Akintürk Hakan, Michel-Behnke Ina, Valeske Klaus, Mueller Matthias, Thul Josef, Bauer Juergen, Hagel Karl-Juergen, Schranz Dietmar
Pediatric Heart Center, Justus-Liebig University, Feulgenstrasse 12, 35385 Giessen, Germany.
Pediatr Cardiol. 2007 Mar-Apr;28(2):79-87. doi: 10.1007/s00246-006-1444-7. Epub 2007 Feb 15.
The outcome of patients with hypoplastic left heart (HLH) is determined by many factors, particularly by the first-step palliative procedure in newborns undergoing the Norwood procedure, its Sano modification, or, rarely, through challenging biventricular repairs. Duct stenting combined with bilateral pulmonary artery banding (PAB) is a new method employed as an alternative first-step approach in a number of centers worldwide. We describe this interventional-surgical "hybrid approach" as an additional strategy for the treatment of newborns with HLH syndrome and HLH complex. Between 1998 and April, 2006, 58 newborns underwent ductal stenting and bilateral PAB. These patients underwent surgical bilateral PAB initially, followed by percutaneous duct stenting; the only exception to this were patients in whom duct stenting was performed as a rescue procedure. Various balloon-expandable and self-expandable stents with different widths and lengths were used during the 8-year period of this study. Balloon dilatation of the atrial septum was performed when indicated. This included 5 patients in whom the atrial septum was stented. Aortic arch reconstruction (AAR) combined with a bidirectional cavopulmonary connection (BCPC) was performed at a median age of 4.8 months (range, 2.6-7.5), and total cavopulmonary connection (TCPC) was performed at a median age of 3.1 years (range, 2.5-4). Nine patients were listed for heart transplantation (HTX) and transplanted with AAR when a donor heart was available. Depending on growth of left ventricular structures, biventricular repair (BVR) was performed at a median age of 7.1 months (range, 3.5-10). Overall, 8 of 58 patients (13.8%) treated by the transcatheter-surgical hybrid approach died during the study period. The mortality rate for duct stenting was 1.7% (l/58), and it was 1.7% for bilateral PAB as well. Twenty-seven patients received an AAR/BCPC; 2 of them died (7.4%). Additionally, 1 of 2 patients with AAR/BCPC died while on the waiting list for HTX, resulting in a total mortality rate of 11% with an actuarial survival rate of 89%. One patient is still awaiting AAR + BCPC. Three patients died while on the waiting list for HTX despite successful bilateral PAB and duct stenting. The 30-day mortality rate for TCPC (n = 11), HTX (n = 8), and 18 patients with BVR was 0. The actuarial survival rate for patients with BVR is 93%. Postnatal transcatheter-surgical hybrid palliation expands the surgical options for newborns with HLH. Using hybrid palliation, Norwood stage I operation can be avoided in the neonatal period, the waiting period for children scheduled for cardiac transplantation can be extended, and observation for left ventricular growth suitable for biventricular repair as well.
左心发育不全（HLH）患者的预后受多种因素决定，尤其是接受诺伍德手术、其佐野改良术的新生儿的第一步姑息治疗，或者很少见地通过具有挑战性的双心室修复手术。导管支架置入联合双侧肺动脉环扎术（PAB）是全球多个中心采用的一种新的替代第一步治疗方法。我们将这种介入性手术“杂交方法”描述为治疗HLH综合征和HLH复合体新生儿的一种额外策略。1998年至2006年4月期间，58例新生儿接受了导管支架置入和双侧PAB。这些患者最初接受了手术双侧PAB，随后进行经皮导管支架置入；唯一的例外是那些将导管支架置入作为抢救程序的患者。在本研究的8年期间使用了各种不同宽度和长度的球囊扩张型和自膨胀型支架。在有指征时进行房间隔球囊扩张。这包括5例房间隔置入支架的患者。主动脉弓重建（AAR）联合双向腔肺连接（BCPC）在中位年龄4.8个月（范围2.6 - 7.5）时进行，全腔肺连接（TCPC）在中位年龄3.1岁（范围2.5 - 4）时进行。9例患者被列入心脏移植（HTX）名单，在有供体心脏时接受了AAR移植。根据左心室结构的生长情况，双心室修复（BVR）在中位年龄7.1个月（范围3.5 - 10）时进行。总体而言，在研究期间，采用经导管 - 手术杂交方法治疗的58例患者中有8例（13.8%）死亡。导管支架置入的死亡率为1.7%（1/58），双侧PAB的死亡率也为1.7%。27例患者接受了AAR/BCPC；其中2例死亡（7.4%）。此外，2例AAR/BCPC患者中有1例在等待HTX名单上时死亡，导致总死亡率为11%，精算生存率为89%。1例患者仍在等待AAR + BCPC。3例患者尽管双侧PAB和导管支架置入成功，但在等待HTX名单上时死亡。TCPC（n = 11）、HTX（n = 8）和18例BVR患者的30天死亡率为0。BVR患者的精算生存率为93%。产后经导管 - 手术杂交姑息治疗扩展了HLH新生儿的手术选择。使用杂交姑息治疗，可以避免新生儿期的诺伍德I期手术，可以延长计划进行心脏移植儿童的等待期，还可以观察适合双心室修复的左心室生长情况。
