Case Report Access this article online Quick Response Code: Downloaded from http://journals.lww.com/neur by BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AW nYQp/IlQrHD3i3D0OdRyi7TvSFl4Cf3VC4/OAVpDDa8KKGKV0Ymy+78= on 04/18/2024 Cardioembolic Stroke in Sinus of Valsalva Aneurysm, A Case Report Vineeth V Thomas, Joel M Thomas, Ramya Iyadurai Website: www.neurologyindia.com DOI: 10.4103/0028-3886.388106 Abstract: A case of cardioembolic stroke in a patient with sinus of Valsalva aneurysm. The patient presented with chief complaints of syncope, with slurring of speech and a history of deviation of angle of mouth to the right side, which resolved over one day. Examination revealed no focal neurological deficit. CT Brain revealed a lacunar infarct over the left frontal cortex. Echocardiography showed sinus of Valsalva aneurysm of the non‑coronary cusp. Hence a diagnosis of cardioembolic stroke secondary to thrombus present in the sinus of Valsalva of the aneurysm was considered. This is a rare presentation of Sinus of Valsalva as a cardioembolic stroke. Key Words: Acute aortic syndrome, cardio‑embolic stroke, ruptured sinus of valsalva Key Message: Ruptured sinus of Valsalva presenting as a cerebrovascular accident is a rare differential for cardio‑embolic stroke. Should be considered as a differential in the appropriate setting. R upture sinus of Valsalva (RSOV) is an uncommon life‑threatening emergency which presents as acute onset chest pain and cardiac failure. Incidence of RSOV is 0.09% in the general population with no data on its presentation as stroke. [1] It has rarely been described in literature as a source of emboli, while our review of literature found only four prior case reports in a similar manner. Here we report a case of RSOV which presented as a cardio‑embolic stroke, with a comparison against previous reported cases. Patient Information and Clinical Findings Department of General Medicine, Christian Medical College, Vellore, Tamil Nadu, India Address for correspondence: Dr. Vineeth V Thomas, Medicine Unit‑V, Department of General Medicine, Christian Medical College, Vellore, Tamil Nadu ‑ 632 004, India. E‑mail: vvt212@gmail.com 998 A 73‑year male had presented to casualty with a deviation of angle of mouth to the left with right upper limb weakness, dizziness and acute onset chest pain for the past 6 days. His chest pain was sudden in onset with moderate severity and decreased effort tolerance which progressed from New York Heart Association class I to IV over 4 days.\Bilateral lower limb swelling was noticed since the event with swelling up to the knee at presentation and associated abdominal distension. There was no history of trauma, fever, cough. He was not diagnosed with diabetes or hypertension. He had no significant past medical This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: WKHLRPMedknow_reprints@wolterskluwer.com history. He consumes ethanol weekly and had a 40 pack‑year history of consuming cigarettes. He did not give any history of iv drug abuse. He had presented to us on the 5th day of illness. On examination, he was in distress and diaphoretic. He had a pulse of 128/min which was regular and bounding in character with a blood pressure of 100/60 mmHg. He had a respiratory rate of 35 per minute and required 60% of oxygen to maintain saturation. His JVP was elevated up to 10 cm with bilateral pedal edema extending up to the knees. He had no pallor, icterus, cyanosis, clubbing or lymphadenopathy. He had no peripheral signs of infective endocarditis or Marfan’s syndrome. Neurological examination revealed a deviation of angle of mouth to the left with right upper limb monoparesis. Cardiovascular examination revealed a loud continuous murmur at the lower sternal border with diastolic accentuation. Investigations His baseline blood investigations revealed mild normocytic anemia with a left shift leukocytosis and thrombocytopenia. His renal function tests showed a pre‑renal acute kidney injury with How to cite this article: Thomas VV, Thomas JM, Iyadurai R. Cardioembolic Stroke in Sinus of Valsalva Aneurysm, A Case Report. Neurol India 2023;71:998-1001. Submitted: 03‑Apr‑2020 Accepted: 28‑Oct‑2020 Revised: 25-Oct-2020 Published: 18-Oct-2023 © 2023 Neurology India, Neurological Society of India | Published by Wolters Kluwer - Medknow Thomas, et al.: Unusual presentation of stroke Downloaded from http://journals.lww.com/neur by BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AW nYQp/IlQrHD3i3D0OdRyi7TvSFl4Cf3VC4/OAVpDDa8KKGKV0Ymy+78= on 04/18/2024 metabolic acidosis and medically correctable hyperkalemia. His liver function tests showed elevated liver enzymes along with coagulopathy and undetectable fibrinogen suggestive which along with low platelet count suggesting disseminated intravascular coagulation [Table 1]. His acute viral hepatitis markers were negative. His ECG revealed sinus tachycardia with non‑specific T wave changes and chest radiograph revealed cardiomegaly with increased vascular markings. An MRI done in view of his deficits revealed acute infarct in the left frontal lobe on diffusion‑weighted imaging. His Echocardiogram subsequently revealed a 5 mm rupture left coronary cusp sinus directly into the right atrium with no associated VSD or involvement of aortic valve [Figures 1-3]. or other connective tissue diseases can predispose to these conditions. Acquired causes are due to aortic wall infection, trauma or inflammatory tissue diseases.[5] A large case series comprising 55 patients with ruptured sinus of Valsalva revealed that there was an 80% prevalence among the male gender. The presentation was also varied with extremes of asymptomatic to frank heart failure Treatment He was initially managed with oxygen supplementation and non‑invasive ventilation along with appropriate anti‑failure medication. He was monitored for features of overt bleed and hepatic encephalopathy. He was given supportive ventilatory care which was weaned off by day 4 of admission, and was clinically better with improvement in renal and liver function tests. His deficits had improved since admission. He underwent angiographic closure of the defect and is on regular follow up. Discussion Figure 1: 2D Echocardiogram Revealing The Location of The Shunt Acute aortic syndromes are a spectrum of life‑threatening aortic conditions which include aortic intramural hematoma, penetrating aortic ulcers and the most common and well known, aortic dissection. [2,3] The prevalence of sinus of Valsalva aneurysms comprises 0.1‑3.5% of all congenital cardiac anomalies with an incidence of 0.09% in the general population.[1] The incidence of the embolic phenomenon of sinus of Valsalva is sporadic and is rare with most data confined to case reports.[4] Congenital causes like Marfan, Ehler‑Danlos Table 1: Relevant laboratory investigations ABG pH: 7.37; pO2: 76; pCO2: 17; Lactate: 7.0 CBC Hb: 10.9 gm%; TC/DC: 15,500 with 80% N; Plt: 45,000/cumm LFT Total bilirubin: 6.19 (0.5‑1.0) Direct bilirubin: 5.22 (< 0.5) Total protein: 6.2 (6.0‑8.5) Albumin: 3.3 (3.5‑5.5) SGOT: 1469 (8‑40) SGPT: 1601 (5‑35) ALP: 151 (40‑125) Coagulation profile APTT: 42.7s (27.8‑40.4) INR: 4.84 Fibrinogen: undetectable (150‑450) RFT Urea: 228 mg/dl (15‑40) Creat: 2.08 mg/dl (0.5‑1.4) Neurology India | Volume 71 | Issue 5 | September-October 2023 Figure 2 : 3D Echocardiogram Revealing Defect In The Left Coronary Cusp Sinus Figure 3: Magnetic Resonance Imaging - Diffuse Weighted Image Revealing A Left Cerebral Infarct In The Frontal Lobe 999 Thomas, et al.: Unusual presentation of stroke Table 2: Comparison of clinical outcomes with other case reports Downloaded from http://journals.lww.com/neur by BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AW nYQp/IlQrHD3i3D0OdRyi7TvSFl4Cf3VC4/OAVpDDa8KKGKV0Ymy+78= on 04/18/2024 CEREBROVASCULAR ACCIDENT Anterior circulation Posterior circulation Vessel involvement ECHOCARDIOGRAM Visualised Thrombus MANAGEMENT 30 DAY SURVIVAL Our case Ruzieh et al. 2016 Shahrabani et al. 1993 Stollberger et al. 1996 Nakajima et al. 2013 Present Absent Medium vessel Present Absent Medium vessel Present Absent Medium vessel Present Absent Medium vessel Present Absent Medium vessel Absent Medical management Yes Present Medical management Yes Absent Medical management Yes Present Medical management Yes Absent Medical management Yes with more than 50% of the cohort however presenting in New York Heart Association (NYHA) class III or higher.[6] Another case series of 53 cases showed that 60% of the cohort had presented with acute breathlessness with 25% having syncopal attacks. Although infective endocarditis is a known predisposing factor, less than 5% of patients in one cohort had the same.[7] An echocardiogram is the ideal first‑line investigation as to its non‑invasiveness & easy availability. In both the largest studies, the rupture of the right coronary cusp was the most common site followed by non‑coronary cusp. The ventricular septal defect was the most common associated cardiac lesion followed by aortic insufficiency.[6,7] The role of transesophageal echocardiogram is further characterizing the lesion size, location, associated endocarditis or cardiac lesion which may not be easily assessed on a trans‑thoracic echo.[8] The role of a computed tomographic angiogram is the modality of choice when assessing for any arch dissection or hematoma and should be considered in hemodynamically stable patients.[9] The common modalities of cerebral ischemia caused due to cardiac abnormalities are in the setting of arrhythmia like atrial fibrillation, ascending aortic atheromatous disease, patent foramen ovale or an atrial septal defect. Other etiologies like rheumatic heart disease, left ventricular thrombus and hypercoagulable states should also be considered. [10‑12] In the case of sinus of Valsalva, thrombosis into the sinus with subsequent artery‑artery embolism during the event is the most likely mechanism of cerebral ischemia.[13] Medical management for the stroke with antiplatelet is indicated with medical stabilization playing an essential role in patient management. Medical management of RSOV involves stabilization with heart failure medication with a perioperative assessment. A transcatheter repair is a feasible option in select cases however surgical repair, especially with the presence of intracardiac shunting, is indicated.[14,15] A comparison table of previously reported RSOV presenting as a cerebrovascular accident is presented in Table 2.[4,13,16,17] All the previous cases were predominantly anterior circulation strokes with a majority presenting as monoplegia or hemiplegia with medium vessel occlusion. Only half of these patients had definitive echocardiographic evidence of a thrombus and all of them were medically managed with subsequent surgical repair of the anatomical defect. 30‑day mortality was favorable in all cases. 1000 Conclusion Acute aortic syndromes are rarely considered in the differential for patients presenting as cardio‑embolic stroke. Common causes such as LV thrombus, arrhythmias & RHD are often the most common. The management of this case was challenging as the clinical presentation was protracted and diagnosis was delayed. An RSOV would need prompt surgery, either by angiographic or surgical means based on imaging. The stabilization of these patients would require aggressive supportive care in an intensely monitored setting. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil Conflict of interest There are no conflicts of interest. References 1. 2. 3. 4. 5. Hope J. A Treatise on the Diseases of the Heart and Great Vessels: And on the Affections Which May be Mistaken for Them, Comprising the Author’s View of the Physiology of the Heart’s Action [Internet]. Philadelphia : Lea & Blanchard; 1846. p. 630. Available from: http://archive.org/details/068973910.5810.emory. edu. [Cited on 2020 Mar 14]. Tsai TT, Nienaber CA, Eagle KA. Acute aortic syndromes. Circulation 2005;112:3802‑13. Harris KM, Braverman AC, Eagle KA, Woznicki EM, Pyeritz RE, Myrmel T, et al. Acute aortic intramural hematoma: An analysis from the International Registry of Acute Aortic Dissection. Circulation 2012;126 (11 Suppl 1):S91‑6. Stöllberger C, Seitelberger R, Fenninger C, Prainer C, Slany J. Aneurysm of the left sinus of Valsalva: An unusual source of cerebral embolism. Stroke 1996;27:1424‑6. Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL, et al. The International Registry of Acute Aortic Dissection (IRAD): New insights into an old disease. JAMA 2000;283:897‑903. Neurology India | Volume 71 | Issue 5 | September-October 2023 Thomas, et al.: Unusual presentation of stroke 6. Downloaded from http://journals.lww.com/neur by BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AW nYQp/IlQrHD3i3D0OdRyi7TvSFl4Cf3VC4/OAVpDDa8KKGKV0Ymy+78= on 04/18/2024 Thomas PA. Working the fundamentals. Eur J Cardiothorac Surg 2013;43:455‑8. 7. Vural KM, Şener E, Taşdemir O, Bayazıt K. Approach to sinus of Valsalva aneurysms: A review of 53 cases. Eur J Cardiothorac Surg 2001;20:71‑6. 8. Dhawan I, Malik V, Sharma KP, Makhija N, Pangasa N. Transthoracic echocardiography versus transesophageal echocardiography for rupture sinus of valsalva aneurysm. Ann Card Anaesth 2017;20:245‑6. 9. Nienaber CA. The role of imaging in acute aortic syndromes. Eur Heart J Cardiovasc Imaging 2013;14:15‑23. 10. Powers WJ, Rabinstein AA, Ackerson T, Adeoye OM, Bambakidis NC, Becker K, et al. Guidelines for the early management of patients with acute ischemic stroke: 2019 update to the 2018 guidelines for the early management of acute ischemic stroke: A guideline for healthcare professionals from the American Heart Association/American Stroke Association. Stroke 2019;50:e344‑418. 11. Harloff A, Handke M, Reinhard M, Geibel A, Hetzel A. Therapeutic strategies after examination by transesophageal echocardiography in 503 patients with ischemic stroke. Stroke 2006;37:859‑64. Neurology India | Volume 71 | Issue 5 | September-October 2023 12. Horowitz DR, Tuhrim S, Weinberger JM, Budd J, Alweiss GS, Goldman ME. Transesophageal echocardiography: Diagnostic and clinical applications in the evaluation of the stroke patient. J Stroke Cerebrovasc Dis Off J Natl Stroke Assoc 1997;6:332‑6. 13. Ruzieh M, Shafiq Q, Murphy L, Bonnell M, Khouri S. Embolic stroke due to sinus of valsalva aneurysm thrombus. Am J Med Sci 2016;352:333‑4. 14. Altekin RE, Karakas MS, Er A, Yanikoglu A, Ozbek S, Yilmaz H. Percutaneous closure of ruptured sinus of Valsalva aneursym with Amplatzer ductal occluder. Acta Cardiol 2011;66:657‑60. 15. Kloppenburg GTL, Sonker U, Post MC, Yilmaz A, Morshuis WJ. Emergency surgery for ruptured sinus of Valsalva aneurysms. Scand Cardiovasc J SCJ 2011;45:374‑8. 16. Shahrabani RM, Jairaj PS. Unruptured aneurysm of the sinus of Valsalva: A potential source of cerebrovascular embolism. Heart 1993;69:266‑7. 17. Nakajima M, Abe A, Nishiyama Y, Harada‑Abe M, Kutsuna A, Goto Y, et al. Multiple vascular accidents including rupture of a sinus of Valsalva aneurysm, a minor ischemic stroke and intracranial arterial anomaly in a patient with systemic congenital abnormalities: A case report. Case Rep Neurol 2013;5:195‑200. 1001