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Successful one-stage extraction of an intracardiac and intravenous leiomyoma through the right atrium under transesophageal ultrasound monitoring

Réussite d’une extraction en une étape d’un léiomyome intracardiaque et intraveineux par l’oreillette droite sous surveillance échographique transœsophagienne

Abstract

Purpose

Intravenous leiomyomatosis is a rare disorder characterized by benign smooth-muscle tumours, termed leiomyomas, which originate from uterine leiomyomas or pelvic veins. Tumours may extend into the right-sided heart chambers, termed intracardiac leiomyomatosis (ICLM), and may be potentially life-threatening due to mechanical interference with cardiac structures or pulmonary arteries. While surgical excision is the optimal therapy, incomplete retrieval of a tumour or fatal retroperitoneal hemorrhage may occur. We present a case where intraoperative transesophageal ultrasound (TEU) guided complete removal of an intracardiac leiomyoma in a single-stage surgery solely through the right atrium without vein injury.

Clinical features

A 46-yr-old female patient presented with a two-week history of exertional dyspnea, palpitations, and syncope. Preoperative imaging modalities revealed a continuous solid mass extending from the inferior vena cava (IVC) into the right atrium, and the patient subsequently underwent open heart surgery for tumour removal and definitive diagnosis. A systematic intraoperative TEU examination performed before resection showed that the serpentine tumour was free from any attachment to the IVC and the heart. Furthermore, the diameter of the intracardiac end of the tumour was wider than that of the IVC. Given these findings, the surgeons carefully drew the cord-like tumour out of the right atrium under close TEU monitoring without vein injury. Post-extraction TEU examination showed complete removal of the tumour. Microscopic examination of the specimen confirmed the diagnosis of intravenous leiomyomatosis.

Conclusions

For cases with ICLM, intraoperative TEU plays a significant role in helping to plan the surgical approach, monitor the movement of the tumour and the IVC during the extraction, and assess the completeness of tumour resection.

Résumé

Objectif

La léiomyomatose intraveineuse est une affection rare caractérisée par des tumeurs bénignes des muscles lisses (appelées léiomyomes) qui ont pour origine des léiomyomes utérins ou des veines pelviennes. Les tumeurs peuvent s’étendre dans les cavités droites du cœur (constituant la léiomyomatose intracardiaque) et menacer la survie des patients en raison de la gêne mécanique engendrée dans les structures cardiaques ou les artères pulmonaires. Bien que le traitement optimal soit l’exérèse chirurgicale, une exérèse incomplète de la tumeur ou une hémorragie rétropéritonéale fatale peuvent survenir. Nous présentons un cas dans lequel une échographie transœsophagienne (ETO) peropératoire a guidé l’extraction complète en un seul temps chirurgical d’un léiomyome intracardiaque, uniquement par voie auriculaire droite, sans lésion veineuse.

Caractéristiques cliniques

Une femme âgée de 46 ans a présenté pendant deux semaines une dyspnée d’effort, des palpitations et une syncope. Les modalités d’imagerie préopératoire ont révélé une masse pleine continue s’étendant de la veine cave inférieure (VCI) jusque dans l’oreillette droite et la patiente a subi en conséquence une intervention chirurgicale à cœur ouvert pour l’ablation de la tumeur et un diagnostic définitif. Un examen peropératoire systématique par ETO réalisé avant la résection a montré que la tumeur allongée et flottante était dépourvue de tout lien à la VCI et au cœur. De plus, le diamètre de son extrémité intracardiaque était supérieur à celui de la VCI. Compte tenu de ces constatations, le chirurgien a précautionneusement tiré sur la tumeur semblable à une corde hors de l’oreillette droite sous monitorage ETO continu sans léser la veine. L’examen par ETO post extraction a confirmé l’ablation complète de la tumeur. L’examen microscopique de l’échantillon a confirmé le diagnostic de léiomyomatose intraveineuse.

Conclusions

Dans les cas de léimyomatose intracardiaque, l’ETO peropératoire apporte une contribution significative à la planification de l’abord chirurgical, à la surveillance des mouvements de la tumeur et de la VCI au cours de l’extraction, et permet de s’assurer que la tumeur a été complètement réséquée.

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Acknowledgments

The authors declare that there was no assistance with the manuscript, no financial support, and no conflicts of interest.

Author information

Correspondence to Jin Liu MD.

Additional information

Author contributions

Hao-Rui Sun, Hai-Bo Song, Ke Lin, and Jin Liu contributed to the conception and design of this manuscript. Hao-Rui Sun and Yan-Zi Zhang participated in data acquisition. All authors made significant contributions to manuscript preparation and final editing.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Video 1 Transesophageal ultrasound examination showing a serpentine solid tumour in the long-axis view that extends up the inferior vena cava into the right atrium and is free from any attachment to the lumen and without invasion into the hepatic veins. (MPG 3088 kb)

Video 2 Live visualization of tumour movement in the inferior vena cava with transesophageal echocardiography during surgical extraction. (MPG 1958 kb)

Video 3 Post-extraction transesophageal ultrasound examination of the inferior vena cava and the right atrium showing complete removal of the tumour. (MPG 4688 kb)

Video 1 Transesophageal ultrasound examination showing a serpentine solid tumour in the long-axis view that extends up the inferior vena cava into the right atrium and is free from any attachment to the lumen and without invasion into the hepatic veins. (MPG 3088 kb)

Video 2 Live visualization of tumour movement in the inferior vena cava with transesophageal echocardiography during surgical extraction. (MPG 1958 kb)

Video 3 Post-extraction transesophageal ultrasound examination of the inferior vena cava and the right atrium showing complete removal of the tumour. (MPG 4688 kb)

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Sun, H., Song, H., Zhang, Y. et al. Successful one-stage extraction of an intracardiac and intravenous leiomyoma through the right atrium under transesophageal ultrasound monitoring. Can J Anesth/J Can Anesth 61, 446–451 (2014) doi:10.1007/s12630-014-0130-0

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Keywords

  • Inferior Vena Cava
  • Right Ventricle
  • Sufentanil
  • Right Atrium
  • Uterine Leiomyomas