172:513-23. 2. Hocking WG, Golde DW. Polycythemia: evaluation and management. Blood Rev 1989;3:59-65. 3. Quintanilla MA, Haque AK. Thrombotic obstruction of prosthetic aortic valve presenting as acute myocardial infarction. AM HEART
4. Kuramoto thrombotic infarction. 5. Lopez JA, thrombotic
J 1989;117:1378-9.
K, Matsushita S, Yamanouchi H. Nonbacterial endocarditis as a cause of cerebral and myocardial Jpn Circ J 1964;48:1000-6. Ross RS, Fishbein MC, Siegel RJ. Nonbacterial endocarditis: a review. AM HEART J 1987;113:773-
84.
6. Gosalakkal JA, Neligan MC. Coronary artery bypass grafting for coronary thrombosis complicating polycythemia rubra Vera. Stand J Thorac Cardiovasc Surg 1991;25:159-60.
Coronary artery aneurysms in association with adult polycystic kidney disease Kwabena Adubofour, MD, Larry Sidaway, DO, and Thomas Glatter, MD Phoenix, Ark
From the Cardiovascular Division, Department of Internal Medicine, Maricopa Medical Center, Phoenix. Reprint requests: Thomas Glatter, MD, Department of Medicine, Maricopa Medical Center, P.O. Box 5099, Phoenix, AZ 85010. AM HEART J 1994;127:1411-3.
Copyright G 1994 by Mosby-Year Book, OOOZ-8703/94/$3.00 +0 4/4/53519
Inc.
Sidaway,
and Glatter
1411
The pathognomonic hallmark of polycystic kidney disease (PKD) is the presence of renal cysts. The most prominent extrarenal manifestations of this condition include hepatic cysts, intracranial berry aneurysms, aortic aneurysms, and gastrointestinal involvement in the form of diverticulosis and annuloaortic ectasia.l PKD has been associated with cardiac and aortic lesions,2 and Hossack et a1.,3in a recent review, comment on the echocardiographic findings in autosomal dominant PKD. Aneurysmal dilatation of the coronary arteries may be congenital or acquired and occurs in about 1.5% of patients studied by autopsy or coronary angiography.4 In reported series these localized areas of coronary dilatation are multiple and seem to have a predilection for the right coronary artery.5 This case report demonstrates the association of aneurysmal dilatation of the coronary arteries and adult PKD in the same patient. Cardisc catheterization and angiography were carried out as a prelude to renal transplantation. The angiographic study revealed the presence of multiple aneurysmal dilatations of the coronary vessels. A MEDLINE search (1966 to 1992) revealed that this association had not been previously reported. A 36-year-old man was referred to the cardiology clinic for evaluation before renal transplantation for treatment of adult PKD complicated by renal failure and hypertension. Five years before presentation he had been diagnosed at another hospital with PKD and dilated cardiomyopathy. A renal sonogram showed that he had multiple large and small cysts, which occupied the entirety of the renal parenchyma bilaterally. An endomyocardial biopsy, done to help define the cause of the dilated cardiomyopathy, _ _ demonstrated no significant abnormality. Two years before this admission, the patient had undergone bilateral nephrectomy for treatment of rapidly enlarging kidneys with worsening renal function and marked abdominal pain. Coronary angiography was performed in expectation of renal transplantation, With the right femoral artery, angiography was carried out with 7F Judkins catheters. The left ventricle showed marked dilatation with globally decreased left ventricular ejection fraction calculated at 30 % . The patient had a dominant left circulation. The small right coronary artery exhibited several fusiform dilatations, which were aneurysmal in type and not associated with atherosclerotic disease. These measured 3 to 5 mm in diameter. The left coronary artery showed normal left main and left anterior descending arteries. However, the circumflex artery demonstrated a long fusiform dilatation in the distal segment measuring 5 to 6 mm in width. Before this aneurysmal dilatation, there did not appear to be any significant atherosclerotic lesion, although in some views streaming of the dye created an apparent lesion. The vessels, aside from these dilatations, generally appeared completely smooth and free of atherosclerotic disease (Figs. 1 and 2). Although many extrarenal manifestations of PKD have been noted, the combination of PKD and coronary artery aneurysms has not been reported. Aneurysmal dilatation of intracranial vessels has been well noted, and hemorrhage from these cerebral aneurysms is one of the leading causes of death in patients with PKD. Intracranial aneurysms occur in 10% to 40?; of patients with PKD.’ One of the hy-