Pulmonary Hypertension

Description

Increased pulmonary vascular resistance

Epidemiology

Pathophysiology

• Structural alterations in pulmonary vessel architecture (remodeling)
• Smooth muscle hypertrophy
• Extension of blood vessel’s smooth muscle into smaller vessels
• Inflammation

Etiology

• Hypoxemia-induced pulmonary hypertension
• Chronic lung disease
  • Cystic fibrosis
  • Bronchopulmonary dysplasia
  • Interstitial lung disease
  • Diaphragmatic hernia with secondary pulmonary hypoplasia
• Upper airway obstruction
  • Tonsillar and/or adenoid hypertrophy
  • Obesity
• Hypoventilation
  • Neurologically mediated process
  • Secondary to muscular weakness
• High pulmonary blood flow secondary to left-to-right shunting (seen in congenital heart disease)
  • Patent ductus arteriosus
  • Atrial septal defect
  • Ventricular septal defect
• Left-sided cardiac disorders that increase pulmonary venous pressure
  • Left ventricular failure
  • Mitral valve stenosis
  • Obstructed anomalous pulmonary veins
• Occlusion of pulmonary vessels
  • Sickle cell disease
  • Veno-occlusive disease
  • Thromboembolism
• Pulmonary vasculitis
  • Systemic lupus erythematosus
  • Rheumatoid arthritis
  • Scleroderma
• Persistent pulmonary hypertension of the newborn
• Idiopathic cases (primary pulmonary hypertension)