Pulmonary Hypertension

Causes

Idiopathic Pulmonary Arterial Hypertension

No specific cause found

Familial Pulmonary Arterial Hypertension

Other Forms of Pulmonary Hypertension

Related to Pulmonary Venous Hypertension

• Aortic
  • Coarctation
  • Supravalvular aortic stenosis
• Left ventricle
  • Aortic stenosis
  • Aortic regurgitation
  • Congenital subaortic stenosis
  • Hypertrophic cardiomyopathy
  • Constrictive pericarditis
  • Restrictive cardiomyopathy
  • Dilated cardiomyopathy
  • Mitral stenosis
  • Mitral regurgitation
• Left atrium
  • Ball-valve thrombus
  • Myxoma
  • Cor triatriatum
• Pulmonary veins
  • Congenital pulmonary vein stenosis
  • Mediastinitis
  • Mediastinal fibrosis
  • Mediastinal neoplasm
  • Radiofrequency ablation for atrial fibrillation

Related to Arterial Obstructive Pulmonary Hypertension

• Thrombotic disease
  • Sickle cell disease
  • Coagulation disorders
• Embolic disease
  • Chronic thromboemboli
  • Tumor emboli
  • Schistosomiasis
• Connective tissue disorders/vasculitis
  • Lupus
  • Systemic sclerosis
• Chronic liver disease (portal hypertension)
• Hematologic problems (myeloproliferative abnormalities)

Related to Hypoxic Pulmonary Hypertension

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

Related to Pulmonary Hypertension due to Left-to-Right shunts

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