Pulmonary hypertension: Difference between revisions
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==Background== | ==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<ref>Ryan, J. et al. (2012) The WHO classification of pulmonary hypertension: A case-based imaging compendium. Pulmonary Circulation, 2(1).</ref>=== | ||
*'''Group 1''': | *'''Group 1''': Pulmonary ''arterial'' hypertension | ||
*'''Group 2''': PH due to left heart disease | **Idiopathic | ||
*'''Group 3''': PH due to lung diseases | **Associated with: | ||
***Drugs or [[toxins]] | |||
***Connective tissue disorders | |||
***[[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 thromboembolic disease | *'''Group 4''': PH due to chronic thromboembolic disease | ||
*'''Group 5''': PH of unclear multifactorial mechanisms | *'''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 | ||
* | *Chronic hypoxia | ||
* | *Chronic thromboembolic disease | ||
*Vasculitis | |||
*Autoimmune disease | |||
*Toxic exposures | |||
*Vasculitis | |||
*Chronic renal failure on dialysis | |||
*Myeloproliferative disorders | |||
==Clinical Features== | ==Clinical Features== | ||
===History=== | ===History=== | ||
*Exertional dyspnea (most common symptom)<ref name="Wilcox"></ref> | *Exertional dyspnea (most common symptom)<ref name="Wilcox"></ref> | ||
*Consider in undifferentiated patients with dyspnea, fatigue, syncope (late PH finding), [[chest pain]], [[palpitations]], | *Consider in undifferentiated patients with [[dyspnea]], fatigue, [[syncope]] (late PH finding), [[chest pain]], [[palpitations]], lower extremity edema | ||
===Physical exam=== | ===Physical exam=== | ||
| Line 31: | Line 60: | ||
==Evaluation== | ==Evaluation== | ||
''Some, all or none of the following findings may be present.'' | |||
*'''[[BNP]]:''' Elevated<ref name="Wilcox"></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 | |||
*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> | |||
===[[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<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''' | |||
**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 | |||
{|class="wikitable" | {|class="wikitable" | ||
| align="center" style="background:*f0f0f0;"| | | align="center" style="background:*f0f0f0;"| | ||
| Line 78: | Line 108: | ||
==Acute Management== | ==Acute Management== | ||
''PH patients do not tolerate rapid changes in hemodynamics well, all therapies should be instituted with caution.'' | ''PH patients do not tolerate rapid changes in hemodynamics well, all therapies should be instituted with caution. Do NOT rate control tachycardias, best to electrically cardiovert or attempt rhythm control with amiodarone'' | ||
===Optimize Circulation=== | ===Optimize Circulation=== | ||
#'''Optimize (usually reduce) RV preload''': | #'''Optimize (usually reduce) RV preload''': | ||
#*Usually euvolemic or hypervolemic, rarely need | #*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> | ||
#**Furosemide 20-40mg IV | #**Furosemide 20-40mg IV | ||
#**Furosemide drip at 5-20mg/hr | #**Furosemide drip at 5-20mg/hr | ||
| Line 98: | Line 127: | ||
#*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 | ||
*May require radiofrequency ablation | *May require radiofrequency ablation | ||
*AVOID calcium channel blockers or | *AVOID calcium channel blockers or β-blockers | ||
===Optimize Oxygenation=== | ===Optimize Oxygenation=== | ||
| Line 107: | Line 135: | ||
===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>=== | ===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 | **Pulmonary arterial catheter | ||
**Inhaled | **Inhaled plumonary vasodilators | ||
**Mechanical support with right ventricular assist device or ECMO | **Mechanical support with right ventricular assist device or ECMO | ||
| Line 114: | Line 142: | ||
===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 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, coumadin, diuretics, home O2. RARELY are they on CCB only if responsive during cath. Consider line infections as complication to chronic infusions. | ||
Revision as of 13:19, 30 March 2019
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]
- Group 1: Pulmonary arterial hypertension
- Idiopathic
- Associated with:
- Drugs or toxins
- Connective tissue disorders
- HIV
- Portal hypertension
- Congenital heart disease
- Group 2: PH due to left heart disease
- Associated with:
- Coronary artery disease
- Hypertension
- Valvular disease
- Advanced age
- Associated with:
- Group 3: PH due to lung diseases
- Associated with:
- COPD
- Interstitial lung disease
- Any other lung disease causing hypoxemia
- Associated with:
- Group 4: PH due to chronic thromboembolic disease
- Group 5: PH of unclear multifactorial mechanisms
- Associated with:
- Sarcoidosis
- Sickle cell anemia
- Chronic hemolytic anemia
- Splenectomy
- Metabolic disease
- Associated with:
Etiologies
- Heritable
- Idiopathic
- Chronic hypoxia
- Chronic thromboembolic disease
- Vasculitis
- Autoimmune disease
- Toxic exposures
- Vasculitis
- Chronic renal failure on dialysis
- Myeloproliferative disorders
Clinical Features
History
- Exertional dyspnea (most common symptom)[2]
- Consider in undifferentiated patients with dyspnea, fatigue, syncope (late PH finding), chest pain, palpitations, lower extremity edema
Physical exam
- JVD
- Hepatomegaly
- Ascites
- Edema
- Stigmata of liver failure
Differential Diagnosis
Acute dyspnea
Emergent
- Pulmonary
- Airway obstruction
- Anaphylaxis
- Angioedema
- Aspiration
- Asthma
- Cor pulmonale
- Inhalation exposure
- Noncardiogenic pulmonary edema
- Pneumonia
- Pneumocystis Pneumonia (PCP)
- Pulmonary embolism
- Pulmonary hypertension
- Tension pneumothorax
- Idiopathic pulmonary fibrosis acute exacerbation
- Cystic fibrosis exacerbation
- Cardiac
- Other Associated with Normal/↑ Respiratory Effort
- Other Associated with ↓ Respiratory Effort
Non-Emergent
- ALS
- Ascites
- Uncorrected ASD
- Congenital heart disease
- COPD exacerbation
- Fever
- Hyperventilation
- Interstitial lung disease
- Neoplasm
- Obesity
- Panic attack
- Pleural effusion
- Polymyositis
- Porphyria
- Pregnancy
- Rib fracture
- Spontaneous pneumothorax
- Thyroid Disease
- URI
Evaluation
Some, all or none of the following findings may be present.
- BNP: Elevated[2]
- ECG findings (similar to acute pulmonary embolism):
- 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
PH patients do not tolerate rapid changes in hemodynamics well, all therapies should be instituted with caution. Do NOT rate control tachycardias, best to electrically cardiovert or attempt rhythm control with amiodarone
Optimize Circulation
- 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 norepinephrine (MAP > 65 mmHg).
- Usually euvolemic or hypervolemic, rarely need IV fluids so diuretics can benefit and treat the RV failure[5]
- Increase cardiac output:
- Once MAP >65 mmHg, start low dose dobutamine (5-10mcg/kg/min)
- Improves inotropic support and theoretically decreases pulmonary vascular resistance
- Reduce RV afterload:
- Avoid hypoxia, maintain O2 sat >90% (increases pulmonary vasoconstriction)
- Avoid hypercapnea (increases pulmonary vascular resistance)
- Avoid acidosis
- 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
- Intubated patients should be optimized to increased O2 delivery and minimize hypercapnea, 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 plumonary 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 modulation
- Bosentan, Ambrisentan
- 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.
Disposition
- Low threshold for admission if acute decompensation
See Also
References
- ↑ Ryan, J. et al. (2012) The WHO classification of pulmonary hypertension: A case-based imaging compendium. Pulmonary Circulation, 2(1).
- ↑ 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
- ↑ 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
- ↑ Critical USG. Echocardiographic assessment of pulmonary artery pressure. 2012. http://www.criticalusg.pl/en/echo/tte/tutorials/echocardiographic-assessment-of-pulmonary-artery-pressures
- ↑ Ternacle, J et al. Diruetics in Normotensive Patients with Acute Pulmonary Embolism and Right Ventricular Dilation. Circulation Journal. Vol 77(10) 2013. 2612-618.