Summary
Definition
History and exam
Key diagnostic factors
- age 20 to 50 years
- female sex
- dyspnoea
- accentuated pulmonic component (P2) to the second heart sound
- tricuspid regurgitation murmur
- FHx
Other diagnostic factors
- peripheral oedema
- cyanosis
- stimulant use
- fatigue
- syncope
- chest pain
- near syncope
- early diastolic, high-pitched murmur in the pulmonary area
- jugular vein distention
Risk factors
- female sex
- FHx
- bone morphogenetic protein receptor type 2 (BMPR2) mutations
- drugs and toxins
Diagnostic investigations
1st investigations to order
- CXR
- ECG
- transthoracic Doppler echocardiography
- right heart catheterisation
- ANA
- PFTs
- nocturnal oximetry
- ventilation-perfusion scintigraphy
- 6-minute walk test
- B-type natriuretic peptide (BNP) or N-terminal proBNP (NT-proBNP)
- full blood count
- LFTs
- TFTs
- HIV serology
Investigations to consider
- high-resolution chest CT scan ± CT pulmonary angiography
- vasodilator testing with inhaled nitric oxide, intravenous epoprostenol, or intravenous adenosine
Treatment algorithm
Contributors
Authors VIEW ALL
Medical Director, Pulmonary Thromboendarterectomy Program
Department of Pulmonary and Critical Care Medicine
Respiratory Institute
Cleveland Clinic
Cleveland
OH
Disclosures
GAH has received speaking and advisory board fees from Bayer Healthcare, the manufacturer of riociguat.
Director
Pulmonary Vascular Program
Department of Pulmonary, Allergy, and Critical Care Medicine
Cleveland Clinic
Cleveland
OH
Disclosures
RAD declares that he has no competing interests.
Peer reviewers VIEW ALL
Division of Pulmonary and Critical Care Medicine
Mayo Clinic College of Medicine
Rochester
MN
Disclosures
MJK declares that he has no competing interests.
Consultant Physician in Respiratory and General Internal Medicine
Sheffield Pulmonary Vascular Disease Unit
Royal Hallamshire Hospital
Sheffield
UK
Disclosures
CE has received reimbursement for attending several conferences as well as lecture fees from Actelion Pharmaceuticals, GSK, and Bayer.
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