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 polysomnography in the workup 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 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 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 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 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 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)?
-
Gross pathology on patient who died of severe pulmonary arterial hypertension secondary to persistent patent ductus arteriosus.
-
Close-up view of gross pathology on patient who died of severe arterial pulmonary hypertension secondary to persistent patent ductus arteriosus.
-
Chest radiograph of patient with nonidiopathic pulmonary hypertension shows enlarged pulmonary arteries. This patient had atrial septal defect.
-
54-year-old woman with history of scleroderma (CREST variety—ie, calcinosis cutis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, and telangiectasia) developed dyspnea that worsened upon exertion. The patient was found to have severe pulmonary arterial hypertension.
-
54-year-old woman with history of scleroderma (CREST variety—ie, calcinosis cutis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, and telangiectasia) developed dyspnea that worsened on exertion. Spiral CT showed enlarged pulmonary arteries but no evidence of thromboembolism.
-
Ventilation-perfusion scan of bilateral mismatched segmental and subsegmental defects, suggesting chronic thromboembolic hypertension.
-
A 34-year-old woman with history of scleroderma (CREST variety—ie, calcinosis cutis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, and telangiectasia) developed dyspnea that worsened upon exertion. The patient was found to have severe pulmonary arterial hypertension.
-
A 34-year-old woman with history of scleroderma (CREST variety—ie, calcinosis cutis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, and telangiectasia) developed dyspnea that worsened upon exertion. The patient was found to have severe pulmonary arterial hypertension.
-
A 34-year-old woman with history of scleroderma (CREST variety—ie, calcinosis cutis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, and telangiectasia) developed dyspnea that worsened on exertion. A CT pulmonary angiogram showed a massively enlarged pulmonary artery.