Cardiology Letters 2024, 33(5):305-315

Unveiling the hidden threat: How to diagnose pulmonary arterial hypertension in time

Milan Luknár¹, Peter Lesný¹, Bibiana Kafková², Pavel Kmeč³, Eva Gonçalvesová¹

¹ Kardiologická klinika Lekárská fakulta a Národný ústav srdcových a cievnych chorôb, a. s., Bratislava

² Východoslovenský ústav srdcových a cievnych chorôb, a. s., Košice

³ Stredoslovenský ústav srdcových a cievnych chorôb, a. s., Banská Bystrica, Slovenská republika

Pulmonary arterial hypertension (PAH) is a rare, debilitating, and often lethal condition primarily affecting the pulmonary arterioles. Vasoconstriction, remodeling, and local thrombosis lead to a reduction in the total cross-sectional area of the pre-capillary pulmonary vascular bed, resulting in increased pulmonary vascular resistance and afterload on the right ventricle (RV). This pressure overload ultimately results in RV failure. Owing to the non-specific nature of its symptoms, the diagnosis of PAH is often delayed. Early diagnosis and intervention may improve the prognosis for patients. This paper presents a practical approach to the early diagnosis of PAH and other types of pulmonary hypertension, emphasizing the importance of early referral to specialized PAH centres. The majority of patients present with progressive dyspnea. Physical examination may reveal an accentuated pulmonary second heart sound and signs of systemic congestion due to RV failure. Electrocardiograms typically show characteristics such as P pulmonale, RV hypertrophy and overload, and right bundle branch block. Chest X-ray can indicate right atrial enlargement, as well as dilation of the pulmonary artery and its branches. Elevated levels of natriuretic peptides are often observed. Echocardiography typically reveals tricuspid regurgitation with increased peak jet velocity, along with dilation and signs of dysfunction of the RV. In conclusion, PAH should be suspected in patients with unexplained dyspnea and related symptoms of RV failure, particularly when their symptomatic burden does not correlate with the degree of heart or lung lesions and persists despite standard treatments. Evidence supporting the suspicion of PAH includes electrical dominance of the right-sided heart compartments, elevated levels of natriuretic peptides, and characteristic findings on chest X-ray and echocardiography. These indicators warrant an active search for PAH. If clinical presentation and additional findings are suggestive of PAH or chronic thromboembolic pulmonary hypertension, patients should be referred to a specialized centre for consideration of right heart catheterization and further diagnostic procedures. Most cases of other types of pulmonary hypertension do not require invasive testing. The ultimate goal of these early diagnosis efforts is to improve the prognosis for patients with PAH.

Keywords: pulmonary hypertension; right ventricle; early diagnosis; electrocardiogram X-ray; echocardiography

Published: May 1, 2024  Show citation