Schranz Dietmar, Krause Ulrich, Kerst Gunter, Esmaeili Anoosh, Paul Thomas
Pediatric Cardiology, Children's Hospital, Johann Wolfgang Goethe University, Theodor-Stern-Kai 7, Frankfurt D-60596, Germany.
Department of Pediatric Cardiology, Georg-August-University Medical Center, Göttingen, Germany.
Eur Heart J Case Rep. 2023 Jan 31;7(2):ytad052. doi: 10.1093/ehjcr/ytad052. eCollection 2023 Feb.
Dilated cardiomyopathy (DCM) is a leading cause of heart transplantation (HTx) in children. Surgical pulmonary artery banding (PAB) is used worldwide to achieve functional heart regeneration and remodelling.
We report for the first-time successful bilateral transcatheter implantation of bilateral pulmonary artery flow restrictors in a case series of three infants with severe DCM based on left-ventricular non-compaction morphology associated with Barth syndrome in one and a non-classified syndrome in another. Functional cardiac regeneration was observed in two patients after almost 6 months of endoluminal banding, and in the neonate with Barth syndrome already after 6 weeks. Accompanied by an improvement in functional class (Class IV to Class I), the left ventricular end-diastolic dimensions -score normalized, as did the elevated serum brain natriuretic peptide levels. A listing for HTx could be avoided.
Percutaneous bilateral endoluminal PAB is a novel minimally invasive approach that enables functional cardiac regeneration in infants with severe DCM and preserved right ventricular function. Interruption of the ventriculo-ventricular interaction, the key mechanism for recovery, is avoided. Intensive care for these critically ill patients is reduced to a minimum. However, investing in 'heart regeneration to avoid transplantation' remains a challenge.
扩张型心肌病（DCM）是儿童心脏移植（HTx）的主要原因。外科肺动脉环扎术（PAB）在全球范围内用于实现功能性心脏再生和重塑。
我们首次报告了在一组3例严重DCM婴儿中成功进行双侧经导管植入双侧肺动脉血流限制器的病例，其中1例与Barth综合征相关的左心室心肌致密化不全形态，另1例为未分类综合征。在腔内环扎近6个月后，2例患者观察到功能性心脏再生，而患有Barth综合征的新生儿在6周后就已出现。随着功能分级的改善（从IV级到I级），左心室舒张末期尺寸评分恢复正常，血清脑钠肽水平升高的情况也得到改善。从而避免了列入心脏移植名单。
经皮双侧腔内PAB是一种新型的微创方法，可使患有严重DCM且右心室功能保留的婴儿实现功能性心脏再生。避免了心室-心室相互作用的中断，而这是恢复的关键机制。对这些危重症患者的重症监护降至最低限度。然而，投资于“心脏再生以避免移植”仍然是一项挑战。
