What is the management of acute severe pulmonary hypertension?

From the Guidelines

The management of acute severe pulmonary hypertension requires immediate intervention with inhaled nitric oxide (iNO) at 10-40 ppm as first-line therapy, due to its selective pulmonary vasodilation without systemic effects, as recommended by the most recent guidelines 1.

Key Considerations

• The goal of treatment is to reduce pulmonary arterial pressure, improve right ventricular function, and maintain adequate systemic perfusion.
• Supplemental oxygen should be administered to maintain oxygen saturation above 92%, and ventilatory support should be provided if respiratory failure is present.
• Pulmonary vasodilators, such as iNO, inhaled epoprostenol, or inhaled iloprost, are the cornerstone of therapy.
• Hemodynamic support may require careful fluid management and vasopressors like norepinephrine to maintain systemic blood pressure.
• Dobutamine or milrinone can be used to improve cardiac output.
• Mechanical circulatory support with ECMO may be necessary in refractory cases.
• The underlying cause of pulmonary hypertension must be identified and treated simultaneously.

Treatment Options

• Inhaled nitric oxide (iNO) at 10-40 ppm
• Inhaled epoprostenol (Flolan) at 10-50 ng/kg/min
• Inhaled iloprost (Ventavis) at 2.5-5 mcg every 2-4 hours
• Intravenous prostacyclins like epoprostenol starting at 2 ng/kg/min and titrating upward
• Sildenafil 20-40 mg orally every 8 hours
• Norepinephrine (0.05-0.5 mcg/kg/min) to maintain systemic blood pressure
• Dobutamine (2-10 mcg/kg/min) or milrinone (0.25-0.75 mcg/kg/min) to improve cardiac output

Monitoring and Assessment

• Continuous hemodynamic monitoring via arterial line and pulmonary artery catheter is essential to guide therapy and assess response to treatment.
• Regular assessment of oxygen saturation, cardiac output, and systemic blood pressure is necessary to adjust treatment as needed.
• Identification and treatment of the underlying cause of pulmonary hypertension is crucial to improve patient outcomes 1.

From the FDA Drug Label

FLOLAN is indicated for the treatment of pulmonary arterial hypertension (PAH) (WHO Group I) to improve exercise capacity Initiate intravenous infusions of FLOLAN at 2 ng/kg/min. Alter the infusion by 1- to 2-ng/kg/min increments at intervals sufficient to allow assessment of clinical response.

The management of acute severe pulmonary hypertension involves the use of epoprostenol (IV), which is indicated for the treatment of pulmonary arterial hypertension (PAH). The initial dose is 2 ng/kg/min, and it can be adjusted by 1- to 2-ng/kg/min increments at intervals of at least 15 minutes to assess clinical response 2.

• Key points:
  • Initiate intravenous infusions of epoprostenol at 2 ng/kg/min
  • Adjust the infusion rate by 1- to 2-ng/kg/min increments
  • Monitor clinical response at intervals of at least 15 minutes
  • Epoprostenol is indicated for the treatment of PAH (WHO Group I) to improve exercise capacity 2.

From the Research

Management of Acute Severe Pulmonary Hypertension

The management of acute severe pulmonary hypertension is complex and requires a careful balance to maintain perfusion while optimizing right-sided heart function 3.

• Pathogenesis and Physiology: Pulmonary hypertension may be encountered in the intensive care unit in patients with critical illnesses such as acute respiratory distress syndrome, left ventricular dysfunction, and pulmonary embolism, as well as after cardiothoracic surgery 4.
• Diagnostic Tools and Therapies: The utility of various diagnostic tools and the use of vasopressors and pulmonary vasodilators in intensive care units are crucial for the management of pulmonary hypertension 4.
• Vasopressors and Pulmonary Vasodilators: The use of dobutamine, milrinone, inhaled nitric oxide, and intravenous prostacyclin have the greatest support in the literature for the management of pulmonary hypertension 4.
• Aerosolized Prostacyclin and Iloprost: Aerosolized prostacyclin and its stable analog iloprost have been shown to cause selective pulmonary vasodilatation, increase cardiac output, and improve venous and arterial oxygenation in patients with severe pulmonary hypertension 5.
• Inhaled Epoprostenol: Inhaled epoprostenol may be an alternative therapy to minimize systemic hypotension, which often accompanies rapid intravenous titration, and has been shown to significantly decrease pulmonary pressures without lowering systemic blood pressure 6.
• Sildenafil, Nitric Oxide, and Iloprost: Different vasodilators such as sildenafil, nitric oxide, and iloprost have been compared in terms of their hemodynamic effects in primary pulmonary hypertension, and all have been shown to improve pulmonary hemodynamics 7.

Treatment Strategies

Treatment of pulmonary hypertension resulting from critical illness or chronic lung diseases should address the primary cause of hemodynamic deterioration, and pulmonary vasodilators usually are not necessary 4.

• Right Ventricular Failure: Patients with decompensated pulmonary hypertension, including those with pulmonary hypertension associated with cardiothoracic surgery, require therapy for right ventricular failure 4.
• Selective Pulmonary Vasodilatation: Aerosolization of prostacyclin or its stable analog iloprost may offer a new strategy for treatment of severe pulmonary hypertension by causing selective pulmonary vasodilatation 5.