Panaioli Elena, Khraiche Diala, Pontailler Margaux, Ader Flavie, Raisky Olivier, Gaudin Regis, Bonnet Damien
Unité Médico-Chirurgicale de Cardiologie Congénitale et Pédiatrique, Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C, Hôpital Universitaire Necker-Enfant-Malades, 75015 Paris, France.
Service de Biochimie Métabolique, UFCardiogénétique et Myogénétique, Département Médico-Universitaire BioGEM, APHP, Hôpital Universitaire Pitié-Salpêtrière, 75013 Paris, France.
J Cardiovasc Dev Dis. 2024 Feb 27;11(3):79. doi: 10.3390/jcdd11030079.
Dilated cardiomyopathy (DCM) is the most common childhood cardiomyopathy and is associated with considerable early mortality. Heart transplantation is often the only viable life-saving option. Pulmonary artery banding (PAB) has been recently proposed as a bridge or alternative to transplantation for DCM. In our cohort, PAB was selectively addressed to heritable DCM or DCM with congenital left ventricle aneurysm (CLVA). This study aimed to describe the clinical evolution and left ventricle reverse remodeling (LVRR) over time (6 months and 1 year after surgery). Ten patients with severe DCM received PAB between 2016 and 2021 and underwent clinical and postoperative echocardiography follow-ups. The median age at PAB was <1 year. The in-hospital mortality was zero. Two patients died two months after PAB of end-stage heart failure. The modified Ross class was improved in the eight survivors with DCM and remained stable in the two patients with CLVA. We observed a positive LVRR (LV end-diastolic diameter Z-score: 8.4 ± 3.7 vs. 2.8 ± 3; < 0.05; LV ejection fraction: 23.8 ± 5.8 to 44.5 ± 13.1 ( < 0.05)). PAB might be useful as part of the armamentarium available in infants and toddlers with severe DCM not sufficiently responding to medical treatment with limited probability of spontaneous recovery.
扩张型心肌病(DCM)是儿童期最常见的心肌病,与相当高的早期死亡率相关。心脏移植往往是唯一可行的救命选择。肺动脉环缩术(PAB)最近被提议作为DCM移植的桥梁或替代方案。在我们的队列中,PAB被选择性地应用于遗传性DCM或伴有先天性左心室动脉瘤(CLVA)的DCM。本研究旨在描述随着时间推移(手术后6个月和1年)的临床演变和左心室逆向重构(LVRR)。2016年至2021年间,10例重度DCM患者接受了PAB,并进行了临床和术后超声心动图随访。PAB时的中位年龄<1岁。住院死亡率为零。2例患者在PAB后两个月死于终末期心力衰竭。8例存活的DCM患者改良Ross分级得到改善,2例CLVA患者保持稳定。我们观察到LVRR呈阳性(左心室舒张末期直径Z评分:8.4±3.7对2.8±3;<0.05;左心室射血分数:23.8±5.8至44.5±13.1(<0.05))。对于对药物治疗反应不佳且自发恢复可能性有限的重度DCM婴幼儿,PAB可能是可用治疗手段的一部分。