Departments of 2Surgery and 3Internal Medicine, 1College of Medicine, Imam Abdulrahman Bin Faisal University, Dammam, Saudi Arabia *Corresponding Author’s e-mail: feras_a_k@yahoo.com ورم خماطي يف الصمام األهبري يعرض كسكتة دماغية تقرير حالة و مراجعة لألدبيات الطبية فرا�س اأحمد الكويتي، يا�رص الغنيمي، �شامي غزال abstract: Myxomas originating from the aortic valve are rare. We report a 40-year-old male patient who presented to the King Fahd Hospital of the University, Khobar, Saudi Arabia, in 2017 with a stroke. Transoesophageal echocardiography indicated a mobile mass measuring 6 × 2 mm attached to the right coronary cusp of the aortic valve and a mobile interatrial septum with a small patent foramen ovale (PFO). The patient underwent surgical excision of the mass and direct closure of the PFO. Histopathology confirmed the mass to be a myxoma. Despite their rarity, the recognition and treatment of valvular myxomas is very important; moreover, clinicians should be aware that affected patients may present with an embolic stroke. Keywords: Aortic Valve; Myxoma; Patent Foramen Ovale; Stroke; Transesophageal Echocardiography; Case Report; Saudi Arabia. امللخ�ص: يعد الورم املخاطي النا�شئ عن �شمامات القلب حالة نادرة احلدوث. نعر�س هنا حالة مري�س رجل يبلغ من العمر 40 عاًما تقدم اإىل م�شت�شفى امللك فهد اجلامعي، اخُلرب، اململكة العربية ال�شعودية، يف عام 2017 ب�شكتة دماغية. اأ�شار تخطيط �شدى القلب عرب املريء اإىل وجود كتلة متحركة بحجم 2 × 6 مم مت�شلة بال�رصفة التاجية اليمنى لل�شمام الأبهري واحلاجز الأذيني مع وجود ثقب بي�شاوي قلبي �شغري. خ�شع املري�س لالإ�شتئ�شال اجلراحي واإغالق مبا�رص للثقب البي�شاوي القلبي. اأكد تقرير مر�شيات الأن�شجة على اأن الكتلة هي عبارة عن ورم خماطي. على الرغم من ندرة احلالة، اإل اأن معرفة وعالج الورم احلبيبي لل�شمامات يعترب مهمة للغاية؛ عالوة على ذلك، يجب اأن يكون الأطباء على دراية باأن املر�شى امل�شابني قد يظهرون ب�شكتة دماغية �شمّية. اململكة حالة؛ تقرير املريء؛ عرب القلب �شدى تخطيط الدماغية؛ ال�شكتة القلبي؛ البي�شاوي الثقب خماطي؛ ورم الأبهري؛ ال�شمام املفتاحية: الكلمات العربية ال�شعودية. Aortic Valve Myxoma Presenting with a Stroke A case report and review of the literature *Feras A. Alkuwaiti,1 Yasser Elghoneimy,2 Sami Ghazal3 Sultan Qaboos University Med J, November 2018, Vol. 18, Iss. 4, pp. e537–540, Epub. 28 Mar 19 Submitted 27 May 18 Revision Req. 26 Jun 18; Revision Recd. 25 Jul 18 Accepted 9 Aug 18 case report doi: 10.18295/squmj.2018.18.04.019 Myxomas originating from the cardiacvalves are uncommon.1,2 These benign tumours typically occur between the fourth and seventh decades of life and usually affect women more comm- only than men.1 The condition is mostly sporadic in nature, but can also be familial.2 Myxomas are true neo- plasms in that they are derived from primitive multipotent mesenchymal cells which may exist as embryonic rem- nants in the heart wall.3 Rarely, myxomas may arise from both the ventr- icular aspect and the margin of the aortic valve cusps; moreover, one or both leaflets may also be affected.4 Although transoesophageal echocardiography (TEE) aids in the assessment of such masses, histopathology is considered the gold-standard method to confirm a diagnosis.4,5 This report describes an atypical case of aortic valve myxoma and patent foramen ovale (PFO) presenting with a cerebral stroke. Such cases serve to increase awareness of the varying presentation of aortic valve myxomas and highlight appropriate investigations and management of this condition. Case Report A 40-year-old male patient presented to the King Fahd Hospital of the University (KFHU), Khobar, Saudi Arabia, in 2017 with sudden-onset dysphasia, left ataxia, nausea and vomiting over the preceding two hours. The patient reported having had a previous stroke at the age of 11 years; however, he was unable to provide any information about the event. At presentation, the patient was alert, oriented and had stable vital signs. All laboratory investigation findings were within normal limits and electrocardiography showed normal sinus rhythm. As a result, the case was transferred to the stroke team. Urgent computed tomography (CT) of the head showed evidence of the prior ischaemic stroke event in the right occipital lobe and right cerebellum. An intra- venous thrombolytic was administered in accordance with hospital guidelines. Subsequently, the patient was admitted to the inpatient department and received 150 mg of aspirin and 75 mg of clopidogrel. Symptoms improved over the course of the following three days. However, Aortic Valve Myxoma Presenting with a Stroke A case report and review of the literature e538 | SQU Medical Journal, November 2018, Volume 18, Issue 4 further investigations were undertaken to determine the source of the stroke. Brain magnetic resonance imaging (MRI) revealed that the patient had suffered an acute ischaemic stroke, with a multifocal area of diffusion restriction within the right cerebral and superior cerebellar peduncles and both cerebellar hemispheres, as well as hyperintensities on T2-weighted fluid-attenuated inversion recovery MRI sequences. However, transthoracic echocardiography (TTE) did not show any clear source of emboli. Acc- ordingly, TEE was performed and revealed a small mobile mass measuring 6 × 2 mm attached to the right coronary cusp (RCC) of the aortic valve and a mobile interatrial septum with a small PFO visible with agitated saline contrast [Figure 1]. No significant arrhythmia was noted on a Holter monitor. A papillary fibroelastoma (PFE) was suspected. After two weeks of stroke management, the decision was made excise the mass along with closure of the PFO to prevent any future embolic events. The surgical procedure was performed while the patient underwent cardiopulmonary bypass. During the surgery, the PFO was visible as an opening between the right and left atria [Figure 2]. The mass was success- fully excised from the RCC and the PFO was closed. The postoperative period was uneventful and the patient was discharged five days later. A histopathological exam- ination of the excised mass showed a paucicellular lesion composed of stellate and oval cells with a moderate amount of eosinophilic cytoplasm, fine chromatin and indistinct cell borders [Figure 3]. These features were consistent with a diagnosis of myxoma. Discussion To the best of the authors’ knowledge, only 11 cases of aortic valve myxoma have been previously reported in the literature [Table 1].4–14 Interestingly, nine of these previously reported patients were male, as in the current case; this is contrary to the usual female pre- ponderance of myxoma cases.1,4–9,11,13,14 Overall, six aortic valve myxoma patients were symptomatic, with symptoms largely attributable to distal embolisation and ischaemia, including three cases with stroke, two with myocardial infarctions and one with limb ischaemia.4,6,8,11–13 Clinicians should therefore be aware of stroke as a potential presentation of aortic valve myxoma. The pathogenesis of ischaemic events in aortic valve myxoma could be due to either embolic debris derived from the tumour itself or from a thrombus around the tumour.12 In the current case, the patient was also diagnosed with PFO, a potential cause of embolic stroke in which a paradoxical embolism may pass through a PFO via right-to-left shunting (RLS).15 However, in the current case, the observed PFO was very small in size and therefore would not be able to cause the RLS of an embolus. Figure 1: Transoesophageal echocardiography of a 40- year-old male stroke patient in (A) bi-plane short-axis view showing a small mass in the right coronary cusp of the aortic valve measuring 3 × 5 mm and (B) three- dimensional enfaced view showing the mass measuring 6 × 2 mm. Figure 2: Intraoperative photograph of the heart of a 40-year-old male stroke patient showing an opening between the right and left atria measuring 10 × 10 mm. Figure 3: Haematoxylin and eosin stains at (A) x40 magnification showing a hypocellular lesion in a myxoid matrix and (B) at x200 magnification showing stellate and oval cells with eosinophilic cytoplasm and indistinct cell borders. Feras A. Alkuwaiti, Yasser Elghoneimy and Sami Ghazal Case Report | e539 as filiform fronds located near valve closure lines.20 Radiologically, TEE is superior to TTE in ident- ifying a cardiac mass and defining its exact location, attachment to the underlying tissue and relation to surrounding structures.21,22 Among previously reported cases of aortic valve myxoma, 10 were identified by TTE, of which four were further confirmed via TEE.5–14 In one case, an aortic valve abnormality was first revealed via CT.4 In the current case, TEE revealed the mass to be located on the RCC of the aortic valve, thus precl- uding a thrombus or cardiac vegetation; however, the preoperative diagnosis was of a PFE. As with previous cases, the correct diagnosis was eventually confirmed by histopathological examination revealing a myxoid matrix with ovoid myxoma cells.4–14 Typically, the management of aortic valve myxoma cases involves the surgical excision of the tumour as well as the surrounding tissue to minimise the risk of local recurrence, as in the present case.4,10–12 During the procedure, the native aortic valve should be conserved as much as possible; however, if the tumour is too large The differential diagnosis of an aortic valve myxoma includes vegetation, a thrombus, PFE and Lambl’s excre- scences; these conditions can be distinguished by their microscopic and immunohistochemical characteristics.7,10,13 In addition, thrombi and myxomas can be differentiated using two-dimensional echocardiography; the former typically present with a layered appearance, while an area of echolucency may be observed within the tumour in the latter.7 Moreover, vegetations of the heart valves are a sign of infective endocarditis; however, this was ruled out in the current case due to a lack of history of this condition.10,16 On imaging, the size, shape, attachment site, mobility and location of a cardiac mass can aid in the exact diagnosis, along with the patient’s clinical presentation.17–19 For example, in PFE cases, the tumour appears as a small mass attached to the mitral or aortic valve, usually located downstream and attached by a small pedicle; such masses are more common and often mobile and appear irregularly-shaped with delicate frond-like surfaces.7,17,18 In contrast, Lambl’s excrescences appear Table 1: Literature review of reported aortic valve myxoma* cases4–14 Author and year of case* Age/ gender Presentation History Imaging Site Size in cm Complication Surgery Kennedy et al.8 (1995) 23/M Leg pain None TTE RCC and LCC 1.5 Limb ischaemia AVR Watarida et al.9 (1997) 58/M Asymptomatic None TTE RCC 1.1 × 1 None AVR Ramsheyi et al.6 (1998) 34/M Facial haemiparesis None TTE and TEE RCC 1 Stroke AVR Okamoto et al.10 (2006) 61/F Endocarditis HTN and DM TTE LCC 1 × 1 None Resection Dyk et al.11 (2009) 15/M Chest pain None TTE and TEE NCC 4 × 1 MI Resection Koyalakonda et al.12 (2011) 60/F Paroxysmal AF AF and HTN TTE and TEE RCC 1 × 1 Stroke Resection Fernández et al.13 (2012) 28/M Haemiparesis Epilepsy TTE RCC and LCC 1.5 × 0.7 Stroke AVR Kim et al.7 (2012) 72/M Shortness of breath HTN TTE NCC 1.5 × 0.8 None AVR Javed et al.4 (2014) 81/M Leg pain HTN, CKD and PAD CT, angiography and TEE LCC 1.8 × 1.2 Acute MI CABG and resection Prifti et al.5 (2015) 13/M Dyspnoea and angina None TTE and TEE RCC and LCC 0.6 × 0.2 None AVR Ji et al.14 (2017) 17/M Heart murmur None TTE NCC 2 None AVR Present case (2018) 40/M Dysphasia, ataxia and nausea Previous stroke TTE and TEE RCC 0.6 × 0.2 Stroke and PFO Excision and PFO closure TTE = transthoracic echocardiography; RCC = right coronary cusp; LCC = left coronary cusp; AVR = aortic valve replacement; TEE = transoesophageal echo- cardiography; HTN = hypertension; DM = diabetes mellitus; NCC = non-coronary cusp; MI = myocardial infarction; AF = atrial fibrillation; CKD = chronic kidney disease; PAD = peripheral arterial disease; CT = computed tomography; CABG = coronary artery bypass graft; PFO = patent foramen ovale. *In all cases, the diagnosis was confirmed via histopathological examination. Aortic Valve Myxoma Presenting with a Stroke A case report and review of the literature e540 | SQU Medical Journal, November 2018, Volume 18, Issue 4 and/or degeneration of the valvular structure occurs, aortic valve replacement (AVR) may be necessary. Among previously reported cases, seven underwent AVR, while resection alone was sufficient in three cases and one required resection and a coronary artery bypass graft.4–14 Nevertheless, regardless of treatment option, aortic valve myxoma cases require careful follow-up due to the high probability of local recurrence and distal tumour growth at the site of embolisation.5 Conclusion An aortic valve myxoma is uncommon and may present with serious complications, such as stroke. In patients with embolic phenomena, TEE should be performed, with special attention to the valvular morphology. 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