Rahnama Nour, Kubangumusu Linda, Pasquet Agnes, Robert Annie, Pouleur Anne-Catherine, Carbonez Karlien, Kefer Joelle, Moniotte Stéphane, Poncelet Alain, de Becco Geoffroy, Ghaye Benoit, Pierard Sophie
Division of Cardiology, Cliniques Universitaires Saint-Luc, Brussels, Belgium.
Division of Cardiology, Clinique Sainte-Elisabeth, Brussels, Belgium.
Int J Cardiol Congenit Heart Dis. 2022 Nov 24;11:100426. doi: 10.1016/j.ijcchd.2022.100426. eCollection 2023 Mar.
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.
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).
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.
部分性肺静脉异位回流（PAPVR）是一种罕见病症，可能导致肺动脉高压（PAH）。我们旨在确定PAPVR的患病率、偶然发现的PAPVR的随访率、容量和压力超负荷对右心室（RV）的影响以及PAH的患病率和预测因素。
纳入了50例年龄≥18岁的PAPVR患者。所有患者均接受经胸超声心动图和多层成像检查。PAPVR患病率为0.2%（23/13606份胸部计算机断层扫描报告）。在26例偶然诊断的患者中，14例（54%）未进行心脏随访。7例（14%）患者患有PAH，其中三分之一（28%）患有艾森曼格综合征。左向右分流比（Qp/Qs）是PAH的唯一预测因素。PAPVR引起的容量超负荷导致右心室腔容积增加（右心室基底直径45±8mm，右心室中间直径38±9mm，右心室/左心室比值1.2±0.2，右心室指数舒张末期面积14±4cm/m，右心室指数收缩末期面积8.6±3.2cm/m），并使右心室功能参数增加（三尖瓣环平面收缩期位移27±5mm，面积变化分数[FAC]42±10%，整体纵向应变[GLS]-22±5）。相比之下，PAH引起的压力超负荷对右心容积没有影响，但对右心室功能参数有影响，与非PAH患者相比有所降低（FAC 35±13%对43±10%，P=0.049；GLS -18±5对-23±4%，P=0.01）。
PAPVR患病率为0.2%。偶然诊断时，尽管可能有严重后果（14%患有PAH），但其管理往往被忽视。只有Qp/Qs比值可预测PAH。PAPVR引起的容量超负荷会增加右心容积，而PAH引起的压力超负荷会导致右心室功能参数改变。
