PAH associated with CHD is linked to significant reductions in survival and quality of life, especially for patients progressing to Eisenmenger syndrome.
Although pulmonary hypertension as a result of interstitial lung disease is linked to significant morbidity and mortality, there is no consensus regarding screening or management of the disease.
Emerging evidence suggests that the extrapulmonary effects of pulmonary arterial hypertension may manifest in the form of vascular dysfunction in systemic circulation, metabolic/endocrine dysfunction, and skeletal and respiratory muscle dysfunction.
An abridged version of the REVEAL risk calculator, the REVEAL Lite 2, represents a simpler and accurate tool for predicting mortality risk in patients with pulmonary arterial hypertension.
Oral combination therapy does not appear to help achieve or maintain low risk status in most treatment-naive patients with pulmonary arterial hypertension.
Pulmonary arterial compliance was found to be strongly associated with all-cause mortality and clinical worsening in patients with systemic lupus erythematosus-associated pulmonary arterial hypertension.
Nighttime nasal high flow therapy at 20 L/min reduced sympathetic drive in patients with precapillary pulmonary hypertension and obstructive sleep apnea.
Pulmonary artery denervation in patients with residual chronic thromboembolic pulmonary hypertension after pulmonary endarterectomy was associated with improved hemodynamics and clinical and functional status compared with medical therapy.
Female candidates for liver transplant who have portopulmonary hypertension have higher pulmonary vascular resistance, lower Model for End-Stage Liver Disease scores, and are more likely to have autoimmune liver disease, compared with male candidates.
A combination of C-arm computed tomography with conventional digital subtraction angiography of the pulmonary arteries can enhance the diagnostic work-up of patients with suspected CTEPH.
