Pulmonary Vascular Diseases

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Pulmonary Embolism - Clot Commotion

Obstruction of pulmonary artery/branches, often by dislodged thrombus from deep veins (DVT).

  • Risk Factors: 📌 Virchow's Triad: SHE

    • Stasis (e.g., immobilization, surgery)
    • Hypercoagulability (e.g., OCPs, malignancy, genetic)
    • Endothelial injury (e.g., trauma, surgery)
  • Types of Emboli: Thrombus (>95%), fat, air, amniotic fluid, tumor cells, septic emboli.

  • Pathophysiology: ↑Dead space → V/Q mismatch; hypoxemia; ↑Pulmonary Vascular Resistance (PVR) → right ventricular strain.

  • Clinical Features: Sudden onset dyspnea, pleuritic chest pain, hemoptysis, tachypnea, tachycardia, syncope (massive PE).

  • Diagnosis:

    • Clinical probability: Wells score, PERC rule.
    • D-dimer: High sensitivity, low specificity.
    • CT Pulmonary Angiography (CTPA): Gold standard. CTPA showing saddle pulmonary embolus
    • V/Q Scan: If CTPA contraindicated/inconclusive.
    • ECG: S1Q3T3 pattern (classic but uncommon), sinus tachycardia, RBBB.
    • Echocardiogram: RV dysfunction, McConnell's sign.
  • Management:

    • Anticoagulation: LMWH/UFH, then DOACs/Warfarin.
    • Thrombolysis (e.g., alteplase): For massive PE (hypotension).
    • Embolectomy (catheter/surgical): For massive PE if thrombolysis fails/contraindicated.
  • Complications: Chronic Thromboembolic Pulmonary Hypertension (CTEPH), right heart failure, death.

⭐ A normal D-dimer has a high negative predictive value to rule out PE in low-probability patients.

Pulmonary Hypertension - Pressure Overload

  • Definition: Mean Pulmonary Arterial Pressure (mPAP) >20 mmHg at rest (2022 ESC/ERS).
  • Pathophysiology: Vasoconstriction, vascular remodeling (e.g., intimal fibrosis, medial hypertrophy, plexiform lesions), microthrombosis, inflammation. Histology of plexiform lesion in pulmonary hypertension
  • WHO Classification (📌 Mnemonic: PAH, Left heart, Lung disease, CTEPH, Others - PLLCO)
    GroupTypeExamples
    1PAHIdiopathic, heritable, drug, CTD
    2PH due to Left Heart DiseaseLHD (LV dysfxn, valvular)
    3PH due to Lung Diseases/HypoxiaLung disease/hypoxia (COPD, ILD, sleep apnea)
    4CTEPHCTEPH
    5PH with Unclear Multifactorial MechanismsUnclear (hematologic, systemic, metabolic)
  • Clinical Features: Dyspnea, fatigue, chest pain, syncope; RHF signs (edema, JVD, loud P2, RV heave).
  • Investigations:
    • Echocardiography: Initial screening (estimates PASP, RV size/function).
    • Right Heart Catheterization (RHC): Gold standard (diagnosis, severity: mPAP, PVR >3 Wood units for pre-capillary PH; vasoreactivity testing).
  • Management Principles:
    • Treat underlying cause.
    • General measures: Diuretics, oxygen, anticoagulation (select groups).
    • Targeted (Group 1 PAH): Prostanoids (epoprostenol), ERAs (bosentan), PDE5 inhibitors (sildenafil), sGC stimulators (riociguat).

⭐ Plexiform lesions are characteristic histopathological findings in advanced Group 1 Pulmonary Arterial Hypertension (PAH), indicating severe vascular remodeling.

Pulmonary Edema - Fluid Fiasco

  • Definition: Abnormal accumulation of excess extravascular fluid in lung interstitium & alveoli.
  • Pathophysiology: Imbalance in Starling forces: $J_v = L_p S ([P_c - P_i] - \sigma[\pi_c - \pi_i])$.
  • Clinical Features: Dyspnea, orthopnea (esp. cardiogenic), pink frothy sputum.

Pulmonary Edema Pathogenesis

FeatureCardiogenic EdemaNon-cardiogenic Edema
Primary Defect↑ $P_c$ (Pulm. Cap. Hydrostatic Pressure)↑ Capillary Permeability / ↓ $\pi_c$ (Plasma Oncotic Pressure) / Lymphatic Obstruction
CausesLV failure, mitral stenosisARDS, sepsis, trauma, pancreatitis, drug reactions, HAPE (📌 Think ALI causes)
PCWP>18 mmHg (↑)Normal / Low
Edema FluidLow proteinHigh protein (due to ↑ permeability)
Chest X-rayBatwing, Kerley B lines, cardiomegaly, pleural effusionPatchy/diffuse infiltrates, normal heart size (initially)

High‑Yield Points - ⚡ Biggest Takeaways

  • Pulmonary embolism (PE): Most commonly from DVT; Lines of Zahn indicate pre-mortem clot formation.
  • Pulmonary hypertension (PHTN): Defined as mean pulmonary arterial pressure >20 mmHg; BMPR2 gene mutation is often implicated in primary PHTN.
  • Common causes of secondary PHTN include COPD, mitral stenosis, recurrent PEs, and connective tissue diseases.
  • Plexiform lesions in pulmonary arteries are pathognomonic for severe, irreversible PHTN.
  • Fat embolism syndrome: Classic triad of respiratory distress, neurological symptoms, and petechial rash, typically after long bone fractures.
  • Amniotic fluid embolism: Characterized by sudden DIC, profound shock, and severe hypoxia during or immediately post-delivery; high mortality rate.

Practice Questions: Pulmonary Vascular Diseases

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Drug not used in pulmonary hypertension is:

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Flashcards: Pulmonary Vascular Diseases

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Early hematogenous seedling in the apex of lungs is known as _____ focus.

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Early hematogenous seedling in the apex of lungs is known as _____ focus.

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