A 45-year-old woman with WHO Functional Class IV pulmonary arterial hypertension is on maximal medical therapy, including macitentan, tadalafil, and high-dose intravenous treprostinil. She is now mechanically ventilated and requires norepinephrine for hemodynamic support. Despite these measures, she has worsening right ventricular failure (CVP 22 mmHg, cardiac index 1.6 L/min/m²) and rising BNP. What is the MOST appropriate next step in management?

A 58-year-old man with pulmonary hypertension presents to the ICU in acute right heart failure with severe hypoxemia. The team needs an inhaled pulmonary vasodilator that provides the most rapid, titratable decrease in pulmonary artery pressure, while recognizing institutional cost and resource constraints. Which agent is the most appropriate initial choice?

A 52-year-old woman with idiopathic pulmonary arterial hypertension (WHO Group 1) on oral ambrisentan 10 mg daily and tadalafil 40 mg daily is admitted to the ICU with worsening dyspnea and peripheral edema over the past 24 hours. On arrival her heart rate is 112 bpm, blood pressure 78/50 mmHg on norepinephrine 0.05 µg/kg/min, respiratory rate 28/min, and SpO₂ 88% on room air. Point-of-care echocardiography shows severe right ventricular dilation, an estimated RV systolic pressure of 75 mmHg, central venous pressure 18 mmHg, and a cardiac index of 1.4 L/min/m². Lactate has risen to 3.2 mmol/L and urine output has decreased over the last 6 hours. The team is considering initiation of continuous intravenous prostacyclin or urgent veno-arterial ECMO for progressive right heart failure and hemodynamic instability. Given the high risk and burden of these interventions, what should the critical care pharmacist prioritize next?

A 62-year-old woman with chronic thromboembolic pulmonary hypertension (CTEPH) has been stabilized in the ICU after an acute decompensation. She has normal renal function (creatinine clearance 80 mL/min), no history of major bleeding, platelet count 230 × 10^3/µL, and no contraindications to anticoagulation. According to the 2022 ESC/ERS Guidelines, which long-term anticoagulation strategy is most appropriate to prevent recurrent thromboembolism in this patient?

A 58-year-old man with Group 1 pulmonary arterial hypertension (PAH) is admitted to the ICU with severe sepsis complicated by acute kidney injury on continuous renal replacement therapy (creatinine 3.5 mg/dL; BUN 48 mg/dL) and vasopressor-dependent hypotension requiring norepinephrine at 0.1 µg/kg/min to maintain a mean arterial pressure of 65 mmHg. He takes macitentan 10 mg once daily and tadalafil 40 mg once daily at home, with his last doses taken 10 hours ago. Laboratory studies reveal AST 65 U/L and ALT 78 U/L. Which of the following is the most appropriate initial management strategy for his chronic PAH therapies?

A 48-year-old woman (weight 70 kg, BSA 1.8 m²) with WHO Functional Class IV pulmonary arterial hypertension (PAH) was admitted to the ICU and started on continuous IV epoprostenol at 35 ng/kg/min. Baseline right heart catheterization showed mPAP 55 mmHg, PVR 9 Wood units, and CI 1.9 L/min/m². After 6 weeks of therapy, she improved to WHO Class II, with repeat hemodynamics mPAP 38 mmHg, PVR 5 WU, CI 2.5 L/min/m², and is preparing for discharge. Which strategy is safest and most appropriate for de-escalating her PAH therapy?

A 45-year-old woman with idiopathic World Health Organization (WHO) Group I pulmonary arterial hypertension (PAH) was admitted to the ICU with an acute decompensation episode. She has been stabilized on oral bosentan 125 mg twice daily and tadalafil 40 mg once daily, remains WHO functional class III, and requires supplemental oxygen at 4 L/min to maintain SpO₂ > 90%. As the critical care pharmacist, you are tasked with optimizing her long-term management and ensuring a safe transition of care upon discharge. According to current guidelines, which of the following is the MOST appropriate next step?

A 45-year-old woman (65 kg) with severe pulmonary arterial hypertension is admitted to the ICU with a 2-day history of fever (38.5 °C), erythema, and tenderness at her right internal jugular central venous catheter site. She is receiving continuous intravenous epoprostenol at 15 ng/kg/min (1.5 mL/hr) via a portable infusion pump. Nursing reports that the pump alarmed for occlusion and troubleshooting has been unsuccessful. Vital signs are: HR 110 bpm, BP 100/60 mmHg; labs show WBC 14,200/mm3. As the critical care pharmacist, which of the following is the MOST appropriate initial intervention regarding her epoprostenol therapy and central line management?

A 45-year-old woman with severe pulmonary arterial hypertension (PAH) is being prepared for discharge from the critical care unit after stabilization. She has been successfully weaned from mechanical ventilation and is now stable on a continuous intravenous treprostinil infusion via a central venous catheter. Her husband will be her primary caregiver at home. During evening rounds, the medical team confirms she is medically ready for discharge, pending comprehensive home care arrangements.

Given the critical, life-sustaining nature of her continuous IV treprostinil infusion, which of the following discharge planning interventions should the critical care pharmacist prioritize to ensure the safest transition to home?

A 45-year-old patient with pulmonary arterial hypertension (PAH) has been stabilized in the ICU on continuous parenteral prostacyclin therapy. Discharge planning reveals the following social factors: the patient’s health insurance coverage recently lapsed; transportation to the specialty pharmacy and home nursing visits is unreliable; a family member is present but has not yet received formal training in pump management; and access to a specialty pharmacy capable of compounding and delivering prostacyclin is limited. Which factor represents the most significant immediate barrier to safe discharge home?