Updated: 12/16/2019

Pulmonary Hypertension

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Snapshot
  • A 48-year-old woman presents to the emergency department with worsening exertional dyspnea over the course of a few weeks. She reports mild chest pain when climbing the stairs or walking a few blocks. She has also noticed some swelling in both of her legs. Medical history is remarkable for sarcoidosis and she denies any alcohol, cigarette, or illicit drug use. Physical examination is notable for a loud P2 heart sound, bilateral lower extremity pitting edema, and jugular venous distension. Troponin and B-natruietic peptide are not elevated. An ECG demonstrates right axis deviation. She is admitted to the cardiac step down unit where she undergoes a right heart catheterization, which demonstrates a mean pulmonary arterial pressure of 67 mmHg at rest. 
Introduction
  • Definition
    • a state of increased mean pulmonary arterial pressure ≥ 25 mmHg (at rest) in the absence of lung or left-sided heart disease
  • Etiology
    • primary pulmonary arterial hypertension
      • idiopathic
      • familial
      • connective tissue disease (e.g., systemic lupus erythematosus and rheumatoid arthritis)
      • HIV
      • congenital left-to-right shunt
    • left heart disease (e.g., mitral stenosis, mitral regurgitation, atrial or ventricular (diastolic or systolic) dysfunction)
    • lung disease (e.g., chronic obstructive pulmonary disease, sleep apnea, and interstitial lung disease) 
    • chronic thromboembolic disease (e.g., pulmonary embolism)
    • sarcoidosis
    • malignancy compressing pulmonary vessels
  • Prognosis
    • can result in right-sided heart failure
Presentation
  • Symptoms 
    • exertional dyspnea, chest pain, and syncope
    • fatigue and lethargy
  • Physical examination
    • loud P2 on auscultation
    • right ventricular heave
    • right-sided 4th heart sound
    • murmurs
      • holosystolic murmur of tricuspid regurgitation
      • systolic ejection murmur
      • diastolic pulmonic regurgitation (in severe cases)
Imaging
  • Echocardiogram
    • estimates pulmonary artery systolic pressure 
    • evaluates the right ventricle size, thickness, and function
    • also evaluates the left ventricular function and valvular function
  • Radiography of the chest
    • may demonstrate
      • central pulmonary arterial dilatation
      • loss of peripheral blood vessels
      • may find right atrial and ventricular enlargement (suggestive of advanced disease)
Studies
  • ECG
    • can demonstrate right ventricular hypertrophy (e.g., right axis deviation)
  • Right heart catheterization  
    • confirms the diagnosis of pulmonary hypertension 
      • mean pulmonary artery pressure is ≥ 25 mmHg at rest (8-20 mmHg at rest is considered normal)
    • vasoreactivity testing can be performed
      • this involves administering a short-acting vasodilator followed by measuring the hemodynamics of the pulmonary artery
        • agents that are used include
          • nitric oxide
          • epoprostenol
          • adenosine
          • diltiazem (in patients with positive vasoreactivity testing)
Differential
  • Myocardial infarction
    • differentiating factors
      • left heart catheterization will demonstrate a blockage in the coronary vessels
Treatment
  • Conservative
    • continuous long-term oxygen therapy
      • indication
        • to maintain adequate oxygenation
  • Medical
    • note that there are a myriad of causes of pulmonary hypertension
      • treatment should be aimed at treating and optimizing the underlying condition (e.g., anticoagulation in patients with recurrent pulmonary embolisms)
    • vasoactive agents
      • indication
        • pulmonary hypertension targeted therapy
      • medication options
        • calcium channel blockers
        • prostacyclin pathway agonists (e.g., epoprostenol, treprostinil, and iloprost)
        • endothelin receptor antagonists (e.g., bosentan, macitentan, and ambrisentan)
        • nitric oxide-cyclic GMP enhancer (e.g., sildenafil and tadalafil)
Complications
  • Right ventricular dysfunction
  • Right heart failure
  • Arrhythmia

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Questions (3)
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(M3.PL.16.65) A 62-year-old man with history of heart failure with preserved ejection fraction (HFpEF) and COPD presents to his cardiologist for a right heart catheterization procedure after a recent echocardiogram revealed pulmonary artery pressure (PAP) of 55 mmHg. Intraoperative administration of intravenous adenosine causes the PAP to decrease to 35 mmHg. What pharmacological therapy is most likely to provide long-term benefit for this patient? Tested Concept

QID: 103006
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Epoprostenol

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Adenosine

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Bosentan

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Amlodipine

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Sildenafil

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(M2.PL.14.63) A 30-year-old previously healthy woman with no significant past medical history presents to the emergency department with gradually worsening shortness of breath for the past year. The patient reports worsening dyspnea on exertion, fatigue, shortness of breath, and occasional exertional chest pain. Her temperature is 97.2°F (36.2°C), blood pressure is 137/98 mm Hg, pulse is 90/min, respirations are 20/min, and oxygen saturation is 95% on room air. Physical exam is notable for bilateral clear breath sounds and an increased intensity of the P2 heart sound. A chest radiograph is seen in Figure A and an ECG is seen in Figure B which is a change from her previous sinus rhythm ECG. Which of the following is the most accurate test for this patient's condition? Tested Concept

QID: 103004
FIGURES:
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Cardiac MRI

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CT angiogram

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Echocardiogram

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Pulmonary function tests

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Right-sided heart catheterization

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