Management of Pulmonary Hypertensive Crisis

Immediate Recognition and ICU Admission

The American College of Cardiology recommends immediate ICU admission with continuous hemodynamic monitoring for all patients with suspected pulmonary hypertensive crisis, as mortality rates for PAH patients admitted to ICU can reach 41% 1, 2
A pulmonary hypertensive crisis is characterized by high heart rate, low systolic blood pressure, low urine output, and rising lactate levels, indicating imminent right ventricular failure 1

The American College of Cardiology recommends initiating inhaled nitric oxide immediately as the preferred first-line pulmonary vasodilator, as it decreases pulmonary vascular resistance and improves cardiac output 1, 2, 3
The American College of Cardiology suggests starting dobutamine as the preferred inotrope for all hypotensive PAH crisis patients, as it has neutral or beneficial effects on pulmonary vascular resistance 1, 2
The American College of Cardiology recommends initiating continuous IV epoprostenol if the patient fails to stabilize on inhaled nitric oxide and dobutamine, as epoprostenol is the only therapy for PAH that has been shown to prolong survival 1, 4, 5, 6

The American College of Cardiology recommends maintaining oxygen saturation >90% at all times to prevent hypoxia-induced increases in pulmonary vascular resistance 1, 2, 4, 5, 6
The American College of Cardiology suggests avoiding mechanical ventilation if possible, as positive intrathoracic pressure reduces venous return and worsens right ventricular failure 1, 2

The American College of Cardiology recommends optimizing fluid balance with IV diuretics for symptomatic management of right ventricular volume overload, as volume overload worsens right ventricular function 1, 2, 4, 5, 6
The American College of Cardiology suggests avoiding aggressive volume expansion in patients with right ventricular failure, as this may worsen right ventricular function 1, 2

The American College of Cardiology recommends direct measurement of central venous pressure via central line placement, as non-invasive estimates may be misleading 2
The American College of Cardiology suggests continuously monitoring the systemic-to-pulmonary vascular resistance ratio, as maintaining systemic vascular resistance greater than pulmonary vascular resistance is crucial 2

The American College of Cardiology recommends initiating veno-arterial ECMO as a bridge to recovery or transplantation for patients with pulmonary hypertensive crisis, low cardiac output, or right ventricular failure despite optimal medical therapy 1, 2

The American College of Cardiology recommends maintaining oral anticoagulation with warfarin in patients with idiopathic PAH, with a target INR of 1.5-2.5 in North American centers or 2.0-3.0 in European centers 1, 7
The American College of Cardiology suggests ensuring current immunization against influenza and pneumococcal pneumonia, as respiratory infections can be devastating in these patients 1, 7

The American College of Cardiology recommends avoiding the use of beta-blockers in patients with PAH, as they may worsen right ventricular function 8
The American College of Cardiology suggests avoiding non-selective systemic vasodilators that may cause systemic hypotension without reducing pulmonary pressure 8
The American College of Cardiology recommends avoiding the combination of riociguat and PDE-5 inhibitors due to the risk of hypotension 8