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Pulmonary Arterial Hypertension

Nasheena Jiwa, MD Reviewed 06/2020
 


BASICS

DESCRIPTION

  • Pulmonary arterial hypertension (PAH) is a category of pulmonary hypertension (PH) characterized by abnormalities in the small pulmonary arteries (precapillary PH) that produce incre...

DIAGNOSIS

Symptoms of PAH are nonspecific, which can lead to missed or delayed diagnosis of this serious disease. 

HISTORY

Dyspnea, weakness, syncope, dizziness, chest pain, palpitations, lower extremit...

TREATMENT

  • Treat underlying diseases/conditions that may cause PAH to relieve symptoms and improve quality of life and survival.

  • Reasonable goals of therapy include the following:

    • Modified NYHA FC I or I...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • Pneumococcal and influenza vaccines

  • Exercise: walking or low-level aerobic activity, as tolerated, once stable; respiratory training

Patient Monitoring

Frequently ev...

REFERENCES

1
Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol.  2013;62(25 Suppl):D34–D41.
2
McLaughlin VV, Gaine SP, Howard L...

ADDITIONAL READING

Taichman DB, Ornelas J, Chung L, et al. Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report. Chest.  2014;146(2):449–47...

CODES

ICD10

  • I27.0 Primary pulmonary hypertension

  • I27.2 Other secondary pulmonary hypertension

ICD9

  • 416.0 Primary pulmonary hypertension

  • 416.8 Other chronic pulmonary heart diseases

SNOMED

  • 11399002 Pulmonary ...

CLINICAL PEARLS

  • PAH involves abnormalities in the small pulmonary arteries (precapillary PH) which increase PAP and vascular resistance leading to right heart failure. Diagnosis is made by right heart...

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