Topic map

1. Basic physiology. The normal pulmonary circulation: low pressure, high compliance, PVR, West zones, hypoxic pulmonary vasoconstriction (HPV), and RV-PA coupling.
2. Definitions and classification. PH is now defined as mean PAP above 20 mmHg. Pre-capillary, post-capillary and combined disease are separated by PVR and wedge pressure. WHO Groups 1 to 5.
3. Pathology and molecular biology. Endothelin, NO/cGMP and prostacyclin pathways, remodeling, plexiform lesions, and how the RV fails.
4. Left heart and valve disease PH (Group 2). This covers mitral stenosis, aortic stenosis and TR. It includes how PH develops and regresses after surgery, and why it sometimes doesn't.
5. Pediatric PAH. PPHN, congenital heart disease (shunts, Eisenmenger), BPD, pulmonary vein stenosis, and pulmonary hypertensive crises.
6. Diagnosis. Echo, right heart catheterization, vasoreactivity testing, and risk stratification.
7. Chronic therapy. Calcium channel blockers, ERAs, PDE5 inhibitors, riociguat, prostanoids, selexipag, sotatercept, and when each applies.
8. Anesthesia for adult PAH. Induction, ventilation, drugs to use and avoid, inhaled NO and epoprostenol, vasopressin, inotropes, and RV protection.
9. Anesthesia and ICU care for pediatric PAH. Crisis prevention and treatment, sedation depth, ventilation, and ECMO.
10. Post-cardiac-surgery PH and RV failure. Monitoring, weaning, and troubleshooting.
11. Your patient, in detail.

Brief overview

• Pre-capillary PH (Group 1, 3, 4) has a high PVR and a normal wedge pressure. The disease is in the pulmonary arterioles.
• Post-capillary PH (Group 2, left heart and valve disease) has a high wedge or left atrial pressure. It begins as passive back-pressure. Over time reactive vasoconstriction and remodeling add a pre-capillary component, called combined pre- and post-capillary PH.

Your patient

• Severe MS raises left atrial pressure. Severe AS adds LV pressure overload, diastolic dysfunction and further LA hypertension. Chronic back-pressure then caused remodeling and a pulmonary pressure of 120 mmHg.
• Severe TR means the RV was dilated and failing, with a volume-loaded RA.
• After surgery, PASP is still 70 to 80 mmHg. Oxygen requirement and congested lungs point to persistent left-sided pressure elevation until proven otherwise. The remaining pulmonary vascular disease may also be fixed.

1. Residual or new mitral obstruction, such as a stenotic repair or prosthesis-patient mismatch.
2. Poor LV diastolic compliance after long-standing AS (a stiff, hypertrophied LV).
3. LV systolic dysfunction after surgery.
4. Residual MR or AR.
5. Fixed pre-capillary disease from long-standing exposure.
6. RV failure with persistent TR.
7. Fluid overload, atrial fibrillation or loss of atrial kick, pulmonary venous obstruction, or lung causes such as atelectasis, effusion or ARDS.

• Treat the cause first: look for residual obstruction, optimize rhythm, and manage volume with diuretics.
• Support the RV and LV: inotropes, and avoid hypoxia and hypercarbia.
• Use inhaled pulmonary vasodilators selectively. Pulmonary vasodilators can worsen pulmonary edema in left-sided disease, because they increase flow into an obstructed or stiff left heart. Caution is essential.

What I need from you

Topic map

1. Basic physiology. The normal pulmonary circulation: low pressure, high compliance, PVR, West zones, hypoxic pulmonary vasoconstriction (HPV), and RV-PA coupling.
2. Definitions and classification. PH is now defined as mean PAP above 20 mmHg. Pre-capillary, post-capillary and combined disease are separated by PVR and wedge pressure. WHO Groups 1 to 5.
3. Pathology and molecular biology. Endothelin, NO/cGMP and prostacyclin pathways, remodeling, plexiform lesions, and how the RV fails.
4. Left heart and valve disease PH (Group 2). This covers mitral stenosis, aortic stenosis and TR. It includes how PH develops and regresses after surgery, and why it sometimes doesn't.
5. Pediatric PAH. PPHN, congenital heart disease (shunts, Eisenmenger), BPD, pulmonary vein stenosis, and pulmonary hypertensive crises.
6. Diagnosis. Echo, right heart catheterization, vasoreactivity testing, and risk stratification.
7. Chronic therapy. Calcium channel blockers, ERAs, PDE5 inhibitors, riociguat, prostanoids, selexipag, sotatercept, and when each applies.
8. Anesthesia for adult PAH. Induction, ventilation, drugs to use and avoid, inhaled NO and epoprostenol, vasopressin, inotropes, and RV protection.
9. Anesthesia and ICU care for pediatric PAH. Crisis prevention and treatment, sedation depth, ventilation, and ECMO.
10. Post-cardiac-surgery PH and RV failure. Monitoring, weaning, and troubleshooting.
11. Your patient, in detail.

Brief overview

• Pre-capillary PH (Group 1, 3, 4) has a high PVR and a normal wedge pressure. The disease is in the pulmonary arterioles.
• Post-capillary PH (Group 2, left heart and valve disease) has a high wedge or left atrial pressure. It begins as passive back-pressure. Over time reactive vasoconstriction and remodeling add a pre-capillary component, called combined pre- and post-capillary PH.

Your patient

• Severe MS raises left atrial pressure. Severe AS adds LV pressure overload, diastolic dysfunction and further LA hypertension. Chronic back-pressure then caused remodeling and a pulmonary pressure of 120 mmHg.
• Severe TR means the RV was dilated and failing, with a volume-loaded RA.
• After surgery, PASP is still 70 to 80 mmHg. Oxygen requirement and congested lungs point to persistent left-sided pressure elevation until proven otherwise. The remaining pulmonary vascular disease may also be fixed.

1. Residual or new mitral obstruction, such as a stenotic repair or prosthesis-patient mismatch.
2. Poor LV diastolic compliance after long-standing AS (a stiff, hypertrophied LV).
3. LV systolic dysfunction after surgery.
4. Residual MR or AR.
5. Fixed pre-capillary disease from long-standing exposure.
6. RV failure with persistent TR.
7. Fluid overload, atrial fibrillation or loss of atrial kick, pulmonary venous obstruction, or lung causes such as atelectasis, effusion or ARDS.

• Treat the cause first: look for residual obstruction, optimize rhythm, and manage volume with diuretics.
• Support the RV and LV: inotropes, and avoid hypoxia and hypercarbia.
• Use inhaled pulmonary vasodilators selectively. Pulmonary vasodilators can worsen pulmonary edema in left-sided disease, because they increase flow into an obstructed or stiff left heart. Caution is essential.

What I need from you

Pulmonary Hypertension: Basics to Advanced, for an Anesthesia Resident, and Your Patient

1. Basic physiology of the pulmonary circulation

• Mean PAP is about 14 mmHg (normal upper limit about 20). PA pressure is roughly 25/10, and PAWP is 6 to 12.
• PVR = (mPAP - PAWP) / CO. Normal is under 2 Wood units (WU), about 160 dyn.s/cm⁻⁵.
• The lung takes the entire cardiac output at about one tenth of systemic pressure. This works because the vessels are thin, compliant and recruitable.

• It is thin-walled and crescent-shaped and does about a quarter of the LV's work. It tolerates volume well and tolerates pressure poorly.
• An RV that has never faced high afterload, such as after an acute PE, can generate only about 40 to 50 mmHg mean PAP. A chronically hypertrophied RV can reach systemic pressures but has little reserve.
• RV perfusion. The LV is perfused only in diastole. The RV is perfused in systole and diastole, but with PH the transmural pressure rises and systolic RCA flow falls. Hypotension therefore collapses RV perfusion. Miller makes this point directly: avoiding hypotension is key (Miller's Anesthesia 10e, p. 7089).
• Ventricular interdependence. A dilated RV shifts the septum into the LV ("D-shaped LV"). Reduced LV preload and filling follow, and cardiac output falls.
• The vicious circle: High PVR → RV dilation and ↑ wall stress → TR, ↓ RV coronary perfusion → ↓ RV output → ↓ LV preload → ↓ systemic BP → worse RV ischemia → further RV failure.
• Pressure versus flow. In pediatrics, a pulmonary flow increase from a shunt raises PA pressure with a normal PVR initially. Always ask whether the pressure is high because of flow, resistance or downstream pressure.

2. Definitions and classification

1. PAH. Idiopathic, heritable (BMPR2), drug or toxin (fenfluramine, methamphetamine), connective tissue disease (scleroderma), HIV, portal hypertension, congenital heart disease, schistosomiasis. Subgroups include PVOD/PCH and PPHN.
2. Left heart disease. HFrEF, HFpEF, valvular disease (MS, MR, AS, AR), congenital inflow or outflow obstruction. This is the most common cause worldwide.
3. Lung disease and hypoxia. COPD, ILD, OSA, high altitude, developmental lung disease (BPD, CDH).
4. Pulmonary artery obstruction. CTEPH.
5. Unclear or multifactorial. Haematological disease (sickle cell), sarcoid, renal failure.

3. Pathology and molecular biology

• Medial hypertrophy → intimal proliferation → concentric laminar intimal fibrosis ("onion-skin") → plexiform lesions → fibrinoid necrosis (Heath-Edwards grades I to VI).
• Dysfunctional endothelium causes an imbalance:
  • ↓ NO (cGMP pathway) and ↓ prostacyclin (cAMP pathway).
  • ↑ endothelin-1 and ↑ thromboxane A2.
• Others include serotonin, inflammation, in situ thrombosis, BMPR2 loss-of-function (the TGF-β/BMP imbalance, now targeted by sotatercept), K⁺ channel dysfunction and metabolic reprogramming.
• These pathways are the targets for the drugs in section 7.

1. Passive phase. A raised LAP is transmitted backward. Capillary pressure rises, and a PAWP above about 25 causes pulmonary edema.
2. Adaptive phase. The alveolar-capillary membrane thickens. This protects against edema (less breathlessness at rest) but impairs diffusion. Pulmonary veins and arterioles remodel, and endothelin-1 rises (Schwartz's cites endothelin-1 as a predictor of PH regression after mitral surgery).
3. Reactive phase. Active arteriolar vasoconstriction (a "second stenosis") further raises PVR and protects against edema at the expense of the RV. This is CpcPH.
4. Fixed phase. Established arterial and venous structural change. This may only partly regress after the valve is fixed.

4. Valve disease and PH (the key concept for your ICU)

1. Residual or new mitral obstruction (stenotic repair, prosthesis-patient mismatch, pannus, thrombosis).
2. Stiff, hypertrophied, underfilled LV (diastolic dysfunction). Acute LV failure may appear after MV relief ("sudden loading of a small LV").
3. Residual MR or AR, or paravalvular leak.
4. A fixed pre-capillary component (CpcPH).
5. RV failure and persistent TR.
6. Loss of atrial kick (AF) or tachycardia.
7. Lung causes, such as CPB lung injury, protamine, transfusion, atelectasis, effusion, phrenic palsy or pneumonia.
8. Fluid overload.

5. Pediatric PH

• Reactive pulmonary vasculature, especially under 1 year.
• The ratio of lung blood flow to body size is high, and children need indexed values: PVRi, with normal about 1 to 3 WU·m².
• Closing volume lies above FRC in infants, so atelectasis drives HPV.
• Crises develop faster, and the myocardium is less reserved.

• PPHN. Meconium aspiration, sepsis, RDS, CDH, idiopathic. Treatment is oxygen, gentle ventilation, surfactant, iNO (20 ppm, the only FDA-approved indication, per Goodman & Gilman), sildenafil, milrinone, ECMO.
• Congenital heart disease. Large left-to-right shunts (VSD, AVSD, PDA, truncus, TAPVR) cause high flow, then PVD, then Eisenmenger syndrome (shunt reversal, cyanosis, erythrocytosis). Children with Down syndrome are at high risk of early PVD. Repair is considered if PVRi is low (roughly under 4 WU·m²). Higher values need individual assessment, and very high values (about 8 or more) usually mean repair is not advised.
• Lung disease. BPD, CDH, interstitial lung disease.
• Left heart. Obstructed TAPVR, pulmonary vein stenosis, cor triatriatum, congenital MS.
• Idiopathic and heritable PAH. Treated as in adults, with specialist-directed dosing.

• Triggers: hypoxia, hypercarbia, acidosis, hypothermia, pain, airway manipulation, suctioning, agitation.
• Picture: sudden rise in PAP toward or above systemic pressure, falling SpO2 and EtCO2, hypotension, bradycardia, then RV ischemia and low output. Suprasystemic PAP was the strongest predictor of complications, not the anesthetic type or airway (Miller's Anesthesia 10e, p. 10814).
• Response:
  1. 100% O2 with manual ventilation, and treat hypoxia and hypercarbia aggressively.
  2. Hyperventilate to PaCO2 30 to 35 and pH above 7.5. Miller notes that PaCO2 about 20 with pH 7.6 reliably lowers PVR in infants (p. 10815).
  3. Deepen sedation and use neuromuscular blockade.
  4. Start or increase iNO (20 to 40 ppm).
  5. Give inotropes: epinephrine, milrinone. Add vasopressin to support systemic pressure.
  6. If there is arrest or refractory low output, early CPR and ECMO (right VAD in selected patients).
• Ventilation in the child: large tidal volumes and slow rates (15 to 25 breaths/min in neonates) keep lungs at FRC. Use a long expiratory phase and low PEEP (3 to 5). Higher PEEP compresses alveolar capillaries and raises PVR (Miller, p. 10816). Positive-pressure inspiration also helps the RV by raising the lung-to-LA gradient, though mean airway pressure must be balanced.
• Drugs: milrinone reduces PVR and SVR and boosts RV contractility. Sildenafil acts with NO. Bosentan helps with liver-enzyme monitoring. IV prostacyclin and PGE1 cause systemic hypotension. Low-dose NO (1 to 5 ppm) is used routinely after Fontan in some centers.
• Eisenmenger syndrome: avoid dehydration and use IV air filters. Braunwald recommends filters for repaired or unrepaired shunt lesions around labor, and the same logic applies to all IV lines.

6. Diagnosis and risk assessment

• History and exam: exertional dyspnea, syncope, chest pain, hepatomegaly, edema. Look for a loud P2, RV heave, TR murmur, raised JVP.
• ECG: right axis, RV hypertrophy, P pulmonale. CXR: enlarged PAs, "pruned" vessels.
• Echo:
  • TR velocity above 2.8 m/s suggests PH.
  • Septal flattening, RV/LV ratio above 1, TAPSE low, PA acceleration time short, RA area large, pericardial effusion.
  • TAPSE/PASP is a prognostic index (above 0.32 low risk, below 0.19 high risk in the ESC risk table).
• RHC (the gold standard): mPAP, PAWP, CO, PVR, SvO2, RAP.
  • Vasoreactivity testing (iNO) is done only for idiopathic, heritable or drug-induced PAH. A positive test is a fall in mPAP of at least 10 to a value of 40 or below, with unchanged or increased CO.
  • Pitfalls: in MS, PAWP overestimates LVEDP. In severe TR, thermodilution CO is unreliable. Large V waves mislead.
  • Pediatrics: cath carries real anesthetic risk, so plan carefully.
• V/Q scan to exclude CTEPH. Biomarkers: BNP/NT-proBNP. Function: 6MWD, WHO functional class.
• Risk stratification (ESC/ERS): low, intermediate and high strata, using WHO class, 6MWD, NT-proBNP, echo, CI, RAP and SvO2. This guides initial therapy and follow-up.

7. Chronic therapy

• General: diuretics, O2 if SaO2 is below 90%, exercise training, vaccinations, pregnancy avoidance, iron if deficient.
• Vasoreactive responders (about 5 to 10%): high-dose calcium channel blockers (nifedipine, diltiazem, amlodipine).
• Everyone else: initial combination therapy stratified by risk.
  • Low or intermediate risk: ERA + PDE5 inhibitor (for example ambrisentan + tadalafil, or macitentan + sildenafil).
  • High risk: triple therapy including IV or SC prostanoid (epoprostenol or treprostinil).

• Refractory disease: atrial septostomy, Potts shunt, lung transplant.
• Anticoagulation: not routine in PAH. Indicated in CTEPH.

8. Anesthesia for the adult with PH

1. Maintain systemic pressure above pulmonary pressure (RV coronary perfusion).
2. Avoid PVR triggers: hypoxia, hypercarbia, acidosis, pain, cold, light anesthesia.
3. Maintain sinus rhythm, a heart rate of about 70 to 100, and RV contractility.
4. Keep preload optimal. Neither dry nor overloaded.
5. Continue all PAH drugs perioperatively without interruption.

• Risk stratify: syncope, resting dyspnea, RV failure, high BNP, suprasystemic PAP, low CI. Elective surgery in decompensated patients should be deferred and optimised at a PH centre.
• Echo and ideally recent haemodynamics.
• Continue pumps with spare cassettes. Minimise fasting and avoid dehydration. Use minimal premedication and monitor SpO2.

• Arterial line before induction. CVC. TEE for RV function and filling.
• PA catheter is reserved for cases where data will change management. Rising PAP is almost always bad. Falling PAP can reflect vasodilation or impending RV failure, so interpret PAP together with CO or SvO2 (Miller, p. 7090).

• Ketamine (titrated) or etomidate, with an opioid. Avoid propofol bolus and large doses of volatile agents. Titrated sevoflurane is acceptable.
• Avoid nitrous oxide. Preoxygenate. Blunt laryngoscopy response. Use cautious positive-pressure ventilation.
• Keep tidal volumes about 6 to 8 mL/kg, low-moderate PEEP, FiO2 high, mild hypocapnia, no acidosis.

• Pressors: norepinephrine, phenylephrine, vasopressin (raises systemic pressure without raising PAP, per Miller).
• Inotropes: dobutamine, milrinone, epinephrine. Inodilators help left heart PH. They can worsen hypotension, and in PH from lung disease they can worsen V/Q mismatch.
• Inhaled pulmonary vasodilators are preferred to IV agents: NO 10 to 40 ppm or inhaled epoprostenol (about 50 ng/kg/min; Barash gives 0.01 to 0.05 µg/kg/min). IV vasodilators drop SVR and cause hypotension.
• Neuraxial and regional: lumbar epidural has been used in obstetric PH. Thoracic epidural removes cardiac sympathetic drive and abolishes the RV's contractility response to rising afterload, so use it with inotrope/vasopressor backup (Miller, p. 7090). Single-shot spinal can drop SVR abruptly.

• Ventilate and recruit before separation. Start iNO or inhaled epoprostenol early.
• Inotropes (epinephrine, milrinone) plus vasopressin. Atrial or AV sequential pacing.
• Give protamine slowly. A catastrophic pulmonary vasoconstrictive reaction is possible.
• If inadequate output persists, mechanical support (Barash 9e, "Discontinuation of Cardiopulmonary Bypass"). Delayed sternal closure can decompress a swollen RV.

9. Pediatric anesthesia and ICU care

• Preop: assess PVRi, shunt direction, PAP:SAP ratio, RV function and current PAH drugs. Suprasystemic PAP is the red flag.
• Induction: IV ketamine, titrated, is often safest. If there is no IV, inhalational induction with sevoflurane at the lowest effective end-tidal, on 100% O2, with IV access obtained quickly (Miller, p. 10814 to 10815).
• Maintenance: adequate depth to blunt stress responses, opioid-based, normothermia, preserved volume, arterial line, and iNO and milrinone available.
• Ventilation: see section 5. Prefer FRC-preserving tidal volumes and low PEEP.
• Post-CPB in children: keep sedated and paralyzed in the first 24 to 48 hours in high-risk cases. Give extra sedation bolus and pre-oxygenation before suctioning. Wean iNO slowly (for example 20 → 10 → 5 → 1 to 2 ppm) and bridge with sildenafil to prevent rebound. Check methemoglobin.
• Mechanical support: early ECMO, or a right VAD when lung function is good (Miller, p. 10813).

10. Post-cardiac-surgery PH and RV failure

• CVP high, CI low, SvO2 low, rising lactate, MAP fall. Echo shows dilated RV, septal flattening, severe TR, small underfilled LV.
• Differentiate from tamponade (localised posterior clots are easy to miss), LV failure, vasoplegia, hypovolemia and pneumothorax.

1. Rule out mechanical problems: tamponade, prosthesis dysfunction, LVOT obstruction, pneumothorax.
2. Optimise rhythm: sinus or AV sequential pacing. Cardiovert AF. Keep rates of 90 to 100 after surgery.
3. Preload: aim CVP about 8 to 12. Diurese if overloaded, give small boluses only if flat.
4. Oxygenation and ventilation: FiO2 high, avoid hypercarbia and acidosis, low-moderate PEEP, plateau pressure kept low.
5. Systemic pressure: vasopressin 0.01 to 0.04 U/min and/or norepinephrine to keep MAP above about 65 to 70.
6. Inotropy: dobutamine (2.5 to 5 µg/kg/min), milrinone (0.25 to 0.5 µg/kg/min, no loading dose if hypotensive), epinephrine (0.02 to 0.1 µg/kg/min).
7. Lower RV afterload: iNO 20 ppm or inhaled epoprostenol. Watch PAWP when LAP is high.
8. Mechanical support (RVAD, ECMO) if refractory.

11. Your patient

What the facts say

• Preop: severe MS + severe AS + severe TR + PASP 120. This was PH from left heart disease (Group 2), probably CpcPH, with a failing RV (severe TR).
• Postop: PASP 70 to 80 (an improvement), but she needs O2 and the lungs are congested. I assume MV and AV were repaired or replaced and that TR was not necessarily addressed. Please tell me if that is wrong.
• A PASP of 70 to 80 with severe TR is only a rough guide (see section 2).

Why keep the PFO patent?

1. Right-to-left shunting (RV failure or high PVR). When RV afterload is very high, RAP rises above LAP. Blood crosses the PFO right to left. This preserves LV preload and systemic cardiac output, at the price of some desaturation. It also decompresses the RA and RV. This is the same logic behind atrial septostomy in refractory PAH. Closing the PFO in this situation removes the escape route and the RV may fail.
2. Left-to-right shunting (LA hypertension). If LAP exceeds RAP, the PFO lets blood leave the LA. This works like a Lutembacher-type decompression of the LA, which helps pulmonary congestion in MS.
3. In your patient. A long-standing severe MS and AS, with severe TR and RV dysfunction, makes both RA and LA pressures high. After MV relief the LAP should drop, but the RA stays high with TR and RV failure. So the PFO may now shunt right to left, which can add to her hypoxemia and not just the lung congestion. Congested lungs and a right-to-left shunt can coexist.

• Hypoxemia that does not respond to oxygen.
• Paradoxical embolism (stroke), including air or thrombus. Be meticulous with IV air filters.
• Reduced benefit if the RV has recovered.
• Later percutaneous closure is possible once RV function and PVR recover. A test occlusion at cath helps decide.

How to treat her now

• Examine perfusion. Check ABG, lactate, ScvO2, Hb, K⁺, Mg²⁺, creatinine, BNP, troponin.
• Chest X-ray for edema, effusion, pneumothorax, atelectasis, diaphragm position.
• ECG/rhythm. Is it AF? Is there AV synchrony?
• Urgent TEE (or good TTE):
  • Mitral and aortic prosthesis or repair: gradients, pressure half-time, EOA, leaflet motion, paravalvular leak, LVOT obstruction or SAM.
  • LV size, wall thickness, systolic and diastolic function.
  • RV size, TAPSE, S', septal position, TR severity.
  • PFO shunt direction and size. Effusion/tamponade.
• PA catheter or cath if unclear: compare PA diastolic with PAWP, calculate TPG and PVR.
  • High PAWP, PVR ≤3: left-sided congestion (IpcPH).
  • High PAWP and PVR >3: CpcPH.
  • Low PAWP, high CVP, low CI: RV failure predominates.

• Keep SpO2 at least 92 to 94% and avoid hypercarbia and acidosis.
• High-flow nasal oxygen is often better tolerated than intubation. Intubation and positive pressure can collapse an RV, so if intubation is needed, use a protected induction with vasopressors ready (ketamine/etomidate).
• Use moderate PEEP and low plateau pressure. NIV helps pulmonary edema but increases intrathoracic pressure, so monitor RV response.

• Inhaled NO or epoprostenol: reasonable for acute RV failure. In a left-sided disease patient, vasodilating the lungs sends more blood to a stiff, obstructed left heart, and pulmonary edema can worsen. Trial with PAWP monitoring and stop if PAWP rises.
• Oral PAH drugs (sildenafil, ERAs): avoid by default in post-valve-surgery PH. SIOVAC showed harm with sildenafil. Consider only after RHC proves a severe pre-capillary component (PVR above about 5 WU) and with a PH centre.
• Beta-blockers for rate control in MS help diastolic filling time, but are risky with a failing RV. Amiodarone or digoxin are alternatives.
• Anticoagulation per valve type. Check INR, as a mechanical prosthesis thrombosis would worsen everything.
• Treat anemia, infection, pain, electrolyte and temperature problems.

• Repeat echo in days if she is unstable and at 3 months for remodelling. PASP often keeps falling for months.
• If PH persists at 3 months after good valve function, do RHC. Then decide on CpcPH management, TR intervention, and rarely a PH-centre drug trial.

What I need from you to be more specific

1. Exactly what was done to each valve (repair versus replacement, type, size, was the TR repaired).
2. Current echo: prosthesis gradients, LV and RV function, TR, PFO direction.
3. Hemodynamics: CVP, PA pressures, PAWP, CI, SvO2, lactate.
4. Rhythm, current drugs, fluid balance and ventilation settings.