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Underlying Cause
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Pulmonary Hypertension Underlying Cause
Three types of pulmonary hypertension include:
- Neonatal pulmonary hypertension
- Primary pulmonary hypertension
- Secondary pulmonary hypertension
Neonatal Pulmonary Hypertension
The ductus arteriosus is a connection between the right and left heart that normally closes at birth in newborns. If the ductus arteriosus remains open, the blood pressure becomes elevated in the arteries that supply blood to the lungs.
Primary Pulmonary Hypertension
The blood pressure in the arteries that supply blood to the lungs is elevated. The cause for primary pulmonary hypertension is unknown.
Secondary Pulmonary Hypertension
The blood pressure in the arteries that supply blood to the lungs is elevated, due to underlying heart or lung disease.
Causes of secondary pulmonary hypertension include:
- Aortic regurgitation
- Aortic stenosis
- Atrial septal defect
- Bronchiectasis
- Chronic obstructive pulmonary disease

- Coarctation of the aorta
- Idiopathic hypertrophic subaortic stenosis
- Cystic fibrosis
- Dilated cardiomyopathy
- Emphysema
- Enlarged tonsils
- Hypertrophic cardiomyopathy
- Left atrial myxoma
- Lupus
- Mitral regurgitation
- Mitral stenosis
- Obstructive sleep apnea
- Patent ductus arteriosus
- Pneumoconiosis
- Pulmonary fibrosis

- Restrictive cardiomyopathy
- Rheumatoid lung
- Pulmonary fibrosis due to underlying rheumatoid arthritis
- Scleroderma

- Sickle cell anemia

- Ventricular septal defect
Continue to Pulmonary Hypertension Types
Last Updated: Feb 9, 2011 References
Authors: Stephen J. Schueler, MD; John H. Beckett, MD; D. Scott Gettings, MD
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PubMed Pulmonary Hypertension References
- Lee SH, Rubin LJ. Current treatment strategies for pulmonary arterial hypertension. J Intern Med. 2005 Sep;258(3):199-215. [16115293]
- McCrory DC, Lewis SZ. Methodology and grading for pulmonary hypertension evidence review and guideline development. Chest. 2004 Jul;126(1 Suppl):11S-13S. [15249492]
- Randall PA, Heitzman ER, Bull MJ, Scalzetti EM, Williams SK, Gordon LP, Markarian B. Pulmonary arterial hypertension: a contemporary review. Radiographics. 1989 Sep;9(5):905-27. [2678297]