Articles: connective-tissue-diseases.
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Review Case Reports
Pulmonary Hypertension Associated with Anti-synthetase Syndrome: A Case Report and Literature Review.
Pulmonary hypertension (PH) is a serious condition in which there is an abnormally high pressure in the pulmonary arteries that can occur as a complication of connective tissue diseases. Although the relationship between PH and systemic lupus erythematosus or systemic sclerosis has been well-characterized, PH rarely occurs in patients with anti-synthetase syndrome (ASS), and little is known about the pathophysiology and clinical outcome of patients with ASS-PH. We herein report a patient with anti-Jo-1-positive ASS complicated by PH and discuss the treatment strategy through a review of previously reported cases.
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Connective tissue disorders, including Marfan syndrome (MS) and Ehlers-Danlos syndrome (EDS), are characterized by genetic mutations affecting connective tissue structural integrity. These disorders significantly elevate the risk of aortic dissection, a life-threatening condition. This comprehensive review delves into the intricate interplay between connective tissue disorders and aortic dissection, shedding light on the clinical features, pathophysiology, genetic underpinnings, diagnostic approaches, clinical management, associated comorbidities, and prognosis, mainly focusing on MS and EDS, while also exploring rare connective tissue disorders and forms of cutis laxa contributing to aortic pathology.
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J. Thorac. Cardiovasc. Surg. · Dec 2023
Aortic valve reimplantation in patients with connective tissue disorders: Are the leaflets durable?
The durability of reimplanted myxomatous aortic valves in root replacements for patients with connective tissue disorders (CTD) is unclear; therefore, we sought to evaluate the long-term resilience of these repairs. ⋯ Aortic valve reimplantation is a durable operation in patients with CTD and root aneurysms. These patients do not experience early degeneration of their reimplanted aortic valves.
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Multisystemic inflammatory syndrome is a syndrome of multisystem involvement caused by a novel coronavirus infection that can lead to cardiogenic shock and death in children. ⋯ Multisystem inflammatory syndrome is often mistaken for Kawasaki disease, fortunately, their treatments are similar, the purpose of this case is to remind clinicians of the need for early management of children with multisystem failure following novel coronavirus infection, increase the detection rate, and save the life of the child.