Presentation
The topics discussed are relevant to chest physicians, thoracic surgeons, nurses, students, and teachers, and a well-balanced mix of contributions ensures that doctors, clinicians, and institutions from all around the world will find the information presented [books.google.com]
Fluffy infiltrates are present on the chest radiograph. [emedicine.medscape.com]
Other symptoms that may be present include: Dizziness Fatigue Swelling around the ankles and feet Fainting Tightening feeling in the chest [aoporphan.com]
Symptoms may be present at rest and any activity causes symptoms. Signs of right heart failure present. F. Over-utilized or “wasted” diagnostic tests associated with this diagnosis. [clinicaladvisor.com]
Entire Body System
- Fatigue
Other symptoms that may be present include: Dizziness Fatigue Swelling around the ankles and feet Fainting Tightening feeling in the chest [aoporphan.com]
Ordinary physical activity causes dysnpea/fatigue/chest pain or near syncope Class III Marked limitation in physical activity. No symptoms at rest. [clinicaladvisor.com]
[…] mean pulmonary artery pressure > 25mmHg @ rest and > 30mmHg with exercise measured by catheterization (gold standard) Severity Mild = 25-40mmHg Moderate = 41-55mmHg Severe = > 55mmHg Functional assessment I – no limitation on physical activity (no SOB, fatigue [lifeinthefastlane.com]
Dyspnea and/or fatigue may be present at rest. Discomfort is increased by any physical activity. [aafp.org]
Get rest Proper rest can help you fight fatigue, a common symptom of PAH. This includes getting the right amount and quality of sleep each night. Talk to a doctor if falling or staying asleep is a problem. [cvsspecialty.com]
Gastrointestinal
- Nausea
[…] reported in a Phase 3 clinical trial occurring with a ≥3% difference between VENTAVIS patients and placebo patients were vasodilation (flushing) (27% vs 9%), increased cough (39% vs 26%), headache (30% vs 20%), trismus (12% vs 3%), insomnia (8% vs 2%), nausea [4ventavis.com]
Advise patients to report symptoms suggesting hepatic injury (nausea, vomiting, right upper quadrant pain, fatigue, anorexia, jaundice, dark urine, fever, or itching). [opsumithcp.com]
For example, to relieve nausea and vomiting, the doctor may suggest: Eating smaller meals Avoiding carbonated drinks Avoiding greasy and fatty foods Drinking cold liquids like ice water or ice tea often PAH medicine can only help if it’s taken exactly [cvsspecialty.com]
The more frequently reported adverse events when using prostanoids were headache, facial flushing, nausea, vomiting and diarrhea. [aetna.com]
Adverse events include headache, nausea, diarrhea, and jaw pain. Oral treprostinil, like all prostacyclin analogs, inhibits platelet aggregation and may increase bleeding risk, particularly in patients receiving oral anticoagulants. [uscjournal.com]
Neurologic
- Headache
dyspepsia, flushing Improves cardiopulmonary hemodynamics and exercise capacity 36, 37 $17 for 20-mg tablet $51 Tadalafil (Adcirca) 2.5 to 40 mg orally once per day (40 mg was needed for statistically significant effects 38 ) Headache, flushing, myalgias [aafp.org]
The most common side effects include nasopharyngitis, headache, and anemia. Female patients must go through a Risk Evaluation and Mitigation Strategy (REMS) program to receive the drug. [uscjournal.com]
Adverse Events Adverse events reported in a Phase 3 clinical trial occurring with a ≥3% difference between VENTAVIS patients and placebo patients were vasodilation (flushing) (27% vs 9%), increased cough (39% vs 26%), headache (30% vs 20%), trismus (12% [4ventavis.com]
ADVERSE REACTIONS Most common adverse reactions (more frequent than placebo by ≥3%) were anemia (13% vs 3%), nasopharyngitis/pharyngitis (20% vs 13%), bronchitis (12% vs 6%), headache (14% vs 9%), influenza (6% vs 2%), and urinary tract infection (9% [opsumithcp.com]
As expected, compared with patients who received placebo, more patients in the macitentan arms exhibited nasopharyngitis, headache, and anemia. [phaonlineuniv.org]
Workup
Both pulmonologists and cardiologists should be aware of the pathophysiology of pulmonary arterial hypertension, the workup and the treatment options that are available. [lungindia.com]
The workup for CTEPH should include a ventilation–perfusion scan. [dovepress.com]
The workup of a patient with suspected PAH is designed to confirm the diagnosis, exclude or confirm the presence of an underlying cause, and assess the severity of the disease. [clevelandclinicmeded.com]
Treatment
Phentermine Phentermine is an anorexigen approved for short-term use as a treatment for obesity. [em-consulte.com]
Know the signs of catheter-related infections and get treatment fast. [cvsspecialty.com]
Exclude pregnancy before start of treatment, monthly during treatment, and one month after treatment discontinuation. Prevent pregnancy during treatment and for one month after treatment discontinuation by use of acceptable methods of contraception. [aetna.com]
Females of reproductive potential: Exclude pregnancy before the start of treatment, monthly during treatment, and 1 month after stopping treatment. [opsumithcp.com]
The treatment is cessation of steroid therapy. [emedicine.medscape.com]
Prognosis
PAH associated with systemic sclerosis carries a worse prognosis than idiopathic PAH. However, PAH associated with congenital heart disease has a better prognosis than idiopathic PAH. [patient.info]
Effects of a combination therapy of anticoagulant and vasodilator on the long-term prognosis of primary pulmonary hypertension. Jpn Circ J 1993;57:63–9. Kawut SM, Horn EM, Berekashvili KK, et al. [acc.org]
Prognosis is poor with a 15% mortality within 1 year on modern therapy (6,7). [escardio.org]
Prognosis and Patient Counseling. Prognosis of IPAH: Prognosis used to be much poorer prior to advancement of therapeutic options, with previous mean life expectancy of 2-3 years after initial diagnosis. [clinicaladvisor.com]
The positive impact has been my condition is being managed with the medication if not my prognosis would have been two years. My parents and I are closer. I have met some life-long friends with pulmonary hypertension. [lungfoundation.com.au]
Etiology
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]
Studies establishing effectiveness included predominantly patients with NYHA Functional Class III-IV symptoms and etiologies of idiopathic or heritable PAH (65%) or PAH associated with connective tissue disease (23%). [4ventavis.com]
The groups are divided based on etiology as shown in the following table. [www1.actelion.com.au]
[…] low, ~2 to 6 per million Drug-induced PAH: 1/25,000 with >3 months of anorectic use HIV associated: 0.5/100 Portal hypertension associated: 1 to 6/100 Scleroderma associated: 6–60% Prevalence PAH: ~15 to 50 cases per million IPAH: ~6 cases per million Etiology [unboundmedicine.com]
Regardless of the etiology, unrelieved pulmonary hypertension can lead to right-sided heart failure. [aetna.com]
Epidemiology
This Review provides a comprehensive update on the epidemiology and pharmacotherapy of PAH. [nature.com]
[…] the breadth of use of the coding system today, improved data will support a host of health care needs including: Measuring the quality, safety, and efficacy of care Designing payment systems and processing claims for reimbursement Conducting research, epidemiological [phaware.global]
PVOD and/or PCH: rare cause of PH characterized by extensive diffuse occlusion of the pulmonary veins (unlike PAH which involves the small muscular pulmonary arterioles) Epidemiology Age: can occur at any age; mean age 37 years Sex (IPAH): female > male [unboundmedicine.com]
Epidemiology [ 2 ] Idiopathic PAH is rare. However, the prevalence of PAH is higher in certain patient groups, such as those with systemic sclerosis, portal hypertension, congenital heart disease and HIV infection. [patient.info]
Author Affiliations From the Centre for Clinical Epidemiology and Community Studies, McGill University and Sir Mortimer B. [nejm.org]
Pathophysiology
Pathophysiology of pulmonary hypertension in acute lung injury. American journal of physiology. Lung cellular and molecular physiology. 302(9):L803-15. 2012. [ pubmed ] [ free full text ] Stamm JA, Risbano MG, Mathier MA. [lifeinthefastlane.com]
[…] per million Drug-induced PAH: 1/25,000 with >3 months of anorectic use HIV associated: 0.5/100 Portal hypertension associated: 1 to 6/100 Scleroderma associated: 6–60% Prevalence PAH: ~15 to 50 cases per million IPAH: ~6 cases per million Etiology and Pathophysiology [unboundmedicine.com]
These drugs include macitentan, riociguat, and treprostinil; all 3 target different pathophysiologic pathways and will be reviewed in this article. [phaonlineuniv.org]
Although PAH can affect individuals of all ages, the mean age of presentation ranges from 36 to 50 years. 2,6 Pathophysiology Since PH in Groups 2 to 5 develops secondary to an underlying etiology, the discussion of pathophysiology in PH will focus on [uspharmacist.com]
Definitions [ 1 ] PH is a haemodynamic and pathophysiological condition defined as an increase in mean PAP ≥25 mm Hg at rest as assessed by right heart catheterisation. [patient.info]
Prevention
In PAH, the enzyme phosphodiesterase-5 (PDE-5), found largely in the lung, inactivates cGMP, preventing cellular response, causing pulmonary smooth muscle vasoconstriction, and leading to platelet activation and proliferation. [uspharmacist.com]
He said, “…physicians must remain vigilant of their patient population and establish close interactions between national drug regulatory agencies, national PH networks, and PAH patient associations…” to devise strategies to recognize and prevent D-PAH [phassociation.org]
Conducting research, epidemiological studies, and clinical trials Setting health policy Operational and strategic planning and designing healthcare delivery systems Monitoring resource utilization Improving clinical, financial, and administrative performance Preventing [phaware.global]
Prevent pregnancy during treatment and for one month after stopping treatment by using acceptable methods of contraception. [opsumithcp.com]
Supportive measures include the use of oxygen for patients who require long-term oxygen therapy, the use of diuretics to help relieve organ congestion and the use of oral anticoagulation to help to prevent thromboembolism to the lungs. [nhcs.com.sg]