Impact of age on pulmonary artery systolic pressures at rest and with exercise.

Aim: It is not well known if advancing age influences normal rest or exercise pulmonary artery pressures. The purpose of the study was to evaluate the association of increasing age with measurements of pulmonary artery systolic pressure at rest and with exercise.

Subjects And Methods: A total of 467 adults without cardiopulmonary disease and normal exercise capacity (age range: 18-85 years) underwent symptom-limited treadmill exercise testing with Doppler measurement of rest and exercise pulmonary artery systolic pressure.

Cardiovascular risk in pulmonary alveolar proteinosis.

We hypothesized that cardiovascular events and/or indices of cardiac dysfunction may be increased in pulmonary alveolar proteinosis (PAP). Systemic and pulmonary arterial hypertension, arrhythmias, pulmonary embolism, stroke and ischemic heart attack were reported. Patients underwent serum anti-GM-CSF antibodies, disease severity score (DSS), Doppler transthoracic echocardiograph, glucose, thyroid hormones, lipids, troponin and pro-Brain natriuretic peptide (BNP) examination.

Early decline in six-minute walk distance from the time of diagnosis predicts clinical worsening in pulmonary arterial hypertension.

Background: The six-minute walk distance (6MWD) is commonly used to assess pulmonary arterial hypertension (PAH). However, the role of 6MWD in predicting outcomes in PAH is controversial. Clinical worsening is being increasingly considered as a clinically meaningful end point in PAH.

Management of the patient with pulmonary arterial hypertension receiving intravenous prostacyclin: an expert nurse practical guide.

Pulmonary arterial hypertension (PAH) is a severely disabling disorder characterized by elevated pulmonary artery pressure ultimately leading to right heart failure and death. Treatment options have significantly increased over the past decade. Intravenous prostacyclins remain the treatment of choice for advanced PAH, leading to long-term clinical benefits and improved survival.

Pulmonary hypertension in β thalassaemia.

Pulmonary hypertension is one of the leading causes of morbidity and mortality in patients with haemolytic disorders and is a frequent finding in echocardiographic screening of patients with β thalassaemia. Substantial progress has been made in understanding of the multifactorial pathophysiology of pulmonary hypertension in β thalassaemia. Haemolysis, reduced nitric oxide bioavailability, iron overload, and hypercoagulopathy are among the main pathogenetic mechanisms.

Pulmonary hypertension in parenchymal lung disease.

Idiopathic pulmonary arterial hypertension (IPAH) has been extensively investigated, although it represents a less common form of the pulmonary hypertension (PH) family, as shown by international registries. Interestingly, in types of PH that are encountered in parenchymal lung diseases such as interstitial lung diseases (ILDs), chronic obstructive pulmonary disease (COPD), and many other diffuse parenchymal lung diseases, some of which are very common, the available data is limited. In this paper, we try to browse in the latest available data regarding the occurrence, pathogenesis, and treatment of PH in chronic parenchymal lung diseases.

Diagnostic evaluation and management of chronic thromboembolic pulmonary hypertension: a clinical practice guideline.

Background: Pulmonary embolism is a common condition. Some patients subsequently develop chronic thromboembolic pulmonary hypertension (CTEPH). Many care gaps exist in the diagnosis and management of CTEPH patients including lack of awareness, incomplete diagnostic assessment, and inconsistent use of surgical and medical therapies.

Right ventricular strain for prediction of survival in patients with pulmonary arterial hypertension.

Background: Pulmonary arterial hypertension (PAH) is a devastating illness of pulmonary vascular remodeling, right-sided heart failure, and limited survival. Whether strain-based measures of right ventricular (RV) systolic function predict future right-sided heart failure and/or death is untested.

Methods: RV longitudinal systolic strain and strain rate were evaluated by echocardiography in 80 patients with World Health Organization group 1 pulmonary hypertension (PH) (72% were functional class [FC] III or IV).

Integration of clinical and hemodynamic parameters in the prediction of long-term survival in patients with pulmonary arterial hypertension.

Background: Current management guidelines for pulmonary arterial hypertension (PAH) recommend a treatment choice based primarily on World Health Organization (WHO) functional class. This study was designed to assess how the incorporation of readily obtained clinical and test-based information may significantly improve the prediction of outcomes over functional class alone.

Methods: Clinical and hemodynamic variables were assessed in 484 consecutive patients presenting with WHO group 1 PAH.

Bosentan for pulmonary hypertension and other pulmonary diseases: emerging evidence.

Endothelin-1 is a potent vasoconstrictor and mitogen that is primarily synthesized and released from vascular endothelial cells. Bosentan is a dual endothelin-receptor antagonist that initially received approval for treatment of WHO group I pulmonary arterial hypertension (PAH) for patients in functional classes III and IV. Analysis of a study conducted in functional class II patients (Endothelin Antagonist Trial in Mildly Symptomatic PAH Patients [EARLY] trial) suggest its efficacy for these less symptomatic patients.

Treatment of right ventricular dysfunction in pulmonary arterial hypertension: theoretical considerations.

Significant progress has been made in the medical therapy of pulmonary hypertension in recent years, but long term results are still disappointing. We describe the theoretical underpinnings of a surgical procedure which we suggest might prevent or delay the occurrence of right ventricular failure and thereby improve long-term survival in pulmonary arterial hypertension. This is a hypothesis which could be tested in an animal model of this condition.

Pulmonary hypertension: diagnosis and management.

Pulmonary arterial hypertension is a progressive, symptomatic, and ultimately fatal disorder for which substantial advances in treatment have been made during the past decade. Effective management requires timely recognition and accurate diagnosis of the disorder and appropriate selection among therapeutic alternatives. Despite progress in treatment, obstacles remain that impede the achievement of optimal outcomes.

Pulmonary capillary endothelial metabolic function in chronic thromboembolic pulmonary hypertension.

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) causes physical plugging of large pulmonary arteries as well as a distal micro-vasculopathy. Pulmonary endothelium is an active metabolic tissue in normal humans. The effects of CTEPH on pulmonary endothelial metabolism are unknown.

Obstructive sleep apnea and cardiovascular risk.

Obstructive sleep apnea (OSA) is a form of sleep disordered breathing characterized by episodes of apnea (during sleep) lasting at least 10 seconds per episode. The apneic periods are associated with arterial hypoxemia and disruption of normal sleep as a result of awakenings. It is increasingly being recognized that OSA is a public health hazard and there is increasing evidence that it is associated with an increase in morbidity (and possibly mortality).

Peripheral blood B lymphocytes derived from patients with idiopathic pulmonary arterial hypertension express a different RNA pattern compared with healthy controls: a cross sectional study.

Background: Idiopathic pulmonary arterial hypertension (IPAH) is a progressive and still incurable disease. Research of IPAH-pathogenesis is complicated by the lack of a direct access to the involved tissue, the human pulmonary vasculature. Various auto-antibodies have been described in the blood of patients with IPAH.

Nesiritide for pulmonary arterial hypertension with decompensated cor pulmonale.

Pulmonary arterial hypertension complicated by decompensated cor pulmonale is a challenging clinical problem with few effective therapeutic options. B-type natriuretic peptide is a pluripotent hormone that promotes diuresis and natriuresis, vasodilates systemic and pulmonary vessels, and reduces circulating levels of endothelin and aldosterone. It may represent a possible therapeutic strategy for decompensated cor pulmonale in the same manner that it is used to treat decompensated left heart failure.

[Pulmonary hypertension--classification and treatment: new guidelines].

Pulmonary arterial hypertension is a severe pulmonary vascular disease characterized by elevated pulmonary vascular resistance and low cardiac output. Prior to prostacyclin therapy, it was a fatal disease with a median survival of 3 years. In recent years, the understanding and treatment of the disease have significantly improved patient outcome.