诊断

一项被称为超声心动图的检查可诊断右心室双出口。该检查利用声波生成心脏跳动的图像。它可显示流经心脏和心脏瓣膜的血流。

若需更多关于心脏的详细信息,可进行其他检查。用于检查心脏的项目可能包括:

  • 心脏 CT 扫描。 这也称为心脏 CT。这项检查利用 X 线生成身体特定部位的横截面图像。
  • 心脏 MRI 扫描。 这项检查利用磁场和无线电波来呈现心脏的详细图片。
  • 心脏导管插入术。 医生会将一根细长软管(称为导管)放入血管(通常是腹股沟或手腕处的血管)。然后引导至心脏。造影剂通过导管流至心脏动脉。造影剂可以让动脉在检查期间拍摄的图像上显示得更清楚。在检查过程中,医务人员还可测量心脏腔室和血管中的压力和氧气水平。

治疗

大多数患有右心室双出口的婴儿需要在出生几个月内接受心脏修复手术。可能需要接受多种手术。手术类型取决于具体的心脏结构。

心脏外科医生可能采取以下一种或多种方式治疗右心室双出口:

  • 在心脏中建立一条通道,将心脏左下腔室与身体的主要动脉(主动脉)建立连接。
  • 利用补片(又称板障)修补心脏孔洞。
  • 修复主动脉和肺动脉错位(若有)。
  • 如有必要,建立心脏右下腔室与肺动脉之间的连接。
  • 若通往肺部的自然通道狭窄,则将其拓宽。

日后可能还需要接受其他手术。右心室双出口患者需终身进行定期健康检查。成人患者应到受过先天性心脏状况评估和治疗专业训练的医生处就诊。此类医生称为成人先天性心脏病科医生。

更多信息

临床试验

探索 Mayo Clinic 的研究 测试新的治疗、干预与检查方法,旨在预防、检测、治疗或控制这种疾病。

妥善处理与支持

Learning that your child has DORV can bring many questions and decisions. Ask your child's care team to explain the differences in the heart and the treatment options.

It may help to ask the heart care team to draw a picture of your child's heart and show:

  • Where the VSD is.
  • How blood flows through the heart.
  • How it would flow after repair.

You also may want to ask:

  • What type of repair may be possible.
  • What the surgery is meant to change.
  • What to expect after treatment.

Your child's heart care team is there to answer questions and support you and your family throughout your child's care.

Sept. 10, 2026

Living with 右心室双出口?

Connect with others like you for support and answers to your questions in the Heart & Blood Health support group on Mayo Clinic Connect, a patient community.

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  1. Xu Z, et al. Double outlet ventricles: Review of anatomic and imaging characteristics. Heart. 2023; doi:10.1136/heartjnl-2022-321955.
  2. Bell-Cheddar Y, et al. Double outlet right ventricle. Frontiers in Pediatrics. 2023; doi:10.3389/fped.2023.1244558.
  3. Holten-Andersen M, et al. Current outcomes of live-born children with double outlet right ventricle in Norway. European Journal of Cardio-Thoracic Surgery. 2023; doi:10.1093/ejcts/ezac560.
  4. Gurvitz M, et al. 2025 ACC/AHA/HRS/ISACHD/SCAI guideline for the management of adults with congenital heart disease: A report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2025; doi:10.1161/CIR.0000000000001402.
  5. Pang K, et al. Long-term surgical outcomes in double outlet right ventricle based on detailed anatomical sub-typology. European Journal of Cardio-Thoracic Surgery. 2025; doi:10.1093/ejcts/ezaf334.
  6. Rickert-Sperling S, et al., eds. Congenital Heart Diseases: The Broken Heart: Clinical Features, Human Genetics and Molecular Pathways. 2nd ed. Springer; 2024. https://link.springer.com/book/10.1007/978-3-031-44087-8. Accessed Aug. 17, 2026.
  7. Öcal A, et al. Navigating the spectrum of double-outlet right ventricle presentations: Outcomes from a contemporary cohort based on subtypes. Echocardiography. 2024; doi:10.1111/echo.15790.
  8. Moon-Grady AJ, et al. Guidelines and recommendations for performance of the fetal echocardiogram: An update from the American Society of Echocardiography. Journal of the American Society of Echocardiography. 2023; doi:10.1016/j.echo.2023.04.014.
  9. Pontailler M, et al. Long-term results after the réparation à l'étage ventriculaire procedure for transposition of the great arteries and double-outlet right ventricle with pulmonary stenosis. European Journal of Cardio-Thoracic Surgery. 2023; doi:10.1093/ejcts/ezad409.
  10. Lacour-Gayet F, et al. Multicentre study on late outcomes of biventricular repair of double outlet right ventricle. European Journal of Cardio-Thoracic Surgery. 2024; doi:10.1093/ejcts/ezad423.
  11. Medical review (expert opinion). Mayo Clinic. Sept. 3, 2026.

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