Pulmonary Hypertension

Causes

Depending on the type, pulmonary hypertension can have many potential causes. Types include:

• Pulmonary arterial hypertension
• Pulmonary venous hypertension
• Chronic thromboembolic pulmonary hypertension
• Hypoxic pulmonary hypertension
• Pulmonary hypertension due to left-to-right shunts.

Idiopathic pulmonary arterial hypertension is the diagnosis when no underlying cause for high blood pressure in the lungs can be found.

Hereditable pulmonary arterial hypertension can be the diagnosis when a patient and a family member or members have the condition. In some instances, a gene mutation can be the cause.

Associated pulmonary arterial hypertension is diagnosed when pulmonary hypertension results from certain medical conditions. Several medical conditions can cause pulmonary arterial hypertension.

Pulmonary venous hypertension is caused by conditions of the left side of the heart or the impaired return of blood to the left side of the heart. These conditions include:

• Aortic regurgitation
• Aortic stenosis
• Ball-valve thrombus
• Coarctation of the aorta
• Congenital pulmonary vein stenosis
• Congenital subaortic stenosis
• Constrictive pericarditis
• Cor triatriatum
• Dilated cardiomyopathy
• Hypertrophic cardiomyopathy
• Mediastinal fibrosis
• Mediastinal neoplasm
• Mediastinitis
• Mitral regurgitation
• Mitral stenosis
• Myxoma
• Restrictive cardiomyopathy
• Supravalvular aortic stenosis

Thromboembolic pulmonary hypertension — clots in the lung vessels — may be cured by a surgical procedure (pulmonary thromboendarterectomy) that is performed at Mayo Clinic in Minnesota.

Hypoxemic pulmonary hypertension can result from a number of lung conditions, including:

• Chronic diffuse parenchymal disease
• Alveolar filling disorders
• Connective tissue disorders (scleroderma, rheumatoid lung)
• Interstitial fibrosis
• Granulomatous disease
• Pneumoconioses
• Chronic lower airway obstruction
• Asthmatic bronchitis
• Bronchiectasis
• Chronic bronchitis
• Cystic fibrosis
• Emphysema
• Chronic upper airway obstruction
• Congenital webs
• Enlarged tonsils
• Obstructive sleep apnea
• Long-term high altitude dwelling
• Obesity
• Kyphoscoliosis
• Lung resection
• Neuromuscular disorders
• Severe pleural fibrosis
• Restrictive respiratory dysfunction

Left-to-right shunt pulmonary hypertension occurs when blood flows abnormally and continuously from the left side to the right side of the heart and may cause pressure to build up in the lungs. Medical conditions that can lead to pulmonary hypertension due to left-to-right shunts include:

• Aortopulmonary window
• Atrial septal defect
• Patent ductus arteriosus
• Rupture of aortic sinus
• Ventricular septal defect