Department of Cardiology and Grown-up Congenital Heart Disease, ICLAS, Rapallo, GE, Italy.
Unit of Pediatric Cardiac Surgery, Gaslini Pediatric Hospital, Genoa, GE, Italy.
Cardiol Young. 2023 May;33(5):699-703. doi: 10.1017/S1047951122001494. Epub 2022 May 18.
Infradiaphragmatic partial anomalous pulmonary venous connection is occasionally diagnosed in adulthood. Management of infradiaphragmatic PAPVC depends on anatomy and clinical presentation.
Over a 10-year period, we observed seven adult patients (median age 29 years) with partial anomalous pulmonary venous connection. We classified our patients in two groups. Group I: isolated partial anomalous pulmonary venous connection from one pulmonary lobe to the inferior vena cava, three patients. Group II: partial anomalous pulmonary venous connection of the entire right lung to IVC, four patients.
The mean term follow-up was 5.4 years. Patients in Group I have been managed conservatively, as they were asymptomatic, without a significant shunt. Patients in Group II were surgically corrected using long right intra-atrial baffles. After 6 months of follow-up, the first two cases were diagnosed with complete tunnel thrombosis and loss of right lung function. Oral anticoagulation failed to recanalize the tunnel. Considering this serious complication, the other two patients were empirically and preventively treated with anticoagulation after surgery, with good outcome on long-term follow-up.
Conservative management should be considered for asymptomatic patients, without a significant shunt. Surgical treatment of infradiaphragmatic partial anomalous pulmonary venous connection of the entire right lung in inferior vena cava is challenging. Slow blood flow inside the long intra-atrial baffles inclines to thrombosis and occlusion, as we observed in two cases. Therefore, oral anticoagulation should be considered for long baffles with slow blood flow.
膈下部分性肺静脉异常连接偶尔在成年期被诊断出来。膈下 PAPVC 的处理取决于解剖结构和临床表现。
在 10 年期间，我们观察了 7 例成人患者（中位年龄 29 岁）有部分性肺静脉异常连接。我们将患者分为两组。组 I：来自一个肺叶到下腔静脉的孤立性部分性肺静脉异常连接，3 例。组 II：整个右肺到 IVC 的部分性肺静脉异常连接，4 例。
平均随访时间为 5.4 年。组 I 的患者无症状，分流不明显，因此保守治疗。组 II 的患者使用长的右房内隔障进行手术矫正。在 6 个月的随访后，前两个病例被诊断为完全隧道血栓形成和右肺功能丧失。口服抗凝剂未能再通隧道。考虑到这种严重的并发症，另外两个患者在手术后经验性和预防性地接受抗凝治疗，长期随访结果良好。
对于无症状、分流不明显的患者，应考虑保守治疗。膈下完全性右肺静脉异常连接至下腔静脉的手术治疗具有挑战性。正如我们在两个病例中观察到的那样，长的房内隔障内缓慢的血流倾向于血栓形成和闭塞。因此，对于血流缓慢的长隔障，应考虑口服抗凝剂。
