[Treatment of pulmonary arterial hypertension]. / Traitement de l'hypertension artérielle pulmonaire.

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. The target of current therapies is endothelial dysfunction of pulmonary arteries. Continuous intravenous infusion of epoprostenol remains the only recommended therapy for the most severe patients with PAH. 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.

Autonomic modulation in a patient with syncope and paroxysmal atrial-fibrillation.

We report a case of a patient with recurrent syncope and paroxysmal atrial fibrillation whose clinical status greatly improved after a period of orthostatic training. The potential efficacy of this non-pharmacological measure in modulating the autonomic tone is discussed below.

Carotid sinus syndrome, should we pace? A multicentre, randomised control trial (Safepace 2).

BACKGROUND: Cardioinhibitory carotid sinus hypersensitivity (CICSH) is highly prevalent among older people with falls. OBJECTIVE: To assess the efficacy of dual-chamber pacing in older patients with CICSH and unexplained falls. DESIGN: A multicentre, double blind, randomised controlled trial. SETTING: Selection from emergency room, geriatric medicine and orthopaedic departments. PATIENTS: Patients aged >50 years, with two unexplained falls and/or one syncopal event in the previous 12 months for which no other cause is evident apart from CICSH. INTERVENTIONS: Patients randomised to either a 700/400 kappa, rate responsive pacemaker or implantable loop recorder (Medtronic Reveal thera RDR, Medtronic, Minneapolis, Minnesota, USA). MAIN OUTCOME MEASURES: The primary outcome was the number falls after implantation. Secondary outcomes were time to fall event, presyncope, quality of life and cognitive function. RESULTS: 141 patients were recruited from 22 centres. Mean age was 78 years and mean follow-up 24 months. The overall relative risk of falling after device implantation compared with before was 0.23 (0.15 to 0.32). No significant reduction in falls was seen between paced and loop recorder groups (RR=0.79; 95% CI 0.41 to 1.50). Data were also consistent in both groups for syncope, quality of life and cognitive function. Conclusions These results question the use of pacing in CICSH. However, the study was underpowered and also patient characteristics differed from those in Safepace 1-participants were physically and cognitively frailer. Further work is necessary to assess cardiac pacing in this setting.

Orthostatic circulatory disorders in early neurorehabilitation: a case report and management overview.

Orthostatic circulatory disorders are a common problem in the mobilization of patients with severe neurological diseases such as paraplegia, the vegetative state or the minimally conscious state. They create difficulties when mobilizing the patient out of bed. Although their incidence has not been clearly established and the severity and length of symptoms differ greatly, a relevant number of the patients in a rehabilitation unit is affected. Rehabilitation specialists should therefore be aware of these disorders and the therapeutic alternatives available. This case study reports on a 45-year-old patient who repeatedly suffered from orthostatic hypotension after a severe traumatic brain injury. The pathogenesis and predisposing factors of orthostatic dysfunction in severely disabled neurological patients as well as therapeutic efforts are subsequently reviewed.

Tilt training: a new challenge in the treatment of neurally mediated syncope.

Clinical experience with repeated tilt testing and standing training has shown that this procedure can be used as a new therapy for the restoration of abnormal autonomic orthostatic reflexes. The introduction of tilt training therapy for neurally mediated syncope is a new and effective treatment. This new therapy has been used recently in several centres with excellent results. Since syncope is a severe social handicap for the patient, successful therapy will normalise the functional status and restore self-confidence. The use of tilt training can be considered as first-line therapy in this disorder. In the majority of the studies on tilt training, patients remain free of syncope during active treatment with this therapy. In about 50% of the patients with neurally mediated syncope already the second consecutive tilt test became negative. This therapeutic effect of in-hospital repeated tilt tests is sustained by continued standing training at home.

a 49-year-old woman with dyspnoea, palpitations and syncope.

Pulmonary hypertension is rarely described in association with Sjögren's syndrome. The authors report the case of a patient in which pulmonary hypertension was the inaugural clinical manifestation of primary Sjögren's syndrome. Clinical assessment, differential diagnosis, etiopathological implications, and therapeutic approach are discussed.