JUSINDO, Vol. 7 No. 1, Januari 2025

p-ISSN: 2303-288X, e-ISSN: 2541-7207

Jurnal Sehat Indonesia: Vol. 7, No. 1, Januari 2025 | 270

Heart Failure Caused by Incidentally Discovered Right Atrial

Myxoma: A Rare Case Report

Rahmatulhusna Atikah

, Budi Patria Meilus

RSUD Dr. Achmad Darwis, Sumatera Barat, Indonesia

Email: atikahch3@gmail.com

ABSTRACT

Keywords:

Cardiac myxoma; right

atrial myxoma;

echocardiography; heart

failure; case report

Primary cardiac tumors, though uncommon, play a significant

role in medical practice. Cardiac myxoma is the most common

benign cardiac tumor, characterized by varying symptoms

depending on the location and size of the tumor. We report the

case of a 44-year-old woman with complaints of shortness of

breath, upper abdominal pain, and leg oedema. Right atrial

myxoma was incidentally found after an echocardiography

examination, which is an essential tool for detecting and

diagnosing cardiac tumors. The patient exhibited clinical

improvement with inotropic therapy and antibiotics and was

referred for surgical removal of the tumor. Cardiac myxoma

surgical procedures generally have a favorable prognosis with

a high survival rate. However, postoperative complications

such as arrhythmias and infections require close monitoring.

Thus, early diagnosis and appropriate management are

essential in addressing cardiac myxoma.

Coresponden Author:Rahmatulhusna Atikah

Email: atikahch3@gmail.com

Artikel dengan akses terbuka dibawah lisensi

Introduction

Primary cardiac tumors are rare, with an overall incidence rate reported to be less

than 0.1%, primarily consisting of benign neoplasms (Vroomen et al., 2015). Cardiac

myxoma is the most common benign cardiac tumor, accounting for nearly 50% of cases,

followed by lipomas and fibromas. Recent research conducted in Spain reported adjusted

incidence rates of approximately 1.6 cases per million population when standardized to

the global population and 2.1 cases per million when standardized to the European

population (Pérez-Andreu et al., 2019).

Unique macroscopic and microscopic features characterize cardiac myxoma.

Microscopically, myxoma consists of spindle-shaped cells of varying sizes, ranging from

small to large. The nucleus of myxoma cells is typically round, oval, or elongated, and

their cytoplasm tends to be eosinophilic, appearing pinkish. Moreover, myxoma cells

often exhibit an excess in cell count. Macroscopically, myxoma can assume a round or

oval shape, sometimes featuring a stalk. It has a gelatinous consistency with a soft texture

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on palpation. The surface can be smooth with regular edges or villous with irregular

borders, and it may even have multiple lobes, resembling fragile gelatine (Cianciulli et

al., 2019; Hasan et al., 2020). The color of myxoma typically ranges from white to

yellowish or brown, with varying sizes, averaging around 2 cm to 6 cm but occasionally

growing conspicuously large, up to 15 cm. The weight of myxoma also varies, ranging

from 15 grams to 180 grams (Mustafa et al., 2018).

Clinical manifestations of myxoma vary depending on factors such as size, location,

shape, growth rate, stalk length, mobility, and degenerative changes within the tumor,

such as hemorrhage or cell necrosis (Yuan et al., 2017). Myxoma can range from being

asymptomatic (when the tumor size is less than 4 cm) to life-threatening symptoms,

including vessel obstruction due to emboli (Aiello & Campos, 2016). While clinical

presentations vary, the classical triad of myxoma, consisting of constitutional or systemic

symptoms, obstructive symptoms, and embolic events, remains a distinct characteristic

of the tumor (Alassal et al., 2019). Systemic symptoms include fever with high body

temperature, weakness, myalgia (muscle pain), arthralgia (joint pain), weight loss, and

loss of appetite (Yuan et al., 2017). Obstructive symptoms may involve dyspnea, recurrent

pulmonary edema, and syncope. Symptoms related to embolic events encompass stroke,

chest pain, limb ischemia, and the discovery of masses in distal extremities (Velez Torres

et al., 2020).

Primary cardiac tumors are rare, with an incidence of less than 0.1%, predominantly

consisting of benign neoplasms such as myxomas. Among these, right atrial myxomas

are even less common, with the majority of cases involving the left atrium. While cardiac

myxomas are well-documented, the occurrence of incidentally discovered right atrial

myxomas presenting as heart failure remains underexplored. Previous studies have

predominantly focused on left atrial myxomas, their clinical manifestations, and surgical

outcomes (Oktaviono et al., 2024), leaving a gap in understanding the distinct

characteristics, clinical presentation, and challenges associated with right atrial myxomas.

This case report addresses this gap by presenting a rare instance of heart failure

caused by a right atrial myxoma discovered incidentally. Unlike typical cases where left

atrial involvement dominates the discussion, this report highlights the unique diagnostic

and management challenges posed by right atrial myxomas. The findings underscore the

critical role of advanced imaging techniques, such as echocardiography, in early detection

and management while also providing insights into the clinical course and outcomes of

this unusual presentation. This contributes to the growing body of knowledge on the less

commonly reported manifestations of cardiac myxomas.

Case

A 44-year-old woman presented with a chief complaint of upper abdominal pain

radiating to the chest for one week before hospital admission. The patient also complained

breathing difficulty, which worsened when lying flat but improved when bending

forward. She experienced orthopnea and paroxysmal nocturnal dyspnea. Additionally, the

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patient complained of edema in both lower extremities. There were no reports of

presyncope or syncope.

Upon physical examination, the patient appeared unwell with increased respiratory

effort, requiring up to 10 liters of oxygen per minute (lpm), while other vital signs were

within normal limits. Heart sounds were normal, and the pulse was regular. The jugular

venous pressure was elevated by 6 cm above the sternal angle, and a grade 2/6 diastolic

murmur was audible at the apex.

Figure 1. a) Patient’s electrocardiography

Figure 1 b) Chest x-ray of the patient with bronchopneumonia, cardiomegaly

without pulmonary congestion, and elevation of the right diaphragm

The patient underwent transthoracic echocardiography (TTE), which revealed

significant enlargement of the right atrium (RA) and right ventricle (RV), along with the

presence of a mass in the RA (Video 1). The patient's left ventricular ejection fraction

(LVEF) was 40%, with a D-shaped morphology. Valvular function assessment revealed

mild aortic regurgitation (AR), mild mitral regurgitation (MR), and moderate tricuspid

regurgitation (TR).

Based on the chest X-ray and echocardiography results, the patient was diagnosed

with right atrial myxoma with accompanying bronchopneumonia. For the initial therapy,

the patient received the following medications: cefotaxime 2x1 gram as per the microbial

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pattern, furosemide 2x20 mg, atorvastatin 1x10 mg, carvedilol 1x6.25 mg, clopidogrel

1x75 mg, and acetylcysteine 3x200 mg. The patient also experienced cardiogenic shock

on day 1 of treatment, and thus received inotropic therapy with dopamine at 10

mcg/kg/minute. During the subsequent days of treatment, the patient responded well to

therapy. After eight days of close monitoring, the patient was discharged with the

following outpatient medications: carvedilol 1x6.25 mg, clopidogrel 1x75 mg,

atorvastatin 1x10 mg, acetylcysteine 3x200 mg, spironolactone 2x25 mg, and was

referred for surgical intervention therapy.

Result and Discussion

Myxoma is the most common primary heart tumor which occurs in the adult

population, with the left atrium often being the most frequently affected location (Saha et

al., 2023). Myxoma of the right atrium has a more stable and compact characteristic

compared to left atrial myxomas, with broader attachments to the septum or atrial wall

(Saha et al., 2023). Left atrial myxomas produce symptoms similar to mitral stenosis,

such as shortness of breath and hemoptysis, typically with a brief, episodic nature, which

can lead to syncope and rapidly progress to heart failure. Right atrial myxomas can also

obstruct the tricuspid valve, with manifestations possibly leading to heart failure (Agstam

et al., 2020; Alamri et al., 2019) or even collapse (Agrawal et al., 2018). Right atrial

myxomas can also exhibit episodic symptoms and may have a rapid progression.

In this paper, we present the case of a 44-year-old woman with the chief complaint of

worsening shortness of breath over the week before hospital admission.

Electrocardiogram (ECG) findings often do not yield specific results, and signs of right

atrial hypertrophy were observed in the patient. Radiographic examination typically

shows a picture of cardiomegaly, as seen in this patient, which represents heart

enlargement. However, cardiomegaly is nonspecific for myxomas and can be observed in

various heart conditions (Hasan et al., 2020).

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Figure 2. Patient’s echocardiography

The patient also underwent echocardiography. Echocardiography, including

transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE), is a

commonly used and highly useful imaging method for diagnosing heart myxomas

(Alassal et al., 2019). Echocardiography can detect the presence of masses in the heart,

and TEE is generally more sensitive than TTE (Hasan et al., 2020). Echocardiography

can also provide information on the size, morphology, site of attachment of the tumor,

and its impact on blood flow in the heart. Echocardiography should be used to confirm

the diagnosis of heart myxoma if there are; 1) a diastolic murmur at the apex that changes

with posture and no history of rheumatic heart disease; 2) recurrent arterial emboli; 3)

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syncopal episodes influenced by postural changes; 4) prolonged subfebrile fever; 5)

anemia without rheumatic heart disease or infective endocarditis, and; 6) refractory heart

failure with a poor response.

The patient underwent general condition improvement and treatment for

bronchopneumonia infection, after which she was referred for outpatient surgery.

Surgical intervention is the primary choice for addressing heart myxoma. The prognosis

for patients after surgery is generally good, both in the short and long term (Alassal et al.,

2019; Kusumoto et al., 2019). The risk of mortality during surgery is low, with

approximately 0.5% mortality rate, and the postoperative mortality rate is around 2%

(Hasan et al., 2020). Even in elderly patients, survival rates after surgery are reported to

be favourable (Cianciulli et al., 2019). One common technique used is the approach

through the right atrium and transeptal approach, where the left atrial tumor is removed

through the atrial septum and followed by simple closure (Cianciulli et al., 2019).

Currently, this technique is typically performed via sternotomy using cardiopulmonary

bypass, as it is considered safe and has a low mortality rate (Hasan et al., 2020). Although

heart myxoma surgery is generally successful, some postoperative complications can

occur. Arrhythmia is the most common complication (33.3%), followed by infection

(19.0%), vasoplegic syndrome (19.0%), pericardial effusion (14.2%), acute kidney injury

(9.5%), atrial septal defect (ASD) (9.5%), stroke (4.8%), and pulmonary embolism

(4.8%) (Cianciulli et al., 2019).

Limitations of the Case Report

Despite its contributions, this case report has several limitations. First, it focuses on

a single patient, limiting the generalizability of findings to broader populations. Second,

follow-up details post-surgery, such as long-term clinical outcomes and quality of life,

were unavailable, which could have provided further insights into the prognosis and

recovery process. Additionally, the case lacks a comprehensive genetic or molecular

analysis of the myxoma, which could contribute to understanding the tumor's etiology

and potential hereditary factors. Future studies with larger sample sizes and a

multidisciplinary approach are necessary to better elucidate the unique characteristics and

management of right atrial myxomas.

Conclusion

The incidental discovery of cardiac myxomas, as demonstrated in this case,

highlights the significance of thorough cardiac evaluation in patients with unexplained

cardiovascular symptoms. Echocardiography remains a valuable diagnostic tool for early

detection and appropriate management of cardiac myxomas, preventing life-threatening

complications such as heart failure. Early diagnosis and timely surgical intervention are

crucial in managing cardiac myxomas successfully.

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