Pulmonary Arterial Hypertension (Guidelines on Diagnosis and Treatment of)

Table of contents: Full Text (ESC Clinical Practice Guidelines)

Clinical classification of pulmonary hypertension

• Idiopathic pulmonary arterial hypertension
• Risk factors and associated conditions
• Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis
• Classification of congenital systemicto-pulmonary shunts

Pathology of pulmonary arterial hypertension

Diagnostic strategy

• Clinical suspicion of pulmonary hypertension
• Detection of pulmonary hypertension
• ECG
• Chest radiograph
• Transthoracic Doppler-echocardiography (TTE)

Pulmonary hypertension clinical class identification

• Pulmonary function tests and arterial blood gases
• Ventilation and perfusion (V/Q) lung scan
• High resolution CT of the lung
• Contrast enhanced spiral CT of the lung, pulmonary angiography and magnetic resonance imaging

Pulmonary arterial hypertension evaluation (type, exercise capacity, hemodynamics)

• Blood tests and immunology
• Abdominal ultrasound scan
• Exercise capacity
• Hemodynamics
• Lung biopsy

Assessment of severity

• Clinical variables
• Exercise capacity
• Echocardiographic parameters
• Hemodynamics
• Blood tests

Treatment

Introduction to level of evidence and grade of recommendation

General measures

• Physical activity
• Travel/altitude
• Prevention of infections
• Pregnancy, birth control and post-menopausal hormonal therapy
• Haemoglobin levels
• Concomitant medications
• Psychological assistance
• Elective surgery

Pharmacological treatment

• Oral anticoagulant treatment
• Diuretics
• Oxygen
• Digitalis and dobutamine
• Calcium-channel blockers

Synthetic prostacyclin and prostacyclin analogues

Endothelin-1 receptor antagonists

Type 5 phosphodiesterase inhibitors: Sildenafil

Combination therapy

Interventional procedures: Balloon atrial septostomy, Lung transplantation

Treatment algorithm