From the Guidelines
Pulmonary hypertension assessment requires a comprehensive approach combining clinical evaluation, diagnostic testing, and risk stratification, with the most recent guidelines recommending a mean pulmonary arterial pressure >20 mmHg at rest for diagnosis, as stated in the 2022 update 1.
Clinical Evaluation
Begin with a thorough history focusing on symptoms like dyspnea, fatigue, chest pain, syncope, and edema, along with risk factors such as connective tissue diseases, HIV, liver disease, congenital heart disease, and medication use. Physical examination should assess for signs including elevated jugular venous pressure, right ventricular heave, loud P2 heart sound, tricuspid regurgitation murmur, and peripheral edema.
Diagnostic Testing
Initial testing includes:
- ECG (looking for right ventricular hypertrophy)
- Chest X-ray
- Pulmonary function tests
- Echocardiography to estimate pulmonary artery pressure and assess right ventricular function, with guidelines for echocardiographic probability of pulmonary hypertension in symptomatic patients provided in the 2015 ESC/ERS guidelines 2 Definitive diagnosis requires right heart catheterization, which confirms pulmonary hypertension when mean pulmonary artery pressure exceeds 20 mmHg at rest, as recently redefined in 2018 and updated in the 2022 guidelines 1.
Additional Testing and Risk Assessment
Additional testing may include:
- Ventilation-perfusion scanning to rule out chronic thromboembolic pulmonary hypertension
- CT angiography
- Sleep studies
- Laboratory tests for underlying conditions Risk assessment tools like the REVEAL score or ESC/ERS risk assessment help determine disease severity and prognosis, as discussed in the 2015 ESC/ERS guidelines 3.
Follow-up Assessments
Regular follow-up assessments every 3-6 months are essential to monitor disease progression and treatment response, with repeat echocardiography, 6-minute walk tests, and BNP/NT-proBNP measurements to guide management decisions, as recommended in the provided evidence 4, 1, 3, 2.
From the FDA Drug Label
Epoprostenol for injection is a prostacyclin vasodilator indicated for the treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to improve exercise capacity If symptoms of pulmonary hypertension persist or recur after improving - the infusion should be increased by 1 ng/kg/min to 2 ng/kg/min increments at intervals sufficient to allow assessment of clinical response; these intervals should be at least 15 minutes.
The assessment for pulmonary hypertension (PH) is done by monitoring symptoms and clinical response to the treatment, with incremental increases in dosage of epoprostenol as needed, with intervals of at least 15 minutes to allow for assessment of clinical response 5.
- Key points: + Monitor symptoms of PH + Assess clinical response to treatment + Increase dosage as needed + Intervals of at least 15 minutes for assessment
From the Research
Assessment of Pulmonary Hypertension
The assessment of pulmonary hypertension (PH) involves various methods, including:
- Right heart catheterization (RHC), which is considered the gold standard for measuring mean pulmonary artery pressure (mPAP) 6, 7, 8, 9
- Echocardiography, which is a noninvasive alternative for estimating hemodynamic parameters and assessing right ventricular function 6, 7, 8, 9, 10
- Cardiac magnetic resonance, which is considered the gold standard for evaluating right ventricular size and function 8
Echocardiographic Assessment
Echocardiography plays a crucial role in the assessment of PH, including:
- Estimating systolic pulmonary arterial pressure using Doppler echocardiography 6
- Evaluating right ventricular size and function 8, 10
- Assessing pulmonary artery pressures and detecting signs of PH, such as pericardial effusion, reduced tricuspid annular plane excursion, and right atrial enlargement 6, 9
- Monitoring the response of patients to therapy and predicting prognosis 6, 10
Prediction of Pulmonary Hypertension
A simple noninvasive echocardiographic score can be useful in predicting the diagnosis of PH at RHC, with a score value more than 57 associated with a high sensitivity and specificity 9
- The score is calculated using right atrium and ventricular diastolic area, tricuspid regurgitation Vmax, tricuspid regurgitation severity degree, and left ventricular ejection fraction 9
Importance of Right Ventricular Assessment
Right ventricular assessment is essential in evaluating the cause and severity of PH, and has prognostic significance 10
- Serial right ventricular assessment is important in risk assessment and prognosis, and achieving normal or near-normal right ventricular performance may serve as a principal goal in the treatment of PH 10