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Pulmonary hypertension: An update of Dx and Tx guidelines

The Journal of Family Practice. 2023 March;72(2):72-83 | doi: 10.12788/jfp.0561
March 7, 2023|Family Medicine
Madhavi Singh, MD;Franklin Berkey, DO;Jason Fragin, DO;Kristen Grine, DO
Author and Disclosure Information

Penn State College of Medicine, State College, PA
msingh1@pennstatehealth.psu.edu

The authors reported no potential conflict of interest relevant to this article.

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

When to refer

Specialty consultation21-23 is recommended for:

  • all patients with PAH
  • PH patients in clinical Groups 2 and 3 whose disease is disproportionate to the extent of their left heart disease or hypoxic lung disease
  • patients in whom there is concern about CTEPH and who therefore require early referral to a specialist for definitive treatment
  • patients in whom the cause of PH is unclear or multifactorial (ie, clinical Group 5).

What are the options for managing PH?

Management of PH is based on the cause and classification of the individual patient’s ­disease.

Treatment for WSPH Group 1

Patients require referral to a specialty clinic for diagnosis, treatment, and monitoring of progression.10

First, regrettably, none of the medications approved by the US Food and Drug Administration for treating PAH prevent progression.7

Drugs and toxins associated with pulmonary arterial hypertension include aminorex, fenfluramine, dexfenfluramine, benfluorex, methamphetamines, dasatinib, and toxic rapeseed oil.

Patients with idiopathic, hereditary, or drug-induced PAH with positive vasoreactivity are treated with a calcium channel blocker (CCB). The dosage is titrated to optimize therapy for the individual patient.

The patient is then reassessed after 3 to 6 months of medical therapy. Current treatment is continued if the following goals have been met:

  • WHO functional classification is I or II
  • BNP < 50 ng/L or NT-proBNP < 300 ng/L
  • hemodynamics are normal or near-normal (mPAP ≤ 30 mm Hg and PVR ≤ 4 WU).

If these goals have not been met, treatment is adjusted by following the algorithm described below.

Continue to: The treatment algorithm...

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