Signs of Heart Failure in PAH Patients May Be Evidence of Pulmonary Artery Aneurysm

Joana Fernandes, PhD avatar

by Joana Fernandes, PhD |

Share this article:

Share article via email
Pulmonary thromboendarterectomy

Almost one-fourth of pulmonary arterial hypertension (PAH) patients in a small recent study had pulmonary artery aneurysm (PAA), a condition marked by serious heart complications. Its researchers recommend that PAH patients with signs of heart failure be screened for aneurysms.

The study, “Cardiac Dysfunction Of Pulmonary Artery Aneurysm In Patients With Pulmonary Arterial Hypertension,” was published in the International Journal of Cardiology.

PAA occurs when the pulmonary arterial trunk dilates (or widens, diameter above 40 mm), affecting blood flow to and from the lungs. Although rare, it can develop as a result of PAH.

“PAAs are considered a consequence of severe [PAH], but the effects of PAAs on left and right heart function in patients with [PAH] have not been studied,” the researchers wrote.

Through echocardiography and right-heart catheterization, researchers measured the maximum dimensions of the main pulmonary artery (MPA) trunk, and evaluated right ventricular and left ventricular function in 130 PAH patients. This allowed them to study cardiac dysfunction associated with PAA in PAH patients.

Of the initial group of patients, 32 (24.6 percent) were found to have both PAH and PAA.

Results indicated that these patients presented significantly higher systolic pulmonary artery pressure (68.0 vs. 58.9 mm Hg) and pulmonary capillary wedge pressure (10.8 vs. 7.7 mm Hg) compared to those with PAH alone.

Patients with both PAA and PAH also showed signs of right ventricle enlargement and deterioration, evidenced by significantly lower values of right ventricle end-diastolic area index, fractional area change and longitudinal strain, and significantly higher E/e′ (a measure of cardiac event risk, 8.1 vs. 6.7) compared to the other group.

Left ventricle diastolic function was also poorer in the PAA group, increasing their chances of a heart attack or other cardiac event.

Ten PAA/PAH patients (31 percent) and seven with PAH died during the study, of causes largely due to right-sided heart failure.

“In PAH patients with PAA, [right ventricle] dysfunction was more severe and [left ventricle] diastolic dysfunction was observed,” the researchers concluded. “It is important to evaluate the presence of PAA using echocardiography in order to diagnose high-risk [PAH] patients with mechanical complications and severe [right and left ventricles] dysfunction.”


A Conversation With Rare Disease Advocates