At present, BMPR2 mutations have been identified in about 80% of the families with FPAH. Information about which specific mutation is present in each family may be available from the research team. [phassociation.org]

Children can present with tachycardia and tachypnea as signs of cardiac failure. [link.springer.com]

Exertional dyspnea, fatigue, chest pains and syncope are the most common presenting complaints. Hemoptysis may also occur and may be of high volume 1. [radiopaedia.org]

A significant difference was identified in the clinical presentation between two WHO functional class groups at baseline. Echocardiography showed a mean pulmonary systolic pressure of 98 mm Hg. [f1000.com]

However, a thorough workup includes a range of additional testing to exclude all reasonable causes of secondary pulmonary hypertension (see Workup). [emedicine.medscape.com]

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]

Figure 2: Diagnostic workup for IPAH/HPAH (modified). 29 CI, cardiac index; CT, computed tomography; CXR, chest x-ray; ECG, electrocardiogram; GI, gastrointestinal; NJ, nasojejeunal; PAp, pulmonary artery pressure; PFTs, pulmonary function tests; PH, [phaonlineuniv.org]

[…] anorexia (from hepatic congestion) Physical examination loud P2 on auscultation right-sided heave from right ventricular hypertrophy if subsequent right heart failure elevated JVP hepatomegaly S3 heart sound Imaging Echocardiogram usually first step in the workup [medbullets.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]

[…] is the future of pulmonary hypertension treatment and several formal combination treatment trials are in progress. [thorax.bmj.com]

Discuss treatment options with your caregivers to decide what care you want to receive. You always have the right to refuse treatment. The above information is an educational aid only. [drugs.com]

FDA Approves Orenitram (treprostinil) Extended-Release Tablets for the Treatment of Pulmonary Arterial Hypertension. PR Newswire. Available at . Accessed: January 6, 2014. FDA approves Orenitram for treatment of PAH. Healio Cardiology. [emedicine.medscape.com]

Treatment and prognosis Treatment and prognosis are discussed in the parent article on pulmonary arterial hypertension. [radiopaedia.org]

Lack of effective treatment was the main cause of poor survival in this study. Our results support the need of an appropriate treatment strategy for this devastating disease in China. [f1000.com]

Treatment and prognosis Treatment and prognosis are discussed in the parent article on pulmonary arterial hypertension. [radiopaedia.org]

Factors determining poor prognosis Rapid progression of symptome H/O Syncope Advanced WHO functional class 6 min walking 45. Combination Therapy 46. [slideshare.net]

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]

PAH represents diverse population groups that vary greatly in aetiology, disease severity, speed of progression, prognosis and response to treatment. [pulmonaryhypertension.ie]

[…] cases per million for idiopathic pulmonary hypertension (IPAH)) or relatively common 10-40% of patients with at-risk associated diseases (below) demographics f emales males up to 9:1 (for IPAH) Etiologies Etiologies of Pulmonary Hypertension Primary Etiologies [medbullets.com]

The treatment of Pulmonary Hypertension (PH) varies depending on the etiology and suspected underlying mechanism of disease progression and thus requires an extensive evaluation. [houstonlungdocs.com]

Additionally, the development of multiple therapies targeting important pathways of disease and the translation of those treatments to pediatrics have led to improved life experience and survival for many children with PAH. 5 CLASSIFICATION Although etiologies [phaonlineuniv.org]

Etiology The strict definition of IPAH is pulmonary hypertension with no known cause. [emedicine.medscape.com]

Studies establishing effectiveness included predominantly patients with NYHA Functional Class III-IV symptoms and etiologies of idiopathic or heritable PAH or PAH associated with connective tissue diseases. [raredr.com]

Articles include: Epidemiology of Pulmonary Arterial Hypertension, Pathology of Pulmonary Hypertension, Genetics of Pulmonary Arterial Hypertension, Diagnosis of Pulmonary Arterial Hypertension, Pulmonary Hypertension Owing to Left Heart Disease, Pulmonary [books.google.com]

EPIDEMIOLOGY 1-2 cases / million Population Female : Male 1.7 : 1 Age range 10 – 40 yrs (mean 35.4 yrs) Females predominate after Pubertal age group(Ref: NIH Study) 12. Predisposing Factors1. Dysfunctional Endothelium2. [slideshare.net]

"Obesity Hypoventilation Syndrome Epidemiology and Diagnosis". Sleep Medicine Clinics. 9 (3): 341–347. doi : 10.1016/j.jsmc.2014.05.007. PMC 4210766. PMID 25360072. Reynolds, PN (February 2011). [en.wikipedia.org]

Epidemiology Incidence : 1-2 cases per 1 million (rare) Female predominance after Puberty in ratio of 1.7 to 1 Most prevalent in ages 20 to 40 years old III. [fpnotebook.com]

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]

Pathophysiology The pathophysiology of IPAH is poorly understood. [emedicine.medscape.com]

Significant advances have been made in this field in the last decade both in the understanding of the pathophysiology and the treatment of PH. [lungindia.com]

Pathophysiology Progressive narrowing of distal pulmonary arteries Processes Inflammation Hypercoagulability Pre-capillary pulmonary arteriole disorder Endothelial dysfunction Excess cell proliferation and dysfunctional apoptosis Vasconstriction Mediators [fpnotebook.com]

[…] low, 2 to 6 per million Drug-induced PAH: 1/25,000 with 3 months of anorectic use HIV associated: 0.5/100 Portal HTN 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]

British Journal of Anaesthesia: "Primary pulmonary hypertension in pregnancy; a role for novel vasodilators" Archived 2011-01-21 at the Wayback Machine March 19, 2011 "Primary Pulmonary Hypertension: Practice Essentials, Background, Pathophysiology". [en.wikipedia.org]

You may have any of the following: Medicines: Blood thinners: These prevent blood clots. They may make you bruise or bleed more easily. Use a soft toothbrush and an electric shaver to prevent bleeding. [drugs.com]

Antitussive medications are also important in children with PH after episodes of hemoptysis, to prevent further bleeding. [link.springer.com]

Treatment Goal To improve symptoms To improve exercise capacities To prevent further functional worsening To improve survival 36. [slideshare.net]

Mensah, MD Division for Heart Disease and Stroke Prevention National Center for Chronic Disease Prevention and Health Promotion This material in this report originated in the National Center for Chronic Disease Prevention and Health Promotion, Janet L [cdc.gov]

On occasion, patients are started on blood thinners to prevent the formation of blood clots. Ultimately, some patients will require lung transplantation. [houstonlungdocs.com]