1. Introduction to Individualized Transition and Discharge Planning

Transitioning patients with severe pulmonary hypertension from ICU to home requires a structured, multidisciplinary protocol that balances hemodynamics, pharmacotherapy, and patient factors.

Rationale for specialized discharge protocols:

• High risk of rebound pulmonary hypertension (PH), right heart failure, and readmission after ICU stay.
• Complexity of chronic regimens often involves multiple oral and parenteral agents.
• Need to integrate hemodynamic stability, medication optimization, and social determinants of health.

Unique challenges of weaning ICU therapies:

• Risk of rebound PH with abrupt prostacyclin cessation.
• Catheter-related infection risk from prolonged parenteral use.
• Altered pharmacokinetics/pharmacodynamics (PK/PD) post–critical illness affecting drug tolerability.

2. Pharmacotherapy Continuation and Adjustment

Maintain or adjust endothelin receptor antagonists, PDE5 inhibitors, and sGC stimulators during and after ICU care, accounting for altered pharmacokinetics and organ function.

A. Endothelin Receptor Antagonists (ERAs)

ERAs block endothelin-1–mediated vasoconstriction and remodeling in WHO Group 1 PAH; selection hinges on receptor selectivity, safety, and monitoring needs.

• Mechanism of action: ETA and/or ETB receptor blockade reduces vasoconstriction and proliferation.
• Indications: WHO Group 1 PAH; avoid in PH due to lung disease (Group 3).
• Agent selection:
  • Bosentan: dual ETA/ETB antagonist; hepatotoxicity risk.
  • Ambrisentan: selective ETA antagonist; lower hepatic risk.
  • Macitentan: dual antagonist; improved tissue penetration and safety.
• Dosing & PK considerations:
  • Bosentan: 62.5 mg BID initially, titrate to 125 mg BID; CYP3A4/2C9 metabolism.
  • Ambrisentan: 5 mg QD initially, titrate to 10 mg QD; minimal CYP interactions.
  • Macitentan: 10 mg QD; long half-life (~16 h).
• Monitoring:
  • LFTs monthly (bosentan); periodic (others).
  • Hemoglobin for anemia (macitentan).
• Contraindications: Pregnancy (teratogenic), severe hepatic impairment.

B. Phosphodiesterase Type 5 Inhibitors (PDE5i)

PDE5 inhibitors enhance NO–cGMP signaling to promote pulmonary vasodilation; choice is based on half-life and dosing convenience.

• Mechanism: Inhibit cGMP degradation, augmenting NO-mediated vasodilation.
• Indications: WHO Group 1 PAH; selected Group 3 (e.g., associated with COPD) with caution.
• Agents:
  • Sildenafil: 20 mg TID; half-life ~4 h.
  • Tadalafil: 40 mg QD; half-life ~35 h.
• Dosing & PK:
  • Adjust sildenafil in renal impairment; watch CYP3A4 interactions.
  • Tadalafil dose reduction in hepatic/renal dysfunction.
• Monitoring:
  • BP for hypotension when combined with other vasodilators.
  • Visual changes (rare NAION – Non-Arteritic Anterior Ischemic Optic Neuropathy).
• Contraindications: Concomitant nitrates or riociguat; potent CYP3A4 modifiers.

C. Soluble Guanylate Cyclase (sGC) Stimulators

sGC stimulators directly activate guanylate cyclase to raise cGMP; riociguat is approved for PAH and CTEPH.

• Mechanism: NO-independent sGC stimulation increases cGMP.
• Indications: WHO Group 1 PAH, Chronic Thromboembolic Pulmonary Hypertension (CTEPH).
• Agent & dosing: Riociguat 1 mg TID initially, titrate by 0.5 mg increments to a maximum of 2.5 mg TID as tolerated.
• PK: Metabolized by CYP1A1/3A4; avoid in severe hepatic dysfunction (Child-Pugh C).
• Monitoring: BP for hypotension; hemoglobin for anemia.
• Contraindications: Pregnancy (teratogenic); do not combine with PDE5i or nitrates.

3. Safe Transition from IV/Inhaled to Home Regimens

Weaning parenteral or inhaled vasodilators requires predefined hemodynamic targets, gradual down-titration, and close monitoring—augmented by telemedicine.

• Patient selection criteria for weaning:
  • Right Atrial (RA) pressure <8 mmHg.
  • Cardiac Index (CI) >2.5 L/min/m².
  • Mixed venous oxygen saturation (SvO₂) >65%.
  • Stable WHO Functional Class (WHO-FC) II–III.
  • No signs of overt right heart failure.
• Stepwise weaning protocol:
  • Reduce dose by 10–20% every 24–48 hours, guided by clinical stability.
  • Monitor symptoms (dyspnea, fatigue), vital signs, BNP/NT-proBNP levels.
  • Consider repeat echocardiography or right heart catheterization (RHC) parameters if concerns arise.
• Adverse event management during weaning:
  • Hypotension: Slow the weaning rate or temporarily hold the dose reduction.
  • Worsening dyspnea or signs of decompensation: Consider re-escalation of therapy and reassessment.
• Telemedicine integration for enhanced monitoring:
  • Remote monitoring of vital signs (BP, heart rate, oxygen saturation) and patient-reported symptoms.
  • Virtual visits for medication adjustment, pump management, and central line assessment.

4. Coordination with PH Specialists and Multidisciplinary Team

Collaboration with PH centers, home health nursing, and pharmacy teams ensures continuity, early detection of issues, and optimized outpatient care.

• Referral pathways and communication:
  • Engage PH specialists pre-discharge from ICU or hospital.
  • Establish clear handoff communication, including medication reconciliation, current status, and follow-up plans.
• Follow-up and risk stratification:
  • Schedule first outpatient PH clinic visit within 1–2 weeks post-discharge.
  • Utilize risk stratification tools (e.g., REVEAL 2.0 score), NT-proBNP levels, and 6-minute walk distance (6MWD) to guide ongoing therapy adjustments.
• Home health and specialty pharmacy liaison:
  • Coordinate with home health nurses for parenteral infusion support, line care education, and monitoring.
  • Involve specialty pharmacists for medication access, detailed patient education on complex regimens, and adherence support.

5. Patient and Caregiver Education, Adherence, and Line Care

Structured teaching and adherence tools empower patients and caregivers to manage complex regimens and prevent line-related infections.

• Adherence support strategies:
  • Provide pillboxes, medication reminder apps, and clear, written schedules.
  • Discuss potential side effects and management strategies to improve persistence.
• Infusion pump management (for parenteral therapies):
  • Conduct hands-on training for patients and caregivers on pump operation, alarms, and troubleshooting.
  • Ensure availability of backup medication syringes/cassettes and emergency contact protocols.
• Central line care (for IV prostacyclins):
  • Teach aseptic techniques for dressing changes and medication administration.
  • Emphasize daily site checks for signs of infection (redness, swelling, pain, discharge).
  • Provide clear instructions on when to seek urgent medical attention.
• Teach-back methods for effective education:
  • Confirm patient and caregiver understanding by having them explain or demonstrate key information and skills.
  • Provide written materials, visual aids, and reputable online resources.

6. Discharge Logistics: Home Infusion and Telemedicine Considerations

Pre-discharge planning for equipment, supplies, insurance, and telehealth setup mitigates early postdischarge gaps in care.

• Equipment and supplies coordination:
  • Confirm timely delivery of infusion pumps, tubing, medications, inhaled therapy devices (e.g., nebulizers, spacers), and backup kits to the patient’s home before discharge.
  • Verify patient/caregiver understanding of supply reordering processes.
• Insurance and financial assistance navigation:
  • Verify insurance coverage for medications, equipment, and home health services.
  • Proactively connect patients with manufacturer assistance programs, co-pay support, or foundations if financial barriers are identified.
• Telemedicine setup and training:
  • If applicable, ensure necessary remote monitoring devices (e.g., blood pressure cuffs, pulse oximeters, scales with transmission capabilities) are provided and functional.
  • Educate patients and caregivers on using telehealth platforms, troubleshooting common issues, and understanding data privacy.

7. Social Determinants of Health Impact Analysis

Assess insurance, home environment, and caregiver support to ensure outpatient therapy safety and feasibility.

• Assessment framework for social determinants:
  • Insurance status and financial resources: Ability to afford medications, co-pays, and necessary supplies.
  • Transportation: Access to reliable transport for follow-up appointments and urgent care.
  • Housing stability and environment: Safe, clean living conditions with adequate space for equipment and medication storage.
  • Caregiver availability and capacity: Presence of a willing and able caregiver, considering their health, work, and other responsibilities.
  • Health literacy and language barriers: Patient’s and caregiver’s ability to understand complex medical information.
• Impact of unaddressed social barriers:
  • Resource gaps can lead to medication non-adherence, therapy interruptions, missed appointments, and increased risk of complications or hospital readmission.
• Intervention strategies:
  • Engage hospital case management and social workers early in the discharge planning process.
  • Connect patients and families to community resources, support groups, and respite services for caregivers.
  • Address food insecurity, utility assistance, and other basic needs that impact health.

8. Case-Based Algorithms and Decision Pathways

Decision algorithms that integrate hemodynamics, pharmacotherapy, and social factors guide safe transitions and troubleshoot barriers.

Core steps in a decision pathway:

• Evaluate current hemodynamic status (e.g., RHC data, echo findings, clinical signs of congestion/perfusion).
• Confirm stability on current oral/inhaled/parenteral PH regimen, including successful weaning if applicable.
• Conduct comprehensive social assessment (caregiver support, home environment, financial resources).
• Complete patient and caregiver education and training, ensuring competency via teach-back.
• Verify all logistical arrangements (medications, equipment, home health, follow-up appointments) are in place for discharge readiness.

Common barriers and troubleshooting:

• Medication access delays: Initiate prior authorizations and specialty pharmacy coordination well before anticipated discharge. Have a plan for bridging therapy if delays occur.
• Caregiver limitations or burnout: Arrange for supplemental home health nursing, explore respite care options, or provide additional targeted training and support.
• Telehealth connectivity or usability issues: Provide clear, simple instructions, offer technical support, and have alternative (e.g., phone-based) monitoring plans if technology fails.