Behçet's Disease, Echocardiographers, and Cardiac Surgeons: Together is Better

Farouk, Heba

doi:10.1111/echo.12524

Cited by 16 publications

(7 citation statements)

References 42 publications

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“…Surgical removal is not the preferred choice because of the high rate of surgical complications and the risk of thrombosis recurrence. However, it should be considered in cases with unclear diagnosis, unresponsive to medical treatment or impaired hemodynamic caused by ICT [24] [25] [26] [27].…”

Section: Discussionmentioning

confidence: 99%

Right Ventricular Thrombus and Tricuspid Valve Dysfunction in a Patient with Beh&ccedil;et’s Disease

Valente¹,

Motet²,

Rusu³

et al. 2020

CRCM

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Background: Behçet's disease (BD) is a multisystemic, chronic inflammatory disorder with a broad range of manifestations including within the cardiovascular system. Cardiac involvement like intracardiac thrombus (ICT) and valvular involvement in BD are rarely seen entities and often associated with poor prognosis. Case Presentation: We present the case of a young patient with vascular-Behçet diagnosed by the presence of intracardiac thrombus in the right ventricle, unresponsive to medical treatment. Even though the intracardiac lesion was successfully treated by surgical excision, he presented a severe tricuspid dysfunction some years later due to the extension of fibrosis with no surgical therapeutic option. Conclusion: Intracardiac thrombosis is a rare but early manifestation of Behçet's disease, which is difficult to diagnose and has a poor prognosis.

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Section: Discussionmentioning

confidence: 99%

Right Ventricular Thrombus and Tricuspid Valve Dysfunction in a Patient with Beh&ccedil;et’s Disease

Valente¹,

Motet²,

Rusu³

et al. 2020

CRCM

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“…Surgery alone does not lead to a complete resolution and carries recurrence risk. 19 We searched PubMed/MEDLINE database and Google Scholar with the keywords of "Behçet's disease" and "intracardiac thrombus" from database inception to 2018. Articles containing pulmonary embolism and pulmonary artery aneurysms were included.…”

Section: Discussionmentioning

confidence: 99%

Is Immunosuppressive and Thrombolytic Therapy Really Effective in a Patient With Intracardiac Thrombosis and Pulmonary Artery Aneurysm due to Behçet’s Disease?

2019

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Behçet's disease (BD) is a rare, multisystemic, chronic vasculitic disorder with unknown etiology. The most common signs of BD are recurrent aphthous oral ulcers, genital ulcers, ocular lesions, dermatological lesions, and arthritis. 1,2 BD may also affect the pulmonary and cardiovascular systems. 2-4 Frequency of BD is low but intracardiac thrombus (IcT) formation is even less common; this serious complication has been reported only as case reports. 5-7 Pulmonary artery aneurysm (PAA) is also very rare, and only a few pediatric patients have been reported in the literature. 6,7 IcT accompanying PAA is extremely rare in the pediatric age group. In this article, we present an unusual case of an adolescent patient with PAA and IcT who did not survive despite all medical therapies. CASE REPORT A 16-year-old male patient was admitted to the hospital with complaints of productive cough, fever, and chills. Despite oral amoxicillin/ clavulanic acid treatment, the patient's complaints increased; he developed shortness of breath and was admitted to another hospital. The patient had weight loss of 8 kg in the prior three months. Two years before admission, the patient was suspected to have BD because of recurrent anterior uveitis and treated with infliximab. The patient never developed oral or genital ulcers, arthralgia, or neurological complaints during follow-up. There was no family history of BD or consanguinity. A written informed consent was obtained from the parents of the patient.

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“…Some patients will meet indications for aortic valve replacement due to severe aortic valve regurgitation, and some may need concomitant aortic root or ascending aortic replacement. The role of immunosuppressive therapy to prevent valve degeneration is the focus of ongoing research [55]. There are no specific recommendations for Behcet disease; however, it is reasonable to follow diagnostic and treatment options used in other diseases known to cause aortic regurgitation.…”

Section: Behçet's Diseasementioning

confidence: 99%

Disorders of the Aorta and Aortic Valve in Connective Tissue Diseases

et al. 2020

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Purpose of Review The incidence of aortic valve disease in inherited connective tissue disorders is well documented; however, recent studies have only begun to unravel the pathology behind this association. In this review, we aim to describe the etiology, clinical manifestations, management, and prognosis of aortic and aortic valvular disorders that co-exist in a variety of connective tissue diseases. An extensive literature review was performed in PubMed. Articles from 2008 to 2018 were included for review. Predetermined search terms used in PubMed include "aortic manifestation of connective tissue diseases" and "aortic valve disorders in rheumatologic disease." Recent Findings Manifestations of aortic valve disease in the context of connective tissue disorders include valvular stenosis, regurgitation, and/or thoracic aortic aneurysms. Both inherited and inflammatory connective tissue disorders contribute to aortic valve damage with increased susceptibility associated with specific gene variants. Summary Anti-inflammatory and immunosuppressive therapies have demonstrated beneficial results in Marfan's syndrome, Behcet disease, rheumatoid arthritis, ankylosing spondylitis, and systemic sclerosis, often leading to remission. Yet, such therapy is less effective in other disorders compared to alternative treatments such as surgical intervention. Additionally, regular echocardiographic studies should be recommended to those suffering from these disorders, especially those at higher risk for cardiovascular involvement. Given the rates of relapse with immunosuppressants, even following aortic valve replacement, further studies are needed to determine if certain dosing and/or combinations of immunosuppressants could be given to those diagnosed with connective tissue diseases to prevent progression of aortic valve involvement.

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Behçet's Disease, Echocardiographers, and Cardiac Surgeons: Together is Better

Cited by 16 publications

References 42 publications

Right Ventricular Thrombus and Tricuspid Valve Dysfunction in a Patient with Beh&ccedil;et’s Disease

Right Ventricular Thrombus and Tricuspid Valve Dysfunction in a Patient with Beh&ccedil;et’s Disease

Is Immunosuppressive and Thrombolytic Therapy Really Effective in a Patient With Intracardiac Thrombosis and Pulmonary Artery Aneurysm due to Behçet’s Disease?

Disorders of the Aorta and Aortic Valve in Connective Tissue Diseases

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Behçet's Disease, Echocardiographers, and Cardiac Surgeons: Together is Better

Cited by 16 publications

References 42 publications

Right Ventricular Thrombus and Tricuspid Valve Dysfunction in a Patient with Beh&amp;ccedil;et’s Disease

Right Ventricular Thrombus and Tricuspid Valve Dysfunction in a Patient with Beh&amp;ccedil;et’s Disease

Is Immunosuppressive and Thrombolytic Therapy Really Effective in a Patient With Intracardiac Thrombosis and Pulmonary Artery Aneurysm due to Behçet’s Disease?

Disorders of the Aorta and Aortic Valve in Connective Tissue Diseases

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Resources

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Right Ventricular Thrombus and Tricuspid Valve Dysfunction in a Patient with Behçet’s Disease

Right Ventricular Thrombus and Tricuspid Valve Dysfunction in a Patient with Behçet’s Disease