PANCE Blueprint Pulmonary (10%)

Pulmonary hypertension

Patient will present as → a 43-year-old woman with a history of COPD presents to your office with worsening dyspnea, especially at rest. She also complains of dull, retrosternal chest pain. On examination, she has persistent widened splitting of S2. Radiographic findings (seen here) demonstrate peripheral “pruning” of the large pulmonary arteries

**Peripheral “pruning” of the large pulmonary arteries is characteristic of pulmonary hypertension in severe emphysema

Blood pressure in the lungs is usually very low 15/5.  In pulmonary hypertension, the pressure increases > 20 mmHg at rest

  • It can be due to left heart disease, chronic lung disease, or conditions that specifically cause pulmonary arterial hypertension.
  • Mitral stenosis is the most common cause of pulmonary HTN due to a cardiac problem. The mitral valve is so tight blood can't pass into the left ventral which backs pressure up into the lungs.
  • The pressure can become so great that the right heart can no longer pump against the vascular resistance which leads to right heart failure and Cor Pulmonale

World Health Organization Pulmonary Hypertension Groups

  1. Pulmonary Arterial Hypertension
  2. Pulmonary Hypertension due to left heart disease
  3. Pulmonary Hypertension due to lung disease
  4. Pulmonary Hypertension due to blood clots in the lungs
  5. Blood and other rare disorders that lead to Pulmonary Hypertension

Pulmonary HTN

Pulmonary hypertension is difficult to diagnose early on as signs and symptoms are often related to the underlying cause

  • Transthoracic echocardiogram (first diagnostic test for pulmonary HTN)
    • Increased pressure in pulmonary arteries, right ventricles → dilated pulmonary artery
    • Dilatation/hypertrophy of right atrium, right ventricle
    • Large right ventricle → bulging septum
  • Right heart catheterization is the GOLD STANDARD for diagnosis of pulmonary HTN → most accurate measure of pressures
  • Chest X-Ray
    • Enlarged pulmonary arteries
    • Lung fields may or may not be clear, dependent on the underlying cause
  • ECG → Right heart strain pattern: T wave inversion in right precordial (V1–V4), and inferior leads (II, III, aVF)

Diagnostic algorithm for pulmonary hypertension

Pulmonary hypertension workup algorithm

Identify and treat the underlying cause

  • Pulmonary hypertension secondary to left ventricular failure → optimize left ventricular function
    • Diuretics (cautiously—individuals may be preload dependent)
    • Digoxin
    • Anticoagulants
  • Cardiogenic pulmonary arterial hypertension
    • Relax smooth muscle (promote vasodilation), reduce vascular remodeling, improve exercise capacity with prostanoids, phosphodiesterase inhibitors, endothelin antagonists
  • Pulmonary arterial hypertension
    • Endothelin receptor antagonists
    • Prostanoids

osmosis Osmosis
Question 1
What is the diagnostic study of choice for pulmonary hypertension?
right heart catheterization
Patients initially undergo chest x-ray, spirometry, and ECG to identify more common causes of dyspnea,
chest X-ray
Patients initially undergo chest x-ray, spirometry, and ECG to identify more common causes of dyspnea,
pulmonary artery pressure is estimated by echocardiography
Question 1 Explanation: 
Gold standard for diagnosis is right heart catheterization. Pulmonary hypertension is increased pressure in the pulmonary circulation. It has many secondary causes; some cases are idiopathic. In pulmonary hypertension, pulmonary vessels become constricted. Severe pulmonary hypertension leads to right ventricular overload and failure. Symptoms are fatigue, exertional dyspnea, and, occasionally, chest discomfort and syncope. Diagnosis is made by finding elevated pulmonary artery pressure (estimated by echocardiography and confirmed by right heart catheterization). Treatment is with pulmonary vasodilators and diuretics. In some advanced cases, lung transplantation is an option. Prognosis is poor overall if a treatable secondary cause is not found.
Question 2
An O2 saturation of 90% corresponds to what PO2 value?
90 mmHg
See D for explanation.
80 mmHg
See D for explanation.
70 mmHg
See D for explanation.
60 mmHg
Question 2 Explanation: 
O2 sat values above 90% correspond with a PO2 >70 mmHg and values less than 94% represent hypoxemia. Less than 90% O2 sat warrants measurement of arterial blood gasses.
Question 3
Pulmonary hypertension is defined as a mean pulmonary arterial pressure of
≥ 5 mm Hg
See D for explanation
≥ 10 mm Hg
See D for explanation
≥ 15 mm Hg
See D for explanation
≥ 25 mm Hg
Question 3 Explanation: 
Blood pressure in the lungs is usually very low < 15 mm HG. In pulmonary hypertension the pressure increases > 25 mmHG at rest!
There are 3 questions to complete.
Shaded items are complete.

References: Merck Manual · UpToDate

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