Aortic valve replacement in systemic sclerosis

Ferrari, Gabriele; Pratali, Stefano; Pucci, Angela; Bortolotti, Uberto

doi:10.2459/jcm.0b013e328365aa9d

Cited by 13 publications

(13 citation statements)

References 7 publications

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“…Even though the involvement of the heart is very common, valvular involvement in systemic sclerosis is uncommon and most often involves the mitral valve causing mitral valve prolapse [ 6 ]. Aortic stenosis is an extremely rare complication [ 7 ]. In 2007, De Groote, et al evaluated 570 patients with systemic sclerosis for cardiac abnormalities with Doppler echocardiography and found aortic stenosis in only 3.3% of the patients [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

An Unusual Case of Aortic Stenosis in Systemic Sclerosis

Kagalwalla

Gupta

Malwi

et al. 2017

Cureus

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Systemic sclerosis is a connective tissue disorder that frequently involves the heart. All cardiac structures can be involved but aortic valve involvement is rare. We report a case of an 83-year-old man with a history of systemic sclerosis presenting with shortness of breath. A transthoracic echocardiogram revealed severe aortic stenosis that could not be explained by other causes. These patients often pose challenges in management as they are not surgical candidates. Transaortic valvular implantation is a viable option for these patients.

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Section: Discussionmentioning

confidence: 99%

An Unusual Case of Aortic Stenosis in Systemic Sclerosis

Kagalwalla

Gupta

Malwi

et al. 2017

Cureus

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“…The patient was not on corticosteroid and did not have infectious complications. Follow-up after 1 year was positive; the patient was in sinus rhythm and in NYHA class 1 and well-functioning prosthesis with a mean transprosthetic gradient of 15 mmHg [25].…”

Section: Systemic Sclerosismentioning

confidence: 98%

“…The third case was that of a 69-year-old woman that presented with severe dyspnea due to aortic stenosis diagnosed via trans-thoracic echocardiography; she had a trans-aortic gradient of 45 mmHg and valve area of 0.9 cm 2 . She had limited scleroderma without lung involvement demonstrated by CT lung imaging [25]. The aortic valves of the 3 patients had 3 different histopathologic findings.…”

Section: Systemic Sclerosismentioning

confidence: 99%

“…Hence, aortic stenosis in scleroderma does not have a specific pathogenesis. The three patients were managed by replacing their aortic valves with biological prostheses [24,25] The first and second patients were asymptomatic after the surgery and were discharged after 7 days, and upon followup after 38 months and 12 months, they were both stable. No infectious complications were noted in both patients after the surgery despite being on corticosteroids.…”

Section: Systemic Sclerosismentioning

confidence: 99%

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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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“…However, some medications, such as ACE-inhibitors, long-acting nifedipine, or β-blockers have been tried in acute valvular diseases or as a bridge to surgery in severely decompensated patients, despite the fact that studies show inconsistent results regarding their efficacy (116). Based on two recently published case reports of SSc patients with aortic valve stenosis, valve replacement in this setting is justified, provided care is taken to avoid hemorrhagic complications and regurgitation during intubation, as well as hypothermia during cardiopulmonary bypass to prevent induction of Raynaud’s phenomenon (117, 118). One additional report described a case series of six SSc patients with severe aortic stenosis who successfully underwent transcatheter aortic valve implantation (TAVI), suggesting that, where indicated, this procedure is a feasible alternative for the management of this high-risk population (119).…”

Section: Valvular Heart Disease: Assessment and Managementmentioning

confidence: 99%

Update on assessment and management of primary cardiac involvement in systemic sclerosis

Bournia

Tountas

Protogerou

et al. 2018

Journal of Scleroderma and Related Disorders

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Primary cardiac involvement is a common and severe complication of systemic sclerosis, which may affect all of the hearts' structural components, including pericardium, myocardium, endocardium, cardiac valves, and conduction system. While cardiac disease can be clinically silent and only diagnosed in autopsy, new imaging modalities such as speckle-tracking echocardiography and cardiovascular magnetic resonance may reveal various abnormal findings in the majority of patients. Cardiovascular magnetic resonance evaluation should include assessment of left and right ventricular function, inflammation (STIR T2-weighted sequences (T2-W) for edema detection), and fibrosis (T1-weighted sequences 15 min after Gd-DTPA contrast medium injection (late-gadolinium enhancement). Notably, cardiac disease is responsible for about one-fourth of systemic sclerosis-related deaths. Systematic studies for the assessment and therapy of systemic sclerosis-related cardiac complications, as well as relevant guidelines from the European League Against Rheumatism and the American College of Rheumatology, are currently lacking. However, research advances reviewed herein allow for a better understanding of the mechanisms that alter cardiac function. Implementation of such knowledge should reduce cardiac morbidity and mortality in systemic sclerosis patients.

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Aortic valve replacement in systemic sclerosis

Cited by 13 publications

References 7 publications

An Unusual Case of Aortic Stenosis in Systemic Sclerosis

An Unusual Case of Aortic Stenosis in Systemic Sclerosis

Disorders of the Aorta and Aortic Valve in Connective Tissue Diseases

Update on assessment and management of primary cardiac involvement in systemic sclerosis

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