Difference between revisions of "Pulmonary hypertension"

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{{Adult top}} [[pulmonary hypertension (peds)]].''
 
==Background==
 
==Background==
*Definition: mean PA pressure >25mmHg assessed by right heart catheterization
+
*Mean PA pressure >25 mmHg as assessed by right heart catheterization  
 +
**Since right ventricle is dependent on preload, RV contractility and afterload, severe pulmonary arterial hypertension causes pathological changes to right ventricle
  
===Types===
+
===WHO Classification<ref>Ryan, J. et al. (2012) The WHO classification of pulmonary hypertension: A case-based imaging compendium. Pulmonary Circulation, 2(1).</ref>===
*'''Group 1''': Idiopathic pulmonary ''arterial'' hypertension, formerly called primary PH
+
*'''Group 1''': Pulmonary ''arterial'' hypertension
*'''Group 2''': PH due to left heart disease (systolic/diastolic dysfunction and valvular heart dz)
+
**Idiopathic
*'''Group 3''': PH due to lung diseases or various causes of [[hypoxemia]], such as [[COPD]], ILD, or sleep-disordered breathing
+
**Associated with:
*'''Group 4''': PH due to chronic thromboembolic disease
+
***Drugs or [[toxins]]
*'''Group 5''': PH of unclear multifactorial mechanisms (myeloproliferative dz, sarcoidosis, glycogen storage dz, etc)
+
***[[Connective tissue disorder]]s
 +
***[[HIV]]
 +
***Portal hypertension
 +
***[[Congenital heart disease]]
 +
*'''Group 2''': PH due to left heart disease
 +
**Associated with:
 +
***Coronary artery disease
 +
***[[Hypertension]]
 +
***[[Valvular disease]]
 +
***Advanced age
 +
*'''Group 3''': PH due to lung diseases
 +
**Associated with:
 +
***[[COPD]]
 +
***[[Interstitial lung disease]]
 +
***Any other lung disease causing [[hypoxemia]]
 +
*'''Group 4''': PH due to chronic [[thromboembolism|thromboembolic disease]]
 +
*'''Group 5''': PH of unclear multifactorial mechanisms
 +
**Associated with:
 +
***[[Sarcoidosis]]
 +
***[[Sickle cell anemia]]
 +
***Chronic [[hemolytic anemia]]
 +
***[[Splenectomy]]
 +
***Metabolic disease
  
 
===Etiologies===
 
===Etiologies===
*heritable
+
*Heritable
*idiopathic
+
*Idiopathic
*associated with connective tissue disease, HIV, portal HTN, congenital heart dz, schistosomiasis, chronic hemolytic anemia
+
*Chronic [[hypoxia]]
*due to lung disease, left heart disease, chronic exposure to high altitudes, chronic thromboembolic disease, myeloproliferative disorders, sarcoidosis, vasculitis, glycogen storage disease, Gaucher disease, chronic renal failure on dialysis
+
*Chronic [[thromboembolism|thromboembolic disease]]
 +
*[[Vasculitis]]
 +
*Autoimmune disease
 +
*Toxic exposures
 +
*Chronic [[renal failure]] on [[dialysis complications|dialysis]]
 +
*[[Myeloproliferative disorders]]
  
 
==Clinical Features==
 
==Clinical Features==
 
===History===
 
===History===
*Exertional dyspnea (most common symptom)<ref>Wilcox et al. "Pulmonary Hypertension and Right Ventricular Failure in Emergency Medicine." Annals of EM. Dec 2015. 66(6):619-631</ref>
+
*Exertional [[dyspnea]] (most common symptom)<ref name="Wilcox"></ref>
*Consider in undifferentiated patients with dyspnea, fatigue, syncope (late PH finding), [[chest pain]], [[palpitations]], LE edema
+
*Consider in undifferentiated patients with [[dyspnea]], fatigue, [[syncope]] (late PH finding), [[chest pain]], [[palpitations]], lower extremity edema
  
 
===Physical exam===
 
===Physical exam===
 
*JVD
 
*JVD
*hepatomegaly
+
*[[Hepatomegaly]]
*ascites
+
*[[Ascites]]
*edema
+
*Edema
*stigmata of liver failure
+
*Stigmata of [[liver failure]]
  
 
==Differential Diagnosis==
 
==Differential Diagnosis==
 
{{SOB DDX}}
 
{{SOB DDX}}
  
==Diagnosis==
+
==Evaluation==
===Testing===
+
''Some, all or none of the following findings may be present.''
*'''[[BNP]]:''' Elevated<ref>Wilcox et al. "Pulmonary Hypertension and Right Ventricular Failure in Emergency Medicine." Annals of EM. Dec 2015. 66(6):619-631</ref>
+
*'''[[BNP]]:''' Elevated<ref name="Wilcox"></ref>
*'''ECG findings''' (similar to acute pulmonary embolism):
+
*'''[[ECG]] findings''' (similar to acute pulmonary embolism):
 
**Right axis deviation
 
**Right axis deviation
**Evidence of right heart strain
+
**Evidence of right heart strain on bedside ultrasound or CT
**S1Q3T3
+
**S1Q3T3 [[ECG]] finding
 +
**Twave inversions on [[ECG]] in inferior and anteroseptal leads
 
**Right ventricular hypertrophy
 
**Right ventricular hypertrophy
***Large R waves in precordial leads
+
*Large R waves in precordial leads
**Tachyarrhythmias (aflutter or afib)
+
*Tachyarrhythmias ([[atrial flutter]] or [[atrial fibrillation]] if new portend poorer prognosis)<ref>Geibel A et al. Prognostic value of the ECG on admission in patients with acute major pulmonary embolism. European Respiratory Journal. 2005. 25: 843-848</ref>
*'''Radiologic findings:'''
+
 
**CXR:
+
===[[CXR]] Abnormalities===
***RA enlargement (obliteration of retrosternal space on lateral CXR)
+
**RA enlargement (obliteration of retrosternal space on lateral CXR)
***Prominent pulmonary vasculature (congestion)
+
**Prominent pulmonary vasculature (congestion)
***PA dilation
+
**PA dilation
**CT Chest:
+
===CTA Chest Abnormalities===
***Pulmonary artery > ascending aorta suggests PH  
+
*Pulmonary artery > ascending aorta suggests PH  
***Pulmonary artery diameter greater than 30 mm suggest PH
+
*Pulmonary artery diameter greater than 30 mm suggest PH
***Right heart enlargement
+
*Right heart enlargement
**Echocardiogram:
+
===[[Echocardiography|Echocardiographic]] Findings===
***RVH, RV dilatation and hypokinesis
+
*D sign (McConnel's Sign)
***RV close to LV size (+/- septal flattening/bowing)
+
*RV close to LV size (+/- septal flattening/bowing)
***Tricuspid valve regurgitation
+
*Tricuspid valve regurgitation
**Estimate systolic pulmonary artery pressure (SPAP) with echo<ref>Critical USG. Echocardiographic assessment of pulmonary artery pressure. 2012. http://www.criticalusg.pl/en/echo/tte/tutorials/echocardiographic-assessment-of-pulmonary-artery-pressures</ref>
+
*Estimate systolic pulmonary artery pressure (SPAP) with echo<ref>Critical USG. Echocardiographic assessment of pulmonary artery pressure. 2012. http://www.criticalusg.pl/en/echo/tte/tutorials/echocardiographic-assessment-of-pulmonary-artery-pressures</ref>
***SPAP = '''Max TR gradient''' + '''Mean RAP'''
+
**SPAP = '''Max TR gradient''' + '''Mean RAP'''
***Cannot use this method with vent-dependent pts, pulmonic stenosis
+
**Cannot use this method with vent-dependent patients, pulmonic stenosis
***Max TR gradient as measured by tricuspid regurg (TR) jet, which >90% of adults have
+
**Max TR gradient as measured by tricuspid regurgitation (TR) jet, which >90% of adults have
****Use parallel CW Doppler line across TR jet in apical view
+
**Use parallel CW Doppler line across TR jet in apical view
****Obtain dense TR profile below the line with well-defined envelope and measure peak = '''Max TR gradient'''
+
**Obtain dense TR profile below the line with well-defined envelope and measure peak = '''Max TR gradient'''
***Estimate right atrial pressure (RAP) with IVC diameter from subcostal view
+
**Estimate right atrial pressure (RAP) with IVC diameter from subcostal view
 
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| align="center" style="background:*f0f0f0;"|
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===Evaluation===
 
===Evaluation===
*Initial diagnosis not typically made in the ED because right-sided heart catheterization needed for definitive diagnosis<ref>Wilcox et al. "Pulmonary Hypertension and Right Ventricular Failure in Emergency Medicine." Annals of EM. Dec 2015. 66(6):619-631</ref>
+
*Initial diagnosis not typically made in the ED because right-sided heart catheterization needed for definitive diagnosis<ref name="Wilcox"></ref>
  
 
==Acute Management==
 
==Acute Management==
''PH patients do not tolerate rapid changes in hemodynamics well, all therapies should be instituted with caution.''
 
  
===Optimize Circulation===  
+
===General Principles of Management for Hypotensive Patient with Pulmonary Hypertension===
 +
''Consult specialist early.  These patients are critically-ill with altered physiology and have very high mortality''
 +
 
 +
 
 +
''First intervention should always be to check any medication pumps.  Resume medication immediately.  If malfunctioning or empty, most pumps/patients have card with hot-line to call if their pulmonologist is not available''
 +
#Assess for triggers for decompensation (Treat infection.  Consider pulmonary embolism.  Evaluate for arrhythmia)
 +
#Assess volume status.  IVC ultrasound less helpful as will be dilated at baseline.  Generally want to avoid fluids.  PH patients do not tolerate rapid changes in hemodynamics.
 +
#Consider early ionotropes nad pressors
 +
#For tachydysarrhythmia, avoid AV nodal blocking agents like beta blockers or calcium channel blockers.  Decrease in inotropy can be fatal.  Do NOT rate control.  Consider cardioversion and rhythm control
 +
#Avoid intubation if at all possible.  PPV can diminish preload which can lead to rapid decompensation and death
 +
#Further details below
 +
 
 +
===Optimize Circulation===
 
#'''Optimize (usually reduce) RV preload''':
 
#'''Optimize (usually reduce) RV preload''':
#*Usually euvolemic or hypervolemic, rarely need IVF
+
#*Usually euvolemic or hypervolemic, rarely need IV fluids so [[diuretics]] can benefit and treat the RV failure<ref>Ternacle, J et al. Diruetics in Normotensive Patients with Acute Pulmonary Embolism and Right Ventricular Dilation. Circulation Journal. Vol 77(10) 2013. 2612-618.</ref>
#*Diuretics to treat RV failure:
+
#**[[Furosemide]] 20-40mg IV  
#**Furosemide 20-40mg IV  
+
#**Furosemide drip at 5-20mg/hr
#**Furosemide drip @ 5-20mg/hr
+
#*If suspect [[sepsis]] or [[hypovolemia]], small (250-500cc) NS challenge to assess fluid responsiveness.  If not responsive to IVF challenge, start [[vasopressin]] or [[norepinephrine]](MAP > 65 mmHg).  
#*If suspect sepsis or hypovolemia, small (250-500cc) NS challenge to assess fluid responsiveness.  If not responsive to IVF challenge, start norepinephrine (MAP > 65 mmHg).
 
 
#'''Increase cardiac output''':
 
#'''Increase cardiac output''':
#*Once MAP >65 mmHg, start low dose dobutamine (5-10mcg/kg/min)
+
#*Once MAP >65 mmHg, start low dose [[dobutamine]] (5-10mcg/kg/min)
 
#*Improves inotropic support and theoretically decreases pulmonary vascular resistance
 
#*Improves inotropic support and theoretically decreases pulmonary vascular resistance
 
#'''Reduce RV afterload''':
 
#'''Reduce RV afterload''':
#*Avoid hypoxia, maintain O2 sat >90% (increases pulmonary vasoconstriction)
+
#*Avoid [[hypoxia]], maintain O2 sat >90% (increases pulmonary vasoconstriction)
#*Avoid hypercapnea (increases pulmonary vascular resistance)
+
#*Avoid [[hypercapnia]] (increases pulmonary vascular resistance)
#*Avoid acidosis
+
#*Avoid [[acidosis]]
#'''Treat arrhythmias:'''
+
#'''Treat [[arrhythmias]]:'''
 
#*[[SVT]] most common although may also become bradycardic (aflutter and afib occur equally)
 
#*[[SVT]] most common although may also become bradycardic (aflutter and afib occur equally)
 
#*Treatment of aflutter is often more successful than afib
 
#*Treatment of aflutter is often more successful than afib
 
#*Do not tolerate negative inotropy, deteriorate to RV failure
 
#*Do not tolerate negative inotropy, deteriorate to RV failure
;Do NOT rate control tachycardias, best to electrically cardiovert or attempt rhythm control with amiodarone
 
 
*May require radiofrequency ablation
 
*May require radiofrequency ablation
*AVOID calcium channel blockers or beta blockers
+
*AVOID calcium channel blockers or β-blockers
  
 
===Optimize Oxygenation===
 
===Optimize Oxygenation===
*'''Intubated patients''' should be optimized to increased O2 delivery and minimize hypercapnea, maintain low tidal volumes and low PEEP as tolerated
+
*'''noninvasive oxygenation:''' attempt nonrebreather mask or high flow nasal cannula to maximize oxygenation. Use NIPPV with caution as positive pressure decreases preload and increases right sided pressures.
 +
*'''Intubate as a last resort:''' RSI meds cause hypotension worsening RV ischemia. hypercapnea and hypoxia worsen pulmonary artery vasoconstriction. positive pressure ventilation decreases preload and increases right sided pressures.
 +
*'''Intubated patients''' should be optimized to increased O2 delivery and minimize hypercapnia, maintain low tidal volumes and low PEEP as tolerated
  
===Early Consultation<ref>Wilcox et al. "Pulmonary Hypertension and Right Ventricular Failure in Emergency Medicine." Annals of EM. Dec 2015. 66(6):619-631</ref>===
+
===Early Consultation<ref name="Wilcox">Wilcox et al. "Pulmonary Hypertension and Right Ventricular Failure in Emergency Medicine." Annals of EM. Dec 2015. 66(6):619-631</ref>===
 
*May require interventions not readily available in the ED:
 
*May require interventions not readily available in the ED:
**Pulmonary arterial cathteter
+
**Pulmonary arterial catheter
**Inhaled pumonay vasodilators
+
**Inhaled pulmonary vasodilators
**Mechanical support with right ventricular assist device or ECMO
+
**Mechanical support with right ventricular assist device or [[ECMO]]
  
 
==Chronic Therapies==
 
==Chronic Therapies==
===Prostacyclins===  
+
===Prostacyclins===
 
'''Mechanisms of action''': vasodilatation, inhibit platelet aggregation
 
'''Mechanisms of action''': vasodilatation, inhibit platelet aggregation
*Epoprostenol, Iloprost, Treprostinil, Beraprost
+
*[[Epoprostenol]], Iloprost, Treprostinil, Beraprost
 
**Complications include acute decompensation if stopped abruptly, diarrhea, edema, headache
 
**Complications include acute decompensation if stopped abruptly, diarrhea, edema, headache
 
===Phosphodiesterase Type 5 (PDE5) Inhibitors===
 
===Phosphodiesterase Type 5 (PDE5) Inhibitors===
 
'''Mechanism of Action''': vasodilation, increases RV contractility
 
'''Mechanism of Action''': vasodilation, increases RV contractility
*Sildenafil
+
*[[Sildenafil]]
 
*Complications include hypotension with administration of nitrates, flushing, epistaxis, headache
 
*Complications include hypotension with administration of nitrates, flushing, epistaxis, headache
 
===Endothelin receptor antagonists===
 
===Endothelin receptor antagonists===
'''Mechanism of Action''': vasodilation via vascular modulation modulation
+
'''Mechanism of Action''': vasodilation via vascular modulation
*Bosentan, Ambrisentan
+
*[[Bosentan]], Ambrisentan
** Complications include liver failure, supratherapeutic INR,  
+
**Complications include liver failure, supratherapeutic INR,  
*Patients also usually taking digoxin, coumadin, diuretics, home O2. RARELY are they on CCB only if responsive during cath. Consider line infections as complication to chronic infusions.
+
*Patients also usually taking [[digoxin]], [[warfarin]], [[diuretics]], home [[O2]]. RARELY are they on [[CCBs]] only if responsive during cath. Consider line infections as complication to chronic infusions.
  
 
==Disposition==
 
==Disposition==
Line 135: Line 176:
 
<references/>
 
<references/>
  
[[Category:Cards]]
+
[[Category:Cardiology]]
[[Category:Pulm]]
+
[[Category:Pulmonary]]

Latest revision as of 16:05, 1 July 2020

This page is for adult patients. For pediatric patients, see: pulmonary hypertension (peds).

Background

  • Mean PA pressure >25 mmHg as assessed by right heart catheterization
    • Since right ventricle is dependent on preload, RV contractility and afterload, severe pulmonary arterial hypertension causes pathological changes to right ventricle

WHO Classification[1]

Etiologies

Clinical Features

History

Physical exam

Differential Diagnosis

Acute dyspnea

Emergent

Non-Emergent

Evaluation

Some, all or none of the following findings may be present.

  • BNP: Elevated[2]
  • ECG findings (similar to acute pulmonary embolism):
    • Right axis deviation
    • Evidence of right heart strain on bedside ultrasound or CT
    • S1Q3T3 ECG finding
    • Twave inversions on ECG in inferior and anteroseptal leads
    • Right ventricular hypertrophy
  • Large R waves in precordial leads
  • Tachyarrhythmias (atrial flutter or atrial fibrillation if new portend poorer prognosis)[3]

CXR Abnormalities

    • RA enlargement (obliteration of retrosternal space on lateral CXR)
    • Prominent pulmonary vasculature (congestion)
    • PA dilation

CTA Chest Abnormalities

  • Pulmonary artery > ascending aorta suggests PH
  • Pulmonary artery diameter greater than 30 mm suggest PH
  • Right heart enlargement

Echocardiographic Findings

  • D sign (McConnel's Sign)
  • RV close to LV size (+/- septal flattening/bowing)
  • Tricuspid valve regurgitation
  • Estimate systolic pulmonary artery pressure (SPAP) with echo[4]
    • SPAP = Max TR gradient + Mean RAP
    • Cannot use this method with vent-dependent patients, pulmonic stenosis
    • Max TR gradient as measured by tricuspid regurgitation (TR) jet, which >90% of adults have
    • Use parallel CW Doppler line across TR jet in apical view
    • Obtain dense TR profile below the line with well-defined envelope and measure peak = Max TR gradient
    • Estimate right atrial pressure (RAP) with IVC diameter from subcostal view
Normal Intermediate High
Mean RAP, mmHg 3 8 15
IVC diameter max 2.1 max 2.1 > 2.1
Resp variation >50% <50% <50%

Evaluation

  • Initial diagnosis not typically made in the ED because right-sided heart catheterization needed for definitive diagnosis[2]

Acute Management

General Principles of Management for Hypotensive Patient with Pulmonary Hypertension

Consult specialist early. These patients are critically-ill with altered physiology and have very high mortality


First intervention should always be to check any medication pumps. Resume medication immediately. If malfunctioning or empty, most pumps/patients have card with hot-line to call if their pulmonologist is not available

  1. Assess for triggers for decompensation (Treat infection. Consider pulmonary embolism. Evaluate for arrhythmia)
  2. Assess volume status. IVC ultrasound less helpful as will be dilated at baseline. Generally want to avoid fluids. PH patients do not tolerate rapid changes in hemodynamics.
  3. Consider early ionotropes nad pressors
  4. For tachydysarrhythmia, avoid AV nodal blocking agents like beta blockers or calcium channel blockers. Decrease in inotropy can be fatal. Do NOT rate control. Consider cardioversion and rhythm control
  5. Avoid intubation if at all possible. PPV can diminish preload which can lead to rapid decompensation and death
  6. Further details below

Optimize Circulation

  1. Optimize (usually reduce) RV preload:
    • Usually euvolemic or hypervolemic, rarely need IV fluids so diuretics can benefit and treat the RV failure[5]
      • Furosemide 20-40mg IV
      • Furosemide drip at 5-20mg/hr
    • If suspect sepsis or hypovolemia, small (250-500cc) NS challenge to assess fluid responsiveness. If not responsive to IVF challenge, start vasopressin or norepinephrine(MAP > 65 mmHg).
  2. Increase cardiac output:
    • Once MAP >65 mmHg, start low dose dobutamine (5-10mcg/kg/min)
    • Improves inotropic support and theoretically decreases pulmonary vascular resistance
  3. Reduce RV afterload:
    • Avoid hypoxia, maintain O2 sat >90% (increases pulmonary vasoconstriction)
    • Avoid hypercapnia (increases pulmonary vascular resistance)
    • Avoid acidosis
  4. Treat arrhythmias:
    • SVT most common although may also become bradycardic (aflutter and afib occur equally)
    • Treatment of aflutter is often more successful than afib
    • Do not tolerate negative inotropy, deteriorate to RV failure
  • May require radiofrequency ablation
  • AVOID calcium channel blockers or β-blockers

Optimize Oxygenation

  • noninvasive oxygenation: attempt nonrebreather mask or high flow nasal cannula to maximize oxygenation. Use NIPPV with caution as positive pressure decreases preload and increases right sided pressures.
  • Intubate as a last resort: RSI meds cause hypotension worsening RV ischemia. hypercapnea and hypoxia worsen pulmonary artery vasoconstriction. positive pressure ventilation decreases preload and increases right sided pressures.
  • Intubated patients should be optimized to increased O2 delivery and minimize hypercapnia, maintain low tidal volumes and low PEEP as tolerated

Early Consultation[2]

  • May require interventions not readily available in the ED:
    • Pulmonary arterial catheter
    • Inhaled pulmonary vasodilators
    • Mechanical support with right ventricular assist device or ECMO

Chronic Therapies

Prostacyclins

Mechanisms of action: vasodilatation, inhibit platelet aggregation

  • Epoprostenol, Iloprost, Treprostinil, Beraprost
    • Complications include acute decompensation if stopped abruptly, diarrhea, edema, headache

Phosphodiesterase Type 5 (PDE5) Inhibitors

Mechanism of Action: vasodilation, increases RV contractility

  • Sildenafil
  • Complications include hypotension with administration of nitrates, flushing, epistaxis, headache

Endothelin receptor antagonists

Mechanism of Action: vasodilation via vascular modulation

  • Bosentan, Ambrisentan
    • Complications include liver failure, supratherapeutic INR,
  • Patients also usually taking digoxin, warfarin, diuretics, home O2. RARELY are they on CCBs only if responsive during cath. Consider line infections as complication to chronic infusions.

Disposition

  • Low threshold for admission if acute decompensation

See Also

References

  1. Ryan, J. et al. (2012) The WHO classification of pulmonary hypertension: A case-based imaging compendium. Pulmonary Circulation, 2(1).
  2. 2.0 2.1 2.2 2.3 Wilcox et al. "Pulmonary Hypertension and Right Ventricular Failure in Emergency Medicine." Annals of EM. Dec 2015. 66(6):619-631
  3. Geibel A et al. Prognostic value of the ECG on admission in patients with acute major pulmonary embolism. European Respiratory Journal. 2005. 25: 843-848
  4. Critical USG. Echocardiographic assessment of pulmonary artery pressure. 2012. http://www.criticalusg.pl/en/echo/tte/tutorials/echocardiographic-assessment-of-pulmonary-artery-pressures
  5. Ternacle, J et al. Diruetics in Normotensive Patients with Acute Pulmonary Embolism and Right Ventricular Dilation. Circulation Journal. Vol 77(10) 2013. 2612-618.