Multiple Infected Cardiac Myxoma In Young Female Patient complicated with multiple systemic infarctions: Case report and review of literature

doi:10.21203/rs.3.rs-4828468/v1

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Case Report

Multiple Infected Cardiac Myxoma In Young Female Patient complicated with multiple systemic infarctions: Case report and review of literature

https://doi.org/10.21203/rs.3.rs-4828468/v1

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Background: Cardiac myxomas are the most common primary heart tumors, yet their occurrence in young patients, particularly when infected and leading to multiple systemic infarctions, is exceedingly rare. This case highlights the critical need for early diagnosis and intervention.

An 18-year-old female presented with orthopnea, low-grade fever, chest tightness, and pulmonary edema. She had experienced dyspnea, weight loss, recurrent abdominal pain, and chronic anemia over the past six months. Examination revealed congested lungs, sweating, tachycardia, and laboratory findings indicative of inflammation. Echocardiography identified multiple obstructing masses in the left atrium moving into the left ventricle during diastole, causing severe mitral regurgitation. Imaging revealed old infarcts in the brain, liver, and spleen. Blood cultures were positive for Enterococcus faecalis, and empirical antibiotics were initiated.

Urgent surgery was performed, including left atrial myxoma resection, mitral valve replacement with a bioprosthetic valve, left atrial appendage closure, and closure of the interatrial septum. Postoperative echocardiography confirmed normal left ventricular function and a well-functioning bioprosthetic mitral valve. The patient tolerated the procedure well and was discharged on a regimen of oral antibiotics for six weeks.

Discussion: Infected cardiac myxomas, though rare, pose significant risks due to their potential for systemic embolization and severe complications. This case emphasizes the necessity of comprehensive diagnostic evaluation and timely surgical intervention to prevent catastrophic outcomes.

Conclusion: This case underscores the importance of a multidisciplinary approach and early surgical intervention in managing complex cases of infected cardiac myxoma with systemic embolization. Our findings reinforce the need for vigilance in diagnosing and treating such rare but life-threatening conditions.

Cardiac Myxoma is a common intracardiac benign neoplasm but a rarely becomes infected, presenting with symptoms of fever or with sepsis of unknown origin. The occurrence of three infected cardiac myxomas in single patient is highly unusual. Herein, we present this rare case and the surgical treatment provided to this condition.

On February 25, 2024, a18-year-old female her height is 150 cm and weight is 55 kg. BMI: 24.4 kg/m2, O positive blood group; presented to our cardiac center with Orthopnea, low-grade fever, chest tightness and pulmonary edema, she was admitted to the critical cardiac unit (CCU). For the past six months, she had experienced Dyspnea, weight loss and recurrent abdominal pain attacks and chronic anemia. Upon her examination she looks sick with congested lungs, sweating, tachycardia, laboratory findings strongly indicated inflammation. Echocardiography showed huge multiple obstructing masses in left atrium (LA) moving to left ventricle (LV) in diastole with severe mitral regurgitation. Imaging studies showed old brain, liver and spleen infarcts. Three Blood cultures were obtained and empirical antibiotic therapy was initiated as infected Myxoma and mitral valve endocarditis were suspected. Chest and lung radiographs showed normal sized a cardiac silhouette, adequate pulmonary markings, and no focal lesions. An electrocardiogram showed sinus tachycardia (heart rate, 115-120 beats/ min) and no myocardial ischemia. Laboratory investigations revealed elevated levels of C-reactive protein (187 mg/L) and Procalcitonin (1.76 µg/L). Transesophageal echocardiography confirmed that huge cauliflower mass arose from the mitral annulus and protruded into the LV during diastole (Fig.1) causing functional moderate mitral valves stenosis. Another small size mass attached to the interatrial septum looks superior to the fossa ovale.

There is severe posterior directed mitral valve regurgitation, no signs of valve destruction or perforation, no annular dilation. Left ventricular systolic function and size is normal. The left atrium is mildly dilated (4.4cm) and no lesion on other valves.

No thrombus is detected in the left atrial appendage.Right ventricular systolic pressure is elevated at

40-50mmHg. The consensus was made to be operated urgently. Blood cultures obtained preoperatively had been positive for bacteria Enterococcus feacalis. Preoperative routine workup done for her which was Computed Tomography (CT) Scan chest /Abdomen / Brain, it revealed left basal pnemonitis, atelectasis, moderate pericardial effusion, small bilateral pleural effusion, moderate hepato-splenomegly with multiple masses suggesting infarcts and multiple brain chronic bilateral infarcts. Lab results showed low Hb% 6gm. All benefits/ risks of LA Myxoma resection and Mitral valve replacement with bioprosthetic valve discussed with the patient and her family then they agreed, and signed the informed consent. Intravenous antibiotics empirically were started. Upon transfer to Operation Theater (OR), the patient still had a low-grade fever.

Procedure details: Left Atrial Myxomas Resection, Mitral valve Replacement by Bioprosthetic valve and Left Atrial Appendage Closure and closure of interatrial septum by synthetic pericardial patch. The patient is shifted from CCU to OR. Access to the left radial artery was done, then she was sedated, intubated and ventilated then the central venous lines were done. Intraoperative transesophageal Echocardiography (TEE) probe inserted. A median longitudinal sternotomy is made. The patient was heparinized and cannulated and cold blood cardioplegia was given. Then through right atrial and trans-septal approach, the Myxoma masses were excised completely with the safety margin bases: 1^st mass 2X2 cm arising from the septum above the Fosa Ovalis with wide base, 2^nd mass size is 4X5cm raising from the roof of the LA, The 3^rdmass size is 5X5cm raising from the septum 5cm above the postero-medial commissure.(Fig1). The LV checked for any mass or fragments. The Mitral valve was assessed there are signs of tumor involvement as multiple myxomatic masses on the anterior leaflets and there is severe reugurge by Saline test. The Mitral valve excised with chordal preservation. The annulus sized and adapt 27mm. Tissue Bioprosthetic Medtronic Mosaic 27MM implanted by using 2/0 Ethibond pledgeted stitches. Test indicates well functioning leaflets. The Left atrial appendage was closed. The Inter-atrial septum has been closed by 4/0 proline suture using Gore pericardial membrane and the other defects were closed by direct Proline suture). The heart resumes normal sinus rhythm. Closure of the right atrium by double layer 5/0 Prolin on beating heart. The pericardial Pacing wires were implanted to right ventricle wall. Gradual weaning off bypass machine was achieved successfully and uneventful. TEE done showed well seated and functioning mitral valve bioprosthesis, normal LV function and no residual masses. The patient tolerated the operation well and was then transferred to CVICU in a stable condition, maintaining adequate mean arterial pressure, urine output, oxygenation and ventilation, good ABG, normal ECG and no bleeding. Smooth recovery and ward course.

Postoperative Echocardiography study done in sinus rhythm. The left ventricle is normal in size. Left ventricular systolic function is normal, estimated Ejection Fraction = 55‐60%.No regional wall motion abnormalities noted. Septal motion is consistent with postoperative state.
The right ventricle is normal in size and function. The Right ventricular systolic pressure is 25-30mmHg.

Bioprosthetic mitral valve is well seated with normal function, the mean gradients across the valve = 6 mmHg. No paravalvular leak.

The antibiotic therapeutic regimen empirically described as per infective endocarditic protocol (Cefatrixone, gentamycine, Amoxicillin ) she had allergy to Vancomycin. Then modified according to the culture and sensitivity result. The cultures from the myxoma specimen was negative as well as another blood sample taken on the 7^th postoperative day was negative then the patient was discharged home on 12^th POD. The intravenous antibiotics were switched to oral and were continued for 6weeks after surgery. After 3 months the warfarin discontinued, the patient had no recurrence of infection or tumor.

The histopathalogical analysis revealed characteristic features consistent with myxoma (Fig 2), supporting the clinical and imaging findings.

Myxoma comprises 50% of all benign cardiac tumors in adults and 15% of such tumors in children. The peak incidence is between the third and sixth decades of life, though cases have been obsereved in younger patients e.g., 18 year old as in our case. 94% of these tumors are solitary with approximately 75% occuring in the left atrium. (1, 2). The incidence of asymptomatic cardiac myxoma varies widely from 0.01–28.4%, and has been increasing with the advancement in imaging technologies and enhanced screening opportunities.(3) Despite being the most common benign cardiac tumour, infected and complicated Cardiac myxoma is very rare. Infected cardiac myxoma is highly fragile, with the potential to embolize, leading to catastrophic conditions such as systemic bacteremia, systemic mycotic embolism, and disseminated intravascular coagulation (DIC). (4) Clinically myxoma is presents with classic triads: intracardiac obstruction with congestive heart failure (67%); signs of embolization (29%); systemic or constitutional symptoms such as fever (19%); weight loss or fatigue (17%); and immunologic manifestations of myalgia, weakness, and arthralgia (5%). All these manifestations were classically present in our case.(1)

The differential diagnosis of infected myxoma primarily includes uninfected myxoma, as fever can

Occur without infection, as well as mural endocarditis and infected intracardiac thrombus.

Myxoma is associated with systemic embolism,(5, 6) although the risk appears to be greater when the myxoma is infected. Since the clinical presentation of infected myxoma may be similar to that of uninfected myxoma, blood cultures should be done whenever a patient with myxoma presents with fever. Trans thoracic Echocardiography and even transesophageal (TEE) should be performed in patients with fever of unknown origin when initial investigations are inconclusive.(7, 8)

Our patient presented with an intermittent fever, which occurs in approximately 10–20% of patients with cardiac myxoma (9, 10). Inflammatory markers cytokines may cause the fever, which makes it difficult to differentiate between infected and non-infected myxoma preoperatively which should prompt further diagnostic investigations to rule out infection or other complications. Therefore, in a febrile patient with myxoma, it is necessary to do blood cultures and sensitivity testing before initiating preoperative antimicrobial therapy. Yuan reported that Gram-positive cocci such as Streptococcus and Staphylococcus accounted for about 70% of the causative organisms (11). In our case, Enterococcus Fecalis was detected in preoperative blood cultures and this was very unusual. In some reports, antimicrobial agents are administered as per the treatment guidelines for infective endocarditis (3, 4). Thus, we started the treatment described in the guidelines for infective endocarditis (9) and used intravenous antibiotics for four weeks post-surgery.

30–50% of left atrial myxomas cause embolisms (5, 7) The incidence of embolization was reported to be two to three times higher in patients with infected than non-infected cardiac myxoma because of the fragile fibrin thrombus on the tumor surface, as seen in infective endocarditis (8, 11) In our case, various old and fresh multiple cerebral, splenic, and hepatic infarctions were also detected by CT imaging.

Considering the risk of embolization, surgery should be performed as soon as possible after diagnosis in such cases. We suggest there is no benefit in delaying surgery until the results of blood cultures are known.

We reported the successful urgent surgical management of a case of complicated infected multiple cardiac myxoma. This case underscores the importance of the multidisciplinary approach, comprehensive diagnostic evaluation which reinforcing the need for early detection and timely intervention in patients with cardiac myxomas, especially when complicated by severe mitral regurgitation and systemic embolization.

Author Information: Mohammed Aldahmashi, Department of cardiac surgery, prince Abdallah Bin Abdelaziz Bin Musaad Cardiac Center, Arar City, Saudi Arabia, [email protected]. Assistant professor, Thamar university, Yemen.

Author contributions: M Aldahmashi is the case operator and conceptualized the review, wrote and approved the manuscript.

M abdelwhab has major contributor in writing the manuscript discussion

Khaled yasin performed the histological examination of the mass.

All authors read and approved the final manuscript.

Corresponding author: Mohammed Aldahmashi, MD, Consultant Cardiothoracic surgeon PAAMCC, Assistant Professor Thamar University, Yemen.

Consent for publication: the patient signed informed consent for publication

Competing interests: The authors declare that they have no conflict of interest.

Funding: No funding

Availability of data and materials: The data of this case study are available from the corresponding author on reasonable request.

Acknowledgements I would like to extend my deepest gratitude to my colleagues for their invaluable assistance and support in the preparation and publication of this case study.

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Alassal MD, Nasser A, Ahmed H, Ibrahim S, et al. (2019) An Unusual Presentation of Cardiac Myxoma; a Case Report and Review of Literature. Cardiovascular Thoracic.
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Sato T, Saji N, Kobayashi K, et al. A case of cerebral embolism due to cardiac myxoma presenting with multiple cerebral microaneurysms detected on first MRI scans. Clin Neurol 2016; 56(2): 98–103.
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No competing interests reported.

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Multiple Infected Cardiac Myxoma In Young Female Patient complicated with multiple systemic infarctions: Case report and review of literature

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