What is the best approach for diagnosing and managing pulmonary hypertension?

From the Guidelines

The best type of echo to evaluate for pulmonary hypertension is transthoracic echocardiography (TTE) with Doppler, as it allows for the estimation of pulmonary artery pressure and assessment of right ventricular function. This is supported by the 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension, which recommend the use of echocardiography as a initial diagnostic test to estimate pulmonary artery pressure 1.

Key Points for Evaluation

• Echocardiography should be performed with Doppler to estimate pulmonary artery pressure and assess right ventricular function
• Right heart catheterization is necessary to confirm the diagnosis of pulmonary hypertension, defined as mean pulmonary artery pressure ≥20 mmHg at rest
• Additional testing, including pulmonary function tests, ventilation-perfusion scanning, CT angiography, and blood tests, helps determine the underlying cause and classify the disease into one of five groups

Management of Pulmonary Hypertension

• Management depends on the classification of pulmonary hypertension, but typically includes treating underlying conditions, oxygen therapy when indicated, and specific pulmonary vasodilators
• For Group 1 PAH, medications include endothelin receptor antagonists, phosphodiesterase-5 inhibitors, soluble guanylate cyclase stimulators, and prostacyclin analogs
• Combination therapy is often more effective than monotherapy, and supportive measures include diuretics for fluid overload, anticoagulation in selected cases, and supervised exercise rehabilitation

Recent Guidelines and Recommendations

• The 2019 AHA/ACC guideline for the management of adults with congenital heart disease recommends the use of cardiac catheterization to accurately diagnose pulmonary hypertension syndromes and select optimal therapies 1
• The 2019 Chest guideline and expert panel report on therapy for pulmonary arterial hypertension in adults recommends a visual algorithm to enhance clinical utility and includes updated recommendations for medication therapy 1

From the FDA Drug Label

The FDA drug label does not answer the question.

From the Research

Diagnosis of Pulmonary Hypertension

• Transthoracic Doppler echocardiography (TDE) is a commonly used noninvasive method for diagnosing pulmonary hypertension (PH) 2.
• TDE has acceptable sensitivity but insufficient specificity for detecting PH, making it useful as a first-line surveillance modality in patients with suspected PH 2.
• The combination of TDE with other noninvasive methods for PH detection may improve diagnostic accuracy, but further investigation is needed 2.

Echocardiographic Techniques

• Transthoracic echocardiography (TTE) has a central role as a screening tool in patients with symptoms and those at risk for developing pulmonary vascular disease 3.
• Echocardiographic techniques can estimate pulmonary artery pressure and resistance, right atrial pressure, and derive indirect information about right heart structure and function 3.
• Newer techniques, such as three-dimensional assessment of the right ventricle, can provide more accurate information about right ventricular volume and contractility 3.

Multi-View Approach

• A multi-view approach using TTE can improve the accuracy of diagnosing PH by imaging from multiple ultrasound windows 4.
• This approach can increase sensitivity without decreasing specificity and may change the echocardiographic diagnosis of PH in some patients 4.

Tricuspid Regurgitant Signal

• The "chin" measurement of tricuspid regurgitant velocity (TRVmax) is more accurate than the "beard" measurement for estimating right ventricular systolic pressure (RVSP) and pulmonary vascular resistance (PVR) 5.
• The optimal cut-off for TRVmax "chin" is 2.8 m/s, with a sensitivity of 93% and specificity of 87% for diagnosing PH 5.

Pediatric Pulmonary Hypertension

• TTE is a useful method for non-invasive screening of children and adolescents with suspected or confirmed PH 6.
• A structured approach for non-invasive assessment of pulmonary artery pressure and myocardial function using TTE may help identify patients with early ventricular deterioration and guide tailored therapy 6.