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 JPIC 2(1): 26-31 (2017)

原著Original Article

極小右室および類洞交通を有する純型肺動脈閉鎖症の治療戦略~心内血栓形成を回避するための新規方針の検討~A new strategy for pulmonary atresia with intact ventricular septum associated with extremely small right ventricles and sinusoidal communications to prevent thrombus formation in the blind-end right ventricles

JCHO中京病院中京こどもハートセンター小児循環器科Department of Pediatric Cardiology, Chukyo Children Heart Center, Japan Community Healthcare Organization Chukyo Hospital ◇ Aichi, Japan

受付日:2017年3月29日Received: March 29, 2017
受理日:2017年5月13日Accepted: May 13, 2017
発行日:2017年8月31日Published: August 31, 2017

背景:従来,当院では極小右室および類洞交通を有する純型肺動脈閉鎖症に対して経皮的肺動脈弁形成術(Percutaneous Transluminal Pulmonary Valvuloplasty: PTPV)を施行せずに単心室修復(Uni-ventricular Repair: UVR)方針としていたが,フォンタン循環到達後遠隔期に盲端右室内に巨大血栓を形成し労作時胸痛を発症した症例の経験を機に,右室依存性冠循環を除く全ての肺動脈弁膜様閉鎖の症例に対して右室を盲端として残さない目的でPTPVを施行する方針に変更した.



結果:PTPVは合併症頻度の高いカテーテル治療であるが,Radiofrequency wireの使用,ワイヤーループ・ローテーション法の導入,段階的PTPVなどの工夫により安全に施行できた.2症例とも肺動脈弁閉鎖不全(Pulmonary Regurgitation: PR)が生じている.


Background: Previously, we introduced a uni-ventricular repair (UVR) strategy without performing percutaneous transluminal pulmonary valvuloplasty (PTPV), for pulmonary atresia with intact ventricular septum (PAIVS) associated with extremely small right ventricles and sinusoidal communications (SC). After experiencing a case complicated by thrombus formation in the blind-end right ventricle during the postoperative Fontan procedure, we changed our strategy to perform PTPV for all cases, exept in cases with right ventricular dependent coronary circulations.

Purpose: To investigate the advisability of our new strategy for cases with high possibility of UVR in the future.

Methods: We retrospectively assessed two cases performed PTPV for PAIVS with extremely small right ventricles and SC to determine the benefits, safety, and issues associated with the new strategy.

Results: Although PTPV involves catheter intervention, which is associated with an increased risk of complications, it could be performed safely with the use of radiofrequency wire, the introduction of wire loop rotation technique, and gradual PTPV. Pulmonary regurgitation (PR) occurred in both cases.

Conclusion: PTPV can be performed safely without being influenced by the size of the right ventricle. However, in the cases that finally UVR is unavoidable, it would be necessary to treat the right ventricle considering the effect of PR.

Key words: Pulmonary Atresia with Intact Ventricular Septum; Percutaneous Transluminal Pulmonary Valvuloplasty; Sinusoidal Communication; Blind-end right ventricle; Radiofrequency wire

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