Biçer Bilgehan Betül, Aykan Hayrettin Hakan, Karagöz Tevfik, Tutar Ercan
Hacettepe University Faculty of Medicine, Department of Pediatric Cardiology, Ankara, Türkiye.
Hacettepe University, Life Support Center, Ankara, Türkiye.
Cardiol Young. 2025 Jun 27:1-4. doi: 10.1017/S1047951125001714.
In the presence of fetal cardiomegaly, when there is no cardiac malformation or dysfunction, systemic or pulmonary arteriovenous malformations that may cause volume loading should be sought. We aimed to present a fetus who had cardiomegaly and left pulmonary artery-left atrial fistula and who underwent transcatheter closure in the early postnatal period.
23-week fetus referred because of severe cardiomegaly on screening obstetric ultrasonography. Fetal echocardiography revealed fistulous connection between dilated left pulmonary artery and left atrium with high velocity continuous flow at the left atrial orifice of fistula and retrograde flow from the ductus arteriosus to the pulmonary artery. Initially, the fetus followed by one-to-two weeks intervals for fetal heart failure and hydrops fetalis. Pregnancy was uneventful and the baby was born by caesarean section at 37 weeks, and oxygen saturation level was 95 %. Transthoracic echocardiography confirmed the prenatal diagnosis of a fistula between the left pulmonary artery and the left atrium (CTA showed left lung aplasia. Transcatheter closure was performed from antegrade route with Amplatzer Piccolo® Duct Occluder due to hypoxaemia. The baby showed normal growth and development at 15 months of ageThere is no pulmonary hypertension during the 15-month follow-up.
Pulmonary artery-to-left atrial fistula is a rare anomaly and is frequently described between the right pulmonary artery and the left atrium. Presentation of age depends on the size of the fistulous connection. Patients with large connections are presented in fetal age with cardiomegaly and heart failure or presented in early infancy with profound cyanosis. Although lung hypoplasia has been reported in patients with pulmonary artery-to-left atrial fistula/connection lung aplasia has never been reported in these patients. Surgical or transcatheter closure can be achieved successfully in these patients at neonatal period or early infancy like in our case.
在胎儿存在心脏肥大的情况下，若不存在心脏畸形或功能障碍，则应寻找可能导致容量负荷增加的体循环或肺动静脉畸形。我们旨在介绍一名患有心脏肥大和左肺动脉 - 左心房瘘且在出生后早期接受经导管封堵术的胎儿。
一名23周的胎儿因产科超声筛查发现严重心脏肥大而转诊。胎儿超声心动图显示扩张的左肺动脉与左心房之间存在瘘管连接，瘘管左心房开口处有高速连续血流，且动脉导管有逆向血流至肺动脉。最初，该胎儿每隔一到两周因胎儿心力衰竭和胎儿水肿接受检查。孕期顺利，婴儿在37周时通过剖宫产出生，血氧饱和度为95%。经胸超声心动图证实了产前诊断的左肺动脉与左心房之间的瘘管（CTA显示左肺发育不全）。由于低氧血症，采用Amplatzer Piccolo® 动脉导管封堵器经顺行途径进行经导管封堵。婴儿在15个月大时生长发育正常，在15个月的随访期间未出现肺动脉高压。
肺动脉至左心房瘘是一种罕见的异常情况，常见于右肺动脉与左心房之间。发病年龄取决于瘘管连接的大小。瘘管连接大的患者在胎儿期表现为心脏肥大和心力衰竭，或在婴儿早期表现为严重青紫。虽然肺动脉至左心房瘘/连接的患者中曾有肺发育不全的报道，但这些患者中从未有过肺发育不全的报道。像我们的病例一样，在新生儿期或婴儿早期对这些患者成功进行手术或经导管封堵是可行的。
