Pediatric Pulmonary Hypertension
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Questions and Answers

What is the definition of Pulmonary Hypertension (PH)?

  • mPAP of >25mmHg at rest via cath (correct)
  • Pulmonary capillary wedge pressure ≤ 10 mm Hg
  • mPAP of >20mmHg at rest via cath
  • Increased pulmonary vascular resistance (PVR) > 2 wu x m2

    • What is the most common cause of Pulmonary Hypertension in children?

  • Transient disease processes (correct)
  • Congenital heart disease
  • Progressive PAH
  • Pulmonary diseases

    • What is the characteristic of smooth muscle cells in Pulmonary Hypertension?

  • Hypertrophy and extension onto arterioles (correct)
  • Increased apoptosis and decreased contraction
  • Hypertrophy and contraction
  • Atrophy and decreased contraction

    • What is the prevalence of chronic PAH in children?

    <p>3 cases/million children</p> Signup and view all the answers

    What is the role of endothelin-1 in Pulmonary Hypertension?

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

    What is the name of the consensus that established a new classification system for pediatric Pulmonary Hypertension?

    <p>Panama Consensus</p> Signup and view all the answers

    What is the effect of increased RV afterload on stroke volume?

    <p>Decreases stroke volume</p> Signup and view all the answers

    What is the consequence of RV hypertrophy?

    <p>Decreased RV diastolic function</p> Signup and view all the answers

    What is the genetic cause of PAH?

    <p>Mutations in the BMPR-II gene</p> Signup and view all the answers

    Under what circumstances is sildenafil contraindicated in children?

    <p>children who have not undergone AVT, are nonresponders to AVT, or have RV failure</p> Signup and view all the answers

    What is the lifetime risk of developing PAH in individuals with a BMPR-II mutation?

    <p>10%–20%</p> Signup and view all the answers

    What is the effect of inhibiting PDE5 in vascular smooth muscle cells?

    <p>increase intracellular cGMP and pulmonary vasodilatation</p> Signup and view all the answers

    What is the common consequence of severe lung disease?

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

    What is the common early sign of acute pulmonary hypertensive crisis?

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

    What is the association found with chronic use of sildenafil in long-term outcome data?

    <p>higher rates of death</p> Signup and view all the answers

    What is the effect of tricuspid regurgitation on the RV?

    <p>Decreases RV diastolic function</p> Signup and view all the answers

    What is the primary mechanism of action of prostacyclin in vasodilatation?

    <p>increasing cAMP within vascular smooth muscle cells</p> Signup and view all the answers

    What is the characteristic of epoprostenol (Flolan) that requires careful dosing?

    <p>short half-life</p> Signup and view all the answers

    What is the consequence of increased RV blood volume?

    <p>Atrial dilation</p> Signup and view all the answers

    What is the consequence of abrupt withdrawal of epoprostenol (Flolan) in patients with PAH?

    <p>rebound PH and acute deterioration</p> Signup and view all the answers

    What is the effect of endothelin (ET) in pulmonary vasoconstriction?

    <p>vasoconstriction and proinflammatory effects</p> Signup and view all the answers

    What is the administration route of iloprost?

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

    What is the typical finding on an X-ray in a patient with acute or chronic PH?

    <p>Enlarged cardiac silhouette due to RVH and enlarged central pulmonary arteries</p> Signup and view all the answers

    Which of the following is NOT a contraindication for using calcium-channel blockers in PH?

    <p>Chronic obstructive pulmonary disease</p> Signup and view all the answers

    What is the primary purpose of using inhaled nitric oxide in PH?

    <p>To improve oxygenation</p> Signup and view all the answers

    What is a potential toxicity of inhaled nitric oxide therapy?

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

    Which of the following imaging modalities may be superior to echocardiography for determining right ventricular volume and contraction?

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

    What is the primary goal of using diuretics in PH?

    <p>To reduce congestive symptoms</p> Signup and view all the answers

    What is the typical dose limit for inhaled nitric oxide therapy?

    <p>20-30 ppm</p> Signup and view all the answers

    What is the goal of hyperventilation in PH crisis?

    <p>To improve oxygenation</p> Signup and view all the answers

    What is the main effect of Ambrisentan on the liver?

    <p>It increases liver transaminases in a dose-dependent manner</p> Signup and view all the answers

    What is the purpose of Atrial Septostomy in severe heart failure?

    <p>To permit right-to-left atrial shunting</p> Signup and view all the answers

    What is the 5-year survival rate for patients who have undergone transplantation for Pulmonary Hypertension?

    <p>49%</p> Signup and view all the answers

    What is a common trigger for Acute Pulmonary Hypertension Crisis?

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

    What is a common complication of Acute Pulmonary Hypertension Crisis?

    <p>Myocardial ischemia</p> Signup and view all the answers

    Which patient population is at a higher risk of Acute Pulmonary Hypertension Crisis?

    <p>Patients with Trisomy 21</p> Signup and view all the answers

    Study Notes

    Pediatric Pulmonary Hypertension

    • Definition: Pulmonary Hypertension (PH) is defined as a mean pulmonary artery pressure (mPAP) of >25mmHg at rest via catheterization.
    • PAH (Pulmonary Arterial Hypertension) is a type of PH with mPAP of >25mmHg at rest via catheterization, pulmonary capillary wedge pressure ≤ 15 mm Hg, and increased pulmonary vascular resistance (PVR) > 3 wu x m2.

    WHO Classification

    • New classification system for Pediatrics established in 2011 in Panama Consensus.

    Epidemiology

    • Most common causes of PH in children: Transient disease processes (82%) such as PPHN, systemic-to-pulmonary shunts.
    • Chronic PAH is rare in children (3 cases/million children), compared to PPHN, which is about 30 cases/million children.

    Pathophysiology

    • Abnormal function of the pulmonary vasculature leading to:
      • Excessive vasoconstriction
      • Smooth muscle hypertrophy and extension of smooth muscle cells onto arterioles
      • Pulmonary blood vessels obstruction due to monoclonal expansion of endothelial cells and plexiform lesions
      • Chronic thrombosis
      • Ongoing inflammation
    • RV hypertension increases RV afterload, leading to:
      • Decreased stroke volume
      • RV hypertrophy
      • Tricuspid regurgitation
      • Diastolic dysfunction
      • RAS system activation
      • Atrial dilation
      • Septal flattening

    Genetics

    • Bone morphogenetic factor receptor type-II (BMPR-II) gene mutations are the most common identified genetic cause of PAH, found in 50%–90% of adults with HPAH.
    • Presence of a BMPR-II mutation is associated with a 10%–20% lifetime risk of developing PAH.

    Pulmonary Hypertension in Acute Lung Disease

    • PH can occur as a consequence of increased PVR in severe lung disease, such as:
      • Acute lung injury/ARDS
      • Bronchiolitis
      • Pertussis

    Clinical Presentation of PH

    • Acute Pulmonary Hypertensive Crisis:
      • Hypotension is a common early sign
      • Late signs include bradycardia with hypotension and signal impending cardiac arrest

    PH Diagnostic Evaluation

    • Imaging:
      • X-ray: Enlarged cardiac silhouette due to RVH and enlarged central pulmonary arteries
      • CT: Helpful for excluding pulmonary causes of PH, such as interstitial lung disease or emphysema
      • MRI: May be superior to echocardiography for determining right ventricular volume and contraction
    • Lung Biopsy: May be used for PH diagnosis

    Treatment

    • Oxygen and General Supportive Measures:
      • Oxygen
      • NIPPV
      • Inotropic medications (Adrenalin and/or milrinone)
      • Diuretics (for congestive symptoms, avoiding excessive reduction in intravascular volume)
      • Intubation and initiation of invasive mechanical ventilation
      • Hyperventilation (for severe, acute PH crisis)
    • Pharmacotherapy:
      • Inhaled Nitric Oxide:
        • Vasodilatation by increasing cGMP within the vascular smooth muscle cells
        • Accumulation of nitrogen dioxide (NO2) and methemoglobin are potential toxicities
      • Calcium-Channel Blockers:
        • Nifedipine, diltiazem, and amlodipine
        • Only a small number of patients with IPAH will respond
      • Phosphodiesterase Type-5 Inhibitors:
        • Sildenafil
        • Increase intracellular cGMP and pulmonary vasodilatation
        • Reduce PAP and vascular resistance, effect is additive to that of NO
      • Prostacyclins:
        • Epoprostenol (Flolan)
        • Treprostinil (Remodulin)
        • Iloprost
      • Endothelin Receptor Antagonists:
        • Bosentan
        • Ambrisentan

    Atrial Septostomy and Transplantation

    • Atrial septostomy: for severe heart failure, refractory PH
    • Transplantation: single lung, double lung, or heart–lung

    Acute Pulmonary Hypertensive Crisis/RV Failure

    • Adaptation of the RV to an increased afterload is critical
    • Triggers:
      • Pain
      • Anxiety
      • Tracheal suctioning
      • Hypoxia
      • Acidosis
    • Commonly described after:
      • Cardiac surgery
      • Rapid withdrawal of PH-specific therapy
      • Intercurrent illness or noncardiac interventions

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    Description

    Introduction to pediatric pulmonary hypertension, including definitions and WHO classification system. Covers diagnosis and symptoms of pulmonary hypertension and pulmonary arterial hypertension in children.

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