Journal of JCIC

Online edition: ISSN 2432–2342
JCIC学会事務局 JCIC学会事務局
〒162-0801東京都新宿区山吹町358-5アカデミーセンター Academy Center, 358-5 Yamabuki-cho, Shinju-ku, Tokyo 162-0801, Japan
Journal of JCIC 6(1): 1-4 (2021)


心腔内エコーガイド下にNykanen™ RF wireで行った自然閉鎖した開窓の再作成Reopening a spontaneously closed fenestration with Nykanen™ RF wire using intracardiac echocardiography: A case report

兵庫県立こども病院 循環器内科Department of Cardiology, Kobe Children’s Hospital, Hyogo, Japan

受付日:2021年3月8日Received: March 8, 2021
受理日:2021年5月10日Accepted: May 10, 2021
発行日:2021年8月31日Published: August 31, 2021

フォンタン術後合併症の蛋白漏出性胃腸症は難治性であり,治療法は確立されていない.血行動態への介入は治療法の一つであり,開窓作成が行われることがある.経皮的な開窓作成にはBrockenbrough法が行われることが多い.症例は4歳男児.左心低形成症候群に対して2歳4か月時に心外導管開窓フォンタン手術を行ったが,2歳7か月時に開窓の閉鎖を確認した.4歳9か月時に蛋白漏出性胃腸症を発症し,自然閉鎖した開窓の再作成を企図した.経食道エコー,血管造影では開窓閉鎖部位は特定できなかった.心腔内エコーでは壁厚が薄く,後方エコーの増強を認め,開窓閉鎖部位を特定した.同部位をNykanen™ RF wireで穿通し,順次バルーンで拡大,最大6 mmまで拡大した.開窓閉鎖部位の特定に心腔内エコーが有用であった.自然閉鎖した開窓の穿通にはNykanen™ RF wireの使用も選択肢の一つになりうる.

Protein-losing enteropathy (PLE) after the Fontan operation is a serious complication that is difficult to manage. One treatment option for PLE is the creation of a fenestration to improve hemodynamic status. The Brockenbrough puncture has traditionally been used to create a Fontan fenestration. The current patient was a 4-year-old boy with hypoplastic left heart syndrome who had undergone a fenestrated extracardiac Fontan operation at the age of 2 years and 4 months. The surgical fenestration closed spontaneously 3 months after Fontan completion. The patient developed PLE at the age of 4 years and 9 months, and underwent catheterization at the age of 4 years and 11 months. The site of Fontan fenestration could not be identified on angiography or transesophageal echocardiography. Intracardiac echocardiography indicated that the region of the spontaneously closed fenestration was thinner than the Fontan conduit and associated with posterior echo enhancement. The region was penetrated with Nykanen™ RF wire, and balloon dilatation was performed up to a final diameter of 6 mm. The present case indicates that intracardiac echocardiography can be useful for identifying the region of fenestration, and that Nykanen™ RF wire can be a viable option for reopening a surgically created fenestration.

Key words: fenestration; radiofrequency wire; intracardiac echography; Fontan

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