Presse Med
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Patients with pulmonary arterial hypertension (PAH) must be referred to expert centers (reference center and competences centers in the French PAH network). Despite progresses in the knowledge of PAH pathophysiology, it is still a devastating disease needing an aggressive approach of therapy to improve long-term outcomes. ⋯ If treatment goals are not met, a sequential combination therapy is recommended (i.e. combination of drugs targeting different dysfunctional pathways). Lung transplantation remains the only curative treatment of PAH.
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Chronic thromboembolic pulmonary hypertension is a rare but underdiagnosed disease. The development of imaging played a crucial role for the screening and the decision of operability over the past few years. Indeed, chronic thromboembolic pulmonary hypertension is the only type of pulmonary hypertension with a potential curative treatment: pulmonary endarterectomy. ⋯ If there is a correlation between the vascular obstruction assessed by imaging and the pulmonary resistance, pulmonary endarterectomy carries a postoperative mortality of less than 3% and has a high rate of success. If the surgery is performed at a later stage of the disease, pulmonary arteriolitis developed mainly in unobstructed territories and participated in the elevated vascular resistance. At this stage, postoperative risk is higher.
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Pulmonary arterial hypertension (PAH) is a classical complication of connective tissue diseases (CTD), particularly in systemic sclerosis (SSc), systemic lupus erythematous (SLE) or mixed connective tissue diseases (MCTD). The prevalence of PAH in SSc, as measured by right heart catheterization (RHC), is estimated between 7.85 to 13%. The detection of PAH in SSc is based on trans-thoracic echocardiography. ⋯ The main characteristic of PAH related to CTD other than SSc is a good response to immunosuppressive treatment, with an improvement in 50% of cases in SLE or MCTD. The prognosis of PAH associated with CTD seem to improve with the diversification of treatments available, but remains reserved. Therapeutic combinations and new molecules should allow to improve the prognosis.
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Ciguatera, an ichtyosarcotoxism linked to the consumption of usually healthy coral fish is a common poisoning in the Pacific, Caribbean and Indian Ocean where it is endemic. However, increased tourism and commercial transportation of tropical fish for consumption make it an unexceptional intoxication in countries away from its endemic area. ⋯ The diagnosis is clinical and relatively easy in endemic areas but much less for physicians who are rarely confronted with, which is a source of prolonged diagnostic delays and a significant increase in spending. Treatment of ciguatera is symptomatic but new treatments, still experimental, give a real hope for the future.