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Pulmonary Arterial Hypertension

Nasheena Jiwa, MD Reviewed 04/2024
 


BASICS

DESCRIPTION

Pulmonary arterial hypertension (PAH) is characterized by abnormalities in the small pulmonary arteries (precapillary PH) that produce increased pulmonary arterial pressure (PAP) and...

DIAGNOSIS

HISTORY

Dyspnea, weakness, syncope, dizziness, chest pain, palpitations, lower extremity edema 

PHYSICAL EXAM

  • Pulmonary component of S2 (at apex in >90% of patients)

  • Early systolic click of p...

TREATMENT

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

  • Reasonable goals of therapy include:

    • Modified NYHA FC I or II

    • ECG/CMR of no...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

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

DIET

Fluid and salt restrictions, especially with RV failure 

PATIENT EDUCATION

REFERENCES

1
McLaughlin  VV, Gaine  SP, Howard  LS, et al. Treatment goals of pulmonary hypertension. J Am Coll Cardiol.  2013;62(Suppl 25):D73–D81. [View Abstract on OvidM...

CODES

ICD10

  • I27.0 Primary pulmonary hypertension

  • I27.2 Other secondary pulmonary hypertension

SNOMED

  • 11399002 Pulmonary hypertensive arterial disease (disorder)

  • 697898008 Idiopathic pulmonary arterial hype...

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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