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
- family history
Other diagnostic factors
- fatigue
- peripheral oedema
- 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 x-ray
- ECG
- transthoracic Doppler echocardiography
- right heart catheterisation
- antinuclear antibodies (ANA)
- pulmonary function tests
- arterial blood gas (ABG)
- nocturnal oximetry
- ventilation-perfusion (V/Q) scintigraphy
- 6-minute walk test
- B-type natriuretic peptide (BNP) or N-terminal proBNP (NT-proBNP)
- FBC
- LFTs
- thyroid function tests
- HIV serology
Investigations to consider
- high-resolution chest CT scan ± CT pulmonary angiography
- cardiac MRI
- vasodilator testing with inhaled nitric oxide, inhaled iloprost, or intravenous epoprostenol
Treatment algorithm
Contributors
Authors
Muhammad Sameed, MD
Director Advance Lung Disease Program
Department of Pulmonary & Critical Care Medicine
Assistant professor of Medicine
Thomas Jefferson University Hospital - Einstein Health
Philadelphia
PA
Disclosures
MS declares that he has no competing interests.
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.
Acknowledgements
Dr Muhammad Sameed and Dr Gustavo A. Heresi would like to gratefully acknowledge Dr Raed A. Dweik, a previous contributor to this topic.
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.
Maria Giovanna Triveri, MD, PhD
Assistant Professor of Cardiology,
Medical Director of the Pulmonary Hypertension Program
Icahn School of Medicine at Mount Sinai Hospital
New York
NY
Disclosures
MGT has participated on Advisory Boards of Bayer and Actelion and received financial compensation for attendance.
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