Mariucci Elisabetta, Guidarini Marta, Bartolacelli Ylenia, Tchana Bertrand, Careddu Lucio, Gargiulo Gaetano, Esposito Susanna Maria Roberta, Donti Andrea
Pediatric Cardiology and Adult Congenital Heart Disease Program, Department of Cardio-Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 9 Massarenti Street, 40138, Bologna, Italy.
Cardiologia Pediatrica, Azienda Ospedaliero-Universitaria di Parma, 14 Gramsci Street, 43126, Parma, Italy.
Int J Cardiol Congenit Heart Dis. 2022 Apr 28;8:100385. doi: 10.1016/j.ijcchd.2022.100385. eCollection 2022 Jun.
Currently there are no data regarding medical therapy of aortic dilatation in pediatric patients with normally functioning bicuspid aortic valve (BAV). Aim of the study was to describe the rates of change of aortic root diameters in untreated pediatric patients with normally functioning BAV and in patients with documented progressive dilatation treated with medical therapy.
Retrospective analysis performed on 191 pediatric patients with normally functioning BAV followed from 2005 to 2021 with serial examinations.
Aortic root dilatation was observed in 46.3% of patients, was mainly localized at the proximal ascending aorta and judged mild. After a mean follow-up of 3.7 ± 2.7 years among 175 untreated patients 52.6% presented a new onset or progressive aortic root dilatation ("progressive") while 47.4% presented normal and stable aortic diameters. Eight percent of untreated patients with a mild aortic dilatation at baseline presented a normalization of aortic diameters. "Progressive" patients presented more frequently a BAV with a raphe (73.9% vs 57.8%, p = .037) and a mild aortic regurgitation (76% vs 45.8%, p = .00007). Thirty "progressive" patients were treated with medical therapy. After a mean follow-up of 3.3 ± 2.3 years no significant differences were observed between aortic root z score progression in "stable", "progressive" and "treated" patients.
In a small cohort of patients with normally functioning BAV a raphe and a mild regurgitation are common in progressive aortic dilatation. Medical therapy didn't affect aortic dilatation in patients with progressive and mild dilatation. A randomized controlled trial is needed.
目前尚无关于正常功能的二叶式主动脉瓣（BAV）小儿患者主动脉扩张药物治疗的数据。本研究的目的是描述未经治疗的正常功能BAV小儿患者以及接受药物治疗的有记录的进行性扩张患者的主动脉根部直径变化率。
对191例2005年至2021年期间接受连续检查的正常功能BAV小儿患者进行回顾性分析。
46.3%的患者观察到主动脉根部扩张，主要位于升主动脉近端，且判断为轻度。在175例未经治疗的患者中，平均随访3.7±2.7年后，52.6%出现新发或进行性主动脉根部扩张（“进行性”），而47.4%的患者主动脉直径正常且稳定。8%基线时轻度主动脉扩张的未经治疗患者主动脉直径恢复正常。“进行性”患者更常出现带有嵴的BAV（73.9%对57.8%，p = 0.037）和轻度主动脉瓣反流（76%对45.8%，p = 0.00007）。30例“进行性”患者接受了药物治疗。平均随访3.3±2.3年后，“稳定”、“进行性”和“接受治疗”患者的主动脉根部z评分进展之间未观察到显著差异。
在一小群正常功能BAV患者中，嵴和轻度反流在进行性主动脉扩张中很常见。药物治疗对进行性和轻度扩张患者的主动脉扩张没有影响。需要进行一项随机对照试验。
