A position statement by the Canadian Cardiovascular Society/Canadian Thoracic Society outlines recommendations for the detection, care, and treatment of pulmonary hypertension (PH). This statement was published in the Canadian Journal of Cardiology.
Since the publication of the previous guidelines on PH in 2005, the prevalence of this condition has drastically increased globally, from an estimated 1% to 10% among individuals ≥65 years. The phenotype of PH has evolved and now includes older patients with more comorbidities that include left heart or lung disease. With recently approved treatment options and diagnostic tools available to clinicians, the expert panel made 31 updated recommendations.
When a patient is suspected to have PH or unexplained dyspnea, the first diagnostic tool that should be used is transthoracic echocardiography and a complete echocardiographic assessment. The echocardiograph should include a systolic pulmonary artery pressure estimation using tricuspid regurgitation jet velocity, measurement of the inferior vena cava size, and degree of inspiratory collapse. Secondary signs (ie, atrial enlargement, right ventricular [RV] hypertrophy, septal flattening, or RV dysfunction) should be noted.
If a patient is suspected of pulmonary arterial hypertension (PAP) or chronic thromboembolic pulmonary hypertension (CTEPH), a right heart catheter should be used to confirm a hemodynamic diagnosis of precapillary PH.
When a patient has residual dyspnea or exercise intolerance after 3 months of anticoagulation therapy, the presence of chronic thromboembolic pulmonary hypertension should be assessed by echocardiography ventilation/perfusion lung scan.
Once PH has been established, abnormality of left-sided chambers or valves should be established by transthoracic echocardiography, as these symptoms could indicate postcapillary PH. A baseline follow-up that includes echocardiogram, tricuspid annular plane systolic excursion, and 2-dimensional speckle tracking is recommended. Patients with PH and scleroderma should also be evaluated annually for lung diffusion capacity for carbon monoxide.
Among patients with pulmonary arterial hypertension (PAH), cardiac magnetic resonance should be performed to assess RV size and function. These patients should be educated on appropriate self-management strategies, have supervised exercise sessions, and be prescribed diuretics. Patients with idiopathic PAH, heritable PAH, or drug- or toxin-induced PAH should be prescribed warfarin and undergo acute vasodilator testing. All patients with PAH should be regularly assessed.
For patients with PH due to left heart disease, ventricular filling pressure should be optimized by identifying underlying causes. For patients with PH due to lung disease or hypoxia, right heart catheterization should not be routinely used.
Among patients with chronic thromboembolic PH which is inoperable or residual, riociguat monotherapy after pulmonary endarterectomy is recommended. When pulmonary endarterectomy is not possible, a balloon pulmonary angioplasty may be considered as an alternative.
When PH is severe despite maximal medicinal therapy attempts, patients should be assessed for lung transplantation. For patients with RV failure or hemodynamic instability, extracorporeal life support should be considered.
“Future advancements in the identification of novel pathways and therapies, personalized approaches to direct therapy, as well as interventional approaches such as balloon pulmonary angioplasty for chronic thromboembolic PH promise to continue the rapid
Hirani N, Brunner N W, Kapasi A, et al. Canadian Cardiovascular Society/Canadian Thoracic Society Position Statement on Pulmonary Hypertension. Can J Cardiol. 2020;36(7):P977-922. doi:10.1016/j.cjca.2019.11.041