Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: Definitions and Treatments

Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: Definitions and Treatments

Pulmonary arterial hypertension is becoming a major cause of death in patients with systemic sclerosis.

Idiopathic PAH vs Scleroderma-Associated PAH: Pulmonary Function and Hemodynamic Differences

Idiopathic PAH vs Scleroderma-Associated PAH: Pulmonary Function and Hemodynamic Differences

Results show statistically significant differences between SSc-PAH groups and iPAH groups for variables including age, sex, FVC capacity, total lung capacity, and FEV1, among others.

Impaired Cerebral Pressure-Flow Relationship in PAH

Impaired Cerebral Pressure-Flow Relationship in PAH

Patients with pulmonary arterial hypertension have reduced cerebrovascular reactivity to CO2.

Pulmonary Arterial Hypertension: Mechanisms of Exercise Intolerance

Pulmonary Arterial Hypertension: Mechanisms of Exercise Intolerance

Respiratory muscle dysfunction, iron deficiency, and skeletal muscle dysfunction have been implicated as mechanisms for exercise intolerance in pulmonary arterial hypertension.

Redesigned Inhalation Device for PAH Approved by FDA

Redesigned Inhalation Device for PAH Approved by FDA

The new device features a more ergonomic design with a single-button operation, an intuitive user interface to adjust breath counts, a rechargeable battery, and a display that guides patients through the inhalation process.

Phenotypes Associated With EIF2AK4 Mutations in PAH

Phenotypes Associated With EIF2AK4 Mutations in PAH

The frequency of eukaryotic translation initiation factor 2 alpha kinase 4 gene (EIF2AK4) mutations in patients with pulmonary arterial hypertension was investigated.

Pulmonary HTN Linked to Increased Mortality During Cardiac Catheterization

Pulmonary HTN Linked to Increased Mortality During Cardiac Catheterization

Borderline PH was linked to increased mortality after adjustment for 34 variables.

Pulmonary Edema After Prostacyclin Therapy for PAH Associated With Worse Outcomes

Pulmonary Edema After Prostacyclin Therapy for PAH Associated With Worse Outcomes

Patients who develop pulmonary edema after starting prostacyclin for pulmonary arterial hypertension have a greater risk for mortality.

PAH-Associated Congenital Heart Disease Outcomes Predicted by Pulmonary Artery Compliance

PAH-Associated Congenital Heart Disease Outcomes Predicted by Pulmonary Artery Compliance

Pulmonary vascular compliance may independently predict survival and prognosis in patients with pulmonary arterial hypertension associated with congenital heart disease.

Pulmonary Arterial Compliance Improved With Riociguat vs PDE5 Inhibitors

Pulmonary Arterial Compliance Improved With Riociguat vs PDE5 Inhibitors

Patients with pulmonary arterial hypertension experienced better improvements in pulmonary arterial compliance with riociguat compared with phosphodiesterase type 5 inhibitors.

Bronchoalveolar Lavage for Diagnosis of Idiopathic Pulmonary Fibrosis: Point/Counterpoint

Bronchoalveolar Lavage for Diagnosis of Idiopathic Pulmonary Fibrosis: Point/Counterpoint

Lung disease experts Athol Wells, MD, and Joshua Mooney, MD, MS, debate the diagnostic value of bronchoalveolar lavage in idiopathic pulmonary fibrosis.

Extent of Right Ventricular Epicardial Vascularization in Pulmonary Arterial Hypertension

Extent of Right Ventricular Epicardial Vascularization in Pulmonary Arterial Hypertension

Right ventricular epicardial vasculature is more extensive in patients with pulmonary arterial hypertension compared with individuals without disease.

No Associations Exist Between 6MWD and Maximum Oxygen Uptake in Pulmonary Arterial Hypertension

No Associations Exist Between 6MWD and Maximum Oxygen Uptake in Pulmonary Arterial Hypertension

Results indicate that further study of the relationship between the 6-minute walk distance test and functional capacity in PAH is necessary.

Association Between Pulmonary Hypertension and Hereditary Hemorrhagic Telangiectasia

Association Between Pulmonary Hypertension and Hereditary Hemorrhagic Telangiectasia

A study sought to describe the occurrence of different types of pulmonary hypertension in hereditary hemorrhagic telangiectasia.

Examining Anxiety, Depression Rates in CTEPH, PAH

Examining Anxiety, Depression Rates in CTEPH, PAH

Depression, anxiety, and reduced healthcare-related quality of life are common in patients with either pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension.

Clinical Case: Patient With COPD and Worsening Dyspnea on Exertion

Clinical Case: Patient With COPD and Worsening Dyspnea on Exertion

A 65-year-old man with COPD presents with worsening exertional dyspnea.

Characteristics and Outcomes of Methamphetamine-Associated Pulmonary Arterial Hypertension

Characteristics and Outcomes of Methamphetamine-Associated Pulmonary Arterial Hypertension

Methamphetamine-associated pulmonary arterial hypertension has a higher risk for clinical worsening compared with idiopathic pulmonary arterial hypertension.

Pulmonary Veno-Occlusive Disease Affects Survival in PAH/Systemic Sclerosis

Pulmonary Veno-Occlusive Disease Affects Survival in PAH/Systemic Sclerosis

Having at least 2 features of pulmonary veno-occlusive disease demonstrated a trend toward worse survival in patients with pulmonary arterial hypertension and systemic sclerosis.

Guidelines Needed for 6-Minute Walk Test in Idiopathic Pulmonary Fibrosis

Guidelines Needed for 6-Minute Walk Test in Idiopathic Pulmonary Fibrosis

Specific guidelines should be developed for the use of the 6-minute walk test in idiopathic pulmonary fibrosis.

Pulmonary Arterial Hypertension: Future Treatment May Depend on Race and Ethnicity

Pulmonary Arterial Hypertension: Future Treatment May Depend on Race and Ethnicity

Pulmonary arterial hypertension tends to vary in presentation in patients of different races and ethnicities.

Right Ventricular Morphology Predictive of Pulmonary Hypertension Type

Right Ventricular Morphology Predictive of Pulmonary Hypertension Type

Differences in right ventricular morphology may predict the hemodynamic nature of pulmonary hypertension.

Pulmonary Endarterectomy in Chronic Thromboembolic Pulmonary Hypertension

Pulmonary Endarterectomy in Chronic Thromboembolic Pulmonary Hypertension

Pulmonary endarterectomy is an effective and durable treatment for chronic thromboembolic pulmonary hypertension.

Managing Pulmonary Hypertension in Preterm Neonates: Updates in an Emerging Field

Managing Pulmonary Hypertension in Preterm Neonates: Updates in an Emerging Field

Prenatal factors such as intrauterine growth retardation, infection, and placental inflammation can affect lung development in neonates.

No Increased Risk for VTE After Flu Vaccine in People Older Than 50

No Increased Risk for VTE After Flu Vaccine in People Older Than 50

Influenza vaccination does not increase the risk for venous thromboembolism in patients older than 50 years of age.

Depression and Anxiety Affect QoL in Idiopathic Pulmonary Fibrosis

Depression and Anxiety Affect QoL in Idiopathic Pulmonary Fibrosis

Depression and anxiety may affect quality of life for patients with idiopathic pulmonary fibrosis.

No Difference in Short-Term Outcome Between Saddle and Nonsaddle Pulmonary Embolism

No Difference in Short-Term Outcome Between Saddle and Nonsaddle Pulmonary Embolism

Similar short-term outcomes were seen in patients with saddle and nonsaddle pulmonary embolism.

Survival Rates in Eisenmenger Syndrome Treated With Pulmonary Vasodilator Therapy

Survival Rates in Eisenmenger Syndrome Treated With Pulmonary Vasodilator Therapy

Patients undergoing pulmonary vasodilatory therapy were more functional at initial presentation.

Poorer Right Atrial Function Predicts Worse Prognosis in Pulmonary Arterial Hypertension

Poorer Right Atrial Function Predicts Worse Prognosis in Pulmonary Arterial Hypertension

Poor outcomes in pulmonary arterial hypertension may be linked to worsening right atrial function.

Prediction of Short-, Long-Term Mortality in Elderly With Stable Acute PE

Prediction of Short-, Long-Term Mortality in Elderly With Stable Acute PE

Charleston comorbidity index is an independent predictor of mortality in patients with hemodynamically stable pulmonary embolism.

Pediatric Pulmonary Arterial Hypertension Drug Approved by FDA

Pediatric Pulmonary Arterial Hypertension Drug Approved by FDA

The first FDA-approved drug for pediatric pulmonary arterial hypertension will be available in 32 mg tablets for oral suspension.

Safety, Efficacy of Treprostinil Delivery System for PAH Examined

Safety, Efficacy of Treprostinil Delivery System for PAH Examined

Researchers were able to successfully use a fully implantable delivery system for treprostinil for adult patients with pulmonary arterial hypertension.

Using Hemodynamic Marker Ratio to Predict Outcomes in Pulmonary Hypertension

Using Hemodynamic Marker Ratio to Predict Outcomes in Pulmonary Hypertension

A retrospective analysis demonstrated that the ratio of left to right ventricular end-diastolic pressures can predict outcomes in patients with PH.

ACR Imaging Criteria for Suspected Pulmonary Hypertension

ACR Imaging Criteria for Suspected Pulmonary Hypertension

New criteria by the American College of Radiology detail appropriateness of various imaging techniques for suspected pulmonary hypertension.

Predicting Survival in IPF With CT-Measured Pulmonary Artery: Aorta Ratio

Predicting Survival in IPF With CT-Measured Pulmonary Artery: Aorta Ratio

Computed tomography-derived pulmonary artery-to-aorta ratio can predict pulmonary artery pressure and survival in patients with idiopathic pulmonary fibrosis.

Biomarkers of PAH Risk in Patients With SLE

Biomarkers of PAH Risk in Patients With SLE

Meta-analysis supports anti-RNP and anti-Sm antibodies as serological biomarkers of PAH risk in patients with SLE.

MRIs Can Predict Outcomes in Pulmonary Arterial Hypertension

MRIs Can Predict Outcomes in Pulmonary Arterial Hypertension

MRI measures can predict outcomes in pulmonary arterial hypertension.

Pulmonary Arterial Hypertension: Expert Interview

Pulmonary Arterial Hypertension: Expert Interview

Pulmonary and genetics experts James E. Loyd, MD, and Wendy Chung, MD, PhD, offer insight into the role of genetics in pulmonary arterial hypertension.

Safety and Efficacy of Combination Treatment for CTD-PAH

Safety and Efficacy of Combination Treatment for CTD-PAH

Combination therapy with ambrisentan and tadalafil may improve response in patients with connective tissue disease-associated pulmonary arterial hypertension.

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