Idiopathic Pulmonary Arterial Hypertension Questions & Answers

Updated: Jun 21, 2018
  • Author: Ronald J Oudiz, MD, FACP, FACC, FCCP; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Questions & Answers

Overview

What is idiopathic pulmonary arterial hypertension (IPAH)?

What are the symptoms of idiopathic pulmonary arterial hypertension (IPAH)?

What are the cardiovascular findings in idiopathic pulmonary arterial hypertension (IPAH)?

What are the physical exam findings in idiopathic pulmonary arterial hypertension (IPAH)?

What is the role of cardiac catheterization in idiopathic pulmonary arterial hypertension (IPAH)?

Which lab studies are indicated in the workup of idiopathic pulmonary arterial hypertension (IPAH)?

Which imaging studies are indicated in the diagnosis of idiopathic pulmonary arterial hypertension (IPAH)?

What ECG findings are associated with idiopathic pulmonary arterial hypertension (IPAH)?

What is the role of exercise testing in the workup of idiopathic pulmonary arterial hypertension (IPAH)?

What is the role of calcium channel blocker therapy in the treatment of idiopathic pulmonary arterial hypertension (IPAH)?

What is the approach to treatment of idiopathic pulmonary arterial hypertension (IPAH) in patients who cannot take calcium channel blockers (CCBs)?

What is the role of transplantation and septostomy in the treatment of idiopathic pulmonary arterial hypertension (IPAH)?

What is idiopathic pulmonary arterial hypertension (IPAH) (precapillary pulmonary hypertension)?

How is pulmonary hypertension classified?

How is idiopathic pulmonary arterial hypertension (IPAH) classified?

How is idiopathic pulmonary arterial hypertension (IPAH) defined?

Which medications are most effective for the treatment of idiopathic pulmonary arterial hypertension (IPAH)?

What is the pathophysiology of idiopathic pulmonary arterial hypertension (IPAH)?

Which genetic mutations are associated with idiopathic pulmonary arterial hypertension (IPAH)?

Which genetic defects are associated with idiopathic pulmonary arterial hypertension (IPAH) according to the Nice Classification system related to?

How is thrombotic pulmonary arteriopathy characterized in idiopathic pulmonary arterial hypertension (IPAH)?

Which medical conditions are the associated with idiopathic pulmonary arterial hypertension (IPAH)?

What causes idiopathic pulmonary arterial hypertension (IPAH)?

How common is idiopathic pulmonary arterial hypertension (IPAH)?

What is the prognosis of idiopathic pulmonary arterial hypertension (IPAH)?

How is the REVEAL Registry used in the prognosis of idiopathic pulmonary arterial hypertension (IPAH)?

What patient education is indicated for patients with idiopathic pulmonary arterial hypertension (IPAH)?

Presentation

What are the early symptoms of idiopathic pulmonary arterial hypertension (IPAH)?

What are the most common symptoms of idiopathic pulmonary arterial hypertension (IPAH)?

What are common findings of cardiovascular exam in patients with idiopathic pulmonary arterial hypertension (IPAH)?

What physical findings are associated with idiopathic pulmonary arterial hypertension (IPAH)?

What are the complications of idiopathic pulmonary arterial hypertension (IPAH)?

DDX

What are the diagnostic considerations of idiopathic pulmonary arterial hypertension (IPAH)?

What are the differential diagnoses for Idiopathic Pulmonary Arterial Hypertension?

Workup

What are the approach considerations in the workup of idiopathic pulmonary arterial hypertension (IPAH)?

How is echocardiography used in the workup of idiopathic pulmonary arterial hypertension (IPAH)?

What is the role of radiography in the workup of idiopathic pulmonary arterial hypertension (IPAH)?

How are CT and V/Q lung scanning used in the workup of idiopathic pulmonary arterial hypertension (IPAH)?

How is pulmonary angiography used in the workup of idiopathic pulmonary arterial hypertension (IPAH)?

What is the role of cardiac catheterization in the workup of idiopathic pulmonary arterial hypertension (IPAH)?

What is the role of serologic analyses for idiopathic pulmonary arterial hypertension (IPAH)?

What is the role of thyroid studies in the workup of idiopathic pulmonary arterial hypertension (IPAH)?

What is the role B-type natriuretic peptide studies in the workup of idiopathic pulmonary arterial hypertension (IPAH)?

What is the role of ECG in the workup of idiopathic pulmonary arterial hypertension (IPAH)?

What is the role of exercise testing in the assessment of idiopathic pulmonary arterial hypertension (IPAH)?

What are the histologic findings of idiopathic pulmonary arterial hypertension (IPAH)?

Which studies are indicated in the workup of idiopathic pulmonary arterial hypertension (IPAH) with comorbidities?

How is idiopathic pulmonary arterial hypertension (IPAH) staged?

Treatment

What are the treatment approach considerations of idiopathic pulmonary arterial hypertension (IPAH)?

What is the role of calcium channel blockers (CCBs) in the treatment of idiopathic pulmonary arterial hypertension (IPAH)?

When is calcium channel blocker therapy indicated for the treatment of idiopathic pulmonary arterial hypertension (IPAH)?

How is a calcium channel blocker challenge used in the treatment of idiopathic pulmonary arterial hypertension (IPAH)?

Which medications are used in the treatment of idiopathic pulmonary arterial hypertension (IPAH)?

What were the results of the SERAPHIN trial on the treatment of idiopathic pulmonary arterial hypertension (IPAH)?

Which PAH-specific therapies are indicated for patients with idiopathic pulmonary arterial hypertension (IPAH) in whom calcium channel blockers (CCBs) are contraindicated?

What is the role of goal-oriented therapy for idiopathic pulmonary arterial hypertension (IPAH)?

Why is vasoactivity testing important for patients with idiopathic pulmonary arterial hypertension (IPAH)?

What is the prognostic value of a vasodilator challenge for idiopathic pulmonary arterial hypertension (IPAH)?

What is the risk of ocular toxicity in the treatment of idiopathic pulmonary arterial hypertension (IPAH)?

Which ancillary treatments are used in patients with idiopathic pulmonary arterial hypertension (IPAH)?

What is the role of anticoagulant therapy in the treatment of idiopathic pulmonary arterial hypertension (IPAH)?

What is the role of digoxin in the treatment of idiopathic pulmonary arterial hypertension (IPAH)?

What is the role of diuretics in the treatment of idiopathic pulmonary arterial hypertension (IPAH)?

What is the role of oxygen therapy in the treatment of idiopathic pulmonary arterial hypertension (IPAH)?

What dietary recommendations are indicated for patients with idiopathic pulmonary arterial hypertension (IPAH)?

What physical activity is indicated for patients with idiopathic pulmonary arterial hypertension (IPAH)?

What is the role of transplantation and septostomy for the treatment of idiopathic pulmonary arterial hypertension (IPAH)?

What is the role of long-term monitoring in patients with idiopathic pulmonary arterial hypertension (IPAH)?

What novel therapies for idiopathic pulmonary arterial hypertension (IPAH) are being investigated?

Guidelines

What are the treatment guidelines for idiopathic pulmonary arterial hypertension (IPAH)?

Medications

What is the importance of medication for idiopathic pulmonary arterial hypertension (IPAH)?

Which medications in the drug class Cardiac Glycosides are used in the treatment of Idiopathic Pulmonary Arterial Hypertension?

Which medications in the drug class Anticoagulants are used in the treatment of Idiopathic Pulmonary Arterial Hypertension?

Which medications in the drug class Diuretics are used in the treatment of Idiopathic Pulmonary Arterial Hypertension?

Which medications in the drug class Soluble Guanylate Cyclase (sGC) Stimulators are used in the treatment of Idiopathic Pulmonary Arterial Hypertension?

Which medications in the drug class Endothelin Receptor Antagonists are used in the treatment of Idiopathic Pulmonary Arterial Hypertension?

Which medications in the drug class Prostacyclin Receptor Agonists are used in the treatment of Idiopathic Pulmonary Arterial Hypertension?

Which medications in the drug class Oral Prostacyclin Analogues are used in the treatment of Idiopathic Pulmonary Arterial Hypertension?

Which medications in the drug class Inhaled Prostanoids are used in the treatment of Idiopathic Pulmonary Arterial Hypertension?

Which medications in the drug class Oral Phosphodiesterase (Type 5) Enzyme Inhibitors are used in the treatment of Idiopathic Pulmonary Arterial Hypertension?

Which medications in the drug class Parenteral Prostanoids are used in the treatment of Idiopathic Pulmonary Arterial Hypertension?

Which medications in the drug class Calcium Channel Blockers are used in the treatment of Idiopathic Pulmonary Arterial Hypertension?