Pulmonary Hypertension for Certified Emergency Nursing (CEN)
Included In This Lesson
Outline
Pulmonary Hypertension
Definition/Etiology:
Normal mean pulmonary artery pressure is 12-16 mmHg.
Pulmonary hypertension is >20 mmHg.
Five groups of Pulmonary Hypertension:
- Group 1: pulmonary arterial (PA) hypertension (PAH), which has several causes (eg, inheritable causes, drugs, connective tissue disease)
- Group 2: due to left-sided heart failure (most common PH)
- Group 3: due to chronic lung disorders and hypoxemia
- Group 4: due to acute or chronic PE
- Group 5: due to unidentified mechanisms
Pathophysiology:
- Pulmonary arterial hypertension (group 1): small pulmonary muscular arterioles develop vasoconstriction, hyperplasia, hypertrophy, fibrosis, and thrombosis that involves all three layers of the vascular wall.
- Pulmonary hypertension:
Group 2–elevated left-sided pressure backs up into the pulmonary system. - Group 3–restrictive (kyphosis), obstructive (COPD), hypoxia (sleep apnea, obesity hypoventilation)
- Group 4–rare (1%) complication of PE. Poorly understood. Not related to genetic hypercoagulable states.
Clinical Presentation:
- Exertional dyspnea / chest pain / syncope
- Lethargy / fatigue
Right-sided heart failure signs and symptoms:
- Ascites / hepatomegaly
- Leg edema
- Early satiety
- RUQ pain
Collaborative Management:
- EKG: right axis deviation due to RV/RA enlargement
- CXR: pleural effusions
- Labs: elevated BNP
- Echocardiogram: RV strain, tricuspid regurg
- Accurate history: travel, cocaine, diet pills, cigarettes, HIV, connective tissue disease, sleep apnea, prior DVT/PE
- Possible left and right heart catheterization
- Pulmonary function test
- Sleep study
- VQ scan or CT angio chest (PE protocol)
- Treat underlying process (anticoagulation if from PEs, CPAP if from apnea, diuretics if from CHF, oxygen if from hypoxia)
- Smoking cessation
- Pulmonary rehab / exercise
- Lung transplantation for Group 3
- Heart transplantation for Group 2 (or LVAD)
- Cardiology consult
Evaluation | Patient Monitoring | Education:
- Oxygen saturation monitoring
- Home infusion of IV medications for Group 1
- Transplant center referral PRN
- Importance of follow-up on sleep study / apnea treatment
- Drug / smoking cessation / counseling / support
- Home oxygen use and teaching re: safety
- Importance of exercise / pulmonary rehab
Linchpins: (Key Points)
- Treat underlying disease.
- Thin right ventricular wall can’t push against high pressures.
- Pressure in pulmonary system should be quite low.
- Group 1 (PAH) is really bad.
- LOTS of things can cause PH/PAH. You don’t have to memorize all of the groups and causes.
Transcript
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References:
- Fedullo, P. F. (2022, March 11). Epidemiology, pathogenesis, clinical manifestations and diagnosis of chronic thromboembolic pulmonary hypertension. UpToDate. https://www.uptodate.com/contents/epidemiology-pathogenesis-clinical-manifestations-and-diagnosis-of-chronic-thromboembolic-pulmonary-hypertension
- Hopkins, W. (2022, September 26). Treatment of pulmonary arterial hypertension (group 1) in adults: Pulmonary hypertension-specific therapy. UpToDate. https://www.uptodate.com/contents/treatment-of-pulmonary-arterial-hypertension-group-1-in-adults-pulmonary-hypertension-specific-therapy
- Krishnan, U (2022, June 8). Pulmonary hypertension due to left heart disease (group 2 pulmonary hypertension) in adults. UpToDate. https://www.uptodate.com/contents/pulmonary-hypertension-due-to-left-heart-disease-group-2-pulmonary-hypertension-in-adults
- Rubin, L. J. (2022, September 26). Clinical features and diagnosis of pulmonary hypertension of unclear etiology in adults. UpToDate. https://www.uptodate.com/contents/clinical-features-and-diagnosis-of-pulmonary-hypertension-of-unclear-etiology-in-adults
- Rubin, L. J. (2021, November 8). The epidemiology and pathogenesis of pulmonary arterial hypertension (Group 1). UpToDate. https://www.uptodate.com/contents/the-epidemiology-and-pathogenesis-of-pulmonary-arterial-hypertension-group-1
- Ryu, J. H. (2022, September 7). Pulmonary hypertension due to lung disease and/or hypoxemia (group 3 pulmonary hypertension): Treatment and prognosis. UpToDate. https://www.uptodate.com/contents/pulmonary-hypertension-due-to-lung-disease-and-or-hypoxemia-group-3-pulmonary-hypertension-treatment-and-prognosis
NCLEX
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