Acute decompensated pulmonary hypertension

• Target SpO2 > 90%; consider HFNC for patients with high oxygen demand

• If possible, avoid intubation and positive pressure ventilation (PPV).

• Extreme caution and specialist consultation is advised if mechanical ventilation is unavoidable.

• Targeting euvolemia

  • Requires very close assessment of volume status and fluid balance monitoring as both hypovolemia and hypervolemia are harmful to cardiac function.

  • Typically involves careful balancing of IV diuretics for volume overload and IV fluid challenges for hypovolemia.

• RV afterload reduction: e.g., with pulmonary vasodilators (prescribed by a PH specialist)

• Optimizing cardiac output and peripheral perfusion: typically with a combination of inodilators (e.g., dobutamine) and other vasopressors (e.g, norepinephrine)

Consider extracorporeal life support as a bridge to urgent lung or heart-lung transplant

• Consult the patient's PAH specialist immediately for any mechanical issues (e.g., suspected device failure or problems with tube patency).

• Do not stop the infusion or adjust dosage or flow rates without guidance from the treating specialist.

• Assess for shock (e.g., cardiogenic shock or obstructive shock).

• Consult a PH specialist.

• Admit to ICU and start continuous monitoring.

• Identify and treat any precipitating factors.

  • Supraventricular arrhythmias

  • Intravascular catheter infection

  • Pulmonary embolism

  • Sepsis

  • Missed or interrupted medical treatment, e.g., IV prostacyclin pump failure