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治疗孤立性三尖瓣发育异常的圆锥形成概念及双孔三尖瓣病例

The concept of cone creation to treat isolated tricuspid valve dysplasia and the case of a double-orifice tricuspid valve.

作者信息

Quarti Andrea Giulio, Petridis Francesco Dimitri, Mangerini Valeria Francesca, Careddu Lucio, Angeli Emanuela, Gargiulo Gaetano Domenico

机构信息

Pediatric Cardiac Surgery and Adult Congenital Heart Disease Program, Department of Cardio-Thoracic and Vascular Medicine, IRCCS, Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.

出版信息

JTCVS Tech. 2023 May 9;20:71-78. doi: 10.1016/j.xjtc.2023.04.013. eCollection 2023 Aug.

Abstract

OBJECTIVES

Isolated tricuspid valve dysplasia is a rare disease characterized by a wide spectrum of possible anomalies. We describe the use of the Cone concept to treat a patient with a double-orifice tricuspid valve with massive regurgitation and severe deficit of coaptation.

METHODS

Three adult patients with congenital non-Ebstein tricuspid valve anomaly characterized by severe coaptation deficiency underwent tricuspid valve repair applying the Cone technique. In particular, we describe the case of a symptomatic 21-year-old woman with a double-orifice tricuspid valve, with massive regurgitation and severe right ventricular dilatation. The tricuspid valve was transformed from a double-orifice valve into a single-orifice valve. The most superior orifice was opened, and the tissue surrounding the orifice was used to extend the leaflet of the inferior orifice. A Cone was created, and a ring annuloplasty was used to stabilize the result.

RESULTS

The patient was discharged home after 7 days with trivial residual tricuspid regurgitation and no significant antegrade gradient. The final coaptation height was 2.8 cm. The cardiothoracic ratio decreased from 0.77 to 0.59 after 2 months, and symptoms promptly improved.

CONCLUSIONS

Over the past 2 years, we have applied the Cone creation concept to patients with a severely dysplastic tricuspid valve with excellent early results. One patient had a double-orifice tricuspid valve, and a Cone repair concept was adopted anyway. One orifice was sacrificed, and surrounding tissue was used to augment the leaflets of the other orifice. A Cone was created to improve central coaptation with a good initial result.

摘要

目的

孤立性三尖瓣发育异常是一种罕见疾病,其特征为可能存在广泛的异常情况。我们描述了使用圆锥概念治疗一名患有双孔三尖瓣且大量反流和严重瓣叶对合不良的患者。

方法

三名患有先天性非埃布斯坦三尖瓣异常且以严重瓣叶对合不良为特征的成年患者接受了应用圆锥技术的三尖瓣修复术。特别是,我们描述了一名有症状的21岁女性病例,其患有双孔三尖瓣,伴有大量反流和严重的右心室扩张。三尖瓣从双孔瓣膜转变为单孔瓣膜。打开最上方的孔口,并用孔口周围的组织来延长下方孔口的瓣叶。创建一个圆锥,并使用瓣环成形术来稳定结果。

结果

患者在7天后出院,三尖瓣残余反流轻微,无明显的前向梯度。最终的瓣叶对合高度为2.8厘米。2个月后心胸比率从0.77降至0.59,症状迅速改善。

结论

在过去2年中,我们已将创建圆锥的概念应用于严重发育异常的三尖瓣患者,早期结果良好。一名患者患有双孔三尖瓣,但无论如何都采用了圆锥修复概念。牺牲一个孔口,并用周围组织来增大另一个孔口的瓣叶。创建一个圆锥以改善中央对合,初期结果良好。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0abc/10405194/affd8e9668df/fx1.jpg

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