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Pulmonary Hypertension, Pediatric

Richard M. Kravitz, MD Reviewed 10/2018
 


BASICS

DESCRIPTION

Increased pulmonary vascular resistance 

EPIDEMIOLOGY

Incidence

Incidence in children is unknown. 

PATHOPHYSIOLOGY

  • Structural alterations in pulmonary vessel architecture (remodeling)

  • Smoo...

DIAGNOSIS

HISTORY

  • Dyspnea (usually earliest complaint reported)

  • Fatigue

    • Seen early in course of illness with exercise or exertion (but not at rest)

    • Seen at rest in the later stages of the illness or in se...

TREATMENT

GENERAL MEASURES

  • Provide for patient stabilization.

  • Treat the primary disease process.

  • Treat underlying hypoxia (supplemental oxygen).

  • Treat underlying hypoventilation:

    • Useful for correcting hypo...

ONGOING CARE

PROGNOSIS

  • Dependent on underlying etiology, but the overall prognosis has been improving with the advent of newer therapies

  • In cases of primary pulmonary hypertension, improvement of pulmon...

ADDITIONAL READING

  • Abman SH, Hansmann G, Archer SL, et al; for American Heart Association Council on Cardiopulmonary, Critical Care, Perioperative and Resuscitation; Council on Clinical Cardiology; Co...

CODES

ICD9

  • 416.8 Other chronic pulmonary heart diseases

  • 416.0 Primary pulmonary hypertension

  • 747.83 Persistent fetal circulation

ICD10

  • I27.2 Other secondary pulmonary hypertension

  • I27.0 Primary pulmonary hy...

FAQ

  • Q: How many hours per day should supplemental oxygen be used?

  • A: Studies have shown decreased mortality in patients using oxygen 24 hours per day compared with patients using supplemental oxygen fo...

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