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Pulmonary vascular disorders

Pulmonary vascular disorders

Pulmonary vascular disorders

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Pulmonary Embolism - The Clot Thickens

  • Pathophysiology: Occlusion of pulmonary artery → ↑ pulmonary vascular resistance → RV strain. Creates ventilation/perfusion (V/Q) mismatch (ventilated, not perfused).
  • Origin: >95% from deep vein thrombosis (DVT) in lower extremities.
  • Risk Factors (Virchow's Triad): Stasis, hypercoagulability, endothelial injury.
  • Clinical Features: Sudden-onset dyspnea, pleuritic chest pain, tachypnea, tachycardia. May have hemoptysis.

V/Q Scan: Shows perfusion defects with normal ventilation. The "gold standard" if CTPA is contraindicated (e.g., renal failure, contrast allergy).

  • Management: Anticoagulation (heparin, DOACs). Thrombolysis or embolectomy if hemodynamically unstable (massive PE).
  • Non-Thrombotic Emboli: 📌 FAT BAT: Fat, Air, Thrombus, Bacteria, Amniotic fluid, Tumor.

Pulmonary Hypertension - Pressure Overload

  • Definition: Mean pulmonary arterial pressure (mPAP) > 20 mmHg at rest.
  • Pathophysiology: Endothelial dysfunction → ↑ vasoconstrictors (e.g., endothelin) & ↓ vasodilators (e.g., NO, prostacyclin) → vascular remodeling, smooth muscle hypertrophy, & intimal fibrosis.
  • WHO Classification Groups:
    • 1: Pulmonary Arterial Hypertension (PAH) (idiopathic, heritable, drugs)
    • 2: Due to left heart disease (e.g., mitral stenosis)
    • 3: Due to chronic lung disease/hypoxia (e.g., COPD, OSA)
    • 4: Chronic thromboembolic (CTEPH)
    • 5: Multifactorial
  • Presentation: Exertional dyspnea, fatigue, syncope on exertion. Loud P2, right ventricular heave.
  • Diagnosis: Echocardiogram (screening) → Right Heart Catheterization (gold standard).

Plexiform lesions in severe pulmonary hypertension

⭐ Plexiform lesions, representing tufts of capillary formations, are pathognomonic for severe, long-standing Group 1 PAH (idiopathic/heritable).

Other Emboli - Unwanted Guests

  • Fat Embolism Syndrome (FES):

    • Cause: Long bone fractures (femur), orthopedic surgery, liposuction.
    • Pathophysiology: Mechanical obstruction & inflammatory response to free fatty acids.
    • Classic Triad:
      • Hypoxemia
      • Neurologic dysfunction (e.g., confusion)
      • Petechial rash (axilla/chest) - often delayed.
    • Diagnosis: Clinical; fat in urine/sputum. Fat Embolism Syndrome Symptoms
  • Air Embolism:

    • Cause: Iatrogenic (central lines), trauma, barotrauma (diving).
    • Effect: Obstructs RV outflow tract → acute right heart failure.
    • Management: Left lateral decubitus position.
  • Amniotic Fluid Embolism:

    • Pathophysiology: Fetal material enters maternal circulation.
    • Presentation: Abrupt cardiogenic shock, respiratory failure, & DIC during/after labor.

High-Yield: Amniotic fluid embolism is a clinical diagnosis of exclusion, often presenting with sudden cardiovascular collapse and coagulopathy during or shortly after childbirth. It carries a very high mortality rate.

  • Pulmonary embolism (PE) most commonly originates from deep vein thrombosis (DVT).
  • Suspect PE with sudden-onset dyspnea, pleuritic chest pain, and tachypnea.
  • CT pulmonary angiography is the gold standard for PE diagnosis; use V/Q scan if contraindicated.
  • Pulmonary hypertension (mean PAP >20 mmHg) leads to cor pulmonale (RV hypertrophy and failure).
  • Idiopathic pulmonary arterial hypertension is classically linked to a BMPR2 gene mutation.
  • Fat embolism syndrome triad (post-long bone fracture): neurologic changes, petechial rash, and respiratory distress.

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