L21 Pulmonary Hypertension

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Author:
jknell
ID:
200968
Filename:
L21 Pulmonary Hypertension
Updated:
2013-02-16 14:28:32
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Pulmonary II
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Pulmonary II
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  1. Ohm's Law
    • As resistance goes up:
    • 1. Pressure increases (pulmonary HTN)
    • 2. CO decreases
    • *usually both
  2. Pulmonary Arterial Hypertension
    Mean pulmonary arterial pressure > 25mmHg

    • Often estimated by Echo:
    • -measures tricuspid regurgitation jet (present in most ppl)
    • -systolic PAP = (4V2) + CVP

    • Measure with Right Heart Cath:
    • -need accurate number
  3. Presentation
    • -female predominance (especially idiopathic)
    • -may develop at any age (related to etiology)

    • Symptoms:
    • -SOB
    • -hypoxemia
    • -swollen feet and ankles
    • -chest pain
    • -syncope
    • -cyanosis

    • Physical Exam Findings:
    • -hypoxemia (usually later in course)
    • -elevated JVP
    • -enhanced/delayed P2 (pathologic splitting)
    • -Right sided S3 or S4 gallop
    • -pulmonic bruit (CTPH)
    • -peripheral edema
    • -possibly clubbing

    • Imaging:
    • -enlarged cardiac silhouette (esp RA)
    • -enlarged R pulmonary artery
  4. Types of PAH
    • 1. PAH
    • -idiopathic
    • -Heritable
    • -Drug induced

    1'. Pulmonary veno occlusive disease/Pulmonary capillary hemangiomatosis

    2. Pulmonary HTN due to left heart disease

    • 3. Pulmonary HTN due to lung diseases/hypoxia
    • -COPD
    • -interstitial lung disease
    • -sleep-disordered breathing
    • -high altitude

    4. Chronic thromboembolic pulmonary hypertension

    • 5. PH with unclear or multifactorial mechanisms
    • -hematologic disorders
    • -systemic disorders
    • -metabolic disorders
    • -other
  5. Idiopathic PAH
    • -young/middle aged women
    • -many have had sx for years (asthma, syncope)
    • -no identifiable specific causes (diet drugs, autoimmune disease, PEs, LVF)

    • Imaging:
    • -R heart hypertrophy
    • -lack of anterior clear space in front of heart

    • -chronic pericardial effusion

    • -reflux of contrast into liver due to tricuspid regurgitation/RHF
  6. Familial PAH
    • Bone Morphogenetic Protein Receptor 2 (BMPR2)
    • -70% of familial cases
    • -20% of idiopathic

    • Activin-like kinase type 1 (ALK1)
    • -found in HHT (hereditary hemorrhagic telangectasia)

    • Endoglin (ENG)
    • -also found in HHT
  7. Drug-Induced PAH
    • 1. Anorexic Agents
    • -fen-phen

    2. Methamphetamine

    3. Cocaine

    4. SSRIs? MDMA?
  8. HIV-Associated PAH
    • -attributed to viral effect itself
    • -may be more closely related to other lifestyle choices (amphetamines)
  9. Autoimmune-Associated PAH
    • -primarily seen with systemic sclerosis (scleroderma)
    • -may be severe and rapidly progressive
    • -patients benefit from immunosuppression (cyclophosphamide, prednisone)
  10. Portopulmonary Hypertension
    • -PAH associated with elevated portal venous pressures
    • -typically due to cirrhosis
    • -high mortality if untreated (still poor with tx)
    • -usually treatable with PAH meds
    • -high risk of mortality with liver transplant

    NOT THE SAME AS HEPATOPULMONARY SYNDROME
  11. Systemic-to-Pulmonary Shunts
    1. Congenital abnormalities (VSD, ASD, PDA)

    • 2. Eisenmenger's syndrome
    • -L to R shunt becomes R to L shunt

    Patients are often very sick
  12. Pulmonary veno occlusive disease (PVOD)/ Pulmonary Capillary Hemangiomatosis (PCH)
    • -HTN on arterial side secondary to HTN on venous side
    • ** meds to tx PAH usually make them worse!

    • Imaging:
    • -nodules
    • -well demarcated lymphatics within pulmonary parenchyma
    • -lymph adenopathy
  13. Pulmonary Hypertension due to left heart disease
    • 1. LVF (most common cause of elevated pulmonary artery pressure)
    • 2. Mitral Valve stenosis
    • 3. Mitral Regurg
    • 4. Restrictive cardiomyopathy
    • 5. Aortic valve stenosis

    NOT cor pulmonale (lungs are not the initial insult)

    • Do not typically improve with standard PAH therapy
    • -increased blood flow into LA overloads the L heart --> pulmonary edema, effusions, respiratory failure
  14. Neurohormonal Reflex
    • -increased LA stretch triggers vasoconstriction of pulmonary arterioles

    -protects capillaries from high pressure

    -can be overwhelmed (pulmonary edema, capillary rupture/hemorrhage, pleural effusions)

    -P in PA increases much more than LA pressure
  15. Pulmonary Hypertension due to lung disease and/or hypoxia
    • Causes:
    • -COPD
    • -Interstitial lung disease
    • -Sleep-disordered breathing
    • -Chronic high altitude exposure
    • -Developmental abnormalities

    • Chronic Hypoxemia:
    • -leads to hypoxic vasoconstriction (may develop SM proliferative changes)

    • Parenchymal Destruction:
    • -lose pulmonary vasculature --> same amount of blood/fewer vessels --> increased resistance
  16. Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
    • Accumulate multiple emboli over time
    • -can result from recognized acute PE (estimated in 2-4% of PE)

    • May have a genetic predisposition
    • -mutations in fibrinogen

    • Treatment:
    • -may respond to traditional PAH meds
    • -Surgical resection the mainstay of therapy (Pulmonary thromboendarterectomy)
  17. PH with unclear or multifactorial mechanisms
    • 1. Hematologic disorders
    • -myeloproliferative disorders
    • -splenectomy

    • 2. Systemic disorders
    • -sarcoidosis
    • -pulmonary Langerhans cell histiocytosis
    • -NF
    • -vasculitis

    • 3. Metabolic disorders
    • -glucogen storage disease
    • -gaucher disease
    • -thyroid disorders

    • 4. Other
    • -tumor obstruction
    • -fibrosing mediastinitis
    • -chronic renal failure

    • Treatment:
    • -very difficult to treat
    • -not usually responsive to PAH meds
    • -address underlying cause if able
    • -manage symptomatically
  18. PAH Pathology
    -SM hypertrophy of small arteries and arterioles

    -intimal hyperplasia (luminal obliteration)

    -plexiform (weblike) changes of small arterioles

    -RV concentric hypertrophy, dilation

  19. PAH Morbidity and Mortality
    • -Untreated 50% survival at 2 years
    • -Still poor even with therapy

    • -Frequent hospitalizations
    • -repeated surgical procedures (catheter placement)
    • -electrolyte abnormalities
    • -renal dysfunction
    • -bloodstream infections (catheters)
    • -liver dysfunction
    • -limited exercise tolerance
  20. Classes
    I: no functional limitations

    II: sx with moderate exertion

    III: sx with mild exertion

    IV: sx at rest
  21. Medical Therapy
    • 1. CCB
    • -verapamil
    • -diltiazem

    2. PDE5 inhibitors

    3. Endothelin receptor antagonists

    4. Prostacyclins
  22. Calcium Channel Blockers
    • -verapamil
    • -diltiazem

    -ineffective in most patients (class I)

    -inhaled NO testing during R heart cath to test for vasoresponsiveness

    -patients typically graduate to more effective therapies
  23. PDE5 Inhibitors
    • -sildenafil
    • -tadalafil

    -good oral therapy for mild disease (I or II)

    -typically well tolerated

    -not used first line for class III to IV

    -often started by non-specialists --> may delay necessary evaluation and tx
  24. Endothelin Receptor Antagonists
    • -bosentan
    • -ambrisentan

    • -more potent than other oral agents
    • -can be used in mild-moderate (class II-III)

    -must monitor hepatic function but typically well tolerated

    -HIGHLY TERATOGENIC
  25. Prostacyclins
    • -Epoprostenol
    • -Treprostinil (inhaled)
    • -Iloprost (inhaled)

    • -most potent class of medications (Class III-IV)
    • -prolong survival and improve quality of life

    -difficult administration: continuous IV infusion (need indwelling catheter)

    -drug delivery associated complications (infections, surgery)
  26. Natural Course: drop in PA pressure
    • -remember Ohm's law!
    • -Drop in PA pressure may be due to decreased PVR OR decreased cardiac output!
    • -patients may actually be getting WORSE!
  27. Transplantation
    -reserved for severe progression despite medical therapy

    -typically bilateral (now starting to do more unilateral)

    • High risk associated with surgery/anesthesia
    • -increased intrathoracic pressure from mechanical ventilator leads to increased RV pressure --> right heart failure

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