Pulmonary Hypertension

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Basics

Description

Increased pulmonary vascular resistance

Epidemiology

Incidence

Incidence in children is unknown.

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)

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Basics

Description

Increased pulmonary vascular resistance

Epidemiology

Incidence

Incidence in children is unknown.

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)

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