Summary
Definition
History and exam
Key diagnostic factors
- age 20-50 years
- female sex
- dyspnea
- accentuated pulmonic component (P2) to the second heart sound
- tricuspid regurgitation murmur
- family history
Other diagnostic factors
- fatigue
- peripheral edema
- cyanosis
- stimulant use
- syncope
- chest pain
- near syncope
- early diastolic, high-pitched murmur in the pulmonary area
- jugular vein distension
Risk factors
- family history
- female sex
- bone morphogenetic protein receptor type 2 (BMPR2) mutations
- appetite suppressants
- drugs and toxins
Diagnostic investigations
1st investigations to order
- chest radiography
- ECG
- transthoracic Doppler echocardiography
- right heart catheterization
- ANA
- PFTs
- nocturnal oximetry
- ventilation-perfusion (V/Q) scintigraphy
- 6-minute walk test
- B-type natriuretic peptide (BNP) or N-terminal proBNP (NT-proBNP)
- CBC
- LFTs
- thyroid function tests
- 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
positive response to acute vasoreactivity testing with no contraindication to calcium-channel blockers
negative response to acute vasoreactivity testing or calcium-channel blockers contraindicated
Contributors
Authors
Gustavo A. Heresi, MD, MS

Director, Pulmonary Vascular and CTEPH 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.
Raed A. Dweik, MD

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
Michael J. Krowka, MD
Division of Pulmonary and Critical Care Medicine
Mayo Clinic College of Medicine
Rochester
MN
Disclosures
MJK declares that he has no competing interests.
Charlie Elliot, MB ChB, MRCP
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.
Differentials
- Pulmonary arterial hypertension (PAH) associated with left heart diseases (pulmonary venous hypertension)
- PAH associated with lung respiratory diseases and/or hypoxia
- PAH due to chronic thrombotic and/or embolic disease
More DifferentialsGuidelines
- Guidelines for the diagnosis and treatment of pulmonary hypertension
- Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report
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