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Lambl’s Excrescence Associated with Recurrent Ischemic Strokes

  • Urvish K. PatelEmail author
  • Siddharth Bhesania
  • Preeti Malik
  • Janki Bhesania
  • Alina Barmanwalla
  • Swati Anand
  • Vishal Jani
  • Abhishek Lunagariya
  • Parag Mehta
Medicine
  • 134 Downloads
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  1. Topical Collection on Medicine

Abstract

Background

Lambl's excrescence (LE) is a rare thin, mobile, filiform frond on the endocardial surface of cardiac valves responsible for recurrent ischemic strokes due to embolic phenomena. Due to nonspecific symptoms, sometimes the diagnosis is challenging. It can be diagnosed by transesophageal echocardiogram (TEE) with increased sensitivity. Several case reports have been published with a strategy to manage LE but there is lack of consensus. We report a case of a 75-year-old patient with the LE arising from aortic valve resulting in recurrent ischemic strokes within a short period and its management.

Case

A 75-year-old male with multiple vascular risk factors presented with confusion, altered mental status of unknown onset and aphasia. He denied weakness, numbness, or other focal neurological deficits. National Institute of Health Stroke Scale was 5. IV tPA was not given due to the unknown onset of symptoms. The magnetic resonance imaging (MRI) revealed acute/subacute left thalamic lacunar infarct. TEE demonstrated a 7-mm linear fibrinous mass on the valve. The patient was on aspirin which was changed to clopidogrel. After 2 weeks, the patient readmitted with similar presentation. MRI showed subacute right parietal cortical infarct. The patient was advised to proceed with surgical resection of LE. Due to refusal for surgery, he was kept on 30 days cardiac monitor and had given anticoagulant therapy for secondary prevention of stroke.

Conclusion

Although wide range from single or dual antiplatelet therapy to full dose anticoagulation have been tried, surgical intervention should be considered in case of multiple ischemic strokes to mitigate the risk of embolism.

Keywords

Lambl’s Excrescence Cardiac tumor Papillary fibroelastomas Acute ischemic stroke Embolic stroke Embolic stroke of undetermined source (ESUS) 

Introduction

Primary cardiac tumors are rare and challenging tumors with a prevalence of approximately 0.001–0.03% [1]. In 1856, Vilém Dušan Lambl, a physician, first described an anomalous cardiac growth later on known as the Lambl’s excrescence (LE) [2]. LE is a rare cardiac growth on the endocardial surface. It occurs typically at sites of valve closure; however, it rarely occurs on the endocardial surface as well. It is seen as thin, mobile, filiform fronds at valve closure on echocardiogram. It is hypothesized that they occur as a result of minor endothelial damage due to valve wear and tear [3].

LE can occur even in the absence of any cardiac disease and the majority of the patients are asymptomatic [3]. This structure contributes to the susceptibility for embolization, causing life-threatening conditions like stroke, embolism, and sudden death in asymptomatic patients. Several reports have been published in the medical literature, but clinical significance, as well as management, remains controversial [4]. The diagnosis is challenging to the physicians due to scarcity, nonspecific symptoms, and variety. With the advancement in imaging technology like magnetic resonance imaging (MRI), computed tomography (CT) scan, and transesophageal echocardiogram (TEE), larger secondary tumors can be diagnosed with more sensitivity, but these small LE may be missed [3].

We report a case of a 75-year-old patient with a LE arising from the noncoronary cusp of the aortic valve resulting in recurrent ischemic strokes within a short period and its management.

Case Presentation

A 75-year-old male with history of diabetes mellitus, hyperlipidemia, and hypertension presented with confusion and altered mental status of unknown onset with the last known well 6 h ago. The patient displayed aphasia. The patient had an unwitnessed fall three days prior and was unsure about head trauma. He denied weakness, numbness, or any other focal neurological deficits at the time of presentation. National Institute of Health Stroke Scale (NIHSS) was 5 (level of consciousness 1; level of consciousness questions 1; level of consciousness commands 1; best language 2; dysarthria 1).

He smoked half a pack per day for 30 years but had quitted 6 months ago, and his family history is significant for diabetes mellitus and ischemic stroke. He had a blood pressure of 194/84 mmHg while the rest of the vitals being normal on admission. Physical examination was unremarkable except above. The CT brain without contrast as well as CT angiogram head and neck on admission did not show any early ischemic changes or large vessel disease. Intravenous recombinant tissue plasminogen activator (IV tPA) was not offered due to the unknown onset of symptoms. The patient was admitted for further management. Initial laboratory investigations revealed microcytic anemia and normal basic metabolic profile. His finger stick was 277 mg/dL in emergency department and HbA1C was 8.9%. His urinalysis was positive for 100 mg/dL of protein and > 500 mg/dL of glucose.

He had MRI brain without contrast, revealed acute/subacute infarct of the left thalamus without associated hemorrhage, mild to moderate diffuse volume loss, and chronic periventricular white matter small vessel ischemic disease, as well as few tiny parenchymal foci of chronic microhemorrhage. Based on the MRI as well as medical history, stroke etiology was presumed to be lacunar from small vessel disease. The Mini-Mental State Examination (MMSE) showed 28/30. The speech therapist was consulted as the patient’s speech was not returning to baseline. His memory function had not deteriorated further at this time. A transthoracic echocardiogram (TTE) was performed which revealed no systolic dysfunction with an ejection fraction of 67%, but cardiac wall thickness was markedly increased. The significant finding was an irregular mobile mass measuring 10 mm in length on the noncoronary cusp of the aortic valve (Fig. 1). For further evaluation, a TEE was performed which demonstrated a small, linear fibrinous mass on the aortic side of the noncoronary cusp, representing Lambl’s excrescence, and measuring 7 mm in length without patent foramen ovale (Fig. 2). The patient was on aspirin prior to the presentation. He was switched to clopidogrel 75 mg and atorvastatin 80 mg QHS for further stroke prevention. Diabetes was controlled with insulin and oral medications.
Fig. 1

Transthoracic echocardiogram (TTE) showing 10-mm small mobile mass on the noncoronary cusp of the aortic valve

Fig. 2

Transesophageal echocardiogram (TEE) showing 7-mm small mobile mass on the noncoronary cusp of the aortic valve.

However, 2 weeks later, the patient returned with another episode of confusion and altered mental status. On MRI without contrast during hospitalization showed a 7-mm acute vs. subacute cortical infarct in the right posterior/inferior parietal lobe which was not present on his initial admission. Cardiothoracic surgery was consulted for possible aortic valve mass removal versus an aortic valve repair; however, the patient was not ready for the surgical option. Although the lack of the evidence of atrial fibrillation on 30 days of cardiac event monitor, we put him on an anticoagulant for the prevention of embolic stroke. The patient had kept on close follow-up and surveillance for another neurological event.

Discussion

These rare cardiac growths may present from being asymptomatic to severe life-threatening events. They are mostly located on the aortic and mitral valve and rarely on pulmonary and tricuspid valve. These strands are acellular which are covered by a single layer of endothelium with elastic fibrils arranged in whirls in the hyaline stroma. They are avascular and devoid of granulation tissue and appear as single strand or clusters and approximately 1 mm in diameter and 10 mm in length [3]. Of all the population referred for TEE for any indications, 5.5% have valvular strands. LE is found in both adults and children but the prevalence is higher in adults and increases with age [3].

These filiform strands are often incidental finding and may confuse clinicians to differentiate between mobile thrombus or pedunculated myxoma or cardiac papillary fibroelastomas. There are several evidences to support that these clusters of LE may break from the atrioventricular valve and cause thromboembolic events and predispose the patients to adverse events like myocardial infarction, stroke, pulmonary embolism, ventricular fibrillation, and retinal artery embolism [2, 4, 5, 6, 7, 8, 9, 10]. LE should be considered in the differential diagnosis for a patient with embolic stroke of undetermined source. In our patient, the multiple ischemic stroke events occurred due to obstruction to the blood flow to the brain caused by broken fragments of these fronds at the aortic valve. For LE diagnosis, TEE is superior to TTE, as these small clusters may be missed by TTE [3].

There is no established evidence for the management and treatment of LE [8]. When a LE is detected by TEE, antiplatelet therapy should be considered. However, if a recurrent ischemic stroke occurs while on the therapy, which exactly happened in our patient then promptly start with anticoagulation therapy to prevent thromboembolic events before surgical resection is considered [8].

Most authors agreed that the decision between surgical interventions vs. conservative treatment should be on a case by case basis and associated complications [7]. Aggarwal et al [9] reported a case of subcortical infarction in a 66-year-old female with LE on the aortic valve, initially started on anticoagulation therapy. But after 3 weeks, she had another stroke episode even being on anticoagulants, eventually leading to surgical intervention. Our case supports the findings of this case report. Few cases have reported the use of either antiplatelet therapy or anticoagulation in patients with single/first embolic event [5, 8].

In Chu et al. and in other literature, we have noted that there is no consensus on the treatment of LE, whether to start with antiplatelet or anticoagulant therapy first [11]. Ammannaya GKK mentioned asymptomatic LE should be closely followed up with TEE, LE with first episode of transient ischemic attack or acute ischemic stroke should be treated with aspirin or dual antiplatelet therapy or warfarin or acenocoumarol followed by surgery for poor medical compliance, and subsequent events should be treated with trial of warfarin/acenocoumarol plus aspirin/dual antiplatelet therapy or surgery [12]. The choice between surgical intervention and conservative management needs to be decided on a case-by-case basis like two or more ischemic stroke with treatment failure, or combined other heart disease like coronary occlusion that leads to myocardial ischemia or valve obstruction and should be based on the pre-operative risk stratification [7, 13, 14, 15]. However, for our patient, both treatment options have been used but eventually, surgery was recommended considering the complications associated with ischemic stroke.

Conclusion

LE is rare cardiac growth with debatable clinical significance, which can cause serious complications ranging from frequent transient ischemic attack to embolic stroke. Due to advancement in diagnostics, discovery is more often. Asymptomatic LE should be followed closely with TEE and if symptomatic, and not managed by aspirin/dual antiplatelet/anticoagulant medications then treated with trial of warfarin/acenocoumarol plus aspirin/dual antiplatelet therapy or should be surgically removed keeping pre-operative risk stratification in mind. This report helps us to keep the possibility of LE in patient presenting with abnormal cardiac growth on TEE and having angina like symptoms or neurological symptoms.

Notes

Funding Information

No targeted funding reported.

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

Ethical Approval and Informed Consent to Participate

Patient’s identity is not disclosed anywhere, and IRB or ethics committee approval was not required to publish this work. All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Consent for Publication

Informed consent was obtained from individual participant included in the study to publish the manuscript.

References

  1. 1.
    Reynen K. Frequency of primary tumors of the heart. Am J Cardiol. 1996;77(1):107.  https://doi.org/10.1016/s0002-9149(97)89149-7.CrossRefPubMedGoogle Scholar
  2. 2.
    Lambl VD. Papillare excrescenzen an der semilunar-klappe der aorta. Wien Med Wochenschr. 1856;6:244–7.Google Scholar
  3. 3.
    Kondamareddy D, Masood W. Lambls Excrescences. StatPearls. Treasure Island: StatPearls Publishing, StatPearls Publishing LLC.; 2019.Google Scholar
  4. 4.
    Roldan CA, Shively BK, Crawford MH. Valve excrescences: prevalence, evolution and risk for cardioembolism. J Am Coll Cardiol. 1997;30(5):1308–14.  https://doi.org/10.1016/S0735-1097(97)00315-X.CrossRefPubMedGoogle Scholar
  5. 5.
    Kalavakunta JK, Peddi P, Bantu V, Tokala H, Kodenchery M. Lambl’s excrescences: a rare cause of stroke. J Heart Valve Dis. 2010;19(5):669–70.PubMedGoogle Scholar
  6. 6.
    Al-Ansari S, Hindori V, Riezebos RK, Yilmaz A. Multiple Lambl’s excrescences with subvalvular extension, a rare cause of cryptogenic stroke: treated by port-access cardiac surgery. BMJ Case Rep. 2013;2013.  https://doi.org/10.1136/bcr-2013-201161.Google Scholar
  7. 7.
    Davogustto G, Fernando RR, Loghin C. Lambl's excrescence, migrainous headaches, and “tiger stripes”: puzzling findings in one patient. Tex Heart Inst J. 2015;42(1):70–2.  https://doi.org/10.14503/thij-13-3808.CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Kamran H, Patel N, Singh G, Pasricha V, Salifu M, McFarlane SI. Lambl's excrescences: a case report and review of the literature. Clin Case Rep Rev. 2016;2(7):486–8.  https://doi.org/10.15761/ccrr.1000254.CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Aggarwal A, Leavitt BJ. Images in clinical medicine. Giant Lambl's excrescences. N Engl J Med. 2003;349(25):e24.  https://doi.org/10.1056/ENEJMicm010900.CrossRefPubMedGoogle Scholar
  10. 10.
    Aziz F, Baciewicz FA Jr. Lambl's excrescences: review and recommendations. Tex Heart Inst J. 2007;34(3):366–8.PubMedPubMedCentralGoogle Scholar
  11. 11.
    Chu A, Aung TT, Sahalon H, Choksi V, Feiz H. Lambl’s excrescence associated with cryptogenic stroke: a case report and literature review. Am J Case Rep. 2015;16:876–81.  https://doi.org/10.12659/ajcr.895456.CrossRefPubMedPubMedCentralGoogle Scholar
  12. 12.
    Ammannaya GKK. Lambl’s excrescences: current diagnosis and management. Cardiol Res. 2019;10(4):207–10.  https://doi.org/10.14740/cr892.CrossRefPubMedPubMedCentralGoogle Scholar
  13. 13.
    Voros S, Nanda NC, Thakur AC, Winokur TS, Samal AK. Lambl’s excrescences (valvular strands). Echocardiography. 1999;16(4):399–414.  https://doi.org/10.1111/j.1540-8175.1999.tb00833.x.CrossRefPubMedGoogle Scholar
  14. 14.
    Liu RZ, Yu SY, Li Y. Migraine-like headache and ischemic strokes in two patients with Lambl's excrescences. Chin Med J. 2012;125(18):3346–8.  https://doi.org/10.3760/cma.j.issn.0366-6999.2012.18.028.CrossRefPubMedGoogle Scholar
  15. 15.
    Chong-lei R, Sheng-li J, Rong W, Cang-song X, Yao W, Chang-qing G. Diagnosis and treatment of Lambl’s excrescence on the aortic valve. HSF [Internet]. 2018;21(3):E148–50.  https://doi.org/10.1532/hsf.1687.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of NeurologyCreighton University School of MedicineOmahaUSA
  2. 2.Department of Internal MedicineNewYork-Presbyterian Brooklyn Methodist HospitalBrooklynUSA
  3. 3.Department of Public HealthIcahn School of Medicine at Mount SinaiNew YorkUSA

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