Abstract
Cardiac myxomas are the most common primary intracardiac tumors in adults. Although benign from a histopathological point of view, they can be life-threatening for the patient. We present a case of an unusually giant left atrial myxoma causing mitral valve obstruction and pulmonary hypertension successfully treated with surgical resection. Our patient was a 54-year-old woman who presented to our emergency complaining of progressive dyspnea of about 1 month duration. On cardiovascular examination, we found crackling rales at both lung bases and a diastolic murmur in the mitral focus. Transthoracic echocardiography revealed a giant mass in the left atrium connected to the interatrial septum and extended into the left ventricle during diastole which caused obstruction of the left ventricular inflow tract and a pulmonary hypertension. The patient underwent a median sternotomy with the removal of left atrial mass and patch closure of the interatrial septum. Histopathological examination confirmed the diagnosis of myxoma. One week later, the patient was discharged without any complications. Giant left atrial myxoma although a benign mass, can induce dramatic symptoms and be life-threatening. In case of suspicion, it must be diagnosed early by transthoracic echocardiography and urgently managed by surgical removal.
Keywords: Cardiac myxoma, cardiac tumors, echocardiography, giant myxoma, left atrium
INTRODUCTION
Myxoma is the most common type of cardiac tumor that occurs primarily in the left atrium.[1] Clinical symptoms depend on the size and location, it can be revealed by complications such as embolic accidents, heart failure, and pulmonary hypertension. Transthoracic echocardiography is the mainstay of diagnosis. Surgical removal of the tumor allows an excellent long-term prognosis. We report a case of a giant left atrial myxoma in a 54-year-old woman that caused mitral valve obstruction and pulmonary hypertension.
CASE REPORT
A 54-year-old womanwith a medical history of hypothyroidism presented to our emergency complaining of progressive dyspnea of about 1 month duration.
On physical examination, she had a normal blood pressure and heart rate. Her oxygen saturation was 92% in ambient air. Pulmonary auscultation found crackling rales at both lung bases. Cardiac auscultation found normal heart sounds and a diastolic murmur in the mitral focus. The electrocardiogram was in sinus rhythm.
Routine laboratory investigations were unremarkable. Frontal chest X-ray showed no lung parenchymal abnormality, heart shadow was normal. Transthoracic echocardiography revealed a 7.5 cm × 4.5 cm mass in the left atrium connected to the interatrial septum and prolapsing into the left ventricle through the mitral valve during diastole [Figure 1a and b], causing severe functional mitral valve stenosis (The mean transmitral gradient = 14 mmHg) [Figure 1c] and a pulmonary hypertension (The systolic pulmonary arterial pressure = 60 mmHg). Left ventricular systolic function and geometry were normal.
Figure 1.
Two dimension echocardiography. (a) parasternal long-axis view, (b) apical four chamber view) and continuous wave doppler (c) showing a giant mass almost completely filling the left atrium and prolapsing into the left ventricle through the mitral valve during diastole causing severe functional mitral valve stenosis (The mean trans-mitral gradient = 14 mmHg)
The patient was admitted to the cardiothoracic surgery department. She underwent a median sternotomy. Surgical resection of the tumor with patch closure of the interatrial septum was performed successfully. Gross examination of the specimen showed a partially encapsulated pedunculated mass measuring 7.5 cm × 4.5 cm × 2.5 cm and weighing 55 g [Figure 2]. The tumor was attached to the interatrial septum with a pedicle. Direct inspection of the mitral valve revealed no abnormalities and intraoperative transesophageal echocardiography (TEE) did not reveal pathologic mitral regurgitation. Histopathological examination of the surgical specimen confirmed the presumed diagnosis of a myxoma. One week later, the patient was discharged without any complications.
Figure 2.
Macroscopic appearance of the extracted tumor: Giant left atrial myxoma
DISCUSSION
Primary tumors of the heart are rare, atrial myxomas are the most common benign primary cardiac tumors and occur in as many as three in 1000 patients,[2] Cardiac myxomas usually develop in the left atrium and most commonly affect women such as the case of our patient.[3] The symptoms are related to location, embolization, and the propensity to obstruct blood flow through the mitral valve.[4] Mitral valve obstruction caused by atrial myxoma represents an important hemodynamic consequence leading to symptoms of congestive heart failure, pulmonary hypertension, syncope, and sudden death.[5] TTE is the gold standard investigation that provides information regarding the structure, location, and hemodynamics of the tumor, which is later confirmed histologically after removal.[6,7] TEE or cardiac magnetic resonance imaging may be indicated when characterization of the mass by TTE is limited due to poor patient echogenicity and poor diagnostic windows. TEE is superior to TTE in evaluating cardiac tumors, in particular for myxomas.[8] It allows the exclusion of differential diagnoses and better analysis of the basis of implantation of the tumor, its relationship with the mitral valve, and a precise morphological study of the heart chambers.[9] In our case, since the patient was echogenic on TTE and the mass was very suggestive of myxoma, further examination by TEE was not indicated. Our patient presented symptoms of left heart failure with a diastolic murmur consistent with severe functional mitral stenosis and pulmonary hypertension. The hypermobile aspect of the mass with a hemodynamic impact is a very urgent indication for surgery in order to avoid embolization.[10]
The surgical excision must include a substantial portion of the normal endocardium near the base of implantation to prevent recurrence. As literature reveals[11] there are low surgical mortality and good long-term results in patients with cardiac myxoma.
The immediate postoperative course of our patient was favorable. After 1 year of follow-up, he remains asymptomatic. The cardiac ultrasound did not show any abnormalities.
CONCLUSION
Giant left atrial myxoma although a benign mass, can induce dramatic symptoms and be life-threatening. In case of suspicion, it must be diagnosed early and urgently managed by surgical removal.
Declaration of patient consent
The authors certify that they have obtained allappropriate 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 thejournal. The patients understand that their names and initial s will not be published and due efforts will bemade to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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