An unusual cause of acute ischemic cardioembolic stroke in a middle aged lady: An incidentally detected large Left Atrial Myxoma – A Case Report.

doi:10.21203/rs.3.rs-1990911/v1

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An unusual cause of acute ischemic cardioembolic stroke in a middle aged lady: An incidentally detected large Left Atrial Myxoma – A Case Report.

https://doi.org/10.21203/rs.3.rs-1990911/v1

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Introduction

Primary cardiac tumors are rare but are surgically curable. Incidence of primary cardiac tumors varied between 0.0017% and 0.03 % in previous studies. Left atrial (LA) myxoma is the most common primary cardiac tumor, locally invading heart or lung tissue, causing left ventricular dysfunction, arrhythmia, pericardial effusion or cardiac tamponade. Again, tumor fragment breakdown or tumor thrombi can result in embolic manifestations, the most deadly manifestations being neurologic. Here we report an unusual cause of acute ischemic cardio-embolic stroke in a middle-aged lady, an incidentally detected large LA myxoma.

Case presentation

A 45-year-old hypertensive lady presented at the Emergency Department (ED) with acute onset aphasia, right facial droop, right sided hemiplegia and left sided subconjunctival haemorrhage. A large LA myxoma was incidentally detected through Transthoracic Echocardiography, which was the cause for the stroke. Excision of LA myxoma was done through a standard median sternotomy, under cardiopulmonary bypass, using the right atrial approach. Histopathology examination of the excised jelly-like mass confirmed it to be an LA myxoma. The patient has been doing well at a follow up period of six months.

Conclusions

Cardiac tumors are rare, but embolic complications can prove to be devastating, especially neurologic. A strong clinical suspicion followed by a thorough physical examination is mandatory as the history can often be misleading, with non-specific signs and symptoms. Trans-esophageal Echocardiography remains the gold standard for diagnosis. Once detected, the tumor needs to be excised at the earliest to prevent further complications.

Intracardiac myxoma

Embolism

Cardio-embolic Stroke

Incidental Finding

Cardiac surgery

Case Report

Primary cardiac tumors are a rare entity but are surgically curable. Incidence of primary cardiac tumors varied between 0.0017% and 0.03 % in previous studies. Seventy-five per cent of these tumors are benign, with myxoma accounting for about 50 - 55% of cases and rhabodomyoma comprising of 25% of lesions [1-3]. Seventy-five per cent of myxomas originate in the left atrium of the heart, 20% of them are seen to occur in the right atrium, whereas ventricular tumors are rare [1].

Usually, myxomas are sporadic, but in 5% cases, autosomal dominant inherited cases have been known to occur. The typical sporadic tumor is seen in women aged 30 to 60 years and is usually solitary in nature. Most commonly, they are located on the limbus of the fossa ovalis. Left Atrial (LA) myxomas cause symptoms via several mechanisms, including obstructing the flow of blood through the cardiac chambers and/or valves, leading to right or left heart failure. They may locally invade surrounding heart or lung tissue, causing left ventricular dysfunction, arrhythmia, pericardial effusion and even cardiac tamponade. Again, the breakdown of tumor fragments or tumor thrombi can result in embolic manifestations, the most deadly manifestations of which are neurologic [4-9]. LA myxomas are slow growing tumors. Larger tumors usually have a smoother surface, while smaller tumors may be friable or villous in appearance and as a result, are more susceptible to embolization (as in this case). Histopathological examination findings include scattered and unstructured cells on a myxoid stromal background of mucopolysaccharides and glycosaminoglycans [4].

LA myxomas classically present with non-specific general and cardio-respiratory symptoms, most commonly dyspnea, orthopnea, paroxysmal nocturnal dyspnea, pulmonary edema, cough, hemoptysis, peripheral edema, and fatigue. Fever and weight loss and deranged biochemical laboratory parameters (including anemia, increased globulins, increased erythrocyte sedimentation rate (ESR), increased C-Reactive protein (CRP)) are seen in about one-thirds of patients. On physical examination, a ‘tumor plop’ (a low-pitched diastolic sound immediately after S2) may be heard on auscultation, which is a giveaway [4, 5].

Echocardiography represents a simple and non-invasive diagnostic method, showing the tumor mass, obstruction in the circulatory pathway, and possible sources of emboli. Trans-esophageal echocardiography is presently the gold standard in diagnosing an LA myxoma.

The course of surgically untreated patients with cardiac myxomas is highly variable. Not much is known about the frequency of embolization in these patients, the chances of repeated embolization if the tumor is not removed surgically, or the natural history of disease in patients presenting with constitutional symptoms alone, without undergoing treatment.

Treatment of LA myxomas focuses on prompt surgical excision of the tumor mass to minimize the possibility of embolic or cardiac complications [10].

Here we report a unique case of acute ischemic cardio-embolic stroke in a middle-aged lady, with no prior clinical history except hypertension. A large Left Atrial (LA) myxoma was incidentally detected, which was the cause for the stroke.

A 45-year-old hypertensive lady presented at the Emergency Department (ED) with acute onset aphasia, right facial droop, right sided hemiplegia and left sided subconjunctival haemorrhage for a day before admission. There was no history of vomiting, loss of consciousness or convulsion. Upon physical examination, she was afebrile, was in atrial fibrillation (pulse 90-102/min), had a blood pressure of 180/100 mm of Hg, room air saturation of 98% and a respiratory rate of 20/min. Her score on the Glasgow Coma Scale was 11 (E₄V₁M₆,where E represents Eye opening, V represents verbal response and M represents Motor response), which suggested moderate brain injury. Her plantar reflex was flexor on the left side and extensor on the right side. Her muscle power was 0/5 and 0/5 for the right upper and lower limbs respectively while it was 4/5 and 4/5 for the left upper and lower limbs respectively. She was on Tablet Amlodipine 5 mg orally, daily and did not give any history of drug allergy. Her pupils were bilaterally 2.5 mm in size and reacting to light. Abdomen was soft and non-tender. Bilateral vesicular breath sounds were heard on auscultating the chest and a “tumor plop” was apparent.

After admission, she underwent the real time Reverse Transcription-Polymerase Chain Reaction (RT-PCR) test for coronavirus disease (COVID-19) which came out to be negative. Computed Tomography (CT) brain done showed a left frontal infarct. Magnetic Resonance Imaging (MRI) of the brain with MR angiography (Figure 1), showed a large area of acute onset infarct with significant diffuse restriction in the left fronto-temporal cortical subcortical and deep white matter region, left basal ganglia and caudate nucleus, with multiple tiny areas of infarcts in the right temporal, bilateral parietal and periventricular region and no obvious intracranial vascular abnormality. CT angiography of the brain revealed degenerative changes of the bilateral carotid system with a focal eccentric plaque in the Right Internal Carotid Artery (ICA), causing 20% intraluminal compromise.

Transthoracic Echocardiography done at the bedside showed an LA myxoma (4.2 cm x 2.2 cm), attached to the inter-atrial septum (IAS) and projecting through the mitral valve in diastole, with no significant left ventricular (LV) inflow obstruction and adequate biventricular systolic function with an LV Ejection Fraction (EF) of 61%. Trans-esophageal Echocardiography done pre-operatively, confirmed these findings (Video 1).

The subconjunctival hemorrhage in the left eye was managed conservatively, as advised by the hospital Ophthalmology team of physicians.

Thus the patient was provisionally diagnosed as a case of LA myxoma, presenting with acute cardio-embolic stroke and was referred to the Department of Cardiothoracic Surgery for excision of the LA myxoma.

Surgical technique

The surgery was done through a standard median sternotomy, via the right atrial approach, under cardiopulmonary bypass, with aortic and bicaval cannulation. A standard right atrial incision was made, approximately 1 cm parallel to the atrioventricular groove. This exposure helped in adequate immediate visualization of the jelly-like mass (Video 2). The tumor was then removed with about 1 cm margin of tissue, taking care not to injure the mitral annulus, the area of conduction tissue, and the tricuspid annulus. The resulting defect was closed primarily with a 6-0 polypropylene suture. A copious amount of normal saline was used to irrigate the ventricular cavity. The right atrium was closed with 5-0 polypropylene, continuous sutures. After adequate deairing of the heart, the patient was weaned off cardiopulmonary bypass.

The histopathology examination of the excised jelly-like mass (shown in Figure 2) confirmed the provisional diagnosis of an LA myxoma. Figure 3 demonstrates the histopathology findings of the LA myxoma. The surgery was uneventful and she was shifted to the intensive therapy unit (ITU) on elective mechanical ventilation and minimal inotropic supports, in a hemodynamically stable condition and in sinus rhythm. She was extubated after 8 hours and her intercostal chest drains were removed on the second day following surgery. She was seen by the Neurology team of physicians on a day-to-day basis and also underwent physiotherapy by the Department of Physical Medicine. She was finally discharged from hospital, eight days after surgery. She was subsequently followed up by the Cardiothoracic Surgical team, the Neurology team of physicians and the physicians from the department of Physical Medicine. She came for her first follow up after one month and could speak and walk with a little help from her husband. Her right sided upper and lower limb muscle power had improved significantly. When she came for follow up next at three months and after six months after surgery, she could talk normally and mobilize without support. She has been hemodynamically stable, in sinus rhythm and fully ambulant at follow up.

The patient and her husband were happy with the way she was managed in the hospital setting and at follow up.

Thus an incidentally detected LA myxoma was the unusual cause of acute ischemic cardioembolic stroke in this middle aged lady, who had no prior clinical history except hypertension. Cardiac tumors are rare, but embolic complications can prove to be devastating, especially neurologic. LA myxomas are easily missed, being relatively slow growing tumors, presenting with constitutional symptoms. A strong clinical suspicion followed by a thorough physical examination is mandatory as the history can often be misleading, with non-specific signs and symptoms, as in this case. Echocardiography clinches the diagnosis and Trans-esophageal Echocardiography remains the gold standard in diagnosing an LA myxoma. Once detected, the tumor needs to be excised at the earliest to prevent further complications. The case in question had an uneventful surgery following incidental detection of LA myxoma, following an acute cardio-embolic stroke and has been doing well at a follow up period of six months.

LA – Left Atrial

ED – Emergency Department

EVM – Eye opening, Verbal response, Motor response (on the Glasgow Coma Scale)

ITU – Intensive Therapy Unit

IAS – Inter-atrial septum

LV – Left Ventricular

EF – Ejection Fraction

RT-PCR - Reverse Transcription-Polymerase Chain Reaction

COVID-19 – Coronavirus disease

CT – Computed Tomography

MRI – Magnetic Resonance Imaging

ICA – Internal Carotid Artery

Ethics approval and consent to participate

The case was approved by the institutional ethical committee, namely the Clinical Research Ethics Committee, for publication. (PHH&RCLCREC/3692/2022 dated 29.06.2022).

All procedures involving human participants were in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Written, informed consent was obtained from the patient and her husband for the procedure and further management.

Consent for publication

Written, informed consent was obtained from the patient and her husband for the use of clinical data and images relevant to this case, for publication.

Availability of data and materials

The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.

Competing interests

The authors declare that they have no competing interests.

Funding

Not applicable

Code availability

Not applicable

Authors’ contributions

SB analyzed and interpreted the patient data and was the major contributor in writing the manuscript. AB conceptualized and designed the study, critically reviewed and edited the manuscript. SB collected the data. SB, AB, SP, SD and AKD were actively involved in drafting the manuscript. All authors read and approved the final version of the manuscript.

Acknowledgments

We gratefully acknowledge the institutional Neurology team and the Physical Medicine team of doctors, nurses and paramedics for their help in management of this case.

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Video1.mp4
Video 1: Pre-operative video of Trans Esophageal Echocardiography.
Video2.mp4
Video 2: Surgical video of the LA myxoma being visualized through the right atrial incision.

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An unusual cause of acute ischemic cardioembolic stroke in a middle aged lady: An incidentally detected large Left Atrial Myxoma – A Case Report.

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Introduction

Case Presentation

Conclusions

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