Monitoring and Supportive Care Strategies for Special Pulmonary Hypertension Populations in the ICU

I. Monitoring and Management of ICU Complications

Early recognition and targeted treatment of arrhythmias, acute kidney injury (AKI), fluid overload, and sepsis are critical to prevent right ventricular (RV) decompensation.

A. Arrhythmia Surveillance and Management

Continuous ECG and telemetry for atrial fibrillation/flutter, premature ventricular contractions (PVCs), ventricular tachycardia (VT).
Optimize electrolytes: maintain Potassium >4.0 mEq/L, Magnesium >2.0 mg/dL.
First‐line antiarrhythmic: amiodarone (minimal negative inotropy) with loading and maintenance doses adjusted for hepatic/renal function.
Rate control: cautious use of diltiazem/verapamil in stable patients; avoid β‐blockers in decompensated RV failure.
Hemodynamically unstable situations: synchronized cardioversion, temporary pacing for high‐grade AV block.

Key Pearls: Arrhythmia Management

New‐onset atrial arrhythmias in PH increase mortality; restore sinus rhythm promptly.
Maintain atrioventricular synchrony to preserve RV filling.

B. AKI Recognition and Fluid Management

Use KDIGO criteria for AKI staging (serum creatinine rise and urine output decline).
Impact: elevated Central Venous Pressure (CVP) impairs renal perfusion and worsens RV congestion.
Diuretics: loop ± thiazide or mineralocorticoid antagonist; monitor weight, electrolytes, renal function.
Continuous Renal Replacement Therapy (CRRT) modalities (CVVH, CVVHD, CVVHDF): use for refractory volume overload or precise preload control.
- Start ultrafiltration at 1–2 mL/kg/h; titrate based on hemodynamics.
- Anticoagulation: regional citrate if bleeding risk, systemic heparin otherwise.

Key Pearls: AKI and Fluid Management

CRRT is as much a preload‐optimizing tool as renal support in RV failure.
Avoid both hypovolemia and hypervolemia; target euvolemia carefully.

C. Sepsis and Multi‐Organ Support

Apply Sepsis‐3 criteria: increase in SOFA score ≥2 with suspected infection.
Hemodynamic goals: Mean Arterial Pressure (MAP) ≥65 mmHg, maintain RV perfusion pressure (norepinephrine first‐line; add vasopressin if needed).
Antimicrobial stewardship: broad spectrum initially, then narrow based on cultures; adjust for organ dysfunction.
Adjuncts: low‐dose hydrocortisone if refractory shock; lung‐protective ventilation to minimize Pulmonary Vascular Resistance (PVR).
Monitor lactate, urine output, liver function for multi‐organ dysfunction.

Key Pearls: Sepsis Management

PH patients can deteriorate rapidly in sepsis; early goal‐directed therapy is vital.
Preserve RV afterload by avoiding hypoxia, hypercapnia, acidosis.

II. Interpretation of Hemodynamic Parameters

Invasive and non‐invasive measurements guide fluid, vasoactive, and inotropic therapies.

A. Pulmonary Artery Catheter Monitoring

Key values: CVP, mean Pulmonary Artery Pressure (mPAP), Pulmonary Artery Wedge Pressure (PAWP), Cardiac Output/Cardiac Index (CO/CI), Mixed Venous Oxygen Saturation (SvO₂).
Calculated PVR = (mPAP – PAWP) ÷ CO; threshold >2 Wood Units (WU) defines precapillary PH.
Calibration: level transducer at mid‐axillary line, zero to atmospheric pressure.
Trend analysis: focus on changes rather than single readings to guide therapy.

Key Pearls: PAC Monitoring

PVR >2 WU is the lowest prognostically relevant cutoff.
Rising PAWP in PH suggests postcapillary component.

B. Non‐Invasive Hemodynamic Assessment

Echocardiography (Transthoracic TTE / Transesophageal TEE): RV size, Tricuspid Annular Plane Systolic Excursion (TAPSE), RV Outflow Tract Velocity Time Integral (RVOT VTI), septal flattening.
Inferior Vena Cava (IVC) diameter and collapsibility to estimate CVP.
Near-Infrared Spectroscopy (NIRS) for tissue oxygenation trends.

Key Pearls: Non-Invasive Assessment

Serial echo guides fluid and vasoactive titration when invasive monitoring is unavailable.

III. Special Population Considerations

Perioperative, peripartum, and congenital heart disease (CHD)–associated PH each demand tailored strategies to avoid RV decompensation.

A. Perioperative PH Management

Preoperative: risk stratify by WHO functional class, RV function, volume status; optimize PH therapies.
Intraoperative: maintain preload, avoid hypoxia/hypercarbia/acidosis; use agents with minimal negative inotropy.
Continue chronic vasodilators and consider inotropes (dobutamine, milrinone) for low output.

Key Pearls: Perioperative PH

Elective surgery should be deferred until PH is optimized.
Multidisciplinary planning reduces perioperative mortality.

B. Peripartum PH Management

Physiologic changes: increased blood volume, increased CO, decreased Systemic Vascular Resistance (SVR) exacerbate RV stress.
Safe therapies: prostacyclins and PDE5 inhibitors; avoid endothelin antagonists (teratogenic).
Delivery planning: early elective delivery, regional anesthesia, ICU monitoring postpartum.

Key Pearls: Peripartum PH

Pregnancy remains high risk (maternal mortality 11–25%); preconception counselling mandatory.

C. CHD‐Associated PH

Shunts: Eisenmenger physiology requires caution to avoid shunt reversal.
Management: balance systemic vs pulmonary flows; surgical repair if feasible; medical therapy if inoperable.

Key Pearls: CHD-Associated PH

Avoid abrupt changes in SVR or PVR to prevent hypoxemia crises.

IV. Pharmacotherapy Adjustments in Organ Dysfunction

Dosage and monitoring of PH drugs must be modified for renal or hepatic impairment to maintain efficacy and safety.

Pharmacotherapy Adjustments for Pulmonary Hypertension Medications in Organ Dysfunction
Drug Class	Drug Name	Standard Dosing / Administration	Key Adjustments / Monitoring
Endothelin Receptor Antagonists	Bosentan	62.5 mg BID for 4 weeks, then 125 mg BID	Monitor LFTs monthly; avoid in moderate–severe hepatic impairment. Check LFTs for ≥3× ULN.
	Ambrisentan	5–10 mg daily	Less hepatotoxic; avoid in Child‐Pugh C hepatic impairment.
	Macitentan	10 mg daily	Monthly LFT monitoring; caution in hepatic dysfunction.
Phosphodiesterase‐5 Inhibitors	Sildenafil	20 mg TID	Reduce dose if CrCl <30 mL/min. Monitor BP, vision changes. Contraindicated with nitrates or riociguat. Adjust doses in renal/hepatic failure to prevent hypotension.
Phosphodiesterase‐5 Inhibitors	Tadalafil	40 mg daily	Reduce to 20 mg if CrCl 31–80 mL/min; avoid if CrCl <30 mL/min. Monitor BP, vision changes. Contraindicated with nitrates or riociguat.
Soluble Guanylate Cyclase Stimulators	Riociguat	Start 1 mg TID, titrate to max 2.5 mg TID	Reduce starting dose to 0.5 mg TID if severe organ impairment. Monitor BP and hemoglobin. Contraindicated with nitrates/PDE5 inhibitors; avoid combination due to profound hypotension risk.
Prostacyclin Analogues	Epoprostenol IV	Start 2 ng/kg/min, increase by 1–2 ng/kg/min as tolerated	No specific organ dosing adjustment but requires central line. Rebound PH can occur after therapy interruption; ensure pump and line integrity. Strict aseptic line care.
	Treprostinil IV/SC	Start 1.25 ng/kg/min	Reduce dose in hepatic impairment; monitor site reactions for SC. Rebound PH on interruption.
	Iloprost inhaled	2.5–5 mcg, 6–9 times per day	Short half‐life. Rebound PH on interruption.

Key Pearls: Pharmacotherapy Adjustments

Bosentan has the highest hepatotoxicity risk among ERAs; diligently check LFTs.
Adjust doses of PDE5 inhibitors and Riociguat in renal/hepatic failure to prevent significant hypotension.
Rebound PH is a critical concern with prostacyclin interruption; meticulous line care and pump management are vital.

V. Management of Group 2 and 3 PH

Treat the underlying disease; reserve PH‐specific therapies for select cases under expert guidance.

A. PH Due to Left Heart Disease (Group 2)

Optimize LV filling pressures: diuretics, ACE inhibitors/ARBs.
PH therapies generally not indicated; fluid challenge during catheterization may unmask LV diastolic dysfunction.

Key Pearls: Group 2 PH

PH‐specific drugs have not shown benefit in Group 2 PH and may cause harm by worsening pulmonary edema or systemic hypotension.

B. PH Due to Lung Disease (Group 3)

Mainstays: supplemental oxygen, optimize ventilator settings to minimize PVR.
Inhaled vasodilators (e.g., aerosolized epoprostenol) may be considered in severe cases; systemic agents risk V/Q mismatch.

Key Pearls: Group 3 PH

Oxygen therapy is the only intervention proven to improve survival in Group 3 PH associated with COPD.

VI. Infection Prevention for Prostacyclin Infusions

Central line-associated bloodstream infections (CLABSIs) threaten continuous prostacyclin therapy and patient safety.

Aseptic dressing changes, chlorhexidine hub scrubbing, maximal barrier precautions during line insertion and access.
Surveillance: routine catheter‐site inspection, prompt blood cultures for fever or signs of sepsis.
Empiric antibiotics covering skin flora (Gram-positives) and Gram‐negatives if CLABSI is suspected; remove catheter if persistent bacteremia or tunnel infection.

Key Pearls: Prostacyclin Line Care

Line infections are a leading cause of morbidity and therapy interruption in patients on IV prostacyclins; education of staff, patients, and caregivers is critical.

VII. Advanced Supportive and Rescue Therapies

Escalate to Extracorporeal Membrane Oxygenation (ECMO) or lung transplant referral when medical therapy fails to stabilize or improve the patient.

A. Extracorporeal Membrane Oxygenation (ECMO)

Veno-Arterial (VA)‐ECMO: supports both heart and lungs in refractory RV failure or cardiogenic shock.
Veno-Venous (VV)‐ECMO: provides isolated respiratory support if RV function is adequate but severe hypoxemia persists.
Anticoagulation: typically heparin or direct thrombin inhibitors; monitor circuit for clot/bleed complications.
Weaning: assess RV recovery (e.g., echo, PAC parameters), end‐organ perfusion, and lactate clearance.

Key Pearls: ECMO

Early referral to ECMO‐capable PH centers improves outcomes for patients with severe, refractory PH and RV failure.

B. Lung Transplantation

Referral criteria: WHO functional class III–IV despite optimized medical therapy, progressive RV failure, significant end‐organ dysfunction related to PH.
Bridging strategies: continuous prostacyclins, ECMO support.
Post‐transplant considerations: immunosuppression, surveillance for rejection, infection prophylaxis.

Key Pearls: Lung Transplantation

Timely referral for lung transplantation is life‐saving in eligible patients with refractory PH before irreversible multi-organ damage occurs.

VIII. Multidisciplinary Collaboration and Family Support

A coordinated team approach and active family engagement enhance care delivery and patient outcomes in complex PH management.

Team composition: critical care pharmacy, specialized nursing, cardiology, pulmonology, anesthesia, respiratory therapy, social work, palliative care.
Communication: structured interdisciplinary rounds, shared electronic care plans, clear role delineation.
Family involvement: education about PH and its ICU management, instruction on home monitoring tools if applicable, inclusion in goals‐of‐care discussions.
Palliative care integration: should be considered early for complex symptom management, psychosocial support, and advanced care planning, not just end-of-life care.

Key Pearls: Team and Family

Care at specialized PH centers with experienced multidisciplinary teams is associated with improved survival and quality of life for patients with pulmonary hypertension.

References

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Implementing Monitoring, Supportive Care, and Specialized Approaches for Pulmonary Hypertension in the ICU

Lesson Objective