Pediatric Heart Center, Justus-Liebig University, Giessen, Germany.
Pediatric Heart Center, Justus-Liebig University, Giessen, Germany.
J Heart Lung Transplant. 2014 May;33(5):542-6. doi: 10.1016/j.healun.2014.01.860. Epub 2014 Jan 24.
The surgical creation of a Potts shunt has been reported in children with suprasystemic idiopathic pulmonary artery (PA) hypertension (IPAH) refractory to any medical therapy. This surgical approach allows acute decompression of the right ventricle (RV) and thereby avoids ventricular failure. We present 4 newborns and infants with different causes of pulmonary hypertension (PH) in whom interventional stenting of the patent ductus arteriosus (PDA) was used to create a "functional" Potts shunt.
Suprasystemic PH was diagnosed by cardiac catheterization in 2 newborns with complex left heart obstructive lesions, a patient with persistent PH of the newborn (PPHN), and an infant with IPAH and was accompanied by RV dilation and imminent RV failure.
Stenting of the PDA was performed uneventfully during cardiac catheterization in all patients and led to stabilization of clinical symptoms. The 2 patients with complex cardiac lesions subsequently underwent successful biventricular repair. In the PPHN patient, the stented PDA was closed interventionally when PA pressures dropped and a significant left-to-right shunt occurred. PA pressures in the patient with IPAH remained high while the stented PDA still served as RV decompression in avoidance of lung transplantation.
Newborns and infants with suprasystemic PH of varying etiologies may benefit from the creation of a functional Potts shunt by stenting the PDA. This strategy should be considered in patients with suprasystemic IPAH or PPHN as a temporary or permanent therapy when a PDA can be identified at cardiac catheterization. It may also be beneficial in patients with PH due to left heart obstructions by serving as a bridge to further corrective surgery.
对于任何药物治疗均无效的系统性肺动脉高压（PA）的儿童，已有外科创建波氏分流术的报道。该手术方法可使右心室（RV）迅速减压，从而避免心室衰竭。我们报告了 4 例因不同原因导致肺动脉高压（PH）的新生儿和婴儿，他们通过介入性动脉导管未闭（PDA）支架置入术来创建“功能性”波氏分流术。
在 2 例患有复杂左心梗阻性病变的新生儿、1 例持续性新生儿 PH（PPHN）患者和 1 例患有特发性 PAH 的婴儿中，通过心导管检查诊断出系统性 PH，同时伴有 RV 扩张和即将发生的 RV 衰竭。
所有患者在心导管检查期间均顺利进行了 PDA 支架置入术，并使临床症状稳定。随后，2 例患有复杂心脏病变的患者成功地进行了双心室修复。在 PPHN 患者中，当 PA 压力下降并出现明显左向右分流时，经介入干预关闭了支架置入的 PDA。在患有 IPAH 的患者中，PA 压力仍然很高，而支架置入的 PDA 仍然作为 RV 减压的方式，避免了肺移植。
患有不同病因的系统性 PH 的新生儿和婴儿可能受益于通过支架置入 PDA 来创建功能性波氏分流术。当在心导管检查时可以识别到 PDA 时，这种策略应考虑用于患有系统性 IPAH 或 PPHN 的患者，作为临时或永久性治疗。对于因左心梗阻导致 PH 的患者，通过作为进一步矫正手术的桥梁，也可能有益。
