Department of Perinatology and Fetal Cardiology, Tehran University of Medical Sciences, Tehran, Iran.
Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran.
BMC Pediatr. 2023 Jul 4;23(1):337. doi: 10.1186/s12887-023-04159-y.
Pulmonary stenosis (PS) is a congenital heart diseases (CHDs) with a spectrum of stenosis. Monochorionic (MC) twins are at increased risk of CHDs, especially acquired CHDs in twin-twin transfusion syndrome (TTTS). PS/Pulmonary atresia (PA) is a rare coincidence with TTTS. MC twin pregnancies have increased in last decades due to increasing in maternal age and extensive use of assisted reproductive technologies. Therefore, attention to this group is important for heart abnormalities, especially in twins with TTTS. Multiple cardiac abnormalities in MC twins with TTTS are to be expected due to cardiac hemodynamic changes and may be eliminated by Fetoscopic laser photocoagulation treatment. Prenatal diagnosis of PS is necessary given the importance of treatment after birth.
We here present a case of coexistence of TTTS with PS in a growth restricted recipient twin who successfully treated with balloon pulmonary valvuloplasty in neonatal period. Also, we detected infundibular PS after valvuloplasty that treated with medical therapy (propranolol).
It is important to detect acquired cardiac abnormalities in MC twins with TTTS, and follow them up after birth to determine the need of intervention in neonatal period.
肺动脉瓣狭窄(PS)是一种具有多种狭窄程度的先天性心脏病(CHD)。单绒毛膜(MC)双胞胎患 CHD 的风险增加,尤其是在双胎输血综合征(TTTS)中获得性 CHD。PS/肺动脉闭锁(PA)是 TTTS 的罕见合并症。由于产妇年龄增加和辅助生殖技术的广泛应用,过去几十年来 MC 双胞胎妊娠的数量有所增加。因此,对于心脏异常,特别是在 TTTS 的双胞胎中,关注这一组非常重要。由于心脏血流动力学的变化,MC 双胞胎 TTTS 中可能会出现多种心脏异常,并且可能通过胎儿镜激光光凝治疗消除。鉴于出生后治疗的重要性,有必要对 PS 进行产前诊断。
我们在此介绍了一例 TTTS 合并 PS 的病例,生长受限的受血儿双胞胎在新生儿期成功接受了球囊肺动脉瓣成形术治疗。此外,我们在瓣环成形术后检测到漏斗部 PS,并用药物治疗(普萘洛尔)。
重要的是要检测 TTTS 的 MC 双胞胎中的获得性心脏异常,并在出生后对其进行随访,以确定新生儿期干预的必要性。