Pulmonary Arterial Hypertension in a Patient with Connective Tissue Disease and ALCAPA Syndrome
نویسندگان
چکیده
منابع مشابه
Connective tissue disease-associated pulmonary arterial hypertension
Although rare in its idiopathic form, pulmonary arterial hypertension (PAH) is not uncommon in association with various associated medical conditions, most notably connective tissue disease (CTD). In particular, it develops in approximately 10% of patients with systemic sclerosis and so these patients are increasingly screened to enable early detection. The response of patients with systemic sc...
متن کاملSildenafil for pulmonary arterial hypertension associated with connective tissue disease.
OBJECTIVE Pulmonary arterial hypertension associated with connective tissue disease (PAH-CTD) is difficult to manage, and has a poor prognosis. The phosphodiesterase-5 inhibitor sildenafil citrate enhances vasodilatation, has antiproliferative effects, and is effective in the treatment of PAH. We examined the efficacy and safety of oral sildenafil in patients with PAH-CTD. METHODS In a 12-wee...
متن کاملConnective tissue disease associated with pulmonary arterial hypertension: management of a patient with severe haemodynamic impairment.
Affiliations: Faculté de Médecine, Université Paris-Sud, Le Kremlin-Bicêtre, France. AP–HP, Centre de Référence de l’hypertension pulmonaire sévère, Service de pneumologie et soins intensifs, Hôpital de Bicêtre, Le Kremlin-Bicêtre, France. INSERM U-999, LabEx LERMIT, Centre chirurgical Marie Lannelongue, Le Plessis-Robinson, France. Service de Médecine Vasculaire et Thérapeutique, Hôpital Nord,...
متن کاملPulmonary arterial hypertension in connective tissue diseases.
Pulmonary arterial hypertension (PAH) is an entity that is known to complicate connective tissue diseases (CTD). PAH in CTD is a very important diagnosis which greatly affects treatment and prognosis. The most commonly affected CTD is scleroderma, although lupus, inflammatory myopathies such as poly and dermatomyositis, and mixed CTD are also associated with PAH. The manifestations of PAH have ...
متن کاملConnective tissue disease-associated pulmonary arterial hypertension in Chinese patients.
We sought to investigate the characteristics, survival and risk factors for mortality in Chinese patients with connective tissue disease (CTD)-associated pulmonary arterial hypertension (APAH) in modern therapy era. 129 consecutive adult patients who visited one of three referral centres in China with a diagnosis of CTD-APAH confirmed by right heart catheterisation during the previous 5 years w...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Respiratory Case Reports
سال: 2021
ISSN: 2147-2475
DOI: 10.5505/respircase.2021.45722