Abstract：
BACKGROUND: Isolated partial anomalous pulmonary venous connection (PAPVC) is difficult to diagnose, and surgical indications remain controversial. We reviewed 10 y of isolated PAPVC cases.
METHODS: The data of patients with isolated PAPVC admitted to the Anzhen Congenital Heart Disease Department from 2010 to 2019 were reviewed retrospectively.
RESULTS: Thirty patients, aged between 4 mo and 32 y, were included in this study. Significant correlations were found between the right ventricle (RV), end-diastolic dimension Z-score (RVED-z) and age (r = 0.398, P = 0.03), and between estimated pulmonary pressure and age (r = 0.423, P = 0.02). However, no significant correlations were found between the RVED-z and the number of anomalous pulmonary veins (r = 0.347, P = 0.061), between estimated pulmonary pressure and the RVED-z (r = 0.218, P = 0.248), and between estimated pulmonary pressure and the number of anomalous veins (r = 0.225, P = 0.232). Transthoracic echocardiography (TTE) confirmed 90% of isolated PAPVC cases. Surgical repair was performed in 29 patients with RV enlargement, persistent low weight, pulmonary hypertension, or respiratory symptoms. Among the surgical patients, nine had elevated pulmonary pressure before surgery, which decreased postoperatively; no mortality or reintervention was observed. The mean duration of echocardiographic follow-up was 1.9 y.
CONCLUSIONS: TTE is recommended for routine assessments, and further clarification can be obtained with computed tomography when TTE proves inconclusive for diagnosis. Transesophageal echocardiography and computed tomography are further recommended for adult patients if TTE fails to provide clear results. PAPVC should be considered as an underlying cause when unexplained RV enlargement is observed. Surgery is recommended for patients with RV enlargement, pulmonary hypertension, or respiratory symptoms.
METHODS: The data of patients with isolated PAPVC admitted to the Anzhen Congenital Heart Disease Department from 2010 to 2019 were reviewed retrospectively.
RESULTS: Thirty patients, aged between 4 mo and 32 y, were included in this study. Significant correlations were found between the right ventricle (RV), end-diastolic dimension Z-score (RVED-z) and age (r = 0.398, P = 0.03), and between estimated pulmonary pressure and age (r = 0.423, P = 0.02). However, no significant correlations were found between the RVED-z and the number of anomalous pulmonary veins (r = 0.347, P = 0.061), between estimated pulmonary pressure and the RVED-z (r = 0.218, P = 0.248), and between estimated pulmonary pressure and the number of anomalous veins (r = 0.225, P = 0.232). Transthoracic echocardiography (TTE) confirmed 90% of isolated PAPVC cases. Surgical repair was performed in 29 patients with RV enlargement, persistent low weight, pulmonary hypertension, or respiratory symptoms. Among the surgical patients, nine had elevated pulmonary pressure before surgery, which decreased postoperatively; no mortality or reintervention was observed. The mean duration of echocardiographic follow-up was 1.9 y.
CONCLUSIONS: TTE is recommended for routine assessments, and further clarification can be obtained with computed tomography when TTE proves inconclusive for diagnosis. Transesophageal echocardiography and computed tomography are further recommended for adult patients if TTE fails to provide clear results. PAPVC should be considered as an underlying cause when unexplained RV enlargement is observed. Surgery is recommended for patients with RV enlargement, pulmonary hypertension, or respiratory symptoms.
摘要：
背景：孤立的部分异常肺静脉连接（PAPVC）难以诊断，和手术适应症仍然存在争议。我们回顾了10年分离的PAPVC病例。
方法：回顾性分析2010-2019年安贞县先心病科收治的孤立性PAPVC患者资料。
结果：30名患者，年龄在4个月到32岁之间，包括在这项研究中。发现右心室（RV）之间存在显着相关性，舒张末期维度Z评分（RVED-z）和年龄（r=0.398，P=0.03），在估计的肺压和年龄之间（r=0.423，P=0.02）。然而,RVED-z与异常肺静脉数之间无显著相关性(r=0.347,P=0.061),在估计的肺压和RVED-z之间（r=0.218，P=0.248），在估计的肺压和异常静脉数量之间（r=0.225，P=0.232）。经胸超声心动图（TTE）证实了90％的孤立PAPVC病例。29例RV扩大患者进行了手术修复，持久的低体重，肺动脉高压,或呼吸道症状。在手术患者中，9人在手术前肺压升高,术后下降;没有观察到死亡率或再干预。超声心动图随访的平均持续时间为1.9y。
结论：建议将TTE用于常规评估，当TTE无法确定诊断时，可以通过计算机断层扫描获得进一步的澄清。如果TTE不能提供明确的结果，则进一步建议成人患者使用经食道超声心动图和计算机断层扫描。当观察到无法解释的RV增大时，PAPVC应被视为根本原因。对于RV增大的患者建议手术,肺动脉高压,或呼吸道症状。
方法：回顾性分析2010-2019年安贞县先心病科收治的孤立性PAPVC患者资料。
结果：30名患者，年龄在4个月到32岁之间，包括在这项研究中。发现右心室（RV）之间存在显着相关性，舒张末期维度Z评分（RVED-z）和年龄（r=0.398，P=0.03），在估计的肺压和年龄之间（r=0.423，P=0.02）。然而,RVED-z与异常肺静脉数之间无显著相关性(r=0.347,P=0.061),在估计的肺压和RVED-z之间（r=0.218，P=0.248），在估计的肺压和异常静脉数量之间（r=0.225，P=0.232）。经胸超声心动图（TTE）证实了90％的孤立PAPVC病例。29例RV扩大患者进行了手术修复，持久的低体重，肺动脉高压,或呼吸道症状。在手术患者中，9人在手术前肺压升高,术后下降;没有观察到死亡率或再干预。超声心动图随访的平均持续时间为1.9y。
结论：建议将TTE用于常规评估，当TTE无法确定诊断时，可以通过计算机断层扫描获得进一步的澄清。如果TTE不能提供明确的结果，则进一步建议成人患者使用经食道超声心动图和计算机断层扫描。当观察到无法解释的RV增大时，PAPVC应被视为根本原因。对于RV增大的患者建议手术,肺动脉高压,或呼吸道症状。
