Current research suggests patients with pulmonary arterial hypertension may benefit from iron and vitamin D supplementation.
Cardiopulmonary exercise testing can be an important diagnostic and prognostic tool in pulmonary arterial hypertension.
The use of a risk calculator aimed at achieving a low-risk profile in PAH trials is discussed.
Recent evidence suggests that systemic sclerosis and systemic lupus erythematosus in pulmonary arterial hypertension should be considered separate diseases.
Lung transplantation may be an option in patients with severe pulmonary arterial hypertension who do not respond well to medical therapy.
Using biomarkers in pulmonary arterial hypertension may be a less invasive strategy for assessing response to treatment.
Pulmonary arterial hypertension is becoming a major cause of death in patients with systemic sclerosis.
Respiratory muscle dysfunction, iron deficiency, and skeletal muscle dysfunction have been implicated as mechanisms for exercise intolerance in pulmonary arterial hypertension.
Pulmonary arterial hypertension tends to vary in presentation in patients of different races and ethnicities.
Prenatal factors such as intrauterine growth retardation, infection, and placental inflammation can affect lung development in neonates.
Pulmonary thromboendarterectomy appears to be a curative resolution in patients with antiphospholipid syndrome related to chronic thromboembolic pulmonary hypertension.
Factors associated with a higher risk for mortality included male sex, exercise desaturation, and diffusing capacity of the lungs for carbon monoxide <50%.
Preterm infants who need mechanical ventilation at day 7 and early echocardiographic evidence of pulmonary vascular disease are strong risk factors for late respiratory disease.
At follow-up, the 6-minute walk distance, cardiac index, stroke volume index, and other hemodynamic variables were independently associated with transplant-free survival in patients with systemic sclerosis-associated pulmonary hypertension.
Riociguat was associated with increases in right ventricular work, right ventricular work index, stroke volume index, stroke volume index, and cardiac efficiency in patients with pulmonary arterial hypertension.
Higher doses of treprostinil significantly delayed time to first pulmonary arterial hypertension-related or all-cause hospitalization.
Recent data suggest that the endothelin receptor antagonist macitentan can be used to target the endothelin pathway in patients with portopulmonary hypertension.
In some patients at risk for pulmonary hypertension, left ventricular end diastolic pressure measurement with standard right heart catheterization should be used.
Tadalafil, a phosphodiesterase 5 (PDE5) inhibitor, is indicated to treat pulmonary arterial hypertension to improve exercise ability.
United Therapeutics announced that the Phase 3 trial of Orenitram for PAH has met its primary endpoint of delayed time to first clinical worsening event.