Scot Med J 1992; 37: 186-187
0036-9330/92/07192/186 $2.00 in USA
© 1992 Scottish Medical Journal
ATRIAL MYXOMA: A RARE CAUSE OF PROGRESSIVE EXERTIONAL DYSPNOEA J B. Gray, A.B. Bridges", G.P. McNeill*. Departments of Pathology and *Medicine, Ninewells Hospital and Medical School, Dundee.
Abstract: A 40 year old man suffered eight years ofvague but disabling symptoms, initially thought to be related to post viral fatigue syndrome, but ameliorated by the removal of a large atrial myxoma. The diagnosis of atrial myxoma is notoriously difficult, but should be excluded by echocardiography if there are predominant symptoms ofprogressive exertional dyspnoea, even in the absence of cardiological signs.
Case report A 40 year old vicar, who previously ran six miles every day and played competitive squash, presented to his general practitioner in 1983 with a sore throat, myalgia, general malaise, 'palpitations' and exertional dyspnoea. Symptoms were thought to be due to a viral illness. However over the next six weeks his fatigue and dyspnoea increased in severity and he was referred for a hospital out-patientconsultation where systemic examination appeared normal. The following investigations were performed: urinalysis showed microscopic haematuria, the erythrocyte sedimentation rate was 34mm/hour, Coxsackie B3 titres I 1t28. Full blood count, electrolytes, chest x-ray, resting and exercise electro-cardiograph, 24 hour cardiac monitoring, ventilation and perfusion scan and pulmonary function tests (before and after exercise) were all normal. The diagnosis considered at this time was the "Royal Free" disease in view of the onset of symptoms shortly after a viral-type illness, and the raised Coxsackie titres. One year later his symptoms of general fatigue and severe exertional dyspnoea with associated palpitations were severe enough to interfere with his ability to work and he was re-referred, Examination revealed slight tenderness in the pectoral muscles, microscopic haematuria was still present on urinalysis and the electrocardiogram was normal. The diagnoses were considered to be a 'post viral effort sysdrome' or a mild myocarditis secondary to his earlier viral infection in 1983. One month later he was admitted to a different hospital with sudden collapse and severe shortness of breath. Right upper lobe pneumonia was identified on chest x -ray. On this occasion the electrodardiogram showed T wave inversion in leads Il, ill, and VI-V4. There was no history of chest pain and serial cardiac enzymes were not elevated. Erythrocyte sedimentation rate was 53rnmlhour on admission, urinalysis showed microscopic haematuria, ventilation and perfusion lung scans were negative. The Mantoux test was negative. Viral titres were raised for adenovirus, Coxsackie titres did not suggest an acute or chronic infection. The pneumonia responded to antibiotic treatment and the patient was discharged. Five months later he was recalled to hospital for cytoscopy and muscle biopsy, both results were normal. Viral titres taken at that time showed elevated Coxsackie B titres at 1/512 and anti-coxsackie IgM "very high", perhaps reflecting chronic infection dating back to the time of presentation in 1983. The patient continued to work despite continuing symptoms. Medical therapy was attempted, propranolol did not help the 'palpitations', and despite being helpful in some post viral fatigue syndrome patients, symptoms were not improved by a six month course of irnmunovir or isoprinosine. The patient was re-referred in December 1990 with increasing dyspnoea with a further reduction in exercise tolerance. Examination revealed atrial fibrillation, a quiet apical systolic murmur but no signs of cardiac failure. Investigations at that time included a chest x-ray showing an enlarged heart, erythrocyte sedimentation rate 30rnm per hour, a full blood count was normal, urinalysis was negative and Coxsackie B titres 'not significantly raised'. An echocardiogram demonstrated a very large Correspondence to: Dr J.R. Gray, Department of Pathology, Ninewells Hospital and Medical School, Dundee DDt 9SY. 186
myxoma almost completely filling the left atrium with slight prolapse through the mitral valve. (Figure lA) Surgical excision was performed (Figure IB) and pathology confirmed the diagnosis. One month post-operatively the patient was in sinus rhythm with a marked improvement in his general condition, he had resumed jogging and suffered no further malaise.
Discussion Atrial myxoma is a rare cardiac condition which can present with a variety of clinical symptoms and signs.' The diagnosis can be difficult and clinical features can be subdivided: 1. Obstructive: relating to left ventricular inflow obstruction producing breathlessness, paroxysmal nocturnal dyspnoea and atrial arrhythmias. 2. Embolic: often whilst in sinus rhythm. The emboli may be large or small with various clinical presentations including haematuria. 3. Constitutional signs relating to the tumour such as fever, weight loss, Raynaud's phenomenon, raised erythrocyte sedimentation rate, anaemia and thrombocytopenia. Post viral fatigue syndrome is a diagnosis which suggests some patients suffer chronic fatigue and malaise due to the continuing effects of an initial viral infection. The symptoms are non-specific and it is difficult to prove or refute a diagnosis in a particular patient? Techniques such as muscle biopsy and electromyography have been attempted with some equivocal results. Approximately one fifth of skeletal muscle biopsies in patients with post viral fatigue syndrome have been found to be positive for enterovirus specific RNA probes? Electromyography has detected clear electrophysical evidence of an abnormality in the peripheral part of the motor unit in patients with post viral fatigue syndrome." Furthermore, seriological tests for detecting Coxsackie B virus antibodies do not help diagnose post viral fatigue syndrome. High titres of antibodies, increased total IgM and the presence of immune complexes may be found as often in healthy individuals as in those with post viral fatigue syndromef Thus problems remain in interpreting laboratory findings when the clinical diagnosis is unproven. This patient had a post viral type illness with exhaustion, malaise, and myalgia, all made worse by exertion or emotion. At presentation examination was normal and ivral titres suggested an initial acute Coxsackie infection at the time of presentation with subsequent chronic infection. The electrocardiogram was also initially normal but later showed T wave changes which
Gray, Bridges, McNeill
Fig lAo Ultrasound of heart showing a massive atrial myxoma almost totally filling the left atrium and partially prolapsing into the ventricle through the mitral valve.
were compatible with a viral myocarditis. The erythrocyte sedimentation rates recorded were 34, 53 and 30 mm/hr A high degree of clinical suspicion is needed to ensure that the full range of diagnoses are considered in patients labelled as post viral fatigue syndrome. The absence of cardiological signs does not exclude an atrial myoxma. In an extensive review only five of 40 cases were correctly diagnosed as atrial myxomas on 6 clinical, chest x-ray and electrocardiagram findings alone. Predominant symptoms in this and a second documented series 7 were exertional dyspnoea and fatigueability. The erythrocyte sedimentation rate may only be elevated in 55% of cases. Chest x-ray signs tend to be vague, with less than 50% showing left atrial enlargement and only 5% showed calcification of the myxoma. Over 75% of the same series had 'non-specific T wave segment changes' on the electrocardiogram. Conclusion Post viral fatigue syndrome and atrial myxoma can present with similar symptomatology. In 1983 the initial viral-type illness with subsequent malaise, in addition to the confirmed Coxsackie infection was strongly suggestive of post viral fatigue syndrome. We now know the Coxsackie titres are less useful, and the diagnosis of post viral fatigue syndrome remains fraught with difficulties. Due to the size of this patient's tumour it seems likely that it was present when he initially presented. At that time he was
Atrial myxoma
Fig lB. Post operative ultrasound showing nonnal atrial anatomy after surgery.
in sinus rhythm with a normal erythrocyte sedimentation rate, chest x-ray and electrocardiogram and no cardiac murmurs were audible. This case highlights the difficulty in making clinical diagnosis of atrial myxoma which can be easily identified by the echocardiogram and be eminently treatable surgically. The diagnosis should be considered in any patient with progressive exertional breathlessness or in whom a diagnosis of myocarditis is entertained. ACKNOWLEDGEMENTS: With thanks to Mr KF Davidson, Depanment of Cardiac Surgery at Glasgow Royal Infirmary and Dr GO Urquhart of the Depanment of Virology at Ninewells Hospital, Dundee. Dr JR Gray is supported by the Scottish Hospitals Endowments Research Trust (Cruden Fellowship). REFERENCES I Nihoyannopoulos P, Venkatesan P, Hackett OJ, Valentine H, Oakley C. Left atrial myxoma: new perspectives in the diagnosis of murmur free cases. Br Heart J 1986; 56: 554-560. 2 Bowman SJ, BrostoffJ, Newman S, Mowbray IF. Post-viral fatigue syndrome - how can a diagnosis be made A study of patients undergoing a Monospot test. JR Soc Med 1989; 82: 712-716. 3 Archard Le, Bowles NE, Behan PO, Bell EJ, Doyle D. Post-viral fatigue syndrome: persistence of interovirus RNA in muscles and elevated creatinine kinase. JR Soc Med 1988; 81: 326-329. 4 Jamal GA, Hansen S. Electrophysiological studies in the post-viral fatigue syndrome. J Neurol Neurosurg. Psychiatry 1985; 48: 691-694. 5 Miller NA, Carmichael HA, Calder BD, Behan PO, Bell EJ, McCanney RA, Hall Fe. Antibody to Coxsackie B virus in diagnosing post-vira1 fatigue syndrome. BMJ 1991; 302: 140-143. 6 St John Sutton M, Mercier LA. Biu1iani E. Lie IT. Atrial myxomas. A review of clinical experience in 40 patients. Mayo CIin Proc June 1980; 55: 371-376. 7 Peters M. Hall R. Cooley D. Leachman R, Garcia E. The clinical syndrome of atrial myxoma. JAMA 1974; 230: 695-701.
187
0036-9330/92/07192/186 $2.00 in USA
© 1992 Scottish Medical Journal
ATRIAL MYXOMA: A RARE CAUSE OF PROGRESSIVE EXERTIONAL DYSPNOEA J B. Gray, A.B. Bridges", G.P. McNeill*. Departments of Pathology and *Medicine, Ninewells Hospital and Medical School, Dundee.
Abstract: A 40 year old man suffered eight years ofvague but disabling symptoms, initially thought to be related to post viral fatigue syndrome, but ameliorated by the removal of a large atrial myxoma. The diagnosis of atrial myxoma is notoriously difficult, but should be excluded by echocardiography if there are predominant symptoms ofprogressive exertional dyspnoea, even in the absence of cardiological signs.
Case report A 40 year old vicar, who previously ran six miles every day and played competitive squash, presented to his general practitioner in 1983 with a sore throat, myalgia, general malaise, 'palpitations' and exertional dyspnoea. Symptoms were thought to be due to a viral illness. However over the next six weeks his fatigue and dyspnoea increased in severity and he was referred for a hospital out-patientconsultation where systemic examination appeared normal. The following investigations were performed: urinalysis showed microscopic haematuria, the erythrocyte sedimentation rate was 34mm/hour, Coxsackie B3 titres I 1t28. Full blood count, electrolytes, chest x-ray, resting and exercise electro-cardiograph, 24 hour cardiac monitoring, ventilation and perfusion scan and pulmonary function tests (before and after exercise) were all normal. The diagnosis considered at this time was the "Royal Free" disease in view of the onset of symptoms shortly after a viral-type illness, and the raised Coxsackie titres. One year later his symptoms of general fatigue and severe exertional dyspnoea with associated palpitations were severe enough to interfere with his ability to work and he was re-referred, Examination revealed slight tenderness in the pectoral muscles, microscopic haematuria was still present on urinalysis and the electrocardiogram was normal. The diagnoses were considered to be a 'post viral effort sysdrome' or a mild myocarditis secondary to his earlier viral infection in 1983. One month later he was admitted to a different hospital with sudden collapse and severe shortness of breath. Right upper lobe pneumonia was identified on chest x -ray. On this occasion the electrodardiogram showed T wave inversion in leads Il, ill, and VI-V4. There was no history of chest pain and serial cardiac enzymes were not elevated. Erythrocyte sedimentation rate was 53rnmlhour on admission, urinalysis showed microscopic haematuria, ventilation and perfusion lung scans were negative. The Mantoux test was negative. Viral titres were raised for adenovirus, Coxsackie titres did not suggest an acute or chronic infection. The pneumonia responded to antibiotic treatment and the patient was discharged. Five months later he was recalled to hospital for cytoscopy and muscle biopsy, both results were normal. Viral titres taken at that time showed elevated Coxsackie B titres at 1/512 and anti-coxsackie IgM "very high", perhaps reflecting chronic infection dating back to the time of presentation in 1983. The patient continued to work despite continuing symptoms. Medical therapy was attempted, propranolol did not help the 'palpitations', and despite being helpful in some post viral fatigue syndrome patients, symptoms were not improved by a six month course of irnmunovir or isoprinosine. The patient was re-referred in December 1990 with increasing dyspnoea with a further reduction in exercise tolerance. Examination revealed atrial fibrillation, a quiet apical systolic murmur but no signs of cardiac failure. Investigations at that time included a chest x-ray showing an enlarged heart, erythrocyte sedimentation rate 30rnm per hour, a full blood count was normal, urinalysis was negative and Coxsackie B titres 'not significantly raised'. An echocardiogram demonstrated a very large Correspondence to: Dr J.R. Gray, Department of Pathology, Ninewells Hospital and Medical School, Dundee DDt 9SY. 186
myxoma almost completely filling the left atrium with slight prolapse through the mitral valve. (Figure lA) Surgical excision was performed (Figure IB) and pathology confirmed the diagnosis. One month post-operatively the patient was in sinus rhythm with a marked improvement in his general condition, he had resumed jogging and suffered no further malaise.
Discussion Atrial myxoma is a rare cardiac condition which can present with a variety of clinical symptoms and signs.' The diagnosis can be difficult and clinical features can be subdivided: 1. Obstructive: relating to left ventricular inflow obstruction producing breathlessness, paroxysmal nocturnal dyspnoea and atrial arrhythmias. 2. Embolic: often whilst in sinus rhythm. The emboli may be large or small with various clinical presentations including haematuria. 3. Constitutional signs relating to the tumour such as fever, weight loss, Raynaud's phenomenon, raised erythrocyte sedimentation rate, anaemia and thrombocytopenia. Post viral fatigue syndrome is a diagnosis which suggests some patients suffer chronic fatigue and malaise due to the continuing effects of an initial viral infection. The symptoms are non-specific and it is difficult to prove or refute a diagnosis in a particular patient? Techniques such as muscle biopsy and electromyography have been attempted with some equivocal results. Approximately one fifth of skeletal muscle biopsies in patients with post viral fatigue syndrome have been found to be positive for enterovirus specific RNA probes? Electromyography has detected clear electrophysical evidence of an abnormality in the peripheral part of the motor unit in patients with post viral fatigue syndrome." Furthermore, seriological tests for detecting Coxsackie B virus antibodies do not help diagnose post viral fatigue syndrome. High titres of antibodies, increased total IgM and the presence of immune complexes may be found as often in healthy individuals as in those with post viral fatigue syndromef Thus problems remain in interpreting laboratory findings when the clinical diagnosis is unproven. This patient had a post viral type illness with exhaustion, malaise, and myalgia, all made worse by exertion or emotion. At presentation examination was normal and ivral titres suggested an initial acute Coxsackie infection at the time of presentation with subsequent chronic infection. The electrocardiogram was also initially normal but later showed T wave changes which
Gray, Bridges, McNeill
Fig lAo Ultrasound of heart showing a massive atrial myxoma almost totally filling the left atrium and partially prolapsing into the ventricle through the mitral valve.
were compatible with a viral myocarditis. The erythrocyte sedimentation rates recorded were 34, 53 and 30 mm/hr A high degree of clinical suspicion is needed to ensure that the full range of diagnoses are considered in patients labelled as post viral fatigue syndrome. The absence of cardiological signs does not exclude an atrial myoxma. In an extensive review only five of 40 cases were correctly diagnosed as atrial myxomas on 6 clinical, chest x-ray and electrocardiagram findings alone. Predominant symptoms in this and a second documented series 7 were exertional dyspnoea and fatigueability. The erythrocyte sedimentation rate may only be elevated in 55% of cases. Chest x-ray signs tend to be vague, with less than 50% showing left atrial enlargement and only 5% showed calcification of the myxoma. Over 75% of the same series had 'non-specific T wave segment changes' on the electrocardiogram. Conclusion Post viral fatigue syndrome and atrial myxoma can present with similar symptomatology. In 1983 the initial viral-type illness with subsequent malaise, in addition to the confirmed Coxsackie infection was strongly suggestive of post viral fatigue syndrome. We now know the Coxsackie titres are less useful, and the diagnosis of post viral fatigue syndrome remains fraught with difficulties. Due to the size of this patient's tumour it seems likely that it was present when he initially presented. At that time he was
Atrial myxoma
Fig lB. Post operative ultrasound showing nonnal atrial anatomy after surgery.
in sinus rhythm with a normal erythrocyte sedimentation rate, chest x-ray and electrocardiogram and no cardiac murmurs were audible. This case highlights the difficulty in making clinical diagnosis of atrial myxoma which can be easily identified by the echocardiogram and be eminently treatable surgically. The diagnosis should be considered in any patient with progressive exertional breathlessness or in whom a diagnosis of myocarditis is entertained. ACKNOWLEDGEMENTS: With thanks to Mr KF Davidson, Depanment of Cardiac Surgery at Glasgow Royal Infirmary and Dr GO Urquhart of the Depanment of Virology at Ninewells Hospital, Dundee. Dr JR Gray is supported by the Scottish Hospitals Endowments Research Trust (Cruden Fellowship). REFERENCES I Nihoyannopoulos P, Venkatesan P, Hackett OJ, Valentine H, Oakley C. Left atrial myxoma: new perspectives in the diagnosis of murmur free cases. Br Heart J 1986; 56: 554-560. 2 Bowman SJ, BrostoffJ, Newman S, Mowbray IF. Post-viral fatigue syndrome - how can a diagnosis be made A study of patients undergoing a Monospot test. JR Soc Med 1989; 82: 712-716. 3 Archard Le, Bowles NE, Behan PO, Bell EJ, Doyle D. Post-viral fatigue syndrome: persistence of interovirus RNA in muscles and elevated creatinine kinase. JR Soc Med 1988; 81: 326-329. 4 Jamal GA, Hansen S. Electrophysiological studies in the post-viral fatigue syndrome. J Neurol Neurosurg. Psychiatry 1985; 48: 691-694. 5 Miller NA, Carmichael HA, Calder BD, Behan PO, Bell EJ, McCanney RA, Hall Fe. Antibody to Coxsackie B virus in diagnosing post-vira1 fatigue syndrome. BMJ 1991; 302: 140-143. 6 St John Sutton M, Mercier LA. Biu1iani E. Lie IT. Atrial myxomas. A review of clinical experience in 40 patients. Mayo CIin Proc June 1980; 55: 371-376. 7 Peters M. Hall R. Cooley D. Leachman R, Garcia E. The clinical syndrome of atrial myxoma. JAMA 1974; 230: 695-701.
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