What are the next best steps for a patient with mild to moderate pulmonary hypertension (PH) diagnosed on echocardiogram (echo)?

From the Guidelines

For a patient with mild to moderate pulmonary hypertension diagnosed on echocardiogram, the next best step is to identify the underlying cause through additional testing, including pulmonary function tests, ventilation-perfusion scan, chest CT, sleep study, and blood tests for connective tissue diseases, rather than immediately confirming the diagnosis with right heart catheterization (RHC), as RHC is not recommended for suspected PH in patients with lung disease, unless therapeutic consequences are to be expected 1. The diagnosis of pulmonary hypertension (PH) is based on echocardiographic criteria, but it is crucial to rule out other potential causes, such as chronic thromboembolic pulmonary hypertension (CTEPH) or pulmonary arterial hypertension (PAH), to determine the best course of treatment.

• The optimal treatment of the underlying lung disease, including long-term O2 therapy in patients with chronic hypoxaemia, is recommended in patients with PH due to lung diseases 1.
• Referral to an expert centre is recommended in patients with echocardiographic signs of severe PH and/or severe right ventricular dysfunction 1.
• The use of drugs approved for PAH is not recommended in patients with PH due to lung diseases 1. According to the 2015 ESC/ERS guidelines, the optimal treatment of the underlying lung disease is recommended, and referral to an expert centre should be considered for patients with signs of severe PH/severe RV failure for individual-based treatment 1. In patients with ACHD and PAH, cardiac catheterization remains the standard for accurate diagnosis of pulmonary hypertension syndromes and for selection of optimal therapies 1. However, for patients with mild to moderate PH, the focus should be on identifying the underlying cause and optimizing treatment for the underlying condition, rather than immediately proceeding with RHC or PAH-specific therapies.
• Pulmonary function tests can help identify underlying lung disease, while ventilation-perfusion scan and chest CT can help rule out CTEPH.
• Blood tests for connective tissue diseases can help identify potential underlying causes of PAH.
• Lifestyle modifications, such as regular light exercise, sodium restriction, and avoiding pregnancy, can help reduce right ventricular strain and improve symptoms. In summary, the next best step for a patient with mild to moderate pulmonary hypertension diagnosed on echocardiogram is to identify the underlying cause through additional testing and optimize treatment for the underlying condition, rather than immediately confirming the diagnosis with RHC or initiating PAH-specific therapies 1.

From the FDA Drug Label

Sildenafil is an inhibitor of cGMP specific phosphodiesterase type-5 (PDE-5) in the smooth muscle of the pulmonary vasculature, where PDE-5 is responsible for degradation of cGMP. Sildenafil, therefore, increases cGMP within pulmonary vascular smooth muscle cells resulting in relaxation In patients with PAH, this can lead to vasodilation of the pulmonary vascular bed and, to a lesser degree, vasodilatation in the systemic circulation.

The next best steps for a patient with mild to moderate pulmonary hypertension (PH) diagnosed on echocardiogram (echo) may include treatment with sildenafil, a phosphodiesterase type-5 (PDE-5) inhibitor, which can help to relax pulmonary vascular smooth muscle and improve symptoms.

• The patient should be evaluated by a pulmonologist or cardiologist to confirm the diagnosis and determine the best course of treatment.
• Monitoring of the patient's condition, including regular echocardiograms and assessments of symptoms, is necessary to adjust treatment as needed 2.

From the Research

Next Best Steps for Mild to Moderate Pulmonary Hypertension

The diagnosis of mild to moderate pulmonary hypertension (PH) on echocardiogram requires careful consideration of the next steps in management. The following points outline the key considerations:

• Confirmatory Diagnosis: Confirmatory right heart catheterization is indicated to determine the severity of PH and guide treatment options 3.
• Treatment Options: For patients with mild to moderate PH, conventional vasodilators or drugs approved for pulmonary arterial hypertension are not recommended due to the lack of evidence supporting their efficacy and potential impairment of gas exchange 3.
• Supportive Therapy: Supportive therapy, including oxygen supplementation, diuretics, and anticoagulation, should be considered in all patients with PH 4.
• Specific Therapy: Specific therapy, directed at the PH itself, includes treatment with vasodilatators, such as calcium channel blockers, and pathogenetic drugs, such as prostanoids, endothelin receptor antagonists, and phosphodiesterase type-5 inhibitors 4.
• Referral to a Specialist Center: Patients with severe PH should be considered for referral to a center with expertise in PH and lung diseases 3.
• Goal-Oriented Therapy: Therapy can be established on the basis of both the clinical classification and the functional class, and a goal-oriented approach can be adopted, where the timing of treatment escalation is determined by inadequate response to known prognostic indicators 4.
• Pharmacological Management: The pharmacological management of PH involves the use of various classes of medications, including prostanoids, endothelin-1 receptor antagonists, and phosphodiesterase type 5 inhibitors, which have different mechanisms of action and require in-depth knowledge of PH and its pathophysiology 5.

Key Considerations for Pharmacological Management

The following points outline the key considerations for pharmacological management:

• Prostanoids: Prostanoids, such as epoprostenol and treprostinil, are administered parenterally or by inhalation to replace the lack of prostacyclin within the pulmonary arterial vasculature 5.
• Endothelin-1 Receptor Antagonists: Endothelin-1 receptor antagonists, such as bosentan, are oral drugs that have been shown to improve exercise capacity and reduce clinical worsening in patients with PAH 6.
• Phosphodiesterase Type 5 Inhibitors: Phosphodiesterase type 5 inhibitors, such as sildenafil, have been shown to improve exercise capacity and are effective in patients with mild to moderate symptoms 6.
• Combination Therapy: Combination therapy has shown promise in patients with severe disease, although data are still lacking 5.