Pulmonary artery aneurysm associated with cardiac dysfunction in PAH
medwireNews: Study results suggest that pulmonary artery aneurysm (PAA) is associated with cardiac dysfunction among patients with pulmonary arterial hypertension (PAH).
Konomi Sakata and colleagues, from Kyorin University School of Medicine in Tokyo, Japan, used echocardiography and right-heart catheterization to measure the dimensions of the main pulmonary artery (MPA) and evaluate ventricular function.
They found that PAA – defined as a dilation of the MPA artery trunk to a diameter of more than 40 mm – occurred in 24.6% of 130 patients. PAH severity was greater in patients with PAA than without, as indicated by significantly elevated mean pulmonary artery pressure (PAP), systolic PAP, and mean right atrial pressure, at 43.1 versus 36.4 mmHg, 68.0 versus 58.9 mmHg, and 10.0 versus 5.4 mmHg, respectively.
Patients with PAA also had poorer right ventricular (RV) function; RV end-diastolic area index was measured at 19.6 cm2/m2 in patients with PAA compared with 14.9 cm2/m2 in those with no PAA, a significant difference.
RV fractional area change and RV longitudinal strain were significantly lower in patients with PAA (32.3 vs 37.3% and –15.4 vs –20.4%, respectively), whereas moderate or severe pulmonary regurgitation or tricuspid regurgitation were more prevalent in the PAA group than the non-PAA group (93.7 vs 11.2% and 53.1 vs 20.6%, respectively).
“The deterioration of RV function and RV dilation were more severe in Group PAA compared to those in Group non-PAA,” explain the researchers in the International Journal of Cardiology.
Cardiac output was significantly impaired in patients with PAA compared with those without, at 4.3 versus 4.9 L/min.
Although the authors found no significant difference in left ventricular (LV) ejection fraction between the two groups, the septal, lateral, and mean ratio of transmitral flow velocity to annulus velocity were significantly higher among patients with than without PAA.
“LV systolic function did not decrease, but LV diastolic function was impaired in Group PAA,” observe the authors.
Over 3 years of follow-up, 31% of patients with PAA and 9% of patients without PAA died. The team notes that one patient with PAA died due to acute coronary syndrome caused by compression of the left main coronary artery (LMCA), whereas the other patients died of right-sided heart failure.
Sakata and team caution that “it is difficult to distinguish the symptoms of severe PAH and those of angina due to extrinsic compression of the LMCA,” and note that they were unable to detect chronic myocardial ischemia in patients with PAA before acute coronary syndrome onset as cardiac stress testing and nuclear imaging were not performed.
And they conclude: “It is important to evaluate the presence of PAA using echocardiography in order to diagnose high-risk [pulmonary hypertension] patients with mechanical complications and severe RV and LV dysfunction.”
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