From the Guidelines
The standard medical work-up for elevated right ventricular (RV) systolic pressure found on echocardiography should include a comprehensive evaluation to determine the underlying cause and severity of pulmonary hypertension, as outlined in the 2014 study by Zamanian et al. 1.
Initial Evaluation
The initial evaluation should include a thorough history and physical examination, focusing on symptoms like dyspnea, fatigue, chest pain, and syncope.
- Laboratory tests should include:
- Complete blood count
- Comprehensive metabolic panel
- Thyroid function tests
- N-terminal pro B-type natriuretic peptide (NT-proBNP)
- Patients should also undergo a full set of pulmonary function tests, a six-minute walk test, and chest imaging with X-ray and high-resolution CT scan to identify parenchymal lung disease.
Further Testing
Further testing may include:
- Ventilation-perfusion (V/Q) scanning to rule out chronic thromboembolic disease
- Right heart catheterization to confirm pulmonary hypertension, measure exact pressures, and assess vasoreactivity
- ECG and overnight oximetry or polysomnography to evaluate for cardiac arrhythmias and sleep-disordered breathing
- Hepatitis serologies, HIV, and hypercoagulable panels as indicated
- Autoimmune antibody panels if screening anti-nuclear antibody is positive
Risk Factor Assessment
A comprehensive risk factor assessment should be performed, including screening for:
- Congenital heart disease
- Autoimmune diseases
- Liver disease
- Thromboembolic disease
- History of stimulant use
- Underlying lung disease
- Heart disease
- Sleep-disordered breathing
- HIV infection
Management
Management should be guided by the underlying cause and severity of pulmonary hypertension, as well as the patient's functional class and co-morbid conditions.
- The 2014 study by Zamanian et al. 1 provides a comprehensive approach to the management of pulmonary hypertension, including the use of calcium channel blockers, vasoreactivity testing, and right heart catheterization.
- The 2009 expert consensus document by the American College of Cardiology Foundation 1 and the 2004 evidence-based clinical practice guidelines by the American College of Chest Physicians 1 also provide guidance on the diagnosis and management of pulmonary hypertension.
From the Research
Standard Medical Work-up for Elevated Right Ventricular Systolic Pressure
The standard medical work-up for echocardiogram (echo) findings of elevated Right Ventricular (RV) systolic pressure involves several steps, including:
- Evaluation of pulmonary hypertension (PH) using echo-Doppler measurements, such as tricuspid regurgitant flow velocity and inferior vena cava collapsibility index (IVCCI) 2, 3
- Assessment of right ventricular function and degree of tricuspid regurgitation to modify diagnostic accuracy 4
- Use of point-of-care ultrasound (POCUS) to evaluate for acute right ventricular strain and suspected pulmonary embolism (PE) 5
- Consideration of clinical context and associated pathophysiology of right ventricular strain to point towards a diagnosis of PE 5
Echocardiographic Findings
Echocardiographic findings that may suggest pulmonary hypertension or right ventricular strain include:
- Increased right ventricle: left ventricle size ratio
- Abnormal septal motion
- McConnell's sign
- Tricuspid regurgitation
- Elevated pulmonary artery systolic pressure
- Decreased tricuspid annular plane systolic excursion
- Decreased S'
- Pulmonary artery mid-systolic notching
- 60/60 sign
- Speckle tracking demonstrating decreased right ventricular free wall strain 5
Diagnostic Approach
A simplified approach to the diagnostic work-up of PH involves:
- Evaluation of echo-Doppler parameters, such as acceleration time of pulmonary artery systolic flow (ACT) and pulmonary artery diastolic pressure obtained utilizing the early phase of the tricuspid regurgitation spectral flow (PAPd/TR) 4
- Use of a new echo-Doppler formula for the evaluation of RVSP based on the estimation of RA pressure by means of IVCCI 3
- Consideration of the diagnostic utility of echocardiography for suspected PE, including the use of POCUS to evaluate for acute right ventricular strain 5