Pulmonary hypertension (peds) - Revision history

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	===WHO Classification<ref>Ryan, J. et al. (2012) The WHO classification of pulmonary hypertension: A case-based imaging compendium. Pulmonary Circulation, 2(1).</ref>===		===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~~'''~~: Pulmonary ''arterial'' hypertension		*Group 1: Pulmonary ''arterial'' hypertension
	**Idiopathic		**Idiopathic
	**Associated with:		**Associated with:
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	***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
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	***[[Valvular disease]]		***[[Valvular disease]]
	***Advanced age		***Advanced age
	*~~'''~~Group 3~~'''~~: PH due to lung diseases		*Group 3: PH due to lung diseases
	**Associated with:		**Associated with:
	***[[COPD]]		***[[COPD]]
	***[[Interstitial lung disease]]		***[[Interstitial lung disease]]
	***Any other lung disease causing [[hypoxemia]]		***Any other lung disease causing [[hypoxemia]]
	*~~'''~~Group 4~~'''~~: PH due to chronic [[thromboembolism\|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]]
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	==Evaluation==		==Evaluation==
	''Some, all or none of the following findings may be present.''		''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 on bedside ultrasound or CT		**Evidence of right heart strain on bedside ultrasound or CT
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	*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 patients, pulmonic stenosis		**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		**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
	{\|class="wikitable"		{\|class="wikitable"
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	===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 [[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 [[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 [[hypercapnia]] (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
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	===Optimize Oxygenation===		===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.		*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.		*'''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		*'''Intubated patients''' should be optimized to increased O2 delivery and minimize hypercapnia, maintain low tidal volumes and low PEEP as tolerated
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	==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		Mechanism of Action: vasodilation via vascular modulation
	*[[Bosentan]], Ambrisentan		*[[Bosentan]], Ambrisentan
	**Complications include liver failure, supratherapeutic INR,		**Complications include liver failure, supratherapeutic INR,

Ostermayer at 17:59, 17 January 2026

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	{{~~Peds top~~}} ~~[[pulmonary hypertension]].''~~		{{PediatricPage\|pulmonary hypertension}}
	==Background==		==Background==
	*Mean PA pressure >25 mmHg as assessed by right heart catheterization		*Mean PA pressure >25 mmHg as assessed by right heart catheterization

Rossdonaldson1: /* Differential Diagnosis */

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Differential Diagnosis

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	==Differential Diagnosis==		==Differential Diagnosis==
	{{SOB DDX}}		{{Pediatric SOB DDX}}
	{{Newborn DDX}}		{{Newborn DDX}}

Rossdonaldson1: /* Differential Diagnosis */

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Differential Diagnosis

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	==Differential Diagnosis==		==Differential Diagnosis==
	{{SOB DDX}}		{{SOB DDX}}
			{{Newborn DDX}}

	==Evaluation==		==Evaluation==

Rossdonaldson1 at 16:05, 1 July 2020

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	{{Peds top}} [[~~pulmonayr~~ hypertension]].''		{{Peds top}} [[pulmonary hypertension]].''
	==Background==		==Background==
	*Mean PA pressure >25 mmHg as assessed by right heart catheterization		*Mean PA pressure >25 mmHg as assessed by right heart catheterization

Rossdonaldson1 at 16:04, 1 July 2020

2020-07-01T16:04:51Z

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			{{Peds top}} [[pulmonayr hypertension]].''
	==Background==		==Background==
	*Mean PA pressure >25 mmHg as assessed by right heart catheterization		*Mean PA pressure >25 mmHg as assessed by right heart catheterization

Rossdonaldson1: Created page with "==Background== *Mean PA pressure >25 mmHg as assessed by right heart catheterization **Since right ventricle is dependent on preload, RV contractility and afterload, severe p..."

2020-07-01T16:04:00Z

Created page with "==Background== *Mean PA pressure >25 mmHg as assessed by right heart catheterization **Since right ventricle is dependent on preload, RV contractility and afterload, severe p..."

New page

==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''': Pulmonary ''arterial'' hypertension
**Idiopathic
**Associated with:
***Drugs or [[toxins]]
***[[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===
*Heritable
*Idiopathic
*Chronic [[hypoxia]]
*Chronic [[thromboembolism|thromboembolic disease]]
*[[Vasculitis]]
*Autoimmune disease
*Toxic exposures
*Chronic [[renal failure]] on [[dialysis complications|dialysis]]
*[[Myeloproliferative disorders]]

==Clinical Features==
===History===
*Exertional [[dyspnea]] (most common symptom)<ref name="Wilcox"></ref>
*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==
{{SOB DDX}}

==Evaluation==
''Some, all or none of the following findings may be present.''
*'''[[BNP]]:''' Elevated<ref name="Wilcox"></ref>
*'''[[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)<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
===[[Echocardiography|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"
| align="center" style="background:*f0f0f0;"|
| align="center" style="background:*f0f0f0;"|'''Normal'''
| align="center" style="background:*f0f0f0;"|'''Intermediate'''
| align="center" style="background:*f0f0f0;"|'''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<ref name="Wilcox"></ref>

==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<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 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).
#'''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<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:
**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==
*[[Shortness of breath]]

==References==
<references/>

[[Category:Cardiology]]
[[Category:Pulmonary]]
[[Category:Pediatrics]]