An unusual right atrial myxoma triggering presumed takotsubo syndrome in a 97-year-old female

Summary

A 97-year-old female was admitted to the emergency department of our hospital due to paroxysmal nocturnal dyspnea and chest pain. At the hospital admission, the patient manifested transient psychomotor agitation and dysarthria. On physical examination, blood pressure was 115/60 mmHg and pulse 96 b.p.m. On blood tests, troponine I was 0.08 ng/mL (normal range <0.04 ng/mL). Electrocardiography (ECG) showed sinus rhythm and ST segment elevation in both inferior and anterior leads except in lead V1. Transthoracic echocardiography (TTE) revealed a right atrial multilobulated, hypermobile, echogenic cauliflower mass (maximum size of 5 cm × 4 cm), which was attached to the tricuspid lateral annulus with a short stalk (Figure 1A). The right atrial mass, which had filiform extremities and was found to prolapse through the tricuspid valve into the right ventricle, was ascribed to a peduncolated myxoma. Its motion was very rapid and uncoordinated, with increased peak antegrade velocity (V_max = 35 cm/s), as precisely measured by pulsed wave tissue Doppler imaging (PW-TDI) (Figure 1B). Estimated left ventricular ejection fraction (LVEF) was normal (60%), and no significant valvulopathy was detected. Finally, a bulging of the interatrial septum with right-to-left shunt, through a patent foramen ovale (PFO), was observed by using colour Doppler (Figure 1C). Acute ischaemic lesions were excluded by brain computed tomography scan.

Given the extremely advanced patient’s age, the absence of tricuspid valve obstruction, and the haemodynamic stability, the patient was conservatively treated with acetylsalicylic acid 100 mg/day, subcutaneous enoxaparin 4000 UI/day, and bisoprolol 2.5 mg/day.

During hospitalization, serial ECG documented diffuse T-wave inversion associated with corrected QT (QTc) interval prolongation (QTc 497 msec). On repeat TTE, apical balloning was diagnosed with significant left ventricular systolic dysfunction (LVEF <35%) (Figure 1D). Finally, repeat blood tests revealed mild troponin I increase (1.7 ng/mL).

Figure 1.

(A). Two-dimensional transthoracic echocardiographic apical four-chamber view revealing a right atrial multilobulated, hypermobile, echogenic cauliflower mass, attached to the tricuspid lateral annulus with a short stalk, compatible with a peduncolated myxoma (arrow). The right atrial mass had filiform extremities and was found to prolapse through the tricuspid valve into the right ventricle. LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle. (B) Pulsed wave tissue Doppler imaging employed for assessing the right atrial mass mobility. Its motion was very rapid and uncoordinated, with increased peak antegrade velocity, measured by positioning the sample volume of pulsed wave tissue Doppler imaging on the body’s mass. (C) Two-dimensional transthoracic echocardiographic apical five-chamber view showing right-to-left interatrial shunt, through a patent foramen ovale (arrow), by using colour Doppler. Ao, aorta; LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle. (D) Two-dimensional transthoracic echocardiographic apical four-chamber view showing apical dyskinesis and ballooning. LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle.

During the subsequent days of hospitalization, all symptoms of agitation disappeared, and the clinical conditions of the patient gradually improved. On Day 6 after presentation, the patient was transferred to a long-term care facility with a cardioprotective treatment. Echocardiographic control performed 4 weeks after hospital discharge documented normalization of LV systolic function (estimated LVEF was 55%).

In the present case, the coronary artery embolization from atrial myxoma was clinically excluded by the trend of ECG changes and the classic apical ballooning pattern on 2D-TTE examination, with rapid recovery of regional apical function within few weeks.

Although coronary angiography and brain magnetic resonance imaging were not performed, the clinical presentation, the typical ECG changes, the classic echocardiographic pattern during the acute phase, and the minimal enzimatic release, together with the complete recovery of LV systolic function within few weeks, allowed us to confirm the diagnosis of presumed takotsubo syndrome (TTS), likely triggered by the cerebral embolization of a right atrial myxoma through a PFO.

To date, only sporadical cases of TTS caused by acute cerebrovascular embolism and the progressive involvement of the central autonomic network from embolization of left atrial myxomas have been reported.^1–4 Conversely, as far as we know, no previous author described the association between right atrial myxoma and TTS in very elderly females aged over 95 years.

Evaluation of intracardiac masses motility by using PW-TDI might be an improvement over visual assessment, as it is able to discriminate the fine movements of intracardiac masses by their characteristic pattern of incoherent motion,⁵ providing a more precise definition of mass mobility.⁶

Lead author biography

Andrea Sonaglioni is a clinical cardiologist with a peculiar skilling in echocardiographic techniques and with a special interest in cardiological research.

Data availability

The data underlying this article will be shared on reasonable request to the corresponding author.

Ethics approval

This study was conducted in accordance with the World Medical Association Declaration of Helsinki. The Local Ethics Committee decided that ethics approval was not required in a single case image. No personal identifying information was included in this manuscript.