Pulmonary hypertension: An update of Dx and Tx guidelines
Here is how to reduce risk factors that can lead to pulmonary hypertension; play a pivotal role in diagnosis; and know when disease requires a referral.
PRACTICE RECOMMENDATIONS
› Employ echocardiography as the first-line diagnostic test when pulmonary hypertension (PH) is suspected. C
› Order a ventilation– perfusion scan in patients with unexplained PH to exclude chronic thromboembolic PH. C
› Order lung function testing with diffusion capacity for carbon monoxide as part of the initial evaluation of PH. C
› Use right heart catheterization to confirm the diagnosis of pulmonary arterial hypertension. C
Strength of recommendation (SOR)
A Good-quality patient-oriented evidence
B Inconsistent or limited-quality patient-oriented evidence
C Consensus, usual practice, opinion, disease-oriented evidence, case series
Once approximately 30% of the pulmonary vasculature is involved, pressure in the pulmonary circulation starts to rise. In all WSPH groups, this increase in PVR results in increased right ventricular afterload that, over time, leads to right ventricular dysfunction.7,11,12
How does PH manifest?
Patients who have PH usually present with dyspnea, fatigue, chest pain, near-syncope, syncope, or lower-extremity edema, or any combination of these symptoms. The nonspecificity of presenting symptoms can lead to a delay in diagnosis.
In addition, suspicion of PH should be raised when a patient:
- presents with skin discoloration (light or dark) or a telangiectatic rash
- presents with difficulty swallowing
- has a history of connective tissue disease or hemolytic anemia
- has risk factors for HIV infection or liver disease
- takes an appetite suppressant
- has been exposed to other toxins known to increase the risk of PH.
A detailed medical history—looking for chronic lung or heart disease, thromboembolism, sleep-disordered breathing, a thyroid disorder, chronic renal failure, or a metabolic disorder—should be obtained.
Common findings on the physical exam in PH include:
- an increased P2 heart sound (pulmonic closure)
- high-pitched holosystolic murmur from tricuspid regurgitation
- pulmonic insufficiency murmur
- jugular venous distension
- hepatojugular reflux
- peripheral edema.
These findings are not specific to PH but, again, their presence warrants consideration of PH.
How best to approach evaluation and diagnosis?
The work-up for PH is broad; FIGURE 113,14 provides an outline of how to proceed when there is a concern for PH. For the work-up of symptoms and signs listed earlier, chest radiography and electrocardiography are recommended.
Continue to: Radiographic findings
