Pulmonary Arterial Hypertension Questions & Answers

Updated: Apr 25, 2018
  • Author: Kristin E Schwab, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Questions & Answers

Overview

What is pulmonary hypertension?

What are the WHO clinical classifications of pulmonary hypertension?

What is the pathophysiology of pulmonary arterial hypertension (PAH)?

What are the subtypes of pulmonary arterial hypertension (PAH)?

What is the prevalence of pulmonary arterial hypertension (PAH)?

What is the prognosis of pulmonary arterial hypertension (PAH)?

Presentation

What should be the focus of history in the evaluation of pulmonary arterial hypertension (PAH)?

What are the nonspecific symptoms of pulmonary arterial hypertension (PAH)?

What are the less common symptoms of pulmonary arterial hypertension (PAH)?

Which physical findings suggest pulmonary arterial hypertension (PAH)?

What are the complications of pulmonary arterial hypertension (PAH)?

DDX

What are the differential diagnoses for Pulmonary Arterial Hypertension?

Workup

Which studies are performed in the workup of pulmonary arterial hypertension (PAH)?

What is the role of lab studies in the workup of pulmonary arterial hypertension (PAH)?

What is the role of imaging studies in the workup of pulmonary arterial hypertension (PAH)?

Which findings on an ECG suggest pulmonary arterial hypertension (PAH)?

Which findings on a two-dimensional echocardiography suggest pulmonary arterial hypertension (PAH)?

Which findings on a Doppler echocardiography suggest pulmonary arterial hypertension (PAH)?

What is the role of ventilation-perfusion scanning for the workup of pulmonary arterial hypertension (PAH)?

What is the role of pulmonary function testing in the workup of pulmonary arterial hypertension (PAH)?

What is the role of polysomnography in the workup of pulmonary arterial hypertension (PAH)?

What is the role of right-sided heart catheterization in the diagnosis of pulmonary arterial hypertension (PAH)?

What is the role of vasoreactivity testing in the workup of pulmonary arterial hypertension (PAH)?

Which histologic findings are characteristic of pulmonary arterial hypertension (PAH)?

What are the functional stages of pulmonary hypertension?

Treatment

What is included in supportive therapy for pulmonary arterial hypertension (PAH)?

What is the role of advanced vasodilatory therapies in the treatment of pulmonary arterial hypertension (PAH)?

What is the role of oxygen supplementation in the management of pulmonary arterial hypertension (PAH)?

What is the role of vaccines in the management of pulmonary arterial hypertension (PAH)?

Why should women with pulmonary arterial hypertension (PAH) be offered birth control?

What is the role of diuretics in the management of pulmonary arterial hypertension (PAH)?

What is the role of digoxin in the management of pulmonary arterial hypertension (PAH)?

What is the role of anticoagulants in the management of pulmonary arterial hypertension (PAH)?

When is calcium channel blocker therapy indicated to treat pulmonary arterial hypertension (PAH)?

What are the types of advanced vasodilatory therapies used in the treatment of pulmonary arterial hypertension (PAH)?

What are the treatment options for functional class II or III pulmonary arterial hypertension (PAH)?

What are the treatment options for functional class IV pulmonary arterial hypertension (PAH)?

What is the role of a balloon atrial septostomy in the treatment of pulmonary arterial hypertension (PAH)?

What is the role of lung transplantation in treatment of pulmonary arterial hypertension (PAH)?

Where should patients with pulmonary arterial hypertension (PAH) be treated?

Which dietary modifications are used in the management of pulmonary arterial hypertension (PAH)?

Which activity modifications are used in the treatment of pulmonary arterial hypertension (PAH)?

How is pulmonary arterial hypertension (PAH) prevented?

What is included in the long-term monitoring of patients with pulmonary arterial hypertension (PAH)?

Guidelines

Which organizations have issued treatment guidelines for pulmonary arterial hypertension (PAH)?

Medications

What is the role of medications in the treatment of pulmonary arterial hypertension (PAH)?

What is the efficacy of calcium channel blockers (CCBs) for the treatment of pulmonary arterial hypertension (PAH)?

What is the role of prostacyclin analogues and agonists in the treatment of pulmonary arterial hypertension (PAH)?

What is the efficacy of epoprostenol for the treatment of pulmonary arterial hypertension (PAH)?

What is the efficacy of treprostinil for the treatment of pulmonary arterial hypertension (PAH)?

What is the efficacy of iloprost for the treatment of pulmonary arterial hypertension (PAH)?

What is the efficacy of selexipag for the treatment of pulmonary arterial hypertension (PAH)?

What is the efficacy of endothelin-1 for the treatment of pulmonary arterial hypertension (PAH)?

What is the efficacy of bosentan for the treatment of pulmonary arterial hypertension (PAH)?

What is the efficacy of ambrisentan for the treatment of pulmonary arterial hypertension (PAH)?

What is the efficacy of macitentan for the treatment of pulmonary arterial hypertension (PAH)?

What is the efficacy of sildenafil for the treatment of pulmonary arterial hypertension (PAH)?

What is the role of tadalafil for the treatment of pulmonary arterial hypertension (PAH)?

What is the role of vardenafil for the treatment of pulmonary arterial hypertension (PAH)?

What is the role of soluble guanylate cyclase (sGC) for the treatment of pulmonary arterial hypertension (PAH)?

What is the efficacy of riociguat for the treatment of pulmonary arterial hypertension (PAH)?

What is the role of cardiac glycosides for the treatment of pulmonary arterial hypertension (PAH)?

What is the role of digoxin for the treatment of pulmonary arterial hypertension (PAH)?

What is the role of diuretics for the treatment of pulmonary arterial hypertension (PAH)?

What is the role of furosemide for the treatment of pulmonary arterial hypertension (PAH)?

What is the role of bumetanide for the treatment of pulmonary arterial hypertension (PAH)?

When is long-term anticoagulation with warfarin indicated in the treatment of pulmonary arterial hypertension (PAH)?

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

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

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

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

Which medications in the drug class Phosphodiesterase-5 Enzyme Inhibitors are used in the treatment of Pulmonary Arterial Hypertension?

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

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

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

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