International Journal of Cardiology 174 (2014) e11–e12
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Anterior mitral valve myxoma: A case report K.M. Karaye ⁎, S. Kana, N.A. Ishaq, U. Abdullahi, M.M. Yakasai, B. Abdullahi, M.U. Sani Cardiology Unit, Department of Medicine, Aminu Kano Teaching Hospital, Kano, Nigeria
a r t i c l e
i n f o
Article history: Received 9 March 2014 Accepted 1 April 2014 Available online 12 April 2014 Keywords: Mitral valve myxoma Left ventricular dysfunction Cardiac cirrhosis
Cardiac myxomas are the most frequently encountered benign intracardiac tumors that if left untreated, are inexorably progressive and potentially fatal [1]. They arise from the endocardial tissue, and between 75 and 83% of them are located in the left atrium (LA), while 10–18% are in the right atrium where they often show a predilection for the area close to fossa ovalis [1,2]. However, cardiac myxomas that are attached to the mitral valve (MV) or tricuspid valve (TV) are rare [1,2]. A recent systematic review has reported that only 55 cases of MV myxoma were reported in the Medline and Embase databases from 2006 to 2011 [2]. Here, we present a case of anterior MV myxoma. Mr. YM is a 40 year old professional driver of a small-scale private company who presented to us in September 2013 with four months history of dyspnea on mild exertion associated with leg swelling, regular palpitations, two episodes of syncopal attacks each lasting some few seconds and epigastric pains during physical activity. The syncopal attacks did not result in trauma to the head or severe injuries. He had no other cardiovascular, respiratory or neurologic symptoms. He had no past history of systemic hypertension, diabetes mellitus, stroke or seizure disorder. There was no family history of cardiac disease or sudden death. Mr. YM gave a written informed consent for his case to be reported. The study conformed to the ethical guidelines of the Declaration of Helsinki; on the principles for medical research involving human subjects [3]. On physical examination at presentation, his oxygen saturation was 97% on room air and at rest, and had minimal pedal edema, grade III digital clubbing and pallor. He had regular heart rate of 100 beats/min, ⁎ Corresponding author at: Cardiology/Medicine and Consultant Cardiologist/Physician, Department of Medicine, Bayero University/Aminu Kano Teaching Hospital, PO Box 4445, Kano, Nigeria. Tel.: +234 803 704 2171. E-mail address: [email protected] (K.M. Karaye).
http://dx.doi.org/10.1016/j.ijcard.2014.04.006 0167-5273/© 2014 Elsevier Ireland Ltd. All rights reserved.
blood pressure of 90/60 mm Hg, raised jugular venous pressure, loud pulmonary component of second heart sound, and grade III MV middiastolic and TV regurgitation murmurs. Abdominal examination revealed a soft, tender and pulsatile hepatomegaly which was 10 cm below the right costal margin, and mild ascites. Examination of his chest revealed fine bilateral basal crepitations while findings in the other systems were unremarkable. There were no observed neurologic deficits. Echocardiogram revealed a single well-circumscribed homogeneous mass with a short stalk attached to the atrial side of anterior MV leaflet, measuring 28 × 29 mm in size. The mass was moving across the MV in diastole causing a dynamic obstruction of the valve orifice. Valve leaflets and sub-valvar apparatus were structurally normal. Left ventricular (LV) geometry was normal with LV end-diastolic dimension of 50 mm, but he had global hypokinesia with calculated ejection fraction of 34%. The left atrium (diameter = 44 mm) and right heart chambers were dilated, with a severe regurgitation of pulmonary and tricuspid valves. The estimated mean pulmonary artery pressure was 49 mm Hg. Pericardium and other structures were normal. (see Figs. 1 and 2). Electrocardiogram confirmed that the patient was in sinus rhythm and had no changes suggestive of myocardial ischemia or infarction. Blood sugar, serum lipids profile and renal function parameters were all normal, with serum creatinine of 86 μmol/L. His complete blood count showed hematocrit of 28.9%, mean corpuscular volume of 67.2 fl, mean corpuscular hemoglobin concentration of 32 g/dl and total white blood cell count of 8.2 × 109/L with a neutrophil count of 5.5 × 109/L. In addition, his alanine transaminase was 49 IU/L, aspartate transaminase was 44 IU/L and alkaline phosphatase was 21 IU/L, while serum albumin and international normalized ratio (INR) were 28 g/L and 2.59 respectively. His activated partial thromboplastin time with kaolin (APTT) was 145 s, and the patient had no history of melena or bleeding from any orifice. Liver scan confirmed enlarged liver with uniform parenchymal echo pattern, and dilated inferior venacava and intrahepatic vascular channels. Serology for hepatitis B and C was negative. The patient is presently clinically stable, has been on the following oral treatment and is being followed up: furosemide, spironolactone, digoxin and iron supplements. He was never treated with anticoagulants. The deranged INR was corrected with vitamin K supplements. The patient has been advised to avoid vigorous physical activity, and to adopt healthy lifestyle measures. At present, our center does not offer open heart surgical or interventional cardiac services. The gentleman has been advised to have coronary angiography and surgical resection of the myxoma at another center, but these are being delayed by financial constraints.
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K.M. Karaye et al. / International Journal of Cardiology 174 (2014) e11–e12
Fig. 1. Anterior mitral valve myxoma obstructing the valve orifice in a parasternal long axis view. Fig. 2. Mass (myxoma) attached to atrial surface of anterior mitral valve leaflet.
The present report is on a patient who presented with anterior MV myxoma and rare complications in the form of global LV hypokinesia probably due to cardiac micro-emboli to the coronary arteries, with severe pulmonary hypertension, cardiac cirrhosis with hypoalbuminemia and coagulopathy, and microcytic hypochromic anemia. The exact incidence of myxomas originating from the mitral valve is not clear. In one study, it was reported as 1.5% (1 case among 68 myxoma cases) [4]. Echocardiography is the most important and widely available method in the diagnosis of myxoma. The observed characteristic narrow stalk is an important feature of cardiac myxoma at any cardiac location, and is helpful when diagnostic confusion exists. The atrial surface of the anterior MV leaflet is the commonest location for solitary MV myxomas representing 43.2% of cases, which supports our finding [4]. Clinical manifestations of myxoma are determined by the location, size, mobility, and friability. Clinical manifestations can be divided into three general categories: systemic symptoms, embolism, and intracardiac obstruction [5,6]. Systemic symptoms such as general weakness, fever, weight loss, digital clubbing, arthralgia, and erythematous rash have been observed, and laboratory abnormalities such as anemia, elevations in erythrocyte sedimentation rate and globulin levels have also been reported in patients with myxoma [5,6]. Our patient had some of these manifestations. Myxoma can cause an embolism by way of the tumor emboli or thromboemboli that are released from or formed on the surface of the tumor [5,6]. From 30% to 60% of the patients with myxoma localization in the left heart shows systemic embolization [1,7]. We hypothesize that our case could have coronary microvascular embolic disease (CMD) which could manifest with global LV hypokinesia. In the case of CMD, the abnormality may not necessarily involve the territory subtended by a major coronary branch, but it may affect the whole left ventricle diffusely or be distributed in a scattered manner [8]. Although coronary angiography is not mandatory in primary heart tumor patients, it is recommended in patients with features of myocardial ischemia or in those aged over 40 with risk factors for coronary heart disease [1,9]. However, the syncopal attacks of our patient were most likely due to the dynamic obstruction of the MV orifice which could completely block LV filling especially during physical exertion, resulting in severe fall in stroke
volume and cerebral perfusion with syncope, but with resolution of symptoms as soon as LV filling resumes. Cardiac cirrhosis has not been well-reported in patients with cardiac myxoma. However, there is a mutual interaction between the function of the heart and the liver. In chronic and acute cardiac hepatopathy, owing to cardiac failure, a combination of reduced arterial perfusion and passive congestion leads to cardiac cirrhosis and cardiogenic hypoxic hepatitis [10]. Chronic liver congestion leads to synthetic function impairment, as shown by prolonged prothrombin time and reduced serum albumin concentration, which is also associated with all-cause mortality in patients with a reduced ejection fraction [10]. Treatment should be directed towards the primary heart disease and seek to secure perfusion of vital organs [10].
References [1] Kuroczyński W, Peivandi AA, Ewald P, Pruefer D, Heinemann M, Vahl CF. Cardiac myxomas: short- and long-term follow-up. Cardiol J 2009;16(5):447–54. [2] Yuan SM. Mitral valve myxoma: clinical features, current diagnostic approaches, and surgical management. Cardiol J 2012;19(1):105–9. [3] World Medical Association Declaration of Helsinki. Ethical principles for medical research involving human subjects. J Postgrad Med 2002;48:206–8. [4] Wold LE, Lie JT. Cardiac myxomas: a clinicopathologic profile. Am J Pathol 1980;101:219–40. [5] Griffiths GC. A Review of primary tumors of the heart. Prog Cardiovasc Dis 1965;7:465–79. [6] Peters MN, Hall RJ, Cooley DA, Leachman RD, Garcia E. The clinical syndrome of atrial myxoma. JAMA 1974;230:695–701. [7] Bjessmo S, Ivert T. Cardiac myxoma: 40 years experience in 63 patients. Ann Thorac Surg 1997;63:697–700. [8] Camici PG, Crea F. Coronary microvascular dysfunction. N Engl J Med 2007;356:830–40. [9] Tillmanns H. Clinical aspects of cardiac tumors. Thorac Cardiovasc Surg 1990;38:152–6. [10] Ambrosy AP, Vaduganathan M, Huffman MD, et al. Clinical course and predictive value of liver function tests in patients hospitalized for worsening heart failure with reduced ejection fraction: an analysis of the EVEREST trial. Eur J Heart Fail 2012;14:302–11.
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Anterior mitral valve myxoma: A case report K.M. Karaye ⁎, S. Kana, N.A. Ishaq, U. Abdullahi, M.M. Yakasai, B. Abdullahi, M.U. Sani Cardiology Unit, Department of Medicine, Aminu Kano Teaching Hospital, Kano, Nigeria
a r t i c l e
i n f o
Article history: Received 9 March 2014 Accepted 1 April 2014 Available online 12 April 2014 Keywords: Mitral valve myxoma Left ventricular dysfunction Cardiac cirrhosis
Cardiac myxomas are the most frequently encountered benign intracardiac tumors that if left untreated, are inexorably progressive and potentially fatal [1]. They arise from the endocardial tissue, and between 75 and 83% of them are located in the left atrium (LA), while 10–18% are in the right atrium where they often show a predilection for the area close to fossa ovalis [1,2]. However, cardiac myxomas that are attached to the mitral valve (MV) or tricuspid valve (TV) are rare [1,2]. A recent systematic review has reported that only 55 cases of MV myxoma were reported in the Medline and Embase databases from 2006 to 2011 [2]. Here, we present a case of anterior MV myxoma. Mr. YM is a 40 year old professional driver of a small-scale private company who presented to us in September 2013 with four months history of dyspnea on mild exertion associated with leg swelling, regular palpitations, two episodes of syncopal attacks each lasting some few seconds and epigastric pains during physical activity. The syncopal attacks did not result in trauma to the head or severe injuries. He had no other cardiovascular, respiratory or neurologic symptoms. He had no past history of systemic hypertension, diabetes mellitus, stroke or seizure disorder. There was no family history of cardiac disease or sudden death. Mr. YM gave a written informed consent for his case to be reported. The study conformed to the ethical guidelines of the Declaration of Helsinki; on the principles for medical research involving human subjects [3]. On physical examination at presentation, his oxygen saturation was 97% on room air and at rest, and had minimal pedal edema, grade III digital clubbing and pallor. He had regular heart rate of 100 beats/min, ⁎ Corresponding author at: Cardiology/Medicine and Consultant Cardiologist/Physician, Department of Medicine, Bayero University/Aminu Kano Teaching Hospital, PO Box 4445, Kano, Nigeria. Tel.: +234 803 704 2171. E-mail address: [email protected] (K.M. Karaye).
http://dx.doi.org/10.1016/j.ijcard.2014.04.006 0167-5273/© 2014 Elsevier Ireland Ltd. All rights reserved.
blood pressure of 90/60 mm Hg, raised jugular venous pressure, loud pulmonary component of second heart sound, and grade III MV middiastolic and TV regurgitation murmurs. Abdominal examination revealed a soft, tender and pulsatile hepatomegaly which was 10 cm below the right costal margin, and mild ascites. Examination of his chest revealed fine bilateral basal crepitations while findings in the other systems were unremarkable. There were no observed neurologic deficits. Echocardiogram revealed a single well-circumscribed homogeneous mass with a short stalk attached to the atrial side of anterior MV leaflet, measuring 28 × 29 mm in size. The mass was moving across the MV in diastole causing a dynamic obstruction of the valve orifice. Valve leaflets and sub-valvar apparatus were structurally normal. Left ventricular (LV) geometry was normal with LV end-diastolic dimension of 50 mm, but he had global hypokinesia with calculated ejection fraction of 34%. The left atrium (diameter = 44 mm) and right heart chambers were dilated, with a severe regurgitation of pulmonary and tricuspid valves. The estimated mean pulmonary artery pressure was 49 mm Hg. Pericardium and other structures were normal. (see Figs. 1 and 2). Electrocardiogram confirmed that the patient was in sinus rhythm and had no changes suggestive of myocardial ischemia or infarction. Blood sugar, serum lipids profile and renal function parameters were all normal, with serum creatinine of 86 μmol/L. His complete blood count showed hematocrit of 28.9%, mean corpuscular volume of 67.2 fl, mean corpuscular hemoglobin concentration of 32 g/dl and total white blood cell count of 8.2 × 109/L with a neutrophil count of 5.5 × 109/L. In addition, his alanine transaminase was 49 IU/L, aspartate transaminase was 44 IU/L and alkaline phosphatase was 21 IU/L, while serum albumin and international normalized ratio (INR) were 28 g/L and 2.59 respectively. His activated partial thromboplastin time with kaolin (APTT) was 145 s, and the patient had no history of melena or bleeding from any orifice. Liver scan confirmed enlarged liver with uniform parenchymal echo pattern, and dilated inferior venacava and intrahepatic vascular channels. Serology for hepatitis B and C was negative. The patient is presently clinically stable, has been on the following oral treatment and is being followed up: furosemide, spironolactone, digoxin and iron supplements. He was never treated with anticoagulants. The deranged INR was corrected with vitamin K supplements. The patient has been advised to avoid vigorous physical activity, and to adopt healthy lifestyle measures. At present, our center does not offer open heart surgical or interventional cardiac services. The gentleman has been advised to have coronary angiography and surgical resection of the myxoma at another center, but these are being delayed by financial constraints.
e12
K.M. Karaye et al. / International Journal of Cardiology 174 (2014) e11–e12
Fig. 1. Anterior mitral valve myxoma obstructing the valve orifice in a parasternal long axis view. Fig. 2. Mass (myxoma) attached to atrial surface of anterior mitral valve leaflet.
The present report is on a patient who presented with anterior MV myxoma and rare complications in the form of global LV hypokinesia probably due to cardiac micro-emboli to the coronary arteries, with severe pulmonary hypertension, cardiac cirrhosis with hypoalbuminemia and coagulopathy, and microcytic hypochromic anemia. The exact incidence of myxomas originating from the mitral valve is not clear. In one study, it was reported as 1.5% (1 case among 68 myxoma cases) [4]. Echocardiography is the most important and widely available method in the diagnosis of myxoma. The observed characteristic narrow stalk is an important feature of cardiac myxoma at any cardiac location, and is helpful when diagnostic confusion exists. The atrial surface of the anterior MV leaflet is the commonest location for solitary MV myxomas representing 43.2% of cases, which supports our finding [4]. Clinical manifestations of myxoma are determined by the location, size, mobility, and friability. Clinical manifestations can be divided into three general categories: systemic symptoms, embolism, and intracardiac obstruction [5,6]. Systemic symptoms such as general weakness, fever, weight loss, digital clubbing, arthralgia, and erythematous rash have been observed, and laboratory abnormalities such as anemia, elevations in erythrocyte sedimentation rate and globulin levels have also been reported in patients with myxoma [5,6]. Our patient had some of these manifestations. Myxoma can cause an embolism by way of the tumor emboli or thromboemboli that are released from or formed on the surface of the tumor [5,6]. From 30% to 60% of the patients with myxoma localization in the left heart shows systemic embolization [1,7]. We hypothesize that our case could have coronary microvascular embolic disease (CMD) which could manifest with global LV hypokinesia. In the case of CMD, the abnormality may not necessarily involve the territory subtended by a major coronary branch, but it may affect the whole left ventricle diffusely or be distributed in a scattered manner [8]. Although coronary angiography is not mandatory in primary heart tumor patients, it is recommended in patients with features of myocardial ischemia or in those aged over 40 with risk factors for coronary heart disease [1,9]. However, the syncopal attacks of our patient were most likely due to the dynamic obstruction of the MV orifice which could completely block LV filling especially during physical exertion, resulting in severe fall in stroke
volume and cerebral perfusion with syncope, but with resolution of symptoms as soon as LV filling resumes. Cardiac cirrhosis has not been well-reported in patients with cardiac myxoma. However, there is a mutual interaction between the function of the heart and the liver. In chronic and acute cardiac hepatopathy, owing to cardiac failure, a combination of reduced arterial perfusion and passive congestion leads to cardiac cirrhosis and cardiogenic hypoxic hepatitis [10]. Chronic liver congestion leads to synthetic function impairment, as shown by prolonged prothrombin time and reduced serum albumin concentration, which is also associated with all-cause mortality in patients with a reduced ejection fraction [10]. Treatment should be directed towards the primary heart disease and seek to secure perfusion of vital organs [10].
References [1] Kuroczyński W, Peivandi AA, Ewald P, Pruefer D, Heinemann M, Vahl CF. Cardiac myxomas: short- and long-term follow-up. Cardiol J 2009;16(5):447–54. [2] Yuan SM. Mitral valve myxoma: clinical features, current diagnostic approaches, and surgical management. Cardiol J 2012;19(1):105–9. [3] World Medical Association Declaration of Helsinki. Ethical principles for medical research involving human subjects. J Postgrad Med 2002;48:206–8. [4] Wold LE, Lie JT. Cardiac myxomas: a clinicopathologic profile. Am J Pathol 1980;101:219–40. [5] Griffiths GC. A Review of primary tumors of the heart. Prog Cardiovasc Dis 1965;7:465–79. [6] Peters MN, Hall RJ, Cooley DA, Leachman RD, Garcia E. The clinical syndrome of atrial myxoma. JAMA 1974;230:695–701. [7] Bjessmo S, Ivert T. Cardiac myxoma: 40 years experience in 63 patients. Ann Thorac Surg 1997;63:697–700. [8] Camici PG, Crea F. Coronary microvascular dysfunction. N Engl J Med 2007;356:830–40. [9] Tillmanns H. Clinical aspects of cardiac tumors. Thorac Cardiovasc Surg 1990;38:152–6. [10] Ambrosy AP, Vaduganathan M, Huffman MD, et al. Clinical course and predictive value of liver function tests in patients hospitalized for worsening heart failure with reduced ejection fraction: an analysis of the EVEREST trial. Eur J Heart Fail 2012;14:302–11.
