Treat-to-target strategies in pulmonary arterial hypertension: the importance of using multiple goals.

Major advances have occurred in the treatment of pulmonary arterial hypertension (PAH) over the past decade. The advent of PAH-specific pharmacological treatments has offered hope to patients with a debilitating, progressive disease and a poor prognosis. Combined drug treatment offers improved benefits over monotherapy, and current treatment guidelines for PAH recommend a sequential add-on approach to combination therapy for patients in New York Heart Association (NYHA)/World Health Organization functional class (WHO FC) II-IV. Goal-oriented therapy determines the timing of treatment escalation by inadequate response to known prognostic indicators. Close monitoring of patients aids the early identification of inadequate response, so that treatment can be escalated promptly and before the patient's condition deteriorates further. Existing treatment goals are based on baseline values of prognostic indicators, but it is vital to identify risk factors that are both relevant during treatment and that can be assessed during follow-up appointments. Data from different PAH aetiologies indicate that NYHA/WHO FC is the most appropriate prognostic marker, with 6-min walk distance and several haemodynamic parameters representing alternatives. Future refinement of goal-oriented therapy could include the use of multiple prognostic markers, while additional, large clinical trials will answer questions concerning choice and combination of treatment goals

The role of the right ventricle in pulmonary arterial hypertension

Although changes in the pulmonary vasculature are the primary cause of pulmonary arterial hypertension (PAH), severity of symptoms and survival are strongly associated with right ventricular function, and right heart failure is the main cause of death in patients with PAH. Echocardiography and cardiac magnetic resonance imaging allow noninvasive evaluation of right ventricular function and structure, and a number of indices have been shown to have potential prognostic value in PAH. Given the importance of the right ventricle in PAH, preservation and improvement of its function should be important aspects of therapy; however, there are currently few data specifically related to this aspect of treatment response. Simple, reproducible, noninvasive measures of right ventricular function would help to improve the management of patients with PAH, and to provide tools with which to help establish the optimal therapeutic approach to manage not only the effects of the disease on the pulmonary vasculature, but also to support and improve right ventricular functio

Acute hand ischemia after radial intervention in patient with CREST-associated pulmonary hypertension: successful treatment with manual thromboaspiration.

We describe the case of a 60-year-old woman with CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) associated pulmonary hypertension undergoing transradial coronary angiography. The day after the procedure, the patient complained of severe symptoms and signs of acute hand ischemia. Urgent right upper extremity angiography showed the lack of ulnar palmar arch and a severe narrowed radial artery with endoluminal filling defect. The patient was successfully treated with manual thromboaspiration leading to a complete flow restoration and symptom relief. This case shows that radial occlusion, one of the most common and usually asymptomatic complications following transradial cardiac catheterization, may cause severe hand ischemia in patients with small-vessel inflammatory disease

Ipertensione polmonare: Necessit\ue0 di chiarezza tra definizione emodinamica-fisiopatologica, stima ecocardiografica e diagnosi clinica finale. Pulmonary hypertension and the relationships between hemodynamic-pathophysiologic definitions, echocardiographic estimate and final clinical diagnosis: clarification is needed.

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Pulmonary hypertension and pulmonary arterial hypertension: a clarification is needed.

[No abstract available

Current therapeutic approaches to pulmonary arterial hypertension.

Pulmonary hypertension is a heterogeneous hemodynamic and pathophysiological state that is observed in a number of clinical conditions, which have been divided into six diagnostic groups. Although the increase in pulmonary pressure observed in these clinical groups may be similar, underlying disease mechanisms, diagnostic methods, and prognostic and therapeutic consequences are completely different. Pulmonary arterial hypertension is associated with several rare conditions that have comparable clinical and hemodynamic characteristics and exhibit virtually identical anatomical and pathological alterations in the lung microcirculation. These conditions include idiopathic and familial forms of the disease and disease forms associated with connective tissue disease, congenital heart defects involving systemic-to-pulmonary arterial shunts, portal hypertension, and HIV infection. It has been shown that treatment with specific drugs (e.g. prostanoids, endothelin-receptor antagonists and phosphodiesterase type-5 inhibitors) is effective in these patients and that these drugs can also be administered in various combinations. An evidence-based treatment algorithm has been developed for these patients. In patients with pulmonary hypertension due to left heart disease or lung disease, treatment focuses on the underlying condition and there is no convincing evidence that agents approved for pulmonary arterial hypertension are effective. For patients with chronic thromboembolic pulmonary hypertension, the treatment of choice is pulmonary endarterectomy. However, drugs intended specifically for the treatment of pulmonary arterial hypertension may be considered in inoperable cases or after suboptimal surger

Liver toxicity of sitaxentan in pulmonary arterial hypertension.

No abstract availabl

Clinical worsening in trials of pulmonary arterial hypertension: results and implications.

Purpose of review: Time to clinical worsening (TTCW) can be used to assess disease progression associated with pulmonary arterial hypertension (PAH). As a consequence, it is highly relevant to patients, clinicians, and regulatory agencies. The majority of clinical trials of PAH-specific drug therapies have included TTCW as a secondary endpoint; this article summarizes the results of randomized controlled clinical trials in PAH, specifically with respect to the clinical worsening endpoint. Recent findings: Some trials have demonstrated a treatment-related delay in TTCW and others have not. Recent results suggest that TTCW shows particular promise in detecting disease progression, even in mildly affected patients. Definitions of clinical worsening have also varied across clinical trials; although all have agreed on the inclusion of all-cause death and hospitalization due to PAH in the definition, the inclusion of additional parameters defining 'disease progression' has differed. Summary: There is a need for a clear and uniform definition of TTCW that can be tailored to the study population being investigated; the endpoint may require adaptation for patients in different functional classes and with different causes. Consistency of event reporting within a trial may be improved by employing a committee to adjudicate events. Trials are beginning to include TTCW as a primary endpoint; the results will be important in establishing the validity of whether this parameter should become the endpoint of choice in PAH trials in the futur