Cardiac dysfunction of pulmonary artery aneurysm in patients with pulmonary arterial hypertension.

BACKGROUND: Pulmonary artery aneurysm (PAA) occurs in some patients with pulmonary arterial hypertension (PAH). We evaluated the characteristics of cardiac dysfunction in patients with PAH complicated by PAA.

METHODS: Echocardiography and right-heart catheterization were performed in 130 PAH patients to measure the maximum dimensions of the main pulmonary artery (MPA) trunk and evaluate right ventricular (RV) and left ventricular (LV) function.

RESULTS: A diameter of >40mm was defined as PAA in the MPA trunk. The maximum MPA diameter was >40mm in 32 of 130 PAH patients (24.6%: Group PAA). Systolic pulmonary artery pressure (68.0±18.5 vs. 58.9±21.6mm Hg, P=0.0354) and pulmonary capillary wedge pressure (10.8±3.9 vs. 7.7±2.6mm Hg, P<0.0001) were significantly higher in Group PAA than in Group non-PAA. RV end-diastolic area index (19.6±6.8 vs. 14.9±5.1 cm²/m², P<0.0001), and RV fractional area change (32.3±7.8 vs. 37.3±8.8%, P=0.0048) and RV longitudinal strain (-15.4±5.1 vs. -20.4±6.1, P=0.0012) were significantly lower, and E/e' was significantly higher (8.1±2.2 vs. 6.7±1.4, P=0.0002) in Group PAA than in Group non-PAA.

CONCLUSIONS: In PAH patients with PAA, RV was larger and RV dysfunction was more severe, and LV diastolic dysfunction appeared. It is important to investigate the appearance of PAA and the severity of RV and LV dysfunction in PAH patients with PAA.