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| CASE REPORT |
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| Year : 2022 | Volume
: 11
| Issue : 2 | Page : 60-62 |
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Left atrial myxoma in a patient with hypertrophic obstructive cardiomyopathy
Jayaprasad Narayanapillai, Suresh Madhavan
Department of Cardiology, Government Medical College, Kottayam, Kerala, India
| Date of Submission | 21-Feb-2022 |
| Date of Decision | 10-Apr-2022 |
| Date of Acceptance | 17-May-2022 |
| Date of Web Publication | 24-Jun-2022 |
Correspondence Address:
DM Jayaprasad Narayanapillai
DM, Department of Cardiology, Government Medical College, Kottayam, Kerala
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/jcpc.jcpc_12_22
Hypertrophic cardiomyopathy and left atrial myxoma are important cardiac causes of syncope. We present an extremely rare combination of these two diseases in a middle-aged man with syncope. Transthoracic echocardiographic images show the typical features of both conditions. There were no features of progressive cardiomyopathic lentiginosis (LEOPARD syndrome) which is known to be associated with both the conditions. Keywords: Echocardiography, hypertrophic cardiomyopathy, left atrial myxoma, syncope
How to cite this article:
Narayanapillai J, Madhavan S. Left atrial myxoma in a patient with hypertrophic obstructive cardiomyopathy. J Clin Prev Cardiol 2022;11:60-2 |
| Introduction | |  |
Cardiac conditions are common causes of syncope. Hypertrophic obstructive cardiomyopathy (HOCM) and left atrial myxoma both can present with syncope. HOCM and atrial myxoma were reported separately in patients with progressive cardiomyopathic lentiginosis (LEOPARD syndrome).[1],[2] Concomitant occurrence of HOCM and atrial myxoma is reported rarely in the literature. We report a patient with recurrent syncope who had coexisting HOCM and left atrial myxoma.
| Case Report | | |
A 50-year-old man presented to our hospital with a history of recurrent syncope for the past 3 months. Syncope was unrelated to exertion and occurred in the upright posture. There was no history of chest pain, dyspnea, or palpitation. He had no comorbidities, and there was no family history of sudden cardiac death (SCD). On examination, he had normal heart sounds and a midsystolic murmur in the lower left sternal border which increased in intensity with standing and strain phase of the Valsalva maneuver. His electrocardiogram showed left ventricular hypertrophy with a strain pattern. A provisional diagnosis of HOCM was made and transthoracic echocardiography was done. His transthoracic echo showed typical features of HOCM including asymmetric septal hypertrophy and dynamic left ventricular outflow tract (LVOT) obstruction. Interventricular septum measured about 18 mm during diastole. In addition, there was a well-circumscribed mass in the left atrium measuring 4.2 cm × 2.5 cm in size attached to the interatrial septum in the fossa ovalis region prolapsing into the left ventricle during diastole. The echocardiographic findings were typical of myxoma [Figure 1]. M-mode evaluation showed systolic anterior motion of the anterior mitral leaflet and the myxoma filling the mitral orifice during diastole. There was systolic fluttering of the aortic valve in M-mode due to subaortic obstruction [Figure 2]. Doppler examination showed a dynamic LVOT gradient of about 45 mmHg and a mean transmitral gradient of 6 mmHg during diastole suggestive of mitral obstruction [Figure 3]. He did not have any features of LEOPARD syndrome. His coronary angiogram was normal. He underwent successful resection of myxoma and the diagnosis was confirmed by histology. A postoperative echocardiogram showed LVOT obstruction and mild mitral regurgitation [Figure 4]. LVOT gradient was 25 mmHg at rest and 40 mmHg on the strain phase of the Valsalva maneuver, and there was no transmitral gradient. Septal myectomy was not considered in this patient as he did not have significant symptoms of LVOT obstruction in the past and LVOT gradient was <50 mmHg. | Figure 1: TTE parasternal long-axis view (a) and apical four-chamber view (b) asymmetrical hypertrophy of LV and myxoma in the left atrium prolapsing to LV through the mitral valve. TTE: Transthoracic echocardiography, LV = Left ventricular
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 | Figure 2: :(a) M-mode echo showing SAM and myxoma filling mitral orifice during diastole. (b) Aortic valve M-mode shows coarse systolic fluttering. SAM = systolic anterior motion
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 | Figure 3: Doppler echo evaluation showing dynamic LVOT obstruction characterized by late-peaking dagger-shaped signal with a peak gradient of 45 mmHg. An elevated transmitral gradient is also seen. LVOT = Left ventricular outflow tract
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 | Figure 4: (a) Postoperative TTE showing HCM with mild mitral regurgitation. (b) Postoperative Doppler evaluation showing LVOT gradient without transmitral gradient. TTE = Transthoracic echocardiography, LVOT = Left ventricular outflow tract, HCM = Hypertrophic cardiomyopathy
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| Discussion | | |
The association of atrial myxoma with hypertrophic cardiomyopathy (HCM) was reported only rarely in the literature.[3],[4] HCM and myxoma were reported separately in patients with LEOPARD syndrome. LEOPARD syndrome is defined by lentigines in combination with at least two other hallmarks: electrocardiographic conduction abnormalities, ocular hypertelorism, structural cardiac abnormalities, abnormal genitalia, retarded growth, and deafness.[1] A literature search by Gi et al. identified six case reports of HCM and myxoma.[5] None of these patients had LEOPARD syndrome. All had myxoma in the left atrium. Among them only one patient had syncope. None of the patients had LVOT gradient of more than 30 mmHg. Both HCM and myxoma can be diagnosed by transthoracic echocardiography although transesophageal echo provides clear details of myxoma.[6] Cardiac magnetic resonance imaging is very useful in diagnosing HCM and cardiac tumors including tissue characterization.[7] Our patient had typical features of HOCM and myxoma in TTE and hence further imaging was not done. Syncope in our patient was most likely due to left atrial myxoma as it was not exertional and was of recent onset. He did not have any family history of HCM or SCD. A study of genetic mutations will be worthwhile in such patients to delineate the pathogenetic mechanism behind the combination of these two diseases.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Polani PE, Moynahan EJ. Progressive cardiomyopathic lentiginosis. Q J Med 1972;41:205-25.  |
| 2. | Rees JR, Ros FG, Keen G. Lentiginosis and left atrial myxoma. Br Heart J 1973;35:874-6. |
| 3. | Kanemoto N, Nishiumi N, Inoue H, Koide S, Kawada S, Shotsu A. Combined apical hypertrophic cardiomyopathy and left atrial myxoma. Chest 1992;101:1149-50. |
| 4. | Abdou M, Hayek S, Williams BR 3 rd. Atrial myxoma in a patient with hypertrophic cardiomyopathy. Tex Heart Inst J 2013;40:462-4. |
| 5. | Gi WT, Sedaghat-Hamedani F, Shirvani Samani O, Kayvanpour E, Herpel E, Arif R, et al. Cardiac Myxoma in a Patient With Hypertrophic Cardiomyopathy. JACC Case Rep. 2020;2:378-383. |
| 6. | Pérez de Isla L, de Castro R, Zamorano JL, Almería C, Moreno R, Moreno M, et al. Diagnosis and treatment of cardiac myxomas by transesophageal echocardiography. Am J Cardiol 2002;90:1419-21. |
| 7. | Abbas A, Garfath-Cox KA, Brown IW, Shambrook JS, Peebles CR, Harden SP. Cardiac MR assessment of cardiac myxomas. Br J Radiol 2015;88:20140599. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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