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连续100例Ross手术的中期结果:生存率良好,但仍未达到治愈效果。

Mid-term Outcome of 100 Consecutive Ross Procedures: Excellent Survival, But Yet to Be a Cure.

作者信息

Zimmermann Corina, Attenhofer Jost Christine, Prêtre René, Mueller Christoph, Greutmann Matthias, Seifert Burkhardt, Valsangiacomo Büchel Emanuela, Kretschmar Oliver, Dave Hitendu Hasmukhlal, Weber Roland

机构信息

Pediatric Heart Centre, University Children's Hospital Zurich, Zurich, Switzerland.

Children's Research Centre, University of Zurich, Zurich, Switzerland.

出版信息

Pediatr Cardiol. 2018 Mar;39(3):595-603. doi: 10.1007/s00246-017-1798-z. Epub 2018 Jan 12.

Abstract

The Ross procedure offers excellent short-term outcome but the long-term durability is under debate. Reinterventions and follow-up of 100 consecutive patients undergoing Ross Procedure at our centre (1993-2011) were analysed. Follow-up was available for 96 patients (97%) with a median duration of 5.3 (0.1-17.1) years. Median age of the patient cohort was 15.2 (0.04-58.4) years with 76 males. 93% had underlying congenital aortic stenosis. Root replacement technique was applied in all. The most common valved conduits used for reconstruction of the right ventricular outflow tract were homografts (66 patients) and bovine jugular vein (Contegra) graft (31 patients). Additional procedures included Ross-Konno procedure (14%), resection of subaortic stenosis/myectomy (11%) and reduction plasty of the ascending aorta (25%). One patient died within the first 30 days (1%). Late deaths occurred in 4 patients (4%) 0.5-4.5 years postoperatively: causes included pulmonary hypertension due to endocardial fibroelastosis (2), subarachnoid haemorrhage (1) and sudden cardiac death (1). Five-year survival was 93.6 (95% CI 88.1-99.1)%. Moderate or severe aortic (autograft) regurgitation needing reoperation occurred in 8 patients with a 5-year freedom from autograft reoperation of 98.5 (95.6-100)%. Five-year freedom from reintervention (surgery or catheter based) on the right ventricular outflow tract conduit was 91.5 (85.5-96.5)%. Univariate predictors of this reinterventions were smaller graft size (p = 0.03) and use of a Contegra graft (p = 0.04). Ross procedure can be performed with low mortality and good survival in the long term. Most of the reinterventions are related to the neo-right ventricular outflow tract and may be partly attributed to the lack of growth. While the Ross Procedure remains an invaluable option for aortic valve disease in children, new solutions for the neo-pulmonary valve as well as for the less often occurring problems on the autograft are needed.

摘要

罗斯手术的短期效果极佳,但长期耐久性仍存在争议。我们分析了在本中心接受罗斯手术的100例连续患者(1993 - 2011年)的再次干预情况及随访结果。96例患者(97%)获得随访,中位随访时间为5.3(0.1 - 17.1)年。患者队列的中位年龄为15.2(0.04 - 58.4)岁,男性76例。93%的患者患有先天性主动脉狭窄。均采用了根部置换技术。用于重建右心室流出道的最常见带瓣管道是同种异体移植物(66例患者)和牛颈静脉(Contegra)移植物(31例患者)。附加手术包括罗斯 - 康诺手术(14%)、主动脉下狭窄切除术/心肌切除术(11%)和升主动脉缩窄成形术(25%)。1例患者在术后30天内死亡(1%)。4例患者(4%)在术后0.5 - 4.5年发生晚期死亡:原因包括心内膜弹力纤维增生症导致的肺动脉高压(2例)、蛛网膜下腔出血(1例)和心源性猝死(1例)。5年生存率为93.6%(95%可信区间88.1 - 99.1)。8例患者出现中度或重度主动脉(自体移植物)反流,需要再次手术,5年无自体移植物再次手术率为98.5%(95.6 - 100)。右心室流出道管道5年无再次干预(手术或基于导管介入)率为91.5%(85.5 - 96.5)。再次干预的单因素预测因素为移植物尺寸较小(p = 0.03)和使用Contegra移植物(p = 0.04)。罗斯手术可在低死亡率的情况下进行,且长期生存率良好。大多数再次干预与新的右心室流出道相关,可能部分归因于生长不足。虽然罗斯手术仍然是治疗儿童主动脉瓣疾病的一个非常有价值的选择,但对于新的肺动脉瓣以及自体移植物较少出现的问题,需要新的解决方案。

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