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Infected atrial myxoma
Infected Atrial Myxoma
H. VONDALE GRAHAM, MD BARRY vonHARTITZSCH, MD JOSE R. MEDINA, MD, FACC
Tulsa, Oklahoma
A patient with an infected atrial myxoma presented with clinical findings simulating bacterial endocarditis. The diagnosis was made with echocardiography, thus demonstrating the value of this rapid, noninvasive screening test. A ruptured mycotic aneurysm in the brain precluded early surgical excision of the tumor. The rapid evolution of this case to a fatal outcome underscores the need for prompt diagnosis.
Atrial myxomas often present clinical findings consistent with bacterial endocarditis. Most often, the embolic p h e n o m e n a are secondary to occlusion of small vessels with sterile t u m o r emboli. Infected atrial myxomas with organisms d e m o n s t r a t e d in the m y x o m a and blood are extremely rare and usually diagnosed at autopsy. This report presents a case of an infected atrial m y x o m a with systemic embolization. The presence of the m y x o m a was confirmed during life with echocardiography. Although the patient was appropriately t r e a t e d with antibiotic therapy, she died of r u p t u r e of a mycotic cerebral aneurysm.
Case Report
From the Clinical Cardiology Service, Hillcrest Medical Center, Tulsa, Okla. Manuscript received February 25, 1976, accepted April 7, 1976. Address for reprints: H. Vondale Graham, MD, Department of Clinical Cardiology, Hillcrest Medical Center, 1120 S. Utica, Tulsa, Okla. 74104.
658
November 4, 1976
A 48 year old white woman presented to the emergency room in a delirious state with fever and hematuria. Three weeks previously, a small furuncule had developed in the occipital region of her neck. She had been treated with Keflex® and ampicillin but had continued to have fever, general malaise and progressive lethargy. At the onset of hematuria, she had sought further medical attention. Past history obtained from the family revealed no previous cardiac or renal problems. Physical examination showed an obese woman (weight 102.5 kg, height 1.65 m) with a temperature of 102 ° F. She was mentally obtunded. Splinter hemorrhages were noted in the fingernails and Osler's nodes at the fingertips; there was a septic infarct of the right index finger (Fig. 1). Spider angiomas were present on the upper trunk, and a few petechiae were present around the right ankle. Funduscopic examination revealed clear disc margins with Roth spots superiorly and medially to the optic disc in the left eye (Fig. 2). A hemorrhage was present temporally. The blood pressure was 120/70 mm Hg and the pulse 86/min and regular. Some investigators heard a high frequency early diastolic sound that could have been consistent with a pericardial knock or opening snap. The respiratory rate was 32/min. The lungs were clear to auscultation. Palpation of the abdomen revealed normal findings; in particular, the spleen was not enlarged. The chest X-ray film (Fig. 3) showed a cardiac silhouette at the upper limit of normal with some pulmonary venous congestion. The electrocardiogram (Fig. 4) revealed sinus tachycardia with right axis deviation. The intravenous pyelogram disclosed poorly functioning bilaterally enlarged and smooth kidneys. The hematocrit was 32 percent and the white blood cell count 11,500 mm 3 with a polymorphonuclear response. The serum electrolyte values were consistent with dilutional hyponatremia. The blood urea nitrogen and creatinine values were 55 and 3.25 mg/100 ml, respectively. Examination of the urine revealed a pH of 5.0, a specific gravity of 1.021 with 1+ protein, numerous white blood cells and 50 to 70 red blood cells per high power field. A clinical diagnosis of acute bacterial endocarditis was made. The hematuria and urinary findings were considered compatible with glomerulonephritis, an
The American Journal of CARDIOLOGY
Volume 38
INFECTED ATRIAL MYXOMA--GRAHAM ET AL.
uncommon consequence of the bacterial endocarditis, and not due ~o emboli. A catheter was placed in the left subclavian artery. The central venous pressure was 20 cm H20. The patient was then given Garamycin ®, 100 mg every 12 hours, and Keflin ®, i g e v e r y 4 hours, intravenously. Twenty-four hours later when the admission blood cultures proved Staphylococcus aureus, coagulase positive, the antibiotic regimen was changed to methicillin, 2 g every 4 hours by continuous intravenous infusion. The consulting cardiologist considered the possibility of an infected left atrial myxoma on the basis of the clinical finding of a loud first heart sound in association with an intermittent early high-pitched diastolic murmur and
an early diastolic knock. The clinical impression was confirmed by the echocardiogram, which demonstrated a pattern consistent with left atrial myxoma (Fig. 5). That evening, the patient suddenly experienced respiratory arrest and had a decerebrate posture. Right-sided flaccid paralysis was also noted. Left carotid arteriograms were performed and revealed a mycotic aneurysm of the posterior branch of the middle cerebral artery measuring 5 by 8 m m with a massive shift to the right of the anterior and internal cerebral veins (Fig. 6). A left parietal craniotomy was performed with evacuation of a rather large intracerebral hematoma. The mycotic an-
FIGURE 1, Splinter hemorrhages demonstrated on fingernails of both hands. A septic infarct of the right index finger had resolved by this time.
FIGURE 2. View of patient's right fundus with Roth spots medially and superiorly. A hemorrhage is present temporally.
FIGURE 3. Admission posteroanterior view of the chest revealing slight cardiomegaly and some prominence of the pulmonary vasculature.
November 4, 1976
The American Journal of CARDIOLOGY
Volume 36
659
INFECTED ATRIAL MYXOMA--GRAHAM ET AL.
eurysm was clipped with a Heifety clip and the dome of the aneurysm was excised. In addition, there was a large occipital brain abscess, which was also evacuated. After surgery, she became afebrile but remained in coma. After 5 weeks of antibiotic therapy, her condition was unchanged and she finally died. At autopsy, the heart weighed 325 g. The left atrial chamber was slightly dilated, and the right ventricle minimally hypertrophied. The left atrium contained a resilient yelloworange mass, 40 by 30 by 20 ram, with an irregular surface mottled by areas of hemorrhage. This oval mass was attached by a 5 mm pedicle approximately 13 mm below the fossa ovalis (Fig. 7). The lobular inferior portion protruded into the mitral valve. Motion of the mass was apparently limited by the short
pedicle. The remaining endocardium was normal. The mitral valve measured 100 mm in circumference. The rest of the heart was normal except for minimal arteriosclerotic changes of the coronary arteries without a p p a r e n t narrowing. The microscopic examination showed the mass to be composed at its base of an a b u n d a n t acellular ground substance
FIGURE 4. Admission electrocardiogram revealing sinus tachycardia.
FIGURE 5. Echocardiogram demonstrating."cloud" or mass of echoes just under the anterior mitral valve leaflet.
FIGURE 6. Lateral cerebral angiogram demonstrating mycotic aneurysm (arrow) of the posterior temporal branch of the middle cerebral artery.
660
November 4, 1976
The American Journal of CARDIOLOGY
FIGURE 7. Pedunculated mass of myxoma in left atrium just above the mitral valve measuring 40 by 30 by 20 mm.
Volume 38
INFECTED ATRIAL MYXOMA--GRAHAM ET AL.
containing scattered plump fibroblasts or cells of the myxomatous type. At the pedicle, vascular and connective tissue proliferation projected into an area of more cellular proliferation. The underlying myocardium was edematous and vascular. Microabscess formation was noted, but organisms were not identified in any area. The rest of the endocardium was normal. Microscopic examination of the brain in the area of the aneurysm revealed only blood clot. No myxomatous material was noted. Cultures of the cerebral specimen grew staphylococcus, coagulase positive. Discussion
Cases of atrial myxoma simulating the clinical presentation of bacterial endocarditis with negative blood cultures have been reported. 1 Reports of atrial myxoma with concomitant findings of positive blood cultures and organisms in the atrial myxoma are rare. We have found three cases reported in the English language during the past 20 years. 2-4 Two of these three cases were diagnosed after death. All three patients presented initially with hemiplegia and fever. One presented with apparent mitral stenosis and bacterial endocarditis; cultures grew Streptococcus faecalis and the mitral valve was subsequently found to be normal. A second patient presented with apparent bacteremia and Staphylococcus aureus. After eradication of the organism with antibiotic therapy, a superinfection with Candida parapsilosis re-
sulted. The organisms were found in the atrial m y x o m a as well as in the blood cultures. The diagnosis was correctly suspected in the only surviving patient. All three patients presented with a loud first heart sound and variable murmurs. Two presented with a third heart sound. Our case is the second reported case of an infected atrial myxoma diagnosed before death. It is unfortunate t hat the untimely rupture of a mycotic cerebral aneurysm prevented prompt surgical excision of the myxoma. Our patient appears to be unique in presenting with the complicating signs and symptoms of subacute bacterial endocarditis. In addition, she had acute glomerulonephritis, a rare complication of bacterial endocarditis. 5,6 T he diagnosis of myxoma was confirmed with echocardiography.7,s Central nervous system embolization has occurred in all cases of infected atrial myxoma; it is therefore urgent t hat the diagnosis be made early. Acknowledgment
We thank Mrs. Nancy Miller for her work in the preparation of the manuscript, Douglas Hubner, MD for his review of the pathologic specimens and Marcel Binstock, MD for the funduscopic photographs.
References 1. Mundth ED, Wheeler EO, Moses JM, et ah Clinical aspects of atrial myxoma. Ann Thorac Surg 5:255-261, 1968 2. Dick HJ, Mullin EW: Myxoma of the heart complicated by blood stream infection by staphylococcus aureus and candida parapsilosis. NY State J Med 56:856-859, 1956 3. Rae A- Two patients with cardiac myxoma: one presenting as bacterial endocarditis, and one as congestive cardiac failure. Postgrad Med J 41:644-648, 1965 4. Malloch Cl, Abbott JA, Rapaport E: Left atrial myxoma with bacteremia. Am J Cardiol 25:353-358, 1970
5. Rames L, Wide B, Goodman JR, et al: Renal disease with Staphylococcus albus bacteremia. JAMA 212:1671-1677, 1970 6. Keslin MH, Messner RP, Williams RC: Glomerulonephritis with subacute bacterial endocarditis. Arch Intern Med 132:578-581, 1973 7. Wolfe SB, Popp RL: Diagnosis of atrial tumors by ultrasound. Circulation 39:615-622, 1969 8. Schattenberg ]1".. Echocardiographic diagnosis of left atrial myxoma. Mayo Clin Proc 43:620-627, 1968
November 4, 1976
The American Journal of CARDIOLOGY
Volume 38
661
H. VONDALE GRAHAM, MD BARRY vonHARTITZSCH, MD JOSE R. MEDINA, MD, FACC
Tulsa, Oklahoma
A patient with an infected atrial myxoma presented with clinical findings simulating bacterial endocarditis. The diagnosis was made with echocardiography, thus demonstrating the value of this rapid, noninvasive screening test. A ruptured mycotic aneurysm in the brain precluded early surgical excision of the tumor. The rapid evolution of this case to a fatal outcome underscores the need for prompt diagnosis.
Atrial myxomas often present clinical findings consistent with bacterial endocarditis. Most often, the embolic p h e n o m e n a are secondary to occlusion of small vessels with sterile t u m o r emboli. Infected atrial myxomas with organisms d e m o n s t r a t e d in the m y x o m a and blood are extremely rare and usually diagnosed at autopsy. This report presents a case of an infected atrial m y x o m a with systemic embolization. The presence of the m y x o m a was confirmed during life with echocardiography. Although the patient was appropriately t r e a t e d with antibiotic therapy, she died of r u p t u r e of a mycotic cerebral aneurysm.
Case Report
From the Clinical Cardiology Service, Hillcrest Medical Center, Tulsa, Okla. Manuscript received February 25, 1976, accepted April 7, 1976. Address for reprints: H. Vondale Graham, MD, Department of Clinical Cardiology, Hillcrest Medical Center, 1120 S. Utica, Tulsa, Okla. 74104.
658
November 4, 1976
A 48 year old white woman presented to the emergency room in a delirious state with fever and hematuria. Three weeks previously, a small furuncule had developed in the occipital region of her neck. She had been treated with Keflex® and ampicillin but had continued to have fever, general malaise and progressive lethargy. At the onset of hematuria, she had sought further medical attention. Past history obtained from the family revealed no previous cardiac or renal problems. Physical examination showed an obese woman (weight 102.5 kg, height 1.65 m) with a temperature of 102 ° F. She was mentally obtunded. Splinter hemorrhages were noted in the fingernails and Osler's nodes at the fingertips; there was a septic infarct of the right index finger (Fig. 1). Spider angiomas were present on the upper trunk, and a few petechiae were present around the right ankle. Funduscopic examination revealed clear disc margins with Roth spots superiorly and medially to the optic disc in the left eye (Fig. 2). A hemorrhage was present temporally. The blood pressure was 120/70 mm Hg and the pulse 86/min and regular. Some investigators heard a high frequency early diastolic sound that could have been consistent with a pericardial knock or opening snap. The respiratory rate was 32/min. The lungs were clear to auscultation. Palpation of the abdomen revealed normal findings; in particular, the spleen was not enlarged. The chest X-ray film (Fig. 3) showed a cardiac silhouette at the upper limit of normal with some pulmonary venous congestion. The electrocardiogram (Fig. 4) revealed sinus tachycardia with right axis deviation. The intravenous pyelogram disclosed poorly functioning bilaterally enlarged and smooth kidneys. The hematocrit was 32 percent and the white blood cell count 11,500 mm 3 with a polymorphonuclear response. The serum electrolyte values were consistent with dilutional hyponatremia. The blood urea nitrogen and creatinine values were 55 and 3.25 mg/100 ml, respectively. Examination of the urine revealed a pH of 5.0, a specific gravity of 1.021 with 1+ protein, numerous white blood cells and 50 to 70 red blood cells per high power field. A clinical diagnosis of acute bacterial endocarditis was made. The hematuria and urinary findings were considered compatible with glomerulonephritis, an
The American Journal of CARDIOLOGY
Volume 38
INFECTED ATRIAL MYXOMA--GRAHAM ET AL.
uncommon consequence of the bacterial endocarditis, and not due ~o emboli. A catheter was placed in the left subclavian artery. The central venous pressure was 20 cm H20. The patient was then given Garamycin ®, 100 mg every 12 hours, and Keflin ®, i g e v e r y 4 hours, intravenously. Twenty-four hours later when the admission blood cultures proved Staphylococcus aureus, coagulase positive, the antibiotic regimen was changed to methicillin, 2 g every 4 hours by continuous intravenous infusion. The consulting cardiologist considered the possibility of an infected left atrial myxoma on the basis of the clinical finding of a loud first heart sound in association with an intermittent early high-pitched diastolic murmur and
an early diastolic knock. The clinical impression was confirmed by the echocardiogram, which demonstrated a pattern consistent with left atrial myxoma (Fig. 5). That evening, the patient suddenly experienced respiratory arrest and had a decerebrate posture. Right-sided flaccid paralysis was also noted. Left carotid arteriograms were performed and revealed a mycotic aneurysm of the posterior branch of the middle cerebral artery measuring 5 by 8 m m with a massive shift to the right of the anterior and internal cerebral veins (Fig. 6). A left parietal craniotomy was performed with evacuation of a rather large intracerebral hematoma. The mycotic an-
FIGURE 1, Splinter hemorrhages demonstrated on fingernails of both hands. A septic infarct of the right index finger had resolved by this time.
FIGURE 2. View of patient's right fundus with Roth spots medially and superiorly. A hemorrhage is present temporally.
FIGURE 3. Admission posteroanterior view of the chest revealing slight cardiomegaly and some prominence of the pulmonary vasculature.
November 4, 1976
The American Journal of CARDIOLOGY
Volume 36
659
INFECTED ATRIAL MYXOMA--GRAHAM ET AL.
eurysm was clipped with a Heifety clip and the dome of the aneurysm was excised. In addition, there was a large occipital brain abscess, which was also evacuated. After surgery, she became afebrile but remained in coma. After 5 weeks of antibiotic therapy, her condition was unchanged and she finally died. At autopsy, the heart weighed 325 g. The left atrial chamber was slightly dilated, and the right ventricle minimally hypertrophied. The left atrium contained a resilient yelloworange mass, 40 by 30 by 20 ram, with an irregular surface mottled by areas of hemorrhage. This oval mass was attached by a 5 mm pedicle approximately 13 mm below the fossa ovalis (Fig. 7). The lobular inferior portion protruded into the mitral valve. Motion of the mass was apparently limited by the short
pedicle. The remaining endocardium was normal. The mitral valve measured 100 mm in circumference. The rest of the heart was normal except for minimal arteriosclerotic changes of the coronary arteries without a p p a r e n t narrowing. The microscopic examination showed the mass to be composed at its base of an a b u n d a n t acellular ground substance
FIGURE 4. Admission electrocardiogram revealing sinus tachycardia.
FIGURE 5. Echocardiogram demonstrating."cloud" or mass of echoes just under the anterior mitral valve leaflet.
FIGURE 6. Lateral cerebral angiogram demonstrating mycotic aneurysm (arrow) of the posterior temporal branch of the middle cerebral artery.
660
November 4, 1976
The American Journal of CARDIOLOGY
FIGURE 7. Pedunculated mass of myxoma in left atrium just above the mitral valve measuring 40 by 30 by 20 mm.
Volume 38
INFECTED ATRIAL MYXOMA--GRAHAM ET AL.
containing scattered plump fibroblasts or cells of the myxomatous type. At the pedicle, vascular and connective tissue proliferation projected into an area of more cellular proliferation. The underlying myocardium was edematous and vascular. Microabscess formation was noted, but organisms were not identified in any area. The rest of the endocardium was normal. Microscopic examination of the brain in the area of the aneurysm revealed only blood clot. No myxomatous material was noted. Cultures of the cerebral specimen grew staphylococcus, coagulase positive. Discussion
Cases of atrial myxoma simulating the clinical presentation of bacterial endocarditis with negative blood cultures have been reported. 1 Reports of atrial myxoma with concomitant findings of positive blood cultures and organisms in the atrial myxoma are rare. We have found three cases reported in the English language during the past 20 years. 2-4 Two of these three cases were diagnosed after death. All three patients presented initially with hemiplegia and fever. One presented with apparent mitral stenosis and bacterial endocarditis; cultures grew Streptococcus faecalis and the mitral valve was subsequently found to be normal. A second patient presented with apparent bacteremia and Staphylococcus aureus. After eradication of the organism with antibiotic therapy, a superinfection with Candida parapsilosis re-
sulted. The organisms were found in the atrial m y x o m a as well as in the blood cultures. The diagnosis was correctly suspected in the only surviving patient. All three patients presented with a loud first heart sound and variable murmurs. Two presented with a third heart sound. Our case is the second reported case of an infected atrial myxoma diagnosed before death. It is unfortunate t hat the untimely rupture of a mycotic cerebral aneurysm prevented prompt surgical excision of the myxoma. Our patient appears to be unique in presenting with the complicating signs and symptoms of subacute bacterial endocarditis. In addition, she had acute glomerulonephritis, a rare complication of bacterial endocarditis. 5,6 T he diagnosis of myxoma was confirmed with echocardiography.7,s Central nervous system embolization has occurred in all cases of infected atrial myxoma; it is therefore urgent t hat the diagnosis be made early. Acknowledgment
We thank Mrs. Nancy Miller for her work in the preparation of the manuscript, Douglas Hubner, MD for his review of the pathologic specimens and Marcel Binstock, MD for the funduscopic photographs.
References 1. Mundth ED, Wheeler EO, Moses JM, et ah Clinical aspects of atrial myxoma. Ann Thorac Surg 5:255-261, 1968 2. Dick HJ, Mullin EW: Myxoma of the heart complicated by blood stream infection by staphylococcus aureus and candida parapsilosis. NY State J Med 56:856-859, 1956 3. Rae A- Two patients with cardiac myxoma: one presenting as bacterial endocarditis, and one as congestive cardiac failure. Postgrad Med J 41:644-648, 1965 4. Malloch Cl, Abbott JA, Rapaport E: Left atrial myxoma with bacteremia. Am J Cardiol 25:353-358, 1970
5. Rames L, Wide B, Goodman JR, et al: Renal disease with Staphylococcus albus bacteremia. JAMA 212:1671-1677, 1970 6. Keslin MH, Messner RP, Williams RC: Glomerulonephritis with subacute bacterial endocarditis. Arch Intern Med 132:578-581, 1973 7. Wolfe SB, Popp RL: Diagnosis of atrial tumors by ultrasound. Circulation 39:615-622, 1969 8. Schattenberg ]1".. Echocardiographic diagnosis of left atrial myxoma. Mayo Clin Proc 43:620-627, 1968
November 4, 1976
The American Journal of CARDIOLOGY
Volume 38
661