Pulmonary hypertension: Difference between revisions
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{{Adult top}} [[pulmonary hypertension (peds)]].'' | |||
==Background== | ==Background== | ||
*Mean PA pressure >25 mmHg as assessed by right heart catheterization | *Mean PA pressure >25 mmHg as assessed by right heart catheterization | ||
Line 7: | Line 8: | ||
**Idiopathic | **Idiopathic | ||
**Associated with: | **Associated with: | ||
***Drugs or toxins | ***Drugs or [[toxins]] | ||
***Connective tissue | ***[[Connective tissue disorder]]s | ||
***HIV | ***[[HIV]] | ||
***Portal hypertension | ***Portal hypertension | ||
***Congenital heart disease | ***[[Congenital heart disease]] | ||
*'''Group 2''': PH due to left heart disease | *'''Group 2''': PH due to left heart disease | ||
**Associated with: | **Associated with: | ||
***Coronary artery disease | ***Coronary artery disease | ||
***Hypertension | ***[[Hypertension]] | ||
***Valvular disease | ***[[Valvular disease]] | ||
***Advanced age | ***Advanced age | ||
*'''Group 3''': PH due to lung diseases | *'''Group 3''': PH due to lung diseases | ||
Line 23: | Line 24: | ||
***[[Interstitial lung disease]] | ***[[Interstitial lung disease]] | ||
***Any other lung disease causing [[hypoxemia]] | ***Any other lung disease causing [[hypoxemia]] | ||
*'''Group 4''': PH due to chronic thromboembolic disease | *'''Group 4''': PH due to chronic [[thromboembolism|thromboembolic disease]] | ||
*'''Group 5''': PH of unclear multifactorial mechanisms | *'''Group 5''': PH of unclear multifactorial mechanisms | ||
**Associated with: | **Associated with: | ||
***Sarcoidosis | ***[[Sarcoidosis]] | ||
***Sickle cell anemia | ***[[Sickle cell anemia]] | ||
***Chronic hemolytic anemia | ***Chronic [[hemolytic anemia]] | ||
***Splenectomy | ***[[Splenectomy]] | ||
***Metabolic disease | ***Metabolic disease | ||
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*Heritable | *Heritable | ||
*Idiopathic | *Idiopathic | ||
*Chronic hypoxia | *Chronic [[hypoxia]] | ||
*Chronic thromboembolic disease | *Chronic [[thromboembolism|thromboembolic disease]] | ||
*Vasculitis | *[[Vasculitis]] | ||
*Autoimmune disease | *Autoimmune disease | ||
*Toxic exposures | *Toxic exposures | ||
*Chronic [[renal failure]] on [[dialysis complications|dialysis]] | |||
*Chronic renal failure on dialysis | *[[Myeloproliferative disorders]] | ||
*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=== | ||
*JVD | *JVD | ||
*Hepatomegaly | *[[Hepatomegaly]] | ||
*Ascites | *[[Ascites]] | ||
*Edema | *Edema | ||
*Stigmata of liver failure | *Stigmata of [[liver failure]] | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
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*Pulmonary artery diameter greater than 30 mm suggest PH | *Pulmonary artery diameter greater than 30 mm suggest PH | ||
*Right heart enlargement | *Right heart enlargement | ||
===Echocardiographic Findings=== | ===[[Echocardiography|Echocardiographic]] Findings=== | ||
*D sign (McConnel's Sign) | *D sign (McConnel's Sign) | ||
*RV close to LV size (+/- septal flattening/bowing) | *RV close to LV size (+/- septal flattening/bowing) | ||
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==Acute Management== | ==Acute Management== | ||
''PH patients do not tolerate rapid changes in hemodynamics | |||
===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 Circulation=== | ||
#'''Optimize (usually reduce) RV preload''': | #'''Optimize (usually reduce) RV preload''': | ||
#*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> | #*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 | ||
#*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). | #*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). | ||
#'''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 | #*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 | ||
Line 131: | Line 142: | ||
===Optimize Oxygenation=== | ===Optimize Oxygenation=== | ||
*'''Intubated patients''' should be optimized to increased O2 delivery and minimize | *'''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 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 catheter | **Pulmonary arterial catheter | ||
**Inhaled | **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== | ||
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*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 | '''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, | *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== |
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]
- 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
- 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
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:
- 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).
- 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 hypercapnia (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
- 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
- ↑ 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.