How to manage hypertensive urgency in patients with pulmonary arterial hypertension?

From the Guidelines

Managing hypertensive urgency in patients with pulmonary arterial hypertension (PAH) requires a cautious approach to avoid worsening pulmonary pressures while controlling systemic hypertension, and the preferred initial strategy is to use medications that reduce systemic blood pressure without significantly impacting pulmonary vascular resistance. When considering the management of hypertensive urgency in PAH patients, it's crucial to prioritize medications that are safe for this specific population. The use of calcium channel blockers, such as amlodipine or diltiazem, is often recommended due to their ability to lower systemic blood pressure without adversely affecting pulmonary vascular resistance 1.

Key considerations in managing hypertensive urgency in PAH include:

• Avoiding non-essential surgery and ensuring care at a pulmonary hypertension center when surgery is necessary, as suggested by guidelines for the management of PAH 1.
• Selecting medications that are known to be safe in PAH, such as certain calcium channel blockers and angiotensin receptor blockers.
• Avoiding medications that can worsen right heart failure or increase pulmonary vascular resistance, such as beta-blockers and certain diuretics.
• Monitoring patients closely for signs of right heart failure, hypoperfusion, and changes in oxygen saturation.

In terms of specific medication choices, calcium channel blockers like amlodipine (5-10 mg daily) or diltiazem (120-360 mg daily in divided doses) can be considered, as well as angiotensin receptor blockers such as losartan (25-100 mg daily) or valsartan (80-320 mg daily). The goal is to reduce systemic blood pressure to safe levels (typically <180/110 mmHg) while maintaining adequate cardiac output and avoiding medications that could increase pulmonary vascular resistance or compromise right ventricular function. Throughout treatment, it's essential to closely monitor the patient's clinical status, including oxygen saturation, heart rate, and signs of right heart failure, to ensure that the management strategy is effective and safe 1.

From the FDA Drug Label

Sildenafil citrate has vasodilatory properties, resulting in mild and transient decreases in blood pressure. Before prescribing sildenafil tablets, carefully consider whether patients with certain underlying conditions could be adversely affected by such vasodilatory effects (e.g., patients on antihypertensive therapy or with resting hypotension [BP less than 90/50], fluid depletion, severe left ventricular outflow obstruction, or autonomic dysfunction).

Monitor blood pressure when co-administering blood pressure lowering drugs with sildenafil citrate.

In patients with pulmonary arterial hypertension (PAH), managing hypertensive urgency requires careful consideration of the potential effects of sildenafil on blood pressure.

• Key consideration: Sildenafil has vasodilatory properties, which can result in mild and transient decreases in blood pressure.
• Recommendation: Monitor blood pressure when co-administering blood pressure lowering drugs with sildenafil citrate, and consider the potential risks of hypotension in patients with certain underlying conditions, such as resting hypotension or severe left ventricular outflow obstruction 2.
• Clinical decision: In the absence of explicit guidance on managing hypertensive urgency in patients with PAH, a conservative approach would be to closely monitor blood pressure and adjust treatment as needed to avoid hypotension, while also considering alternative treatments for hypertensive urgency that do not involve vasodilatory effects.

From the Research

Management of Hypertensive Urgency in Patients with Pulmonary Arterial Hypertension

• The management of hypertensive urgency in patients with pulmonary arterial hypertension (PAH) requires careful consideration of the underlying pathophysiology and the potential consequences of treatment on the pulmonary vasculature and right ventricular function 3, 4.
• The treatment plan should focus on identifying and treating the cause of decompensation, optimizing right ventricular preload, reducing right ventricular afterload, correcting hypotension, and augmenting right ventricular inotropy 4.
• Pulmonary vasodilators, such as prostacyclins, inhaled nitric oxide, and phosphodiesterase type 5 inhibitors, can be used to reduce pulmonary artery pressure and improve right ventricular function, especially in group 1 PAH 3.
• The use of nitrates, such as nitroglycerin, can also be beneficial in reducing pulmonary artery pressure and improving symptoms, but may require careful monitoring to avoid hypotensive events and systemic hypoxemia 5, 6.
• In patients with PAH and heart failure, the combined use of PDE5 inhibitors and nitrates may be effective in reducing pulmonary vascular resistance and improving symptoms, but requires careful monitoring and adjustment of therapy 6.

Diagnostic Considerations

• The diagnosis of PAH and other forms of pulmonary hypertension requires a comprehensive evaluation, including electrocardiography, chest radiography, pulmonary function tests, and transthoracic echocardiography 7.
• Right-sided heart catheterization is essential for accurate diagnosis and classification of PAH and other forms of pulmonary hypertension 7.
• Patients with suspected or confirmed PAH or chronic thromboembolic pulmonary hypertension (CTEPH) should be referred to a specialist center for further evaluation and management 7.

Treatment Options

• Targeted treatments, such as phosphodiesterase type 5 inhibitors, soluble guanylate cyclase stimulators, endothelin receptor antagonists, prostacyclin analogues, and prostacyclin receptor agonists, are licensed for patients with PAH 7.
• Surgical pulmonary endarterectomy is the treatment of choice for eligible patients with CTEPH 7.
• Management of PAH resulting from left-sided heart disease primarily involves treatment of the underlying condition 7.