El Saiedi S A, Attia W A, Abd El-Aziz O M, Lotfy W N, Abd El-Rahim A M, Hassanein H, Qureshi S
Department of Pediatrics, Pediatric Cardiology division, Cairo University, Ali Ibrahim St., Al-Sayeda Zainab, Cairo, Egypt.
Department of Anesthesia, Cairo University, Cairo, Egypt.
Herz. 2018 Nov;43(7):633-641. doi: 10.1007/s00059-017-4606-x. Epub 2017 Aug 23.
Pulmonary atresia with intact ventricular septum (PA-IVS) is an uncommon disorder with significant morphological heterogeneity. The use of percutaneous radiofrequency (RF)-assisted perforation of the atretic valve and subsequent balloon dilation provides a relatively easy but expensive procedure that is expected to establish ante-grade flow through the pulmonary valve in most patients.
The aim of the study was to attempt a cost reduction by using catheters and wires readily available in our catheter laboratory.
A total of 50 patients presenting with PA-IVS to Cairo University Children's Hospital (CUCH) were taken to the catheterization laboratory for radiofrequency perforation using the Baylis RFP-100 generator (Baylis Medical, Montréal, Canada) or the stiff end of a coronary wire. A hybrid approach was used in selected cases.
The overall success rate for valve perforation was 92% (46 cases), 80% of which had successful primary perforation (40 cases). Success correlated with both tricuspid valve (TV) annulus and pulmonary valve (PV) annulus Z-scores, with P values of 0.2 and 0.5, respectively.
The management of PA-IVS is complex. This is a disease that necessitates a dedicated team and working collaboration between the cardiologists and cardiac surgeons. Cost limitation is essential in developing countries and innovative ideas to reduce costs are essential, especially if comparable success can be expected.
室间隔完整的肺动脉闭锁（PA-IVS）是一种罕见的疾病，具有显著的形态学异质性。使用经皮射频（RF）辅助穿通闭锁瓣膜并随后进行球囊扩张是一种相对简便但昂贵的手术，预计在大多数患者中可建立通过肺动脉瓣的前向血流。
本研究的目的是尝试通过使用我们导管室中现有的导管和导丝来降低成本。
共有50例PA-IVS患儿被送往开罗大学儿童医院（CUCH）的导管室，使用Baylis RFP-100发生器（Baylis Medical，加拿大蒙特利尔）或冠状动脉导丝的硬端进行射频穿通。在部分病例中采用了杂交方法。
瓣膜穿通的总体成功率为92%（46例），其中80%一次穿通成功（40例）。成功与三尖瓣（TV）环和肺动脉瓣（PV）环Z值均相关，P值分别为0.2和0.5。
PA-IVS的治疗很复杂。这是一种需要专业团队以及心脏病专家和心脏外科医生合作的疾病。在发展中国家，成本限制至关重要，降低成本的创新想法必不可少，尤其是在有望取得可比成功的情况下。
