Left atrial giant myxoma with mitral valve obstruction

doi:10.21203/rs.3.rs-2954476/v1

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Case Report

Left atrial giant myxoma with mitral valve obstruction

https://doi.org/10.21203/rs.3.rs-2954476/v1

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Myxomas are the most common primary cardiac tumors, predominantly found in the left atrium. They can present with various symptoms, including cardiac manifestations such as heart failure, arrhythmias, stroke, and systemic findings. Surgical resection of myxomas has a favorable prognosis with low complication rates. This case report presents a 41-year-old man with dyspnea, abdominal distension, and edema diagnosed with a giant left atrial myxoma. Successful surgical removal resulted in symptom resolution. Cardiac myxomas are rare, with a prevalence of about 0.03% in the general population. Clinical manifestations, including intracardiac obstruction, embolization, and constitutional symptoms, depend on the tumor's location, size, and mobility. Echocardiography is essential for diagnosis, while additional imaging techniques such as CT and MRI can provide further information. Surgical excision is typically necessary to prevent embolization, with favorable outcomes, although there is a risk of arrhythmias, conduction abnormalities, recurrence, and development of new lesions.

Cardiac tumors

Myxoma

Mitral Stenosis

Left Atrium

Myxomas are tumors that originate from stromal cells, which are capable of neural and endothelial differentiation (1). Myxomas are the most common primary cardiac tumors, and they usually arise in the left atrium (2).

The clinical presentation of myxomas varies from no symptoms up to subtle or evident manifestations of heart failure, syncope, arrhythmias (cardiac symptoms are the most common presentation), or stroke with or without systemic findings such as fever, fatigue, weight loss, leukocytosis, stroke-like signs, etc. Therefore, high clinical suspicion of the tumor is essential, as myxomas are predominantly diagnosed after six months of symptom initiation (3). Myxomas can potentially cause mechanical obstruction of the blood flow on the atrioventricular valves, similar to mitral or tricuspid stenosis (4).

The surgical resection of myxomas has a good prognosis and low risk of complications (5). Cardiac autotransplantation, along with atrial reconstruction or transplantation, are potential treatment options for addressing recurrent atrial myxoma (6, 7).

A 41-year-old man presented to the hospital with a history of dyspnea on exertion, abdominal distension, and edema of the lower extremities. A mid-diastolic murmur and a loud second heart sound were detected on the physical exam. A transthoracic echocardiogram revealed a reduction in left ventricular ejection fraction and a mass, which was approximately 8 cm in size (Fig. 1). The patient underwent a mass surgical removal successfully. The mass was consistent with a myxoma. Six months after the intervention, the patient does not develop symptoms or signs of heart failure.

Cardiac myxoma is an uncommon condition. The known prevalence in the general population is approximately 0.03%. The occurrence of cardiac myxoma per year is estimated to be between 0.5 and 1 case for every million individuals. Myxomas are the most frequent benign primary cardiac tumor type, accounting for 50–85% of cases (8).

How cardiac myxoma manifests clinically depends on where they are located, their size, and their mobility. It is characterized by a triad of intracardiac obstruction, embolization, and constitutional symptoms (4). Intracardiac obstruction is frequently associated with polypoid myxomas, particularly in the mitral valve, resulting in a condition known as mitral valve pseudo-obstruction. This obstruction often leads to cardiac symptom development, and in some cases, it can be accompanied by pulmonary hypertension. Conversely, in cases where right atrial myxomas are present, obstruction of the tricuspid valve can occur, leading to manifestations such as heart failure or even collapse (8). Embolization commonly occurs in papillary-type myxomas due to their delicate texture and fragility. Approximately 30–40% of individuals with myxomas experience embolism (4). Cardiac myxomas often present with constitutional symptoms that resemble inflammatory or connective tissue disorders. These symptoms are more prevalent in women and are more commonly observed in right-sided and larger multicentric myxomas. Typical symptoms include malaise, loss of appetite, fever, joint pain, and weight loss (8). The release of cytokines from the tumor, particularly interleukin-6 (IL-6), may contribute to the underlying pathophysiology (9).

Several methods can be used to confirm the presence of a myxoma. Echocardiography is the essential diagnostic tool for identifying cardiac myxomas. Furthermore, complementary imaging methods such as computed tomography (CT) scanning and magnetic resonance imaging (MRI) have a supporting role. Chest X-ray and electrocardiography (ECG) provide limited value. Histopathology is necessary to confirm the diagnosis. Genetic testing is crucial in cases where myxomas are inherited within families.

Transthoracic echocardiography, which is a practical investigation method, frequently provides sufficient information required for surgical resection. This technique enables the diagnosis, as well as the determination of the tumor's location, size, shape, and connections. On the other hand, transesophageal echocardiography offers higher sensitivity and specificity, allowing the detection of small tumors, tumors situated in unconventional locations, and potential multi-chamber myxomas (10, 11).

MRI offers valuable insights into the dimensions, configuration, surface features, and movement of myxomas. Typically, myxomas appear as a mass that is similar in signal intensity to surrounding tissues on T1-weighted images while appearing brighter on T2-weighted images. Additionally, there may be areas of decreased signal intensity on one or both of these imaging sequences. Moreover, analyzing the tissue properties can help distinguish between a tumor and a thrombus (12). Calcification, which occurs in approximately 10–30% of cases, is best detected using CT as the preferred technique (13, 14).

Surgical excision of cardiac myxoma is frequently required as an urgent measure to minimize the likelihood of tumor embolization (8). The evidence about myxoma surgical removal shows excellent overall outcomes in mortality, short- and long-term survival, and tumor recurrence (15, 16). Nevertheless, in one study, approximately 26 percent of patients exhibited atrial arrhythmias or atrioventricular conduction abnormalities after the surgery. Furthermore, there is a risk of myxoma recurrence, which can be observed in 2 to 5 percent of cases, and the potential development of new lesions (17).

In conclusion, myxomas, the most common primary cardiac tumors, often present with various clinical symptoms, including heart failure, arrhythmias, and embolic events. Prompt diagnosis and surgical resection are crucial for favorable outcomes. Echocardiography remains the cornerstone for diagnosis, while MRI and CT provide valuable complementary information. Surgical excision is usually successful and associated with low mortality rates. However, postoperative complications such as atrial arrhythmia and atrioventricular conduction abnormalities may occur in some patients. Additionally, there is a small risk of myxoma recurrence or the development of new lesions. Overall, early detection and timely intervention improve prognosis in patients with cardiac myxoma.

Ethics approval and consent to participate. All procedures performed in studies involving human participants or on human tissue were in accordance with the ethical standards of the institutional and/or national research committee and with the 1975 Helsinki Declaration and its later amendments or comparable ethical standards.

Consent for publication Written informed consent was obtained from the patient for using the data without patient particular for publication.

Competing interests The authors declare that they have no competing interests.

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Left atrial giant myxoma with mitral valve obstruction

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Abstract

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Introduction

Case history

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