Pediatr Cardiol 11:164-166, 1990
Pediatric Cardiology 9 Springer-Verlag New York Inc. 1990
R i g h t A t r i a l M y x o m a with a N o n e m b o l i c Intestinal M a n i f e s t a t i o n Joon M. Park, Rafael R. Garcia, Jenny K. Patrick, David Waagner, and Sinn Anuras Department of Pediatrics, Texas Tech University Health Sciences Center, Lubbock, Texas, USA
SUMMARY. A 5-year-old male with right atrial myxoma without interatrial communication who presented with abdominal pain, vomiting, fever, and guaiac positive stool is reported. He was later found to have ischemia of a jejunal segment necessitating segmental resection. Although his symptoms persisted postoperatively, surgical removal of a right atrial myxoma was followed by complete resolution of his intestinal symptoms. We demonstrated that the mesenteric vasculitis was of nonembolic origin, and we speculate autoimmune arteritis as a possible mechanism. KEY WORDS: Necrotizing vasculitis--Right atrial myxoma--Tumor embolism
Atrial myxoma often causes diagnostic difficulty because of constitutional and embolic manifestations and subtle cardiac findings. Involvement of extracardiac organs by vasculitis associated with myxomatous embolization is common [4-7, 10]. However, Leonhardt et al. and others suggested that systemic vasculitis may occur as a consequence of an autoimmune process not related to embolism [2, 3, 6]. The purpose of this article is twofold: to report a case with right atrial myxoma initially presenting with necrotizing vasculitis of the intestine and to support the contention that vasculitis may occur without myxomatous embolization.
Case Report This 5-year-old Mexican-American boy was first admitted on September 29, 1985 with abdominal pain and vomiting of 3 days' duration. The pain was described to be crampy and generalized in the periumbilical area and the left upper abdomen. Positive physical findings were limited to fever, diffuse abdominal tenderness, and slight dehydration. Laboratory findings were remarkable for a white blood cell count of 14,400/mm 3 [10] with 88% segmented forms and a positive stool guaiac test. Laboratory tests which were negative included febrile agglutinin, blood lead, antinuclear antibody, blood and urine cultures, urinary vanilmandelic acid, and porphobilinogen. Upper and lower gastrointestinal series and nuclear scan for Meckel's diverticulum were Address offprint requests to: Dr. Joon M. Park, Texas Tech University Health Sciences Center, Department of Pediatrics, Lubbock, Texas 79430, USA.
also negative. Computed axial tomographic scan slaoweU a paraaortic mass near the root of the mesentery. Gallium-67 scintiscan suggested the possibility of an intraabdominal abscess. He was treated with nafcillin, clindamycin, and gentamycin without any relief of persistent abdominal pain. Low-grade fever persisted. On the 17th day of hospitalization, exploratory laparotomy was performed. Four areas of the jejunum were found to be markedly indurated and erythematous. Multiple enlarged lymph nodes were also noted at the base of the mesentery. A jejunal resection with primary anastomosis, lymph node biopsy, and appendectomy were done. Pathological examination of the surgical specimens revealed edematous, hemorrhagic ulceration of the jejunal mucosa which was sharply demarcated from normal bowel. Acute lymphadenitis was present. There was no evidence of tumor embolus, chronic inflammation, granuloma, or fibrosis. These findings were consistent with ischemic-necrotizing jejunitis. On the 31st day of hospitalization, he had an episode of chest pain. A grade 2/6 pansystolie murmur was heard but its significance was not recognized. Chest pain resolved spontaneously. Auscultation subsequently failed to reveal a heart murmur. Abdominal angiography performed for persistent abdominal pain revealed no infarction of the superior mesenteric artery or its branches. The patient was started on prednisone and azulfidine resulting in some symptomatic relief and was discharged home on November 8, 1985. Five days later, he returned to the clinic for a follow-up visit. A grade 3/6 pansystolic murmur and friction rub were heard along the left sternal border. Roentgenography of the chest showed right atrial enlargement but the lungs were normal. Electrocardiography showed right ventricular hypertrophy. Two-dimensional echocardiography showed a large pedunculated mass in the right atrium which moved back and forth through the tricuspid valve during cardiac cycles (Figs. 1 and 2). The left atrium and both ventricles were normal. No pericardial effusion was present. Contrast echocardiography was negative for right-to-left shunt via the atrial septum. A lung perfusion scan was normal with no pulmonary infarction.
Park et al.: Atrial Myxoma
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Fig. 1. An echocardiographic plane in the apical four-chambered view is shown. Arrow indicates that tumor lies within the right atrium.
Fig. 2. Tumor moves forward through the tricuspid valve during the right ventricular diastole.
Laboratory tests showed thrombocytopenia (40,000/mm3) and slight anemia (hemoglobin 11.4 g). No antinuclear antibody test was repeated. During surgery under cardiopulmonary bypass, a large (3 • 4 cm) tumor was removed from the floor of the right atrium, which almost occluded the tricuspid annulus. The atria/and ventricular septa were intact, and no patent foramen ovale was present. Pathological examination of the tumor confirmed the clinical impression of atrial myxoma. Although his convalescence was complicated by postpericardiotomy syndrome, prednisone was tapered off and thrombocytopenia resolved. He was discharged on the 14th postoperative day completely asymptomatic and receiving no medication. He is still asymptomatic 2 years 5 months later without any residual murmur, abdominal symptoms, or echocardiographic evidence of recurrent atrial myxoma.
reported. Although a right atrial m y x o m a causes p u l m o n a r y arterial e m b o l i s m [4], systemic arterial e m b o l i s m was r e p o r t e d w h e n a right-to-left shunt was present through a coexisting atrial septal defect [8]. Necrotizing vasculitis surrounding a m y x o m a t ous embolus [4, 7, 10] has b e e n d e m o n s t r a t e d on muscle biopsy in an affected individual [5]. Therefore, peripheral lesions are attributed to embolic p h e n o m e n a . It is not well known, h o w e v e r , w h e t h e r peripheral lesions o c c u r without t u m o r embolism. It has b e e n suggested that systemic vasculitis m a y be initiated and sustained b y the p r e s e n c e of m y x o m a as a c o n s e q u e n c e o f an a u t o i m m u n e p r o c e s s [2, 3, 6]. S o m e patients with m y x o m a h a v e p r o f o u n d constitutional s y m p t o m s and m a r k e d serological abnormalities which include fever, anorexia, weight loss, arthralgia, R a y n a u d ' s p h e n o m enon, elevated e r y t h r o c y t e sedimentation rate, hypergammaglobulinemia, and thrombocytopenia consistent with clinical features o f collagen vascular disease [1, 5 - 7 , 9]. Such findings h a v e been explained as a nonspecific or i m m u n e r e s p o n s e to a necrotizing t u m o r [4, 8]. It is o f interest to note that Burns et al. [1] d e m o n s t r a t e d that m y x o m a is capable o f erythropoietin production. Our patient had an ischemic-necrotizing vasculitis o f the intestine and his s y m p t o m s persisted after jejunal resection but cleared after r e m o v a l o f the right atrial m y x o m a . N o atrial or ventricular septal defect was present, and there was also no evidence
Discussion Atrial m y x o m a is the m o s t c o m m o n benign t u m o r of the heart. It occurs m o s t frequently in the left atrium and rarely in the right atrium or ventricle [8]. The diagnosis m a y be difficult b e c a u s e cardiac findings m a y be subtle and both constitutional and embolic manifestations are c o m m o n . When present in the left atrium, a m y x o m a often causes systemic arterial embolism affecting various organs in the body [5-6, 10]. M y x o m a t o u s emboli have been found in the vessels of the gastrointestinal tract [5], but, to o u r knowledge, no intestinal disorder as the initial presentation of the atrial m y x o m a has been
166
for pulmonary arteriovenous fistula at any time, ruling out the possibility of systemic embolism from the myxoma in the right atrium. This provides evidence for the occurrence of nonembolic intestinal vasculitis. Persistence of the atrial myxoma in our patient may have provided sufficient antigenic or humoral stimulus to initiate and perpetuate a necrotizing intestinal vasculitis.
References 1. Burns RB, Schulman IC, Murphy MJ (1982) Hematologic manifestations and etiology of atrial myxoma. AJMS 284:17-22 2. Byrd WE, Matthews OP, Hunt RE (1980) Left atrial myxoma presenting as a systemic vasculitis. Arthritis Rheum 23: 240-243
Pediatric Cardiology Vol. 11, No. 3, 1990
3. Currey HFL, Mathews JA, Robinson J (1967) Right atrial myxoma mimicking a rheumatic disorder. Br Med J 1:547548 4. Greenwood WF (1968) Profile of atrial myxoma. Am J Cardiol 21:367-375 5. Hutson KA, Combs JJ Jr, Lie JT, et al. (1978) Left atrial myxoma simulating peripheral vasculitis. Mayo Clin Proc 53:752-756 6. Leonhardt ET, Lullenbert KP (1977) Bilateral atrial myxomas with multiple arterial aneurysms--A syndrome mimicking polyarteritis nodosa. Am J Med 62:793-794 7. Silverman J, Olwin JS, Groettinger JS (1962) Cardiac myxoma with systemic embolization: Review of the literature and report of a case. Circulation 26:99-103 8. Synbas PN, Abbott GA, Logan WD, Hatcher CR Jr (1971) Atrial myxoma: Special emphasis on unusual manifestations. Chest 59:504-510 9. Thomas MH (1981) Myxoma masquerading as potyarteritis nodosa. J Rheumatol 8:133-137 10. Yufe R, Karpati G, Carpenter S (1976) Cardiac myxoma: A diagnostic challenge for the neurologist. Neurology 26:10601065
Pediatric Cardiology 9 Springer-Verlag New York Inc. 1990
R i g h t A t r i a l M y x o m a with a N o n e m b o l i c Intestinal M a n i f e s t a t i o n Joon M. Park, Rafael R. Garcia, Jenny K. Patrick, David Waagner, and Sinn Anuras Department of Pediatrics, Texas Tech University Health Sciences Center, Lubbock, Texas, USA
SUMMARY. A 5-year-old male with right atrial myxoma without interatrial communication who presented with abdominal pain, vomiting, fever, and guaiac positive stool is reported. He was later found to have ischemia of a jejunal segment necessitating segmental resection. Although his symptoms persisted postoperatively, surgical removal of a right atrial myxoma was followed by complete resolution of his intestinal symptoms. We demonstrated that the mesenteric vasculitis was of nonembolic origin, and we speculate autoimmune arteritis as a possible mechanism. KEY WORDS: Necrotizing vasculitis--Right atrial myxoma--Tumor embolism
Atrial myxoma often causes diagnostic difficulty because of constitutional and embolic manifestations and subtle cardiac findings. Involvement of extracardiac organs by vasculitis associated with myxomatous embolization is common [4-7, 10]. However, Leonhardt et al. and others suggested that systemic vasculitis may occur as a consequence of an autoimmune process not related to embolism [2, 3, 6]. The purpose of this article is twofold: to report a case with right atrial myxoma initially presenting with necrotizing vasculitis of the intestine and to support the contention that vasculitis may occur without myxomatous embolization.
Case Report This 5-year-old Mexican-American boy was first admitted on September 29, 1985 with abdominal pain and vomiting of 3 days' duration. The pain was described to be crampy and generalized in the periumbilical area and the left upper abdomen. Positive physical findings were limited to fever, diffuse abdominal tenderness, and slight dehydration. Laboratory findings were remarkable for a white blood cell count of 14,400/mm 3 [10] with 88% segmented forms and a positive stool guaiac test. Laboratory tests which were negative included febrile agglutinin, blood lead, antinuclear antibody, blood and urine cultures, urinary vanilmandelic acid, and porphobilinogen. Upper and lower gastrointestinal series and nuclear scan for Meckel's diverticulum were Address offprint requests to: Dr. Joon M. Park, Texas Tech University Health Sciences Center, Department of Pediatrics, Lubbock, Texas 79430, USA.
also negative. Computed axial tomographic scan slaoweU a paraaortic mass near the root of the mesentery. Gallium-67 scintiscan suggested the possibility of an intraabdominal abscess. He was treated with nafcillin, clindamycin, and gentamycin without any relief of persistent abdominal pain. Low-grade fever persisted. On the 17th day of hospitalization, exploratory laparotomy was performed. Four areas of the jejunum were found to be markedly indurated and erythematous. Multiple enlarged lymph nodes were also noted at the base of the mesentery. A jejunal resection with primary anastomosis, lymph node biopsy, and appendectomy were done. Pathological examination of the surgical specimens revealed edematous, hemorrhagic ulceration of the jejunal mucosa which was sharply demarcated from normal bowel. Acute lymphadenitis was present. There was no evidence of tumor embolus, chronic inflammation, granuloma, or fibrosis. These findings were consistent with ischemic-necrotizing jejunitis. On the 31st day of hospitalization, he had an episode of chest pain. A grade 2/6 pansystolie murmur was heard but its significance was not recognized. Chest pain resolved spontaneously. Auscultation subsequently failed to reveal a heart murmur. Abdominal angiography performed for persistent abdominal pain revealed no infarction of the superior mesenteric artery or its branches. The patient was started on prednisone and azulfidine resulting in some symptomatic relief and was discharged home on November 8, 1985. Five days later, he returned to the clinic for a follow-up visit. A grade 3/6 pansystolic murmur and friction rub were heard along the left sternal border. Roentgenography of the chest showed right atrial enlargement but the lungs were normal. Electrocardiography showed right ventricular hypertrophy. Two-dimensional echocardiography showed a large pedunculated mass in the right atrium which moved back and forth through the tricuspid valve during cardiac cycles (Figs. 1 and 2). The left atrium and both ventricles were normal. No pericardial effusion was present. Contrast echocardiography was negative for right-to-left shunt via the atrial septum. A lung perfusion scan was normal with no pulmonary infarction.
Park et al.: Atrial Myxoma
165
Fig. 1. An echocardiographic plane in the apical four-chambered view is shown. Arrow indicates that tumor lies within the right atrium.
Fig. 2. Tumor moves forward through the tricuspid valve during the right ventricular diastole.
Laboratory tests showed thrombocytopenia (40,000/mm3) and slight anemia (hemoglobin 11.4 g). No antinuclear antibody test was repeated. During surgery under cardiopulmonary bypass, a large (3 • 4 cm) tumor was removed from the floor of the right atrium, which almost occluded the tricuspid annulus. The atria/and ventricular septa were intact, and no patent foramen ovale was present. Pathological examination of the tumor confirmed the clinical impression of atrial myxoma. Although his convalescence was complicated by postpericardiotomy syndrome, prednisone was tapered off and thrombocytopenia resolved. He was discharged on the 14th postoperative day completely asymptomatic and receiving no medication. He is still asymptomatic 2 years 5 months later without any residual murmur, abdominal symptoms, or echocardiographic evidence of recurrent atrial myxoma.
reported. Although a right atrial m y x o m a causes p u l m o n a r y arterial e m b o l i s m [4], systemic arterial e m b o l i s m was r e p o r t e d w h e n a right-to-left shunt was present through a coexisting atrial septal defect [8]. Necrotizing vasculitis surrounding a m y x o m a t ous embolus [4, 7, 10] has b e e n d e m o n s t r a t e d on muscle biopsy in an affected individual [5]. Therefore, peripheral lesions are attributed to embolic p h e n o m e n a . It is not well known, h o w e v e r , w h e t h e r peripheral lesions o c c u r without t u m o r embolism. It has b e e n suggested that systemic vasculitis m a y be initiated and sustained b y the p r e s e n c e of m y x o m a as a c o n s e q u e n c e o f an a u t o i m m u n e p r o c e s s [2, 3, 6]. S o m e patients with m y x o m a h a v e p r o f o u n d constitutional s y m p t o m s and m a r k e d serological abnormalities which include fever, anorexia, weight loss, arthralgia, R a y n a u d ' s p h e n o m enon, elevated e r y t h r o c y t e sedimentation rate, hypergammaglobulinemia, and thrombocytopenia consistent with clinical features o f collagen vascular disease [1, 5 - 7 , 9]. Such findings h a v e been explained as a nonspecific or i m m u n e r e s p o n s e to a necrotizing t u m o r [4, 8]. It is o f interest to note that Burns et al. [1] d e m o n s t r a t e d that m y x o m a is capable o f erythropoietin production. Our patient had an ischemic-necrotizing vasculitis o f the intestine and his s y m p t o m s persisted after jejunal resection but cleared after r e m o v a l o f the right atrial m y x o m a . N o atrial or ventricular septal defect was present, and there was also no evidence
Discussion Atrial m y x o m a is the m o s t c o m m o n benign t u m o r of the heart. It occurs m o s t frequently in the left atrium and rarely in the right atrium or ventricle [8]. The diagnosis m a y be difficult b e c a u s e cardiac findings m a y be subtle and both constitutional and embolic manifestations are c o m m o n . When present in the left atrium, a m y x o m a often causes systemic arterial embolism affecting various organs in the body [5-6, 10]. M y x o m a t o u s emboli have been found in the vessels of the gastrointestinal tract [5], but, to o u r knowledge, no intestinal disorder as the initial presentation of the atrial m y x o m a has been
166
for pulmonary arteriovenous fistula at any time, ruling out the possibility of systemic embolism from the myxoma in the right atrium. This provides evidence for the occurrence of nonembolic intestinal vasculitis. Persistence of the atrial myxoma in our patient may have provided sufficient antigenic or humoral stimulus to initiate and perpetuate a necrotizing intestinal vasculitis.
References 1. Burns RB, Schulman IC, Murphy MJ (1982) Hematologic manifestations and etiology of atrial myxoma. AJMS 284:17-22 2. Byrd WE, Matthews OP, Hunt RE (1980) Left atrial myxoma presenting as a systemic vasculitis. Arthritis Rheum 23: 240-243
Pediatric Cardiology Vol. 11, No. 3, 1990
3. Currey HFL, Mathews JA, Robinson J (1967) Right atrial myxoma mimicking a rheumatic disorder. Br Med J 1:547548 4. Greenwood WF (1968) Profile of atrial myxoma. Am J Cardiol 21:367-375 5. Hutson KA, Combs JJ Jr, Lie JT, et al. (1978) Left atrial myxoma simulating peripheral vasculitis. Mayo Clin Proc 53:752-756 6. Leonhardt ET, Lullenbert KP (1977) Bilateral atrial myxomas with multiple arterial aneurysms--A syndrome mimicking polyarteritis nodosa. Am J Med 62:793-794 7. Silverman J, Olwin JS, Groettinger JS (1962) Cardiac myxoma with systemic embolization: Review of the literature and report of a case. Circulation 26:99-103 8. Synbas PN, Abbott GA, Logan WD, Hatcher CR Jr (1971) Atrial myxoma: Special emphasis on unusual manifestations. Chest 59:504-510 9. Thomas MH (1981) Myxoma masquerading as potyarteritis nodosa. J Rheumatol 8:133-137 10. Yufe R, Karpati G, Carpenter S (1976) Cardiac myxoma: A diagnostic challenge for the neurologist. Neurology 26:10601065
