Medicine Marrow Pg 211-220 (Pulmonology)
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Questions and Answers

Which test is used to assess right ventricular function in pulmonary hypertension?

  • V/Q scan
  • Sleep study
  • ECHO (correct)
  • 6-minute walk test

    • Pregnancy is contraindicated in patients with pulmonary hypertension.

    True

    What is the purpose of the 6-minute walk test in pulmonary hypertension?

    To determine prognosis

    A positive vaso-reactivity test indicates that pulmonary arterial pressure decreases by _____ mmHg when given nitric oxide.

    <p>10</p> Signup and view all the answers

    Match the clinical findings with their respective treatments for pulmonary hypertension:

    <p>Vaso-reactive test + = 240 mg Nifedipine + Tadalafil NYHA 1-3 = Ambrisentan + Prostanoids NYHA class-4 = Surgical management including atrial septostomy or lung transplant</p> Signup and view all the answers

    What is one of the main clinical features of pulmonary hypertension?

    <p>Breathlessness</p> Signup and view all the answers

    In pulmonary hypertension, the endothelin pathway is inhibited.

    <p>False</p> Signup and view all the answers

    Name one treatment option for the prostacyclin pathway in pulmonary hypertension.

    <p>Phosphodiesterase inhibitors</p> Signup and view all the answers

    In RV failure due to pulmonary hypertension, patients may experience ______ and edema.

    <p>ascites</p> Signup and view all the answers

    Match the clinical features with their corresponding signs:

    <p>Loud P2 = Auscultation Wide splitting of P2 = RV failure Palpable epigastric impulse = Palpation JVP a waves = Jugular venous pulse</p> Signup and view all the answers

    What is the most common lobe involved in bronchiectasis?

    <p>Left lower lobe</p> Signup and view all the answers

    Bronchiectasis primarily affects males more than females.

    <p>False</p> Signup and view all the answers

    Which condition has higher mortality rates?

    <p>Pulmonary arterial hypertension (PAH)</p> Signup and view all the answers

    Pulmonary arterial pressure (PAP) is irrelevant in determining mortality rates in pulmonary arterial hypertension.

    <p>False</p> Signup and view all the answers

    What type of bronchiectasis is classified under Interstitial Lung Disease (ILD)?

    <p>Traction bronchiectasis</p> Signup and view all the answers

    The age range commonly affected by bronchiectasis is between ___ years.

    <p>50-70</p> Signup and view all the answers

    What is the acronym for pulmonary arterial hypertension?

    <p>PAH</p> Signup and view all the answers

    Mortality is higher in ______ than in systemic hypertension.

    <p>pulmonary arterial hypertension (PAH)</p> Signup and view all the answers

    Which histological feature indicates inflammation within the bronchiectasis tissue?

    <p>Inflammatory cell infiltrate</p> Signup and view all the answers

    Match the following terms with their corresponding descriptions:

    <p>PAH = Higher mortality than systemic hypertension PAP = Measurement linked to pulmonary arterial pressure Systemic HTN = Common condition affecting blood pressure Mortality = Rate of death in a population</p> Signup and view all the answers

    Match the following types of bronchiectasis with their visual representation:

    <p>Cylindrical = Straight lines Varicose = Wavy lines Cystic = Clustered circles</p> Signup and view all the answers

    Bronchiectasis can be bilateral but is usually unilateral.

    <p>True</p> Signup and view all the answers

    Identify one of the common histological features seen in bronchiectasis.

    <p>Fibrosis and cartilage destruction</p> Signup and view all the answers

    What is considered the gold standard for diagnosing pulmonary embolism?

    <p>Pulmonary Angiography</p> Signup and view all the answers

    A D-Dimer test is typically used in high-risk cases for pulmonary embolism.

    <p>False</p> Signup and view all the answers

    What is the significance of the Wells Score in evaluating pulmonary embolism risk?

    <p>It helps to assess the risk level and determine the appropriate next steps for diagnosis.</p> Signup and view all the answers

    In cases of pregnancy and contrast allergy, a ______ scan is indicated for assessing pulmonary issues.

    <p>V/Q</p> Signup and view all the answers

    Match the following features with their corresponding points in the Wells Score:

    <p>Clinical signs of DVT = 3 Alternative diagnosis less likely = 3 Heart rate &gt; 100/min = 1.5 Hemoptysis = 1</p> Signup and view all the answers

    What is the initial insult that often leads to bronchiectasis?

    <p>Infection, primarily tuberculosis</p> Signup and view all the answers

    Bronchiectasis is characterized by reversible dilatation of the bronchi.

    <p>False</p> Signup and view all the answers

    What are the primary consequences of losing cilia in bronchiectasis?

    <p>Pooling of secretions in the airways</p> Signup and view all the answers

    Bronchiectasis can be a component of __________ lung diseases.

    <p>obstructive</p> Signup and view all the answers

    Match the following conditions with their relationship to bronchiectasis:

    <p>COPD = Associated with bronchiectasis Cystic fibrosis = Causes bronchiectasis Pseudobronchiectasis = Reversible condition Chronic infection = Contributes to bronchiectasis</p> Signup and view all the answers

    What is Carvallo's sign associated with?

    <p>Pan systolic murmur</p> Signup and view all the answers

    Graham Steels murmur is associated with pulmonary regurgitation.

    <p>True</p> Signup and view all the answers

    What characteristic features are seen on a CXR of a patient with PAH?

    <p>Large central pulmonary arteries, right ventricular hypertrophy, rapid attenuation of pulmonary vessels, clear lung fields.</p> Signup and view all the answers

    What is the recommended first dose for unfractionated heparin in patients requiring anticoagulation?

    <p>1 mg/kg s/c LMWH</p> Signup and view all the answers

    The r/s ratio greater than 1 in V1 indicates _____ in the ECG.

    <p>RVH</p> Signup and view all the answers

    Match the murmurs with their descriptions:

    <p>Pan systolic murmur = High pitched, blowing, increases on inspiration Ejection systolic murmur = Due to pulmonary stenosis Graham Steels murmur = Early diastolic murmur due to PA dilatation</p> Signup and view all the answers

    Patients with genetic mutations should discontinue oral anticoagulants after three months.

    <p>False</p> Signup and view all the answers

    What is the target INR range for anticoagulation management?

    <p>2-3</p> Signup and view all the answers

    If a patient is unfit for thrombolysis, the preferred treatment option is catheter-based __________ or embolectomy.

    <p>thrombolysis</p> Signup and view all the answers

    Match the anticoagulation options with their descriptions:

    <p>Heparin = Administered intravenously or subcutaneously for rapid effects Warfarin = Oral anticoagulant that requires monitoring LMWH = Low molecular weight drug with a more predictable effect Alteplase = Used for thrombolysis in massive VTE cases</p> Signup and view all the answers

    Which syndrome is characterized by tracheobroncheomegaly?

    <p>Mounier-Kuhn syndrome</p> Signup and view all the answers

    Cystic fibrosis is not associated with childhood bronchiectasis.

    <p>False</p> Signup and view all the answers

    What is the most common primary immunodeficiency associated with bronchiectasis?

    <p>IgA deficiency</p> Signup and view all the answers

    _____ syndrome is linked with azoospermia and infertility.

    <p>Young's</p> Signup and view all the answers

    Match the following conditions with their associated features:

    <p>Kartagener's syndrome = Primary ciliary dyskinesia Brock's syndrome = Secondary lymphadenopathy Yellow nail syndrome = Pleural effusions and respiratory problems Alpha 1 antitrypsin deficiency = Liver disease and lung disease</p> Signup and view all the answers

    What does the variable 'R' represent in Ohm's Law as applied to pulmonary hypertension?

    <p>Resistance</p> Signup and view all the answers

    A common cause of Class 1 pulmonary hypertension is idiopathic pulmonary hypertension.

    <p>True</p> Signup and view all the answers

    What is the relationship between pulmonary artery pressure (PAP) and left atrial pressure (LAP) when cardiac causes, like mitral stenosis, are involved?

    <p>PAP increases with LAP due to cardiac causes.</p> Signup and view all the answers

    In pulmonary hypertension, if the pulmonary vascular resistance (PVR) increases, this can lead to an increase in _____ pressure.

    <p>pulmonary artery</p> Signup and view all the answers

    Match the classes of pulmonary hypertension with their characteristics:

    <p>Class 1 = Idiopathic pulmonary hypertension Class 2 = Cardiac cause (e.g., mitral stenosis) Class 3 = Pulmonary hypertension due to lung diseases Class 4 = Pulmonary hypertension due to thromboembolic disease</p> Signup and view all the answers

    Study Notes

    Pulmonary Hypertension

    • Pulmonary hypertension (PH) is defined as a mean pulmonary artery pressure (PAP) greater than 25 mmHg at rest
    • Pathology:
      • Vasoconstriction
      • Remodeling
      • Thrombosis
    • Pathophysiology:
      • Endothelin pathway: promoted in PH, treatment involves antagonists of endotheiln receptors
      • Nitric oxide pathway: inhibited in PH, treatment involves NO donors
      • Prostacyclin pathway: inhibited in PH, treatment involves phosphodiesterase inhibitors (PDE-5 inhibitors), prostacyclin analogues
    • Clinical Features:
      • Presentation:
        • Breathlessness (without overt signs of heart and lung disease)
        • Fatigue
        • Right ventricular (RV) dilatation leading to septal push, increased left ventricular (LV) volume, decreased cardiac output (CO), resulting in breathlessness, fatigue, and syncope
        • Decreased RV filling and increased RV afterload leading to RV ischemia → Chest pain
        • RV failure: ascites, edema, hepatomegaly, apex shifts outwards
      • Auscultation:
        • Loud P2
        • In RV failure:
          • Wide splitting of P2
          • S3 (Pulmonary HTN → RA-RV dilatation → Increased diastolic filling pressure)
      • Palpation:
        • Palpable P2
        • Parasternal heave
        • Palpable epigastric impulse
      • JVP: a waves
      • Other findings:
        • Increased jugular venous pulse
        • Prominent V wave if tricuspid regurgitation is present
        • Fixed splitting of S3
        • Left parasternal lift
        • Clear lungs
        • Large pulsatile liver
        • Cold extremities
        • Clubbing (rare)
        • Peripheral edema
    • Treatment:
      • Specific treatment:
        • Vaso-reactive test positive: 240 mg Nifedipine + Tadalafil
        • NYHA class 1-3: Ambrisentan (Endothelin receptor antagonist) + Prostanoids (Epoprostenol [m/c] via a permanent tunnel catheter in IJV, Trepostinil [via microinfusion pump], Selexipag [chemically stable, orally active prostacyclin derivative, not available yet])
        • NYHA class 4:
      • Surgical management:
        • Atrial septostomy
        • Lung transplant (If not responding to drug)
    • Investigations:
      • Pulmonary function test (PFT):
        • Forced expiratory volume (FEV1): Normal
        • Forced vital capacity (FVC): Normal
        • Diffusing capacity of the lung for carbon monoxide (DLCO): Decreased
      • Echocardiogram (ECHO): to assess RV function, tricuspid regurgitation (TR)
      • Ventilation-perfusion (V/Q) scan: to rule out chronic thromboembolic PAH
      • Sleep study: to rule out obstructive sleep apnea
      • High-resolution computed tomography (HRCT): to rule out interstitial lung disease
      • 6-minute walk test: to determine prognosis
      • Vaso-reactivity test:
        • Patient is given nitric oxide (potent PA vasodilator) → PAP is measured
        • Positive test: PAP decreases by 10 mmHg/PAP < 40 mmHg. BP and CO remain the same. Pulmonary HTN responds to high-dose calcium channel blockers (CCBs) (Nifedipine 240 mg)
      • Pulmonary artery catheterisation: Gold standard for diagnosis.
    • Other Investigations:
      • CXR: Large central pulmonary arteries, RV hypertrophy, rapid attenuation of pulmonary vessels, clear lung fields.
      • ECG: r/s ratio > 1 in V1 (Indicates RVH)
    • Murmurs in PAH:
      • Pan systolic murmur
        • Increased on inspiration (Carvallo's sign)
        • Lower left sternal border
        • High pitched, blowing
      • Ejection systolic murmur due to pulmonary stenosis
      • Early diastolic murmur
        • Graham Steels murmur
        • Due to PA dilatation (severe PAH)
        • Sitting up, leaning forward and fixed expiration
        • Indicates pulmonary regurgitation plus

    Bronchiectasis

    • Definition: Abnormal and irreversible dilatation of bronchi (initially medium-sized, progressing to the entire bronchial tree)
    • Types of dilatation:
      • Cylindrical/tubular
      • Varicose
      • Cystic
    • Notes:
      • Traction bronchiectasis is associated with fibrosis (restrictive type classified under ILD)
      • Bronchiectasis is usually unilateral but can become bilateral
      • Most common lobe involved: Left lower lobe
      • More common in females (F > M)
      • Age range: 50-70 years
    • Histological Features:
      • Fibrosis and cartilage destruction
      • Mucosal and mucous gland hyperplasia
      • Excessive mucus and exudate
      • Inflammatory cell infiltrate
    • Etiopathogenesis/Pathogenesis:
      • Initial insult is often infection (primarily tuberculosis (TB) or idiopathic)
      • This leads to a loss of cilia, weakening and inflammation of the bronchial wall, goblet cell hyperplasia and mucus hypersecretion, further leading to microbial colonization & infection, loss of smooth muscle and elastic tissue in bronchi

    Management of Anticoagulation

    • Start anticoagulation at the earliest (unfractionated heparin/LMWH):
      • First dose: 80 units/kg heparin IV, or
      • 1 mg/kg s/c LMWH (Low molecular weight heparin)
    • Determine severity:
      • Massive VTE (Hypotension +)
      • Submassive VTE (RV dilatation +)
      • Extensive clot burden
    • Absent: Continue anticoagulation at 18 units/kg/hr heparin
    • Target: APTT: 1.5-2 times the upper limit; INR: 2-3
    • Fit for thrombolysis: Thrombolysis with Alteplase: 100 mg IV over 2 hours. Within 14 days.
    • Unfit for thrombolysis: Catheter-based thrombolysis/embolectomy (preferred)
    • At the end of 3 months:
      • Restart heparin and replace with warfarin
      • Oral anticoagulants (New oral anticoagulants > warfarin/Vitamin K): Lifelong if genetic mutation (+)
      • If Contraindication: Inferior vena cava filter

    D-Dimer

    • In low-risk cases. # Pulmonology

    Pulmonary Angiography

    • Gold standard
    • Invasive: Cannulates vein to reach pulmonary artery. # Pulmonology

    V/Q Scan

    • Indications:
      • Pregnancy
      • Contrast allergy

    CTPA (Computed Tomography Pulmonary Angiography)

    • Wells Score:
      • Feature | Points
      • Clinical signs of deep vein thrombosis (DVT) | 3
      • Alternative diagnosis less likely | 3
      • Heart rate (HR) > 100/min | 1.5
      • Recent surgery | 1.5
      • Previous history of DVT/PE | 1.5
      • Hemoptysis | 1
      • Malignancy | 1
    • Based on Wells score:
      • Risk level: Low -> Moderate -> High
      • Next Step: Low D-Dimer + CTPA
      • High Risk: Helical CTPA

    Bronchiectasis

    • Obstructive lung diseases associated with bronchiectasis:
      • COPD (including emphysema and bronchitis, and small airway diseases)
      • Bronchial asthma
      • Bronchiolitis
      • Cystic fibrosis

    Pulmonology

    • Mortality: In PAH > systemic HTN.
    • PAP: Pulmonary Artery Pressure.

    Note

    • Any unexplained shortness of breath (dyspnea) with a normal chest X-ray (CXR), Electrocardiogram (ECG), and physical exam is likely pulmonary embolism (VTE), unless proven otherwise.

    Etiology of Bronchiectasis

    • Paediatric:
      • Post infective: Pertussis, measles
      • Association with syndromes (usually cystic upper to mid fields)
      • Mounier-Kuhn: Tracheobroncheomegaly
        • William Campbell: Absence of cartilage
      • Kartagener's syndrome: Primary ciliary dyskinesia (bronchiectasis, sinusitis & situs inversus) → Impaired mucociliary clearance
      • Young's syndrome: Azoospermia → Infertility
      • Cystic fibrosis
      • Yellow nail syndrome
      • Alpha 1 antitrypsin deficiency
    • Adult:
      • Idiopathic (30%)
      • Post-infection (mostly TB [40%])
      • Mechanical obstruction
      • Brock's syndrome: Secondary lymphadenopathy → Right middle lobe bronchiectasis
      • Immunodeficiency (Also seen in childhood): IgA deficiency (m/c)
      • Autoimmune: Sjogren syndrome

    Note Continued

    • Obliterative bronchiectasis due to infection in childhood: McLeod's syndrome
    • Impaired mucociliary clearance: Kartagener's syndrome; cystic fibrosis; Young's syndrome; isolated IgA deficiency: m/c primary immunodeficiency

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    Description

    This quiz covers the essentials of pulmonary hypertension (PH), including its definition, pathology, and pathophysiology. Explore the clinical features and understand how various pathways are involved in the condition, alongside treatment options. Test your knowledge on this critical cardiovascular topic.

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