Increased pulmonary pressures, right ventricular dysfunction, and a stiffer pulmonary vascular bed were typical characteristics of early pulmonary vascular disease onset in young adults who were born preterm.
Neopterin was predictive of clinical deterioration in patients with pulmonary arterial hypertension and inoperable CTEPH.
Although there were no significant differences in pulmonary arterial hypertension survival based on therapy type, age was an important modifier.
Pulmonary arterial stiffness was related to mean pulmonary arterial pressure and heart rate in patients with untreated idiopathic pulmonary arterial hypertension.
The Pediatric Index Pulmonary Hypertension Intensive Care Mortality model was compared with the Pediatric Risk of Mortality 2 and 3 models to determine the best model for predicting mortality.
The fractal dimension of right ventricular trabecular complexity was a good reproducible marker of remodeling in pulmonary hypertension.
Right ventricular base/apex ratio reference values may help clinicians diagnose children with pulmonary arterial hypertension.
Many healthcare providers reported that they often do not have the time to counsel their patients with pulmonary arterial hypertension on pregnancy risks.
Management strategies were discussed for subtypes of PAH-congenital heart disease including Eisenmenger syndrome, PAH with persistent systemic to pulmonary shunts, and PAH with small or coincidental defect or PAH after defect closure.
A 72-year-old man with hypertension and paroxysmal atrial fibrillation presents to the emergency department because he has been experiencing worsening shortness of breath.
Cardiopulmonary exercise testing can be an important diagnostic and prognostic tool in pulmonary arterial hypertension.
After iron supplementation, patients with cyanosis experienced significant improvements in hemoglobin concentration, hematocrit, mean corpuscular volume, ferritin, and transferrin saturation.
In patients with end-stage interstitial lung disease, there was a higher rate of survival to transplantation with venoarterial extracorporeal membrane oxygenation.
Strong relationships were observed between health-related quality-of-life scores and factors such as breathlessness, fatigue, depression, anxiety, and sleep in pulmonary hypertension.
Double lung transplantation appears to offer superior survival outcomes to single lung transplantation in patients with interstitial pulmonary fibrosis who are younger than 70 years.
The interaction of age and obesity with respect to survival in pulmonary arterial hypertension showed an increased risk for mortality in young adults who were morbidly obese.
The majority of deaths in patients with systemic sclerosis-associated pulmonary arterial hypertension occurred within 4 years of diagnosis.
Precapillary pulmonary hypertension and obstructive sleep apnea may be causative factors for central sleep apnea and pulmonary hypertension, respectively.
There was no significant difference in efficacy between sildenafil and bosentan initial combination therapy and sildenafil monotherapy in patients with systemic sclerosis-related pulmonary arterial hypertension.
Researchers raise issues regarding interpretation of clinical trial results in the field of pulmonary arterial hypertension.
Researchers modified the inhalation technique of iloprost in a patient with pulmonary arterial hypertension by attaching a nasal cannula to the inhalator's inlet port, shortening the administration duration.
The use of a risk calculator aimed at achieving a low-risk profile in PAH trials is discussed.
Arrhythmia was a significant predictor of mortality in adult patients with congenital heart disease and pulmonary arterial hypertension.
Clinical worsening in pediatric pulmonary arterial hypertension may be predicted using right ventricular stroke work indexed by ejection fraction.
The variables for the stepwise echocardiographic score included right ventricular systolic pressure, early pulmonary regurgitation gradient, right atrial area size, tricuspid regurgitation velocity, left ventricle eccentricity index, and right ventricle fractional area change.
A definition of a pulmonary vascular phenotype of chronic obstructive pulmonary disease has been proposed.
There was a significant relationship between brain natriuretic peptide levels and mortality in patients with group 1 pulmonary arterial hypertension.
Poor subpleural perfusion in patients with nonoperable chronic thromboembolic pulmonary hypertension may predict balloon pulmonary angioplasty failure.
Although metformin prevented the development of PAH in hypoxia and monocrotalinerat models and reversed PAH in SU5416/hypoxia rats, it failed to reverse pulmonary pressures and vascular remodeling in mice with SU5416/hypoxia-induced PAH.
Recent evidence suggests that systemic sclerosis and systemic lupus erythematosus in pulmonary arterial hypertension should be considered separate diseases.
The 1-year survival rate after lung transplantation in patients with a mean pulmonary arterial pressure ≥25 mm Hg was lower than the survival rate of those with a mean pulmonary arterial pressure <25 mm Hg.
The Pulmonary Arterial Hypertension-Symptoms and Impact questionnaire has good psychometric properties and can be administered in clinical practice.
The "egg-and-banana" sign is a valid computed tomography marker of pulmonary hypertension.
At the end of 6 months, the rate of VTE recurrence was 4% among rivaroxaban patients vs 11% in the dalteparin arm (hazard ratio [HR] 0.43, 95% CI: 0.19 to 0.99).
Serum chloride was a strong and independent predictor of mortality at 6 months after a pulmonary arterial hypertension diagnosis.
D-dimer test adjustment based on pretest probability results may be a safe option to reduce the need for imaging during evaluation for pulmonary embolism.
Copeptin may be useful for identifying normotensive patients with pulmonary embolism who have a higher risk for an adverse outcome.
In patients with pulmonary arterial hypertension who were treated with a parenteral prostanoid, survival was linked with the number of guideline-recommended lower-risk and higher-risk criteria attained.
Walking a distance of >400 meters in the 6-minute walk test was associated with a reduced risk for pulmonary arterial hypertension-related death or hospitalization.
The sNDA is accompanied by results from the MERIT-1 trial, a Phase 2 randomized, placebo-controlled, double-blind study which assessed the efficacy, safety and tolerability of macitentan 10mg in 80 patients with inoperable CTEPH.
Stem cell therapy targeting right ventricular failure may help improve pulmonary circulation in pulmonary arterial hypertension.
Ischemic heart disease and kidney dysfunction were identified as 2 comorbidities that may affect outcomes in idiopathic pulmonary arterial hypertension.
Regardless of neonatal lung disease status, infants born before 29 weeks' gestation demonstrated abnormal pulmonary artery acceleration time at 1-year corrected age.
In patients with pulmonary arterial hypertension or distal chronic thromboembolic pulmonary hypertension, targeted pharmacologic treatment was associated with an increase in achieved therapeutic goals.
A 64-year-old man with chronic obstructive pulmonary disease, diabetes, chronic kidney disease, and morbid obesity seeks preoperative approval for femoral popliteal bypass surgery.
Increased right atrial and pulmonary wedge pressures and increased thyroid disease prevalence were associated with atrial arrhythmia development in idiopathic or systemic sclerosis-related pulmonary arterial hypertension.
Prescribers tend to favor a frequency of 3 times per day for oral treprostinil dosing in patients with pulmonary arterial hypertension.
N-terminal-pro brain natriuretic peptide may be a useful biomarker for both mortality and respiratory exacerbations in pediatric patients with pulmonary hypertension.
Patients with pulmonary hypertension have a high risk for adverse events during and following cardiac catheterization, but high case volume may help ameliorate this risk.
The hemodynamic parameters predict mortality and cardiac hospitalization in patients with heart failure with preserved ejection fraction.
A 57-year-old woman has been experiencing progressive exertional dyspnea during the past 6 months as well as a near syncopal episode while attempting to catch a taxi.
Hemodynamic markers of pulmonary hypertension in heart failure with preserved ejection fraction are associated with mortality
Transpulmonary gradient, pulmonary vascular resistance, and diastolic pulmonary gradient are predictors of mortality and cardiac hospitalizations in pulmonary hypertension associated with heart failure with preserved ejection fraction.
Several months after being diagnosed with pulmonary arterial hypertension, a 32-year-old pregnant woman presents with worsening dyspnea.
Nearly two-thirds of phosphodiesterase-5 inhibitor prescriptions for US veterans are inconsistent with pulmonary hypertension guidelines.
Patients with pulmonary arterial hypertension may be better identified through a claims-based algorithm that includes ICD-9-CM codes, specific pulmonary arterial hypertension medications, echocardiography, and right heart catheterization.
Women with pulmonary arterial hypertension experienced better long-term prognosis than men with PAH.
Enlargement of the right atrium is associated with an increased risk for supraventricular arrhythmias in PAH.
Varicose veins may increase the risk for pulmonary embolism and peripheral artery disease.
Peak circulatory power offers greater value than peak oxygen uptake and ventilation in predicting cardiac events in patients with idiopathic PAH.
Hypoxemia in individuals with idiopathic PAH is associated with increased mortality risk.
Observation in the emergency department followed by outpatient management with anticoagulation may be appropriate treatment for some patients with low-risk pulmonary embolism.
In patients at very low risk for pulmonary embolism, the pulmonary embolism rule-out criteria was noninferior to the conventional strategy of identifying thromboembolic events.
Pulmonary Embolism Rule-out Criteria non-inferior to usual care for patients at low-risk for pulmonary embolism
In this multicenter, randomized controlled study, the Pulmonary Embolism Rule-out Criteria (PERC) strategy resulted in significantly fewer uses of CT pulmonary angiography, reduced ED length of stay, and fewer hospital admissions.
Based on this retrospective cohort study from multiple countries, the prevalence of pulmonary embolism in patients presenting to the emergency department with syncope was low.
Patients with suspected PH should undergo two 6MWTs to improve the accuracy of exercise capacity measurements.
The 6-minute walk test had prognostic value with respect to mortality in a population of patients with pulmonary arterial hypertension.
Individuals treated with riociguat for pulmonary hypertension may have a greater risk for gastrointestinal disorders compared with tadalafil and sildenafil.
Brain natriuretic peptide levels may accurately predict 5-year survival rates in patients with pulmonary arterial hypertension.
Thyroid hormone supplementation may be a promising treatment for idiopathic pulmonary fibrosis [PreClinical]
An enzyme involved in thyroid hormone activation was found to be elevated in patients with pulmonary fibrosis.
Although the Wells and revised Geneva scores are used in the general population to predict pulmonary embolism, researchers found they were not reliable in pregnant and postpartum women.
Pulmonary hemodynamic changes in chronic thromboembolic pulmonary hypertension may be monitored via the 6-minute walk test.
Bosentan may benefit a subset of patients with idiopathic pulmonary fibrosis-associated pulmonary hypertension.
A clinical trial is underway to assess the safety and efficacy of dabigatran in intermediate-risk pulmonary embolism after completing 72 hours of heparin therapy.
The REVEAL risk score gives significant predictive value for long-term survival and changes in mortality risk in patients with PAH.
A fully implantable programmable intravascular delivery system using treprostinil was found to be highly stable in patients with PAH.
Pulmonary hypertension may be more important than age in predicting heart transplant outcomes.
A study evaluated whether changes in estimated glomerular filtration rate were predictive of mortality in patients with pulmonary arterial hypertension.
Prospective validation research is needed before these clinical predictors of pediatric PE can become guidelines.
A systematic review evaluated whether catheter-directed thrombolysis improved outcomes in patients with intermediate-risk pulmonary emboli.
The use of veno-arterial extracorporeal membrane oxygenation as bridge therapy for massive pulmonary embolism may allow clinicians to better triage patients to appropriate destination therapy.
Individuals who are HIV-positive may be at a higher risk for increased pulmonary pressure and mortality.
A study evaluated the quality of targeted anticoagulation in patients with PAH, and examined outcomes associated with poor time spent within targeted anticoagulation range.
Lung transplantation may be an option in patients with severe pulmonary arterial hypertension who do not respond well to medical therapy.
Select pediatric patients with pulmonary arterial hypertension may benefit from treatment with riociguat.
A 40-year-old woman presents with worsening shortness of breath after being treated with long-acting nifedipine for 2 years.
Hemodynamics may improve in patients with chronic thromboembolic pulmonary hypertension after balloon pulmonary angioplasty with complication rates comparable to pulmonary endarterectomy.
Study results confirm the benefits of exercise vs granulocyte-macrophage colony-stimulating factor in improving 6-minute walk test in PAD.
Using biomarkers in pulmonary arterial hypertension may be a less invasive strategy for assessing response to treatment.
Pulmonary and thoracic symptoms often coincide with initial presentation of connective tissue disease.
Pulmonary arterial hypertension is becoming a major cause of death in patients with systemic sclerosis.
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.
Patients with pulmonary arterial hypertension have reduced cerebrovascular reactivity to CO2.
Respiratory muscle dysfunction, iron deficiency, and skeletal muscle dysfunction have been implicated as mechanisms for exercise intolerance in pulmonary arterial hypertension.
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.
The frequency of eukaryotic translation initiation factor 2 alpha kinase 4 gene (EIF2AK4) mutations in patients with pulmonary arterial hypertension was investigated.
Borderline PH was linked to increased mortality after adjustment for 34 variables.
Patients who develop pulmonary edema after starting prostacyclin for pulmonary arterial hypertension have a greater risk for mortality.
Pulmonary vascular compliance may independently predict survival and prognosis in patients with pulmonary arterial hypertension associated with congenital heart disease.
Patients with pulmonary arterial hypertension experienced better improvements in pulmonary arterial compliance with riociguat compared with phosphodiesterase type 5 inhibitors.
Lung disease experts Athol Wells, MD, and Joshua Mooney, MD, MS, debate the diagnostic value of bronchoalveolar lavage in idiopathic pulmonary fibrosis.