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Manal.Touilite, Safaa zahlane, Yasmina Zakaria,Mohammed Chraa, Nissrine Louhab

Neurology department, University Teaching

Hospital Mohammed VI

E-mail: manal.touilite@gmail.com

Introduction

Conclusion

Cardiac myxoma is a tumor that can present with different clinical features and often-positive diagnosis can be difficult. Medical imaging investigations, especially echocardiography, allow the identification, localization and impact of the cardiac tumor, but the positive and differential diagnosis is done with the help of the histo- pathological confirmation exam. The standard therapy is represented by surgical resection and postoperative prognosis is good, with the resumption of structural and functional status of the heart .

A rare cause of multiple ischemic strokes reveling cardiac myxoma complicated by endocarditis and PAD (critical limb ischemia): Case report

Références

Myxoma is the most common primitive cardiac tumor diagnosed in adults; it represents 50–85% of the benign tumors. It is more common in the 4th–7th decades of life, it is rarely diagnosed in children .
The name myxoma comes from the abundant myxoid stroma rich in proteoglycans. Myxoma’s composition and origin generated debate.
Actual data provided by genetic and immune-histo- chemical studies confirmed it as a benign tumor originating from subendothelial multipotent mesenchymal heart cells.
Most myxomas arise in the left atrium (LA), 60–80% of cases, with typical insertion on the fossa ovalis, less often they can be found in other locations.

Introduction: Myxoma is the most common primitive cardiac tumor diagnosed in adults; it represents 50–85% of the benign tumors. It is more common in the 4th–7th decades of life, it is rarely diagnosed in children .The name myxoma comes from the abundant myxoid stroma rich in proteoglycans .

Case presentation: Fifty-year-old male patient, non-smoker, presented sudden onset of left-sided hemicorporal deficit associated with dysarthria and left facial asymmetry with ocular deviation, complicated by altered consciousness, without any other associated signs, particularly no critical events reveling cardiac myxoma complicated by endocarditis and PAD.

Conclusion: Cardiac myxoma is a tumor that can present with different clinical features and often-positive diagnosis can be difficult. Medical imaging investigations, especially echocardiography, allow the identification, localization and impact of the cardiac tumor, but the positive and differential diagnosis is done with the help of the histo- pathological confirmation exam .

Keywords: cardiac myxoma, ischemic stroke , PAD, critical limb ischemia ,endocarditis, TTE

Abstract:

Discussion

1.Wilkes D, Charitakis K, Basson CT. Inherited disposition to cardiac myxoma development. Nat Rev Cancer, 2006, 6(2): 157–165.

2. Griffiths D, Sturm J. Epidemiology and etiology of young stroke. Stroke Res Treat, 2011, 2011:209370.

3 Krikler DM, Rode J, Davies MJ, Woolf N, Moss E. Atrial myxoma: a tumour in search of its origins. Br Heart J, 1992, 67(1):89–91.

4. Kodama H, Hirotani T, Suzuki Y, Ogawa S, Yamazaki K. Cardiomyogenic differentiation in cardiac myxoma expressing lineage-specific transcription factors. Am J Pathol, 2002

Case presentation

Fifty-year-old male patient, non-smoker, presented sudden onset of left-sided hemicorporal deficit associated with dysarthria and left facial asymmetry with ocular deviation, complicated by altered consciousness, without any other associated signs, particularly no critical events. The patient stayed for 4 days in the intensive care unit and was then transferred to our department for further management.
Physical examination (at current hospitalization) showed satisfying general condition, normal body mass index (BMI), A non-proportional and impure left hemi-pyramidal syndrome increased muscle tone and minor motor deficit of the lower left limb, exaggerated muscle stretch reflexes and plantar extension on left side. the patient presented also pain on mobilization of the left calf, reduced calf fluctuation, and a red, warm, and painful inflammatory patch on the left leg, heart rate (HR) 100 beats/min ;blood pressure BP 120/70 mmHg.
Brain MRI showed: multiple ischemic strokes some of which show signs of hemorrhage. Figure 1
Blood analysis showed: increased white blood cell count (WBC) and elevated inflammation markers [erythro- cyte sedimentation rate (ESR) 33 mm/1 h, C-Reactive Protein (CRP) 377 mg/dL], optimal value of low-density lipoprotein (LDL)-cholesterol, a slightly decreased high-density lipoprotein (HDL)-cholesterol value (0.26 g/L).
blood culture was positive (cocci gram positif en chainette :ENTEROCOCCUS FAECIUM MULTIRESISTANT sensible only to TEICOPLANIN).
On electrocardiogram (EKG) were noticed sinus rhythm, no right/left atrium enlargement, no decreased QRS nonspecific negative T waves in inferior leads.
Transthoracic echocardiography (TTE) revealed a left ventricle (LV) with normal volumes and normal global systolic function, ejection fraction (EF) 60%, Enlarged left atrium (volume 25.5 cm2) with a heterogeneous, multilobed, and mobile mass located in the left atrial cavity, attached to the interatrial septum and extending to the anterior mitral valve on its atrial side, measuring 61x28 mm. The inferior part of the mass is mobile (possibly related to a vegetation, given the context), without hemodynamic impact.
Doppler ultrasound of the arterial circulation of both lower limbs: Diffuse atherosclerotic peripheral artery disease of both lower limbs with total occlusion of both anterior tibial and pedal arteries, and the left popliteal artery, resulting in hemodynamic consequences downstream, with dampened flow proximally.Figure 2

Myxoma is the most common primitive cardiac tumor diagnosed in adults; it represents 50–85% of the benign tumors . It has an incidence of 0.5–1 case/106 persons/ year and it is encountered three times more often in women. It is more common in the 4th–7th decades of life, it is rarely diagnosed in children and it is exceptionally rare in neonate(1) . The name myxoma comes from the abundant myxoid stroma rich in proteoglycans. Myxoma’s composition and origin generated debate; initially it was considered an organized thrombus or a chronic inflammatory lesion of endocardial tissue induced by viral infection, then an endothelial cell proliferation caused by turbulent blood flow (Prichard structures) and finally data provided by genetic and immunohisto- chemical studies confirmed it as a benign tumor originating from subendothelial multipotent mesenchymal heart cells(2).

Most myxomas arise in the left atrium (LA), 60–80% of cases, with typical insertion on the fossa ovalis, less often they can be found in other locations in LA, right atrium (15–28% of cases), ventricles (12%) or valves. In 90% of cases, myxoma occurs sporadically, while the rest are familial forms as Carney syndrome, in which multifocal myxomas can affect many organs (heart, skin, breast), the patients having abnormal skin pigmentation (lentigines, pigmented nevi) and other types of tumors (3).

Myxomas can grow from a few millimeters to 15 cm, with an average size of 5–6 cm at the moment of diagnosis. Small tumors are usually asymptomatic, while larger masses can cause many symptoms: hemodynamic, due to mechanical interference with mitral valve opening; embolic, due to tumor friability and systemic/constitutional symptoms caused by cytokine release. Atrial myxoma can mimic a variety of diseases: rheumatic mitral stenosis, infective endocarditis or autoimmune disease. Although benign, myxomas can recur in 3% of sporadic cases and up to 20% in familial forms; this can be due to incomplete resection of the interatrial septum or to cell spread at the moment of surgery or previous embolism (4).

Arteriography of the right lower limb: Thrombosis of the common iliac artery extending into the external iliac artery, thrombosis of the popliteal artery at its articular portion with no recanalization.Figure 3
Arteriography of the left lower limb: Occlusion of the superficial femoral artery.Figure 3
The patient had an increased risk of embolic and hemodynamic complications. He was urgently transferred to a Cardiac Surgery Center, where atrial tumor was removed with repair of interatrial septum and Key mitral valve repair and closure of the foramen ovale. Also, a successful revascularization of the right iliac and popliteal arteries, as well as the left femoropopliteal artery, was performed.
Evolution was favorable, with normalization of left heart chambers dimensions and normal mitral and tricuspid valve function at 30 days postoperatively .

Figure 2 : Doppler ultrasound of the arterial circulation lower limbs:

Figure 3 : Arteriography of lower limbs

Figure 1 : Brain MRI

Diffusion

ADC Sequence