Partial anomalous pulmonary venous return in adults: Insight into pulmonary hypertension.

BACKGROUND

Partial anomalous pulmonary venous return (PAPVR) is a rare condition that may lead to pulmonary arterial hypertension (PAH). We sought to determine the prevalence of PAPVR, the follow-up rate of incidentally discovered PAPVR, the repercussions of volume and pressure overload on the right ventricle (RV), and the prevalence and predictors of PAH.

METHODS AND RESULTS

Fifty PAPVR patients aged ≥18 years were included. All underwent transthoracic echocardiography and multislice imaging. PAPVR prevalence was 0.2% (23/13,606 chest computed tomography reports). Of the 26 incidentally diagnosed patients, 14 (54%) did not have a cardiac follow-up. Seven (14%) patients had PAH, of whom one third (28%) had Eisenmenger syndrome. The left-to-right shunt ratio (Qp/Qs) was the only predictor of PAH. PAPVR-induced volume overload led to increased right chamber volumes (RV basal diameter 45±8 mm, RV mid-diameter 38±9 mm, RV/left ventricle ratio 1.2 ± 0.2, indexed RV end-diastolic area 14±4 cm/m, indexed RV end-systolic area 8.6 ± 3.2 cm/m) and increased RV functional parameters (tricuspid annular plane systolic excursion 27±5 mm, fractional area change [FAC] 42 ± 10%, global longitudinal strain [GLS] -22 ± 5). In contrast, PAH-induced pressure overload had no impact on right heart volumes, but on RV functional parameters, which were decreased compared to non-PAH patients (FAC 35 ± 13% 43 ± 10%,  = 0.049; GLS -18 ± 5 -23 ± 4%,  = 0.01).

CONCLUSIONS

PAPVR prevalence was 0.2%. When incidentally diagnosed, its management was often neglected despite potentially serious consequences (14% PAH). Only the Qp/Qs ratio was predictive of PAH. PAPVR-induced volume overload was shown to increase right heart volumes, while PAH-induced pressure overload caused alterations of RV functional parameters.