Youssef David Edward, Averin Konstantin, Richards Susan, Sheppard Catherine, Seaman Cameron, Pietrosanu Matthew, Bates Angela
Division of Pediatrics, Department of Pediatric Pulmonary Hypertension, Stollery Children's Hospital, Edmonton, AB, Canada.
Division of Pediatric Cardiology, Department of Pediatrics, University of Alberta, Edmonton, AB, Canada.
Front Pediatr. 2023 Jan 25;11:1073336. doi: 10.3389/fped.2023.1073336. eCollection 2023.
The clinical deterioration commonly experienced by pediatric patients with pulmonary arterial hypertension (PAH) has motivated a shift in the treatment of pulmonary hypertension (PH) through innovations in surgical salvage interventions. The Occlutech fenestrated atrial septal defect (FASD) Occluder and the atrial flow regulator (AFR), which provides a protective, atrial-level shunt during hypertensive crises, have found an important role in treating pediatric patients with PAH. Other groups of pediatric patients with PH may also benefit from a similar protective physiology. The primary aim of this work is to present a single center's experience with AFR and FASD devices for managing a heterogeneous group of pediatric PH patients. A secondary goal is to identify hemodynamic changes and complications following device implantation.
We performed a retrospective review of all pediatric PH patients who, after being found suitable, either successfully or unsuccessfully received an FASD or AFR device between January 2015 and December 2021 at the Stollery Children's Hospital in Edmonton, Canada.
Fourteen patients (eight female) with a median age of 4.6 (range 0.3-17.9) years and a median body mass index of 15.1 (Q = 13.8, Q = 16.8) kg/m underwent device implantation: five received FASDs, eight received AFRs, and one was ultimately unable to receive an implant due to thrombosed iliac vessels and required surgical intervention. Of the fourteen patients, seven were in group 1 (PAH), one was in group 3 (lung disease), and six were in group 5 (primarily pulmonary hypertension vascular disease) under the World Symposium PH classification. All patients were on mono-, dual-, or triple-drug PH therapy. Device stabilization was not possible for two patients, who then required a repeat catheterization. Of the group 1 patients, three AFR and three FASD implants were successful, while one FASD implant was unsuccessful due to thrombosed vessels. At a six-month clinical assessment, all group 1 patients had patent devices and improved WHO FCs.
This work presents a single center's experience with AFR and FASD implants in a heterogeneous group of fourteen pediatric patients with severe PH. This treatment strategy is novel in the pediatric population and so this work provides momentum for future studies of interventional cardiac catheterization procedures for pediatric patients with PH. Further collaborations are required to develop criteria to identify ideal pediatric candidates and optimally time interventions in order to maximize the benefits of this treatment.
患有肺动脉高压(PAH)的儿科患者常见的临床病情恶化促使通过手术挽救干预措施的创新来改变肺动脉高压(PH)的治疗方法。Occlutech开窗房间隔缺损(FASD)封堵器和心房血流调节器(AFR)在高血压危象期间可提供保护性的心房水平分流,在治疗患有PAH的儿科患者中发挥了重要作用。其他患有PH的儿科患者群体可能也会从类似的保护生理机制中受益。这项工作的主要目的是介绍一个单一中心使用AFR和FASD装置治疗一组异质性儿科PH患者的经验。次要目标是确定装置植入后的血流动力学变化和并发症。
我们对2015年1月至2021年12月期间在加拿大埃德蒙顿斯托利儿童医院被判定适合后成功或未成功接受FASD或AFR装置的所有儿科PH患者进行了回顾性研究。
14例患者(8例女性)接受了装置植入,中位年龄为4.6岁(范围0.3 - 17.9岁),中位体重指数为15.1(Q₁ = 13.8,Q₃ = 16.8)kg/m²:5例接受了FASD封堵,8例接受了AFR植入,1例因髂血管血栓形成最终未能接受植入,需要手术干预。在这14例患者中,根据世界PH研讨会分类,7例属于第1组(PAH),1例属于第3组(肺部疾病),6例属于第5组(主要是肺动脉高压血管疾病)。所有患者均接受单药、双药或三药PH治疗。2例患者无法实现装置稳定,随后需要再次进行心导管检查。在第1组患者中,3例AFR植入和3例FASD植入成功,而1例FASD植入因血管血栓形成未成功。在6个月的临床评估中,所有第1组患者的装置均通畅,世界卫生组织功能分级(WHO FC)得到改善。
这项工作介绍了一个单一中心对14例患有严重PH的异质性儿科患者进行AFR和FASD植入的经验。这种治疗策略在儿科人群中是新颖的,因此这项工作为未来对患有PH的儿科患者进行介入性心导管检查程序的研究提供了动力。需要进一步合作来制定标准,以识别理想的儿科候选者并优化干预时机,从而使这种治疗的益处最大化。