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Cardiac myxomas are rare clinical findings. They are frequently found in the left atrium and more commonly affect women. Clinical presentation can vary widely and symptoms can be vague and non-specific. We present a case of a 67-year-old woman presenting with 3 weeks of progressive heart failure symptoms that failed to respond to oral diuretic therapy. On physical exam, she was found to have a diastolic murmur, rumble and an early diastolic plop. Transthoracic echocardiogram revealed a 5.6 cm × 2.5 cm × 4.3 cm left atrial mass attached to the mitral valve causing left atrial outflow obstruction. The patient subsequently underwent surgical resection of the mass with resolution of symptoms immediately thereafter. Lack of recognition of this pathologic process as a cause of heart failure symptoms and lack of a quality physical exam can lead to a delay in diagnosis and treatment.
Primary cardiac tumors are rare with a reported incidence approximately 200 per 1,000,000.1 Among primary cardiac tumors, approximately 75% are benign.2 Of the benign tumors, nearly half are cardiac myxomas, which predominantly arise from or near the interatrial septum and extend into the left atrium.1 Cardiac myxomas more commonly affect women and can arise in all age groups but present with a mean age of 62 years old ± 13 years.3
Clinical presentation can vary widely. Most commonly, patients present with symptoms of obstructive heart failure but other presenting symptoms may be vague and non-specific such as arrhythmia, systemic embolization, or constitutional symptoms (ie, fever, night sweats and weight loss).4 In one fifth of the cases, a myxoma can be asymptomatic and discovered as an incidental finding.5
A 67-year-old woman presented to her primary care physician with progressive worsening dyspnea on exertion, orthopnea and bilateral lower extremity edema. She was treated empirically for presumed congestive heart failure with oral diuretic therapy and a non-urgent transthoracic echocardiogram was ordered. Her symptoms initially improved with oral diuretic therapy, but after approximately a week, her symptoms progressively worsened and prompted her to come to the emergency department for further evaluation and treatment.
Upon evaluation in the emergency department, she had normal vitals but a diastolic murmur, rumble and early diastolic plop were heard on cardiac auscultation. She did not have jugular venous distension, hepatomegaly, hepatojugular reflux or any lower extremity edema. Her baseline labs revealed normal electrolytes, renal function, hepatic function, and complete blood count. A troponin I was <0.02 ng/mL (reference < 0.05 ng/mL) and N-terminal pro-brain natriuretic peptide was slightly elevated at 397 pg/mL (reference < 125 pg/mL). A chest X-ray showed cardiomegaly but otherwise without any acute cardiopulmonary pathologic process (Figure 1). Twelve-lead electrocardiogram showed an ectopic atrial rhythm with an incomplete right bundle branch block and non-specific T-wave changes. A transthoracic echocardiogram revealed a 5.6 cm × 2.5 cm × 4.3 cm multi-lobulated, gelatinous left atrial mass attached to the lower portion of the interatrial septum that prolapsed through the mitral valve causing moderate obstruction with a 10 mm transvalvular gradient (Figure 2). A coronary angiogram was performed demonstrating only mild, diffuse atherosclerotic coronary disease. She was then referred to a cardiothoracic surgeon for surgical removal of the tumor. Intraoperative transesophageal echocardiogram images (Figures 3 and and4)4) and gross anatomic specimens (Figures 5 and and6)6) were obtained. The mass was sent to the pathology department for histological analysis, which confirmed the diagnosis of a cardiac myxoma. Her postoperative course was uneventful and she was discharged on post-operative day 4 without any oral diuretic therapy. She has been followed in the clinic with serial transthoracic echocardiograms demonstrating no recurrence of the left atrial mass.
Cardiac neoplasms may present as obstructive, embolic, and constitutional symptoms such as fever, weight loss, or systemic symptoms thought to be due to tumor-induced interleukin-6.6 Left-sided cardiac tumors can obstruct blood flow through the mitral valve mimicking signs and symptoms of mitral stenosis, including dyspnea, orthopnea, syncope, and paroxysmal nocturnal dyspnea.6 In addition, it may induce valvular damage and impair myocardial contractility.6 Some tumors can invade into local myocardium to cause conduction disturbances, which can lead to arrhythmias such as supraventricular tachycardia or ventricular tachycardia.6 Cardioembolic features of left-sided cardiac masses can manifest as stroke, visceral infarction, myocardial infarction or peripheral emboli. 6
Our patient initially presented with heart failure symptoms to her primary care physician. During that initial presentation, a diastolic murmur, rumble and plop were likely missed on physical examination. An outpatient transthoracic echocardiogram was ordered, but unfortunately the patient was not able to get it done in time before her symptoms worsened, which brought her to the emergency department for evaluation. It ultimately led to a delay in her diagnosis and treatment of a left atrial myxoma.
This case stresses the importance of a detailed and thorough physical examination. In addition, the pathologic importance of a diastolic murmur was missed during her initial encounter, resulting in a delay in diagnosis and treatment. On physical examination, a diastolic murmur is pathologic until proven otherwise and should always warrant a provider to promptly perform further diagnostic studies.6 The differential diagnoses of a diastolic murmur include aortic insufficiency, pulmonic regurgitation, mitral stenosis, tricuspid stenosis, atrial myxoma, Austin Flint murmur, and left-to-right shunts.7
The murmur of a left and right atrial myxoma can have similar auscultatory findings as mitral and tricuspid stenosis, respectively7. It is typically a mid-diastolic, crescendo diastolic murmur.7 The diastolic murmur occurs as the atrial myxoma causes atrioventricular valve obstruction. The murmur from a left atrial myxoma can be difficult to distinguish from mitral stenosis but the character and intensity of the murmur from an atrial myxoma may change with position.8 Auscultatory abnormalities were found in 64% of patients with atrial myxomas.6 In addition, a “tumor plop” may be heard in only 15% of patients, which is suspected to represent either sudden tensing of the tumor stalk or impact of the tumor against the septum.9
Even though a thorough physical examination can be suggestive of a cardiac tumor, initial diagnostic tool is typically a transthoracic two-dimensional echocardiogram. It can provide information including location, size, mobility, and potential consequences.6 Depending on the location, a transesophageal echocardiogram can provide greater spatial resolution of the tumor and provide further details with regards to the site of attachment.10 Other diagnostic modalities include cardiac magnetic resonance imaging (MRI) or cardiac computed tomography (CT). Both modalities can provide greater detailed images to better characterize the tumor beyond echocardiography, if needed.
This case reminds us of the significance of a detailed and thorough physical examination and pursuing early diagnostic testing for new heart failure findings. Although diastolic murmurs are often missed on physical exam, any patient presenting with a new clinical syndrome of heart failure should urge a provider to obtain urgent transthoracic echocardiogram. Reluctance to proceed to higher level of diagnostic testing can result in a delay in the patient's diagnosis and treatment.
None of the authors report a conflict of interest.
Personal identifiers were excluded to maintain the patient's confidentiality. Informed consent was not obtained for this case report.