Left atrial myxoma mimicking severe mitral stenosis and severe pulmonary hypertension

Balarabe Sulaiman Aminu; Kamilu Musa Karaye; Nasir Ishaq; Shehu Kana; Muzammil Muhammad Yakasai; Mahmud Umar Sani

doi:10.4103/njc.njc_3_20

CASE REPORT

Year : 2020 | Volume : 17 | Issue : 1 | Page : 84-86

Left atrial myxoma mimicking severe mitral stenosis and severe pulmonary hypertension

Balarabe Sulaiman Aminu¹, Kamilu Musa Karaye², Nasir Ishaq², Shehu Kana², Muzammil Muhammad Yakasai², Mahmud Umar Sani²
¹ Department of Medicine, Muhammadu Abdullahi Wase Specialist Hospital, Kano, Nigeria
² Department of Medicine, Aminu Kano Teaching Hospital, Kano, Nigeria

Date of Submission	12-Jan-2020
Date of Acceptance	01-Apr-2020
Date of Web Publication	30-Jun-2020

Correspondence Address:
Dr. Balarabe Sulaiman Aminu
Department of Medicine, Muhammadu Abdullahi Wase Specialist Hospital, Kano
Nigeria

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/njc.njc_3_20

Abstract

Cardiac myxomas are the most commonly encountered benign intracardiac tumors. Although the left atrium is the most commonly involved in majority of cases, myxoma can arise in any cardiac chamber. We present the case of a 53-year-old female with background hypertension, who presented with the features of heart failure and found to have large left atrial myxoma mimicking severe mitral stenosis and severe pulmonary hypertension. She had the excision of the myxoma mass. The case illustrates that atrial myxoma is the diagnosis to keep in view as an occasional cause of cardiac murmur even in a society with a high prevalence of rheumatic valvular heart disease and cardiomyopathies.

Keywords: Mitral stenosis, myxoma, pulmonary hypertension

How to cite this article:
Aminu BS, Karaye KM, Ishaq N, Kana S, Yakasai MM, Sani MU. Left atrial myxoma mimicking severe mitral stenosis and severe pulmonary hypertension. Nig J Cardiol 2020;17:84-6

How to cite this URL:
Aminu BS, Karaye KM, Ishaq N, Kana S, Yakasai MM, Sani MU. Left atrial myxoma mimicking severe mitral stenosis and severe pulmonary hypertension. Nig J Cardiol [serial online] 2020 [cited 2023 May 29];17:84-6. Available from: https://www.nigjcardiol.org/text.asp?2020/17/1/84/288650

Introduction

Cardiac myxomas are the most commonly encountered benign intracardiac tumors. Although the left atrium is the most commonly involved in 75%–83% of cases, myxomas can arise in any cardiac chamber.^[1],[2] While most cases of cardiac myxomas occur sporadically, familial lesions are seen in 10% of cases. Myxomas can also occur in association with a heterozygous genetic disease, the Carney's Syndrome.^[3],[4] The classical clinical presentation of myxoma includes a triad of intracardiac obstruction, embolism and constitutional symptoms of fever, weight loss, or symptoms resembling connective tissue disease.^[5],[6],[7]

Case Report

Mrs F. S was a 53-year-old homemaker, who presented to us at Muhammadu Abdullahi Wase Specialist Hospital Kano (MAWSHK) in March 2018, with a 12-month history of hypertension, 4-month history of palpitations, 2-month history of chest pain, shortness of breath on mild exertion, and significant weight loss. She had no fever, cough, orthopnea, paroxysmal nocturnal dyspnea, or body swelling. Other aspects of the systemic review were not remarkable. She was not known to have diabetes mellitus or bronchial asthma. Examination revealed an ill-looking middle-aged woman, who was afebrile (body temperature = 36.8°C, not pale, anicteric, no digital clubbing, or pedal edema. Her weight was 73 Kg, with a body mass index of 23.6 Kg/m². Her oxygen saturation (SPO2) was 96% on room air. She had a regular pulse rate of 92 beats/min (bpm), blood pressure of 140/100 mmHg, had a loud pulmonary component of second heart sound and Grade III tricuspid regurgitation (TR) murmur, no tumor flop sound heard. Electrocardiogram showed sinus rhythm with a heart rate of 87 bpm, right axis deviation (QRS axis + 120°), left atrial (LA) enlargement, but no left ventricular hypertrophy or ST-T wave changes. Echocardiogram revealed a single large well-circumscribed echogenic mass in the LA measuring 64 mm × 56 mm, attached to interatrial septum with a stalk, confined to the LA in systole [Figure 1]. In diastole, the mass was seen to be prolapsing across the mitral valve (MV) into the left ventricle, almost completely obstructing it and causing severe functional mitral stenosis [Figure 2]. The mitral and other valves were all structurally normal. The right atrium (RA) and the ventricle (RV) were dilated with RA area in systole of 23 cm², RV basal diameter of 52 mm but had a preserved RV systolic function (tricuspid annular plane systolic excursion = 19 mm). RV systolic tissue Doppler velocity (S') was 18.9 cm/s, RV Myocardial Performance Index was 1.2 (using tissue Doppler image method). There was severe functional TR with a TR peak velocity of 123.3 mmHg and an estimated RV systolic pressure of 138.3 mmHg. Computed tomographic coronory angiography was essentially normal with coronary calcium score of 10 (Agaston score of 10). Other investigations such as complete blood counts, serum lipids profile, and renal and liver function tests were essentially normal. The patient was diagnosed to have a large LA myxoma complicated by severe pulmonary hypertension. She was referred to the Lagos State University Teaching Hospital where she had surgical resection of the tumor and MV repair in June 2018. Histology of the excisional biopsy material confirmed atrial myxoma. She did very well postoperatively and is currently being followed up in MAWSHK.

Figure 1: Apical 4-chamber view showing myxoma mass occupying the entire left atrium

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Figure 2: Myxoma mass prolapsing across mitral valve in diastole [Parasternal long-axis view]

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She is currently on the treatment for her hypertension with lisinopril 5 mg od and metoprolol succinate 25 mg od and doing well.

Discussion

The present report is on a patient with a large LA myxoma mimicking severe MS and with severe pulmonary hypertension, who presented with features suggestive of heart failure. The case illustrates that atrial myxoma is a diagnosis to keep in view as an occasional cause of cardiac murmur even in a society with a high prevalence of rheumatic valvular heart disease and cardiomyopathies.

Primary cardiac tumors occur infrequently, with an incidence of 0.0017%–0.19% in autopsy series performed in nonselected populations.^[1],[2],[3] Cardiac myxomas are more common among women with a female-to-male ratio of 2:1 and occur more frequently between the ages of 30 and 60 years,^[3] incidentally our patient is a female and falls within this age group.

A similar case of giant LA myxoma mimicking severe MV stenosis and severe pulmonary hypertension was reported by Mouine et al.^[1] In addition, there are earlier reports of cardiac myxoma from Nigeria.^[3],[7] Clinical manifestations of myxoma are determined by the location, size, mobility, and friability and can be categorized into triad of systemic symptoms, embolism, and intracardiac obstruction.^[5],[6],[7]

The breathlessness she was having is likely due to severe pulmonary hypertension that developed as a result of retrograde transmission of elevated LA pressure due to LA outflow obstruction by the large myxoma mass. This sustained elevated pressure leads to increased pulmonary capillary pressure, arterial remodeling, impaired vascular reactivity, and endothelial dysfunction, leading to increase in mean pulmonary artery pressure.^[8],[9],[10] The significant weight loss observed in this patient could be as a result of anorexia due to increased production of proinflammatory cytokines.

Echocardiography is the most important and widely available method in the diagnosis of myxoma. Early echocardiographic examination played a vital role in the diagnosis and management of this patient, emphasizing the need for the diagnosis of any patient presenting with similar features. Computed tomography coronary angiography was utilized to exclude obstructive coronary artery disease which saved time and resources for invasive coronary angiography.

Acknowledgments

Our appreciation goes to Dr. Bode Falase for helping the patient to have free open heart surgery at the Lagos State University Teaching Hospital, Lagos, Nigeria (LASUTH).

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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