Summary

Acute pulmonary hypertension crisis is a life-threatening emergency where the blood pressure in the lungs suddenly rises dramatically, causing the right side of the heart to fail. Think of your lungs' blood vessels as a network of pipes—when the pressure suddenly spikes, the right ventricle (which pumps blood to the lungs) can't push against this high pressure and starts to fail. This creates a vicious cycle: high lung pressure → right heart failure → low cardiac output → shock → death. This condition affects patients with pre-existing pulmonary hypertension (PH) who experience a trigger (infection, surgery, medication withdrawal, or other stressors). It's rare but catastrophic, with mortality approaching 50-70% if not treated immediately. The key to survival is rapid recognition (severe breathlessness, right heart failure, shock in a PH patient), immediate supportive care (oxygen, inotropes, avoid things that worsen PH), and urgent escalation to PH specialists and advanced therapies (inhaled nitric oxide, prostacyclin, ECMO). Prevention is critical—avoiding triggers and maintaining PH medications can prevent crises.

Key Facts

• Definition: Acute, life-threatening increase in pulmonary artery pressure leading to right heart failure
• Incidence: Rare (affects <1% of PH patients/year), but catastrophic when occurs
• Mortality: 50-70% if untreated; 20-30% with prompt treatment
• Time to treatment: Immediate—minutes count
• Critical feature: Right heart failure + shock in patient with PH
• Key investigation: Clinical diagnosis (do not delay for imaging), echocardiogram (assess RV function)
• First-line treatment: High-flow oxygen, inotropes (dobutamine), avoid things that worsen PH, urgent PH specialist input

Clinical Pearls

"PH patient + sudden deterioration = Crisis until proven otherwise" — Any patient with known pulmonary hypertension who suddenly becomes breathless, hypotensive, or shows signs of right heart failure should be treated as a PH crisis immediately.

"Right heart failure is the problem" — The right ventricle can't pump against the high lung pressure, so it fails. Support the right heart (inotropes) while treating the underlying PH.

"Avoid things that make PH worse" — Hypoxia, acidosis, high PEEP, vasodilators (unless PH-specific), and stopping PH medications can all trigger or worsen a crisis. Be very careful.

"Time is critical" — PH crises can kill within hours. Don't delay—get PH specialist input immediately, consider transfer to PH center.

Why This Matters Clinically

Acute pulmonary hypertension crisis is rare but devastating. It's a true medical emergency where minutes count. Delayed recognition or inappropriate management (like giving standard vasodilators or stopping PH medications) can be fatal. Rapid recognition, immediate supportive care, and urgent escalation to PH specialists can save lives. This condition requires immediate ICU-level care and often advanced therapies that may only be available at specialized PH centers.

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Structured Approach: ABCDE

A - Airway

• Assessment: Usually patent
• Action: Secure if compromised

B - Breathing

• Look: Tachypnoea, use of accessory muscles, cyanosis
• Listen: Usually clear (unless left heart also affected)
• Measure: SpO2 (low), respiratory rate (high)
• Action: High-flow oxygen; avoid high PEEP if ventilated

C - Circulation

• Look: Elevated JVP, peripheral edema, hepatomegaly
• Feel: Pulse (tachycardic, may be weak), BP (low)
• Listen: TR murmur, S3, arrhythmias
• Measure: BP (low), HR (high), ECG
• Action: IV access, inotropes, monitor closely

D - Disability

• Assessment: GCS, mental status
• Finding: May be confused if hypoxic/hypotensive
• Action: Check glucose; consider if hypoperfusion causing confusion

E - Exposure

• Look: Full body examination
• Feel: Temperature (may be elevated if infection)
• Action: Identify trigger if possible

Specific Examination Findings

Right Heart Failure Assessment:

Inspection:

• Elevated JVP (very elevated)
• Peripheral edema
• Hepatomegaly (may be palpable)

Palpation:

• RV heave: Palpable right ventricular enlargement
• Hepatomegaly: Enlarged liver (hepatic congestion)
• Peripheral edema: Ankle/leg swelling

Auscultation:

• Tricuspid regurgitation: Pansystolic murmur, left lower sternal border
• S3: RV dysfunction
• Loud P2: Pulmonary hypertension
• Arrhythmias: May have (AF, VT)

Respiratory Assessment:

• Usually clear: Unless left heart also affected
• Tachypnoea: Respiratory distress

Special Tests

Test	Technique	Positive Finding	Clinical Use
Jugular venous pressure	Patient at 45°, observe JVP	Very elevated	Right heart failure
Hepatojugular reflux	Firm pressure on liver	JVP rises	Confirms right heart failure
ECG	12-lead ECG	Right axis deviation, RVH, strain	Suggests PH

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Natural History (Without Treatment)

Untreated PH Crisis:

• Mortality: 50-70% within hours to days
• Progression: Rapid deterioration → cardiac arrest → death
• Time course: Death often within 24-48 hours if untreated

Outcomes with Treatment

Variable	Outcome	Notes
In-hospital mortality	20-30%	With prompt treatment
30-day mortality	25-35%	Higher if delayed
Long-term survival	50-70% at 1 year	Depends on underlying PH
Recovery	60-70% recover	But may have residual dysfunction

Factors Affecting Outcomes:

Good Prognosis:

• Early recognition (<1 hour)
• Prompt treatment (specialist input, advanced therapies)
• Reversible trigger (infection, medication withdrawal)
• Mild-moderate PH (before crisis)
• Young, otherwise healthy

Poor Prognosis:

• Delayed recognition (>6 hours)
• Delayed treatment (no specialist input)
• Severe underlying PH (advanced disease)
• Cardiac arrest (mortality 70-80%)
• Elderly, comorbidities

Prognostic Factors

Factor	Impact on Prognosis	Evidence Level
Time to treatment	Each hour increases mortality 1.2x	High
Cardiac arrest	5x mortality if occurs	High
Severity of PH	More severe = worse	High
Age	Older age = worse	Moderate
Trigger reversibility	Reversible = better	Moderate

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Key Guidelines

1. ESC/ERS Pulmonary Hypertension Guidelines (2022) — Comprehensive guidelines. European Society of Cardiology

Key Recommendations:

• Immediate recognition and treatment
• PH specialist input
• Supportive care + PH-specific therapies
• Evidence Level: 1A

2. AHA Scientific Statement (2009) — Pulmonary hypertension. American Heart Association

Key Recommendations:

• Rapid assessment and treatment
• Avoid things that worsen PH
• PH specialist input
• Evidence Level: 1A

Landmark Trials

Multiple studies on PH management and crisis treatment.

Evidence Strength

Intervention	Level	Key Evidence	Clinical Recommendation
Supportive care	1A	Guidelines	Primary treatment
Inotropes	1B	Studies	Support RV
PH-specific therapies	1B	Studies	With specialist input
ECMO	2B	Case series	If refractory

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What is Acute Pulmonary Hypertension Crisis?

Acute pulmonary hypertension crisis happens when the blood pressure in your lungs suddenly rises very high, causing the right side of your heart (which pumps blood to your lungs) to fail. Think of your lungs' blood vessels as a network of pipes—when the pressure suddenly spikes, the right ventricle can't push blood through, so it gives up and stops working properly. This causes a dangerous drop in blood pressure and can lead to shock and death if not treated immediately.

In simple terms: The pressure in your lungs suddenly becomes too high, making your right heart fail and causing you to go into shock. This is a medical emergency that needs immediate treatment.

Why does it matter?

Acute pulmonary hypertension crisis is very serious and life-threatening. Without quick treatment, about 5-7 out of 10 people don't survive. Even with the best treatment, about 2-3 out of 10 people don't survive. The good news? With rapid recognition and expert treatment, many people do recover. The key is getting help immediately and being treated at a center with expertise in pulmonary hypertension.

Think of it like this: It's like a critical pipe in your house suddenly blocking completely—everything downstream stops working, and you need emergency plumbers (specialists) to fix it immediately.

How is it treated?

1. Immediate Support: Doctors will act quickly to support your breathing and heart:

• Extra oxygen: To help your lungs and reduce pressure
• Medicines to help your heart: Inotropes to make your right heart pump stronger
• Monitoring: Very close monitoring in intensive care

2. Specialized Treatments: Doctors may use special treatments that are specific for pulmonary hypertension:

• Inhaled nitric oxide: A gas you breathe that helps relax your lung blood vessels
• Special medicines: Medicines that specifically treat high lung pressure
• Advanced support: Sometimes special machines (like ECMO) to support your heart and lungs

3. Treating the Trigger: Doctors will identify and treat whatever caused the crisis:

• If it's an infection: Antibiotics
• If you stopped your PH medicines: Restart them immediately
• If it's something else: Treat that cause

4. Avoiding Things That Make It Worse: Doctors will be very careful to avoid things that can make the lung pressure worse, like low oxygen, certain medicines, or stopping your PH medications.

The goal: Support your heart and lungs immediately, reduce the lung pressure, treat the cause, and help you recover.

What to expect

In the Hospital:

• Intensive Care: You'll be in ICU, very closely monitored 24/7
• First few hours: Most critical period—doctors will support your heart and lungs with medicines and machines
• Days 1-3: If improving, doctors will gradually reduce support
• Days 3-7: If stable, you may move to a regular ward
• Going home: Usually after 1-2 weeks if you're recovering well

After Going Home:

• Medications: You'll need your PH medications every day (very important—never stop them)
• Follow-up: Regular doctor visits with PH specialists
• Lifestyle: Avoid things that can trigger another crisis (infections, stopping medications)
• Recovery: Can take weeks to months to feel back to normal

Recovery Time:

• In hospital: Usually 1-2 weeks
• At home: 2-6 months to feel stronger
• Long-term: Most people can live normal lives with proper PH management

When to seek help

Call 999 (or your emergency number) immediately if:

• You have pulmonary hypertension and suddenly feel very unwell
• You suddenly can't breathe
• You feel very weak or faint
• Your blood pressure drops
• You feel like something is very wrong

See your doctor urgently if:

• You have PH and feel more breathless than usual
• You've stopped your PH medications (even for a short time)
• You have an infection and feel unwell
• You notice swelling in your legs or abdomen

Remember: If you have pulmonary hypertension and suddenly feel very unwell, especially if you're more breathless, feel faint, or have stopped your medications, don't wait—get emergency help immediately. PH crises can be fatal, but with prompt treatment, many people recover.

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Conditions to Consider

Acute pulmonary hypertension crisis must be distinguished from other causes of acute dyspnea and right heart failure:

Condition	Key Distinguishing Features	Investigation	Management Difference
Acute PE	Acute onset, no previous PH, chest pain	CTPA	Anticoagulation, thrombolysis
Acute LV failure	Left-sided signs (crackles, S3), high BNP	Echo (LV dysfunction)	Diuretics, ACE-I, beta-blockers
COPD exacerbation	Wheeze, productive cough, normal JVP	CXR, ABG	Bronchodilators, steroids
Asthma	Wheeze, younger, no JVP elevation	Peak flow, spirometry	Bronchodilators, steroids
Pneumothorax	Sudden onset, hyperresonance	CXR	Chest drain
Cardiac tamponade	Muffled heart sounds, pulsus paradoxus	Echo (pericardial effusion)	Pericardiocentesis
Tension pneumothorax	Hyperresonance, tracheal deviation	Clinical diagnosis	Immediate needle decompression
Acute MI	Chest pain, ECG changes	ECG, troponin	PCI, antiplatelet, anticoagulation

PH Crisis vs. Acute Pulmonary Embolism

Clinical Challenge:

• Both present with acute dyspnea, chest pain, right heart strain, and hypoxia
• Key Difference: PH crisis in patient with known PH; PE usually in previously well patient

Feature	PH Crisis	Acute PE
History	Known PH, on PH medications	No previous PH (usually)
Onset	Hours-days	Sudden (minutes-hours)
Risk factors	PH triggers (infection, meds stopped)	VTE risk factors (surgery, immobility)
JVP	Very high	May be elevated
Heart sounds	Loud P2, TR murmur	May have S1Q3T3
ECG	RVH (chronic), may have RBBB	S1Q3T3, tachycardia, new RBBB
Echo	Severe RV dysfunction, TR, high PA pressure	RV strain, normal/mild TR, normal PA pressure (unless massive PE)
CTPA	No PE (but may show dilated PA, RV)	Filling defect in pulmonary arteries
D-dimer	Normal or mildly elevated	Very high

Key Point: If CTPA negative for PE but RV dysfunction present in known PH patient → PH crisis

PH Crisis vs. Acute Left Heart Failure

Feature	PH Crisis	Acute LV Failure
JVP	Very high	May be elevated
Lung crackles	Absent or minimal	Widespread crackles
S3 gallop	Absent	Present (left-sided)
BNP	Very high	Very high
Echo	RV dysfunction, normal/high LVEF	LV dysfunction, low LVEF
CXR	Clear or oligaemia	Pulmonary edema
Treatment response	No response to diuretics	Improves with diuretics

Key Point: PH crisis = right heart failure with clear lungs; LV failure = left heart failure with pulmonary edema

Differentiating Causes of Right Heart Failure

Acute Right Heart Failure Can Be Caused By:

Cause	Clinical Clue	Investigation	Treatment
PH crisis	Known PH, trigger identified	Echo (severe RV dysfunction, high PA pressure)	PH-specific therapy
Massive PE	Sudden onset, VTE risk factors	CTPA (large PE)	Thrombolysis
RV infarction	Inferior MI, chest pain	ECG (ST elevation II, III, aVF; V4R), troponin	PCI to RCA
Cardiac tamponade	Muffled sounds, pulsus paradoxus	Echo (pericardial effusion, RA/RV collapse)	Pericardiocentesis
Tension pneumothorax	Hyperresonance, tracheal deviation	Clinical, CXR	Needle decompression
ARDS	Hypoxia, bilateral infiltrates	CXR, ABG	Mechanical ventilation

"Can't Miss" Diagnoses

1. Massive Pulmonary Embolism:

• Clue: Sudden onset, VTE risk factors, CTPA shows large PE
• Key: Can look identical to PH crisis
• Investigation: CTPA mandatory if PE suspected
• Management: Thrombolysis if hemodynamically unstable

2. Right Ventricular Infarction:

• Clue: Chest pain, inferior MI on ECG, raised JVP
• Key: ECG shows ST elevation in II, III, aVF; check V4R (RV leads)
• Investigation: ECG, troponin, urgent coronary angiography
• Management: PCI to RCA, avoid nitrates (drop preload), give fluids

3. Cardiac Tamponade:

• Clue: Muffled heart sounds, pulsus paradoxus, no improvement with standard therapy
• Key: Echo shows pericardial effusion with RA/RV collapse
• Investigation: Echo (diagnostic)
• Management: Urgent pericardiocentesis

4. Tension Pneumothorax:

• Clue: Sudden onset, hyperresonance, tracheal deviation, no improvement with oxygen
• Key: Clinical diagnosis, don't wait for CXR
• Investigation: Clinical (confirm with CXR after treatment)
• Management: Immediate needle decompression then chest drain

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Primary Prevention (Preventing PH Development)

Primary prevention focuses on preventing pulmonary hypertension from developing in at-risk populations:

Strategy	Target Population	Evidence Level	Effectiveness
Treat underlying cause	COPD, ILD, sleep apnea	High	Reduces PH development risk
Avoid hypoxia	Chronic lung disease	High	Critical for preventing PH
Screen high-risk	Scleroderma, portal hypertension	Moderate	Early detection allows treatment
Avoid appetite suppressants	General population	High	Known PH trigger
Avoid stimulants	General population	Moderate	Methamphetamine, cocaine linked to PH

High-Risk Groups Requiring Screening:

• Scleroderma: Annual echo (PH develops in 10-15%)
• Portal hypertension: Echo before liver transplant
• Congenital heart disease: Regular cardiology follow-up
• HIV: If symptomatic, consider echo
• Family history of PAH: Genetic counseling, regular screening

Secondary Prevention (Preventing Crises in Established PH)

For patients with diagnosed pulmonary hypertension, preventing crises is critical:

1. Medication Adherence (CRITICAL):

Medication	Why Stopping Triggers Crisis	Prevention Strategy
Prostacyclin analogues	Rebound PH if stopped suddenly	Never stop; backup supply; pump alarms
Phosphodiesterase inhibitors	Loss of pulmonary vasodilation	Daily compliance, set reminders
Endothelin receptor antagonists	Loss of vasodilation	Monthly monitoring, don't skip doses
Riociguat	Rebound vasoconstriction	Daily compliance

Key Point: NEVER stop PH medications abruptly—can trigger fatal crisis

2. Avoid Triggers:

Infection Prevention:

• Annual flu vaccine: Reduces respiratory infection risk
• Pneumococcal vaccine: Prevents pneumonia
• COVID-19 vaccine: Critical for PH patients
• Early treatment of infections: Prompt antibiotics for chest infections
• Avoid sick contacts: Infection can trigger crisis

Surgical/Procedural Management:

• PH specialist input: Always involve before elective surgery
• Regional > general anesthesia: If possible (less cardiac stress)
• Continue PH medications: Through perioperative period
• Experienced anesthetist: PH-trained if possible
• Post-op monitoring: ICU/HDU level

Medication Avoidance:

• NSAIDs: Can worsen fluid retention
• Beta-blockers: Can depress RV function
• Calcium channel blockers: Unless PH-specialist prescribed (vasodilator testing positive)
• Vasodilators: Can drop systemic BP, worsen RV perfusion

3. Regular Monitoring:

Monitoring	Frequency	Purpose	Action if Abnormal
Clinical review	Every 3-6 months	Assess symptoms, WHO class	Escalate therapy if deteriorating
6-minute walk test	Every 6 months	Objective exercise capacity	less than 330m = high risk
BNP/NT-proBNP	Every 6 months	RV stress biomarker	Rising = worsening RV function
Echocardiogram	Annually	RV function, PA pressure	Deteriorating RV = consider escalation
Right heart catheterization	If clinically indicated	Gold standard hemodynamics	Guide therapy escalation

4. Patient Education:

Warning Signs to Report:

• Increased breathlessness (especially at rest)
• New/worsening leg swelling
• Dizziness or syncope
• Chest pain
• Infections (fever, productive cough)
• Medication running out or pump alarm

Lifestyle Measures:

• Oxygen: Use as prescribed (usually nocturnal or with exertion)
• Avoid high altitude: >1500m can worsen PH
• Avoid flying long-haul: If severe PH (WHO class III-IV), may need supplemental oxygen
• Contraception: Pregnancy extremely high-risk in PH (maternal mortality 30-50%)
• Exercise: Gentle (walking), avoid strenuous activity
• Salt restriction: less than 2g/day if fluid overload

Tertiary Prevention (Managing Recurrent Crises)

For patients who have had one PH crisis, preventing recurrence is paramount:

1. Optimize PH Therapy:

Escalation Strategy:

Current Therapy	Next Step if Crisis Occurred	Evidence
Monotherapy	Add second agent (combination therapy)	High
Dual therapy	Add third agent (triple therapy) or escalate to parenteral prostacyclin	High
Triple therapy	Consider lung transplant assessment	Moderate

Combination Therapy Approach:

• ERA + PDE5i: Common first-line combination
• ERA + PDE5i + prostacyclin: Triple therapy for severe PH
• Parenteral prostacyclin: For WHO class IV or recurrent crises

2. Address Underlying Triggers:

If Infection Triggered Crisis:

• Aggressive early treatment: Low threshold for antibiotics
• Prophylactic antibiotics: Consider if recurrent chest infections
• Vaccination: Ensure up to date

If Medication Non-adherence:

• Identify barriers: Cost, side effects, complexity
• Simplify regimen: Combination pills if available
• Support: Medication organizers, reminders, family involvement
• Pump backup: Ensure backup pump and supply for parenteral prostacyclin

If Surgery Triggered Crisis:

• Avoid elective surgery: If possible
• PH specialist involvement: Mandatory for future procedures
• Regional anesthesia: Preferred over general

3. Advanced Therapies:

Atrial Septostomy:

• Indication: Recurrent syncope/crises despite maximal medical therapy
• Mechanism: Create ASD to decompress RV (right-to-left shunt)
• Risk: Can worsen hypoxia
• Bridge to transplant: Usually used as bridge

Lung Transplantation:

• Indication: Refractory PH despite maximal therapy, recurrent crises
• Timing: Refer early (before too unwell for surgery)
• Outcomes: 5-year survival ~50-60%
• Consider if: WHO class IV, recurrent admissions, declining despite therapy

4. Palliative Care:

For patients not suitable for transplant or advanced therapies:

• Symptom management: Oxygen, diuretics, opiates for dyspnea
• Advance care planning: Discuss wishes, DNAR decisions
• Support: Psychological, spiritual, family support
• End-of-life care: Hospice referral when appropriate

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Pregnant Patients with PH (HIGH RISK)

Critical Fact: Pregnancy in pulmonary hypertension carries 30-50% maternal mortality

Specific Considerations:

• Pregnancy contraindicated: In all forms of PH (WHO recommendation)
• Physiological stress: 50% increase in cardiac output during pregnancy
• Right heart cannot cope: RV fails under increased demand
• Delivery: Highest risk peripartum and first 24h postpartum

Contraception (Essential):

Method	Safety in PH	Notes
Barrier methods	Safe	Condoms, but high failure rate
IUD (copper)	Safe	Effective, no hormonal effect
Progestogen-only	Safe	Pill, depot, implant—avoid estrogen
Combined pill	Contraindicated	Estrogen increases VTE risk
Sterilization	Consider	Permanent, but requires surgery

ERA Warning: Endothelin receptor antagonists (bosentan, ambrisentan) are teratogenic—effective contraception mandatory

If Pregnancy Occurs Despite Counseling:

Management Approach:

Trimester	Management	Risks
First	Discuss termination (safest option)	Maternal mortality 30-50% if continue
Second	If continuing: PH specialist team, fetal medicine, ICU planning	Escalating RV strain
Third	Plan early elective delivery (32-34 weeks), regional anesthesia, ICU postpartum	Highest risk peripartum

Delivery Plan (If Pregnancy Continues):

• Timing: Elective at 32-34 weeks (balance fetal maturity vs. maternal risk)
• Mode: Vaginal preferred (less stress than C-section), assisted second stage
• Anesthesia: Regional (epidural), avoid general anesthesia
• Monitoring: Invasive arterial line, central line, continuous echo
• Location: Delivery suite with ICU backup
• Postpartum: ICU care for 72h (highest risk period)

Medication Adjustments:

• Continue PH medications: Except ERA (stop, use alternatives)
• Alternatives to ERA: Sildenafil, inhaled prostacyclin (safer in pregnancy)
• Anticoagulation: LMWH (not warfarin—teratogenic)

Elderly Patients (>75 years)

Specific Considerations:

• Higher mortality: Age itself is poor prognostic factor
• More comorbidities: IHD, CKD, COPD complicate management
• Polypharmacy: Drug interactions common
• Frailty: May not tolerate aggressive interventions

Management Adjustments:

Issue	Standard Approach	Adjustment for Elderly	Rationale
PH therapy	Triple therapy if severe	May start with mono/dual therapy	Tolerability concerns
Inotropes	Dobutamine first-line	Lower doses, monitor for arrhythmias	Higher arrhythmia risk
Mechanical support	ECMO if young, reversible	Higher threshold for ECMO	Poor outcomes if very frail
Transplant	List if age less than 65	Usually not suitable	Age limit for lung transplant

Common Comorbidities:

• IHD: Can worsen RV ischemia in crisis
• CKD: Adjust drug doses, fluid balance tricky
• COPD: May have PH due to hypoxia (group 3 PH)

Goals of Care:

• Quality of life: May prioritize comfort over aggressive intervention
• Advance care planning: Discuss wishes early
• DNAR: Consider if very frail or multiple comorbidities

Patients with Group 2 PH (Left Heart Disease)

Specific Considerations:

• Most common PH: 65-80% of PH is due to left heart disease (HFpEF, HFrEF, valvular)
• Different pathophysiology: PH is secondary to high left-sided pressures
• PH-specific medications: Not recommended (can worsen pulmonary edema)

Approach to Crisis in Group 2 PH:

1. Treat the Left Heart:

• Diuretics: Reduce left-sided pressures → reduces PA pressure
• HFrEF: Optimize GDMT (ACE-I, beta-blocker, MRA, SGLT2i)
• HFpEF: Diuretics, SGLT2i, treat comorbidities
• Valvular disease: Surgical correction if severe (TAVI, mitral repair)

2. Avoid PH-Specific Medications (Usually):

• Prostacyclin: Can cause pulmonary edema
• Endothelin antagonists: Fluid retention
• Phosphodiesterase inhibitors: Can worsen LV failure
• Exception: Selected patients with out-of-proportion PH may benefit (specialist decision)

3. Management of Acute Decompensation:

• Diuretics: IV furosemide
• Vasodilators: GTN, nitroprusside (reduce afterload)
• Inotropes: Dobutamine if low cardiac output
• Avoid fluid overload: Careful fluid balance

Patients with Group 3 PH (Lung Disease)

Specific Considerations:

• Cause: COPD, ILD, OSA causing hypoxic vasoconstriction
• Severity: Usually mild-moderate PH
• PH-specific medications: Not recommended (treat underlying lung disease)

Approach:

1. Optimize Lung Disease Treatment:

• COPD: Bronchodilators, steroids if exacerbation, pulmonary rehab
• ILD: Antifibrotics (nintedanib, pirfenidone), immunosuppression if inflammatory
• OSA: CPAP therapy (improves PH dramatically)

2. Correct Hypoxia:

• Long-term oxygen therapy: If PaO2 less than 7.3 kPa or less than 8 kPa with cor pulmonale
• Target: SpO2 88-92% (avoid hyperoxia in COPD)
• Duration: >15 hours/day for survival benefit

3. Avoid PH-Specific Medications:

• No benefit: Studies show no improvement in group 3 PH
• May worsen VQ matching: Can worsen hypoxia

4. Consider Lung Transplant:

• If severe PH + end-stage lung disease: Combined lung and heart transplant may be considered

Patients on Chronic Anticoagulation

Specific Considerations:

• PH patients often anticoagulated: Especially if PAH, low cardiac output
• Bleeding risk: Hemoptysis can occur in PH
• Balance: Thrombosis risk vs. bleeding risk

Indications for Anticoagulation in PH:

• IPAH/HPAH: Recommended (reduces in-situ thrombosis)
• CTEPH: Mandatory (lifelong)
• Other PH: Case-by-case (if low cardiac output, AF, VTE)

Management in Crisis:

Scenario	Anticoagulation Management	Rationale
No bleeding	Continue anticoagulation	Thrombosis risk high
Minor bleeding (hemoptysis)	Consider holding temporarily, restart when stable	Balance risk
Major bleeding	Reverse anticoagulation, restart cautiously	Bleeding takes priority
Preparing for procedure	Bridge with heparin (if CTEPH or mechanical valve)	Maintain anticoagulation

Reversal Agents:

• Warfarin: Vitamin K + PCC
• DOACs: Idarucizumab (dabigatran), andexanet alfa (Xa inhibitors)

Patients Post-Lung Transplant

Specific Considerations:

• Cure for PH: Lung transplant removes PH
• RV recovery: Can take weeks-months for RV to recover
• Early post-transplant: Risk of primary graft dysfunction (PGD)

Early Post-Transplant (First 72h):

• PGD: Acute lung injury post-transplant (graded 0-3)
• Presents like ARDS: Hypoxia, infiltrates on CXR
• Management: Supportive (ventilation, ECMO if severe)
• Not a PH crisis: But can cause acute RV failure

Late Post-Transplant (Months-Years):

• Chronic rejection (CLAD): Can cause recurrent PH
• Surveillance: Regular spirometry, imaging
• Treatment: Adjust immunosuppression, may need re-transplant

Immunosuppression Considerations:

• Drug interactions: Prostacyclins, endothelin antagonists interact with calcineurin inhibitors
• Infection risk: Higher risk, vigilant for infections

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Last Reviewed: 2025-12-24 | MedVellum Editorial Team

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Medical Disclaimer: MedVellum content is for educational purposes and clinical reference. Clinical decisions should account for individual patient circumstances. Always consult appropriate specialists. This information is not a substitute for professional medical advice, diagnosis, or treatment.