Int J Cardiovasc Imaging (2007) 23:329–332 DOI 10.1007/s10554-006-9153-0
CASE REPORT
Left ventricular outflow tract myxoma accompanied by dextrocardia with situs inversus: a case report Kenan Yalta Æ Okan Onur Turgut Æ Ahmet Yilmaz Æ Mehmet Birhan Yilmaz Æ Ali Ozyol Æ Filiz Karadas Æ Izzet Tandogan
Received: 4 July 2006 / Accepted: 21 August 2006 / Published online: 23 September 2006 Springer Science+Business Media B.V. 2006
Abstract Myxoma is the most frequently encountered primary tumour of the heart, comprising more than 50% of all benign cardiac tumours. The case presented here was diagnosed as having dextrocardia with situs inversus and concomitant left ventricular outflow tract myxoma which has been very rarely encountered, particularly consistent with the scarcity of reported cases. Keywords Left ventricular outflow tract myxoma Æ Dextrocardia Æ Situs inversus
1 Case report About 49-year-old female patient was admitted to our department with symptoms of atypical chest pain and palpitation. The physical examination was found to be normal except for the heart sounds audible only on the right side of the chest and the liver palpable on the left side of abdomen (dextrocardia with situs inversus). Serum parameters were within normal range except for the mildly elevated leukocyte count (WBC) (12,000/
K. Yalta (&) Æ O. O. Turgut Æ A. Yilmaz Æ M. B. Yilmaz Æ A. Ozyol Æ F. Karadas Æ I. Tandogan Department of Cardiology, Cumhuriyet University, Sivas 58100, Turkey e-mail:
[email protected]
ml) and moderately elevated ESR (erhythrocyte sedimentation rate) (50 mm/h). Cultures of various samples (blood, urine, sputum, feces etc.) were found to be negative, and serum concentrations of all ANA (antinuclear antibody) subgroups were found to be within normal range. ECG demonstrated a pattern of nonspecific T wave inversion in precordial leads and negative deflection of P, T waves and QRS complexes in leads D1-aVL (due to dextrocardia) (Fig. 1). The next step was exercise testing which failed to demonstrate myocardial ischemia at any stage of the test. Chest X-ray demonstrated the right sided cardiac sillhouette with no signs of calcification (Fig. 2). TTE (Transthoracic echocardiography) confirmed the presence of dextrocardia, and demonstrated a swinging mass (2.3 · 1.6 cm) with a glistening appearance, attached to the anteroseptal portion of left ventricular outflow tract (LVOT) by a short stalk (Fig. 3a, b). More careful examination by slight movement of the probe revealed interspersed hyperechoic and hypoechoic zones, supposed to be due to hemorrhage and calcification. Aortic velocity was found to be within normal range (1.5 m/s). There was no evidence of any other co-existent acquired abnormality (mass, valvular regurgitation etc.) or associated congenital anomaly (transposition of great arteries etc.) on TTE. The images on TTE were consistent with solitary LVOT myxoma.
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Fig. 1 ECG demonstrating dextrocardia
Likewise, thorax CT (computed tomography), the next step in diagnosis, demonstrated a LVOT mass (2 · 1.5 cm) consistent with myxoma (Fig. 4). Thorax CT also confirmed the presence of dextrocardia with situs inversus (inversely located liver, stomach and spleen). TEE (transesophageal echocardiography) could not be performed due to the intolerance of the patient. The patient underwent surgical excision of the LVOT mass, and recovered uneventfully within a week. The pathology of the tumor was found to be consistent with myxoma and confirmed the diagnosis. Close follow-up was recommended for future risk of probable recurrence on discharge of the patient.
2 Discussion Myxoma is the most common cardiac tumor, constituting more than 50% of all primary cardiac tumours. Approximately 75% of myxomas occur in the left atrium, where the site of attachment is almost always in the region of the limbus of the fossa ovalis. Myxomas less often, may involve right atrium (15–20%) and ventricles. Some authors have reported very unusual sites for cardiac myxomas. Choi et al. [1] reported a cardiac myxoma attached to both atrial and ventricular sides of the mitral valve. Left ventricle has also been a very unusual predilection site for cardiac myxomas. There have been 47 cases of LV myxoma reported in the world literature since 1957 [2]. Myxomas may be sporadic (90%) or familial, solitary (90%) or multiple. Familial tumours tend
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to be multiple in nature. The mean age at the time of presentation in patients with sporadic myxoma is 50 years [3] while two-thirds of patients are female. The tumors average 5–6 cm in diameter but range from less than 1–15 cm or greater [4]. The features of the patient presented here were consistent with the general features described above, but the location of the mass was very uncommon in contrast to the majority of myxoma cases. Myxomas tend to create obstructive, embolic and constitutional symptoms to a variable extent. The rarely encountered LV myxomas generally give rise to symptoms (92.7%), particularly due to systemic embolism (the most common manifestation of LVOT myxomas) (50%) [2]. Two thirds of patients having myxoma suffer cerebral emboli leading to transient ischemic attacks, strokes or seizures while half have peripheral limb emboli
Fig. 2 Chest X-ray demonstrating dextrocardia and right sided fundus
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Fig. 3 (a) Image of LVOT myxoma on transthoracic echocardiography (apical five-chamber view) (b) Image of LVOT myxoma on transthoracic echocardiography (apical two-chamber view)
[4]. Coronary embolization may also occur. Yavuz et al. [5] reported a left atrial myxoma associated with acute myocardial infarction in a 38 year-old male patient with normal coronary arteries. Fortunately, our case did not have any evident sign or history of systemic embolism. The constitutional symptoms and signs including myalgia, atypical chest pain, fatigue, weight loss fever and laboratory findings including elevated WBC, ESR and anemia may be confounding, and sometimes lead to the misdiagnosis of infection such as endocarditis or collagen vascular disease. Our patient had atypical chest pain, elevated WBC (12,000/ml) and ESR (50 mm/h) that might be misconceived as markers of infection or collagen vascular disease. Cultures of various samples (blood, urine, sputum, feces etc.) were found to be negative, and serum concentrations of all ANA (antinuclear antibody) subgroups were found to be within normal range, favoring a myxoma associated elevation of ESR and WBC. Syncopal attacks and heart failure has been regarded as clinically important symptoms of obstructive myxomas [4]. Mechanical effects of myxoma can be important in valvular regurgitation. Previous workers have reported that ‘‘wrecking ball effect’’ of a mobile myxoma can damage mitral or aortic valve to produce regurgitation [6–8]. Mittal et al. [9] reported aortic and tricuspid incompetence in a case with left atrial myxoma and propounded that constant trauma to the central fibrous body (fibrous support of mitral, tricuspid and aortic valves) by the prolapsing left atrial myxoma might exaggerate the physiological
incompetence of structurally normal aortic and tricuspid valves. The case presented here had neither any history of syncopal attack nor any symptom of heart failure including dyspnea, orthopnea etc. as it was clearly proven by TTE which demonstrated the absence of LVOT gradient and the absence of any valvular regurgitation. TTE and CT were the next steps in the diagnosis after routine chest X-ray in this case. Calcification can be evident in the tumor even on a routine chest film, [10] but the chest X-ray of the patient failed to demonstrate any sign of calcification. The value of ultrasound in the noninvasive diagnosis of intracavitary tumors has been well documented [11–13] whereas techniques such as CT has been used to achieve excellent visualization of intracavitary myxomas, providing
Fig. 4 Magnified tomographic image of LVOT myxoma
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information about size, shape and attachment of these tumors. It was proven that CT findings correlated well with operative and pathologic findings. Tsuchiya et al. [14] considered CT as an important diagnostic imaging modality to diagnose atrial myxomas by the location and nature of the intracardiac mass and to differentiate it from thrombus. Likewise, in a series of 23 cases, the diagnostic imaging manifestations (CT and TTE) of 22 cases were confirmed by operation and pathology [15]. CT was found to be superior in differentiating intracardiac thrombus and lipoma from myxoma [15]. These findings particularly corroborate the diagnostic certainty and reliability of CT, emphasizing the concordance of diagnostic imaging manifestations with the subsequent histopathologic confirmation. The pathologic examination of the tumor, as a final step, also confirmed the diagnosis of myxoma in this case. The patient had also concomitant dextrocardia with situs inversus which was found to be unassociated with any variety of congenital cardiac anomaly (transposition of great arteries etc.). It first appeared as the absence of audible heart sounds in the usual location during physical examination and was later proven by the diagnostic imaging manifestations. In conclusion, the case presented here was found to have a rarely encountered solitary LVOT myxoma and concomitant dextrocardia with situs inversus.
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Int J Cardiovasc Imaging (2007) 23:329–332 2. Thongcharoen P, Laksanabunsong P, Thongtang V (1997) Left ventricular outflow tract obstruction due to a left ventricular myxoma: a case report and review of the literature. J Med Assoc Thai 80(12):799–806 3. Yoon DH, Roberts W (2002) Sex distribution in cardiac myxomas. Am J Cardiol 90:563–565 4. Pinede L, Duhaut P, Laire R (2001) Clinical presentation of left atrial cardiac myxoma: a series of 112 consecutive cases. Medicine (Boltimore) 80:159–172 5. Yavuz T, Peker O, Ocal A, Ibrisim E (2005) Left atrial myxoma associated with acute myocardial infarction. Int J Cardiovasc Imaging 21(2–3):235–238 6. Rose MR, Fox AC, Glassman E, Reed GE (1974) Left atrial myxoma and Aortic regurgitation. J Thorac Cardiovasc Surg 68:797–801 7. Harvey WP (1968) Clinical aspects of cardiac tumors. Am J Cardiol 21:328–343 8. Case records of the Massachusetts General Hospital, weekly clinicopathological exercises: case 42, (1973) N Eng J Med 289:853–859 9. SR Mittal, UB Jain (1990/91) Doppler documentation of aortic and tricuspid incompetence in left atrial myxoma. Int J Cardiac Imaging 6:97–99 10. Sharratt GP, Grover ML, Monro JL (1979) Calcified left atrial myxoma with floppy mitral valve. Br Heart J 42:608–610 11. Peters MN, Hall RJ, Cooley DA, Leachman RD, Garcia E (1974) The clinical syndrome of atrial myxoma. JAMA 230:695–701 12. St John Sutton MG, Mercier LA, Giuliani ER, Lie JT (1980) Atrial myxomas: a review of clinical experience in 40 patients. Mayo Clin Proc 55:371–376 13. Fyke FE, Seward JB, Edwards WD, Miller FA, Reeder GS, Schattenberg TT et al (1985) Primary cardiac tumours: experience with 30 consecutive patients since the introduction of two – dimensional echocardiography. J Am Coll Cardiol 5:1465–1473 14. Tsuchiya F, Kohno A, Saitoh R, Shigeta A (1984) CT findings of atrial myxoma. Radiology 151(1):139–143 15. Liu ZC (1989) Imaging diagnosis of intracardiac myxoma (report of 23 cases). Zhonghua Fang She Xue Za Zhi 23(3):134–136
