体肺側副血管と肺動脈の塞栓後に開窓作成術を行い中心静脈圧の低下が得られた肺静脈閉塞を合併したFailing Fontanの一例

Shigehito Baba; 馬場恵史; Tadaaki Abe; 阿部忠朗; Masanori Tsukada; 塚田正範; Junichi Ozawa; 小澤淳一; Ai Sugimoto; 杉本愛; Fujito Numano; 沼野藤人; Shuichi Shiraishi; 白石修一; Akihiko Saito; 齋藤昭彦

doi:10.9794/jspccs.37.320

体肺側副血管と肺動脈の塞栓後に開窓作成術を行い中心静脈圧の低下が得られた肺静脈閉塞を合併したFailing Fontanの一例A Case of Failing Fontan with Pulmonary Vein Occlusion Obtained Decrease in the Central Venous Pressure by Fenestration Creation after Embolization of Minor Aortopulmonary Collateral Artery and Pulmonary Artery

馬場恵史¹，阿部忠朗¹，塚田正範¹，小澤淳一¹，杉本愛²，沼野藤人¹，白石修一²，齋藤昭彦¹Shigehito Baba¹, Tadaaki Abe¹, Masanori Tsukada¹, Junichi Ozawa¹, Ai Sugimoto², Fujito Numano¹, Shuichi Shiraishi², Akihiko Saito¹

¹ 新潟大学医歯学総合病院小児科Department of Pediatrics, Niigata University Graduate School of Medical and Dental Sciences ◇ Niigata, Japan

² 新潟大学医歯学総合病院心臓血管外科Department of Thoracic and Cardiovascular Surgery, Niigata University Graduate School of Medical and Dental Sciences ◇ Niigata, Japan

受付日：2021年3月4日Received: March 4, 2021

受理日：2021年7月19日Accepted: July 19, 2021

発行日：2021年12月1日Published: December 1, 2021

肺静脈閉塞を合併したFontan術後患者に対する有効な治療法はなく予後は不良である．今回我々はFontan術後に右肺静脈閉塞を来し，多量の胸水，浮腫，心房性不整脈が出現した急性心不全の3歳女児例に対して体肺側副血管と右肺動脈の塞栓後に開窓作成術を行った．体肺側副血管の塞栓は3回のセッションに分けて51本のコイルを使用した．右肺動脈塞栓は計5個のAmplatzer Vascular Plug（AVP）と計2本のコイルを用いて，手技時間は3時間程度で合併症なく完全閉塞できた．その後に開窓作成術を行い，中心静脈圧は閉塞術前の20 mmHgから7 mmHgへ低下した．手技に伴う合併症はなく，手技時間も短く施行し得た．しかし術後9日目に心タンポナーデを発症し死亡した．過去に肺静脈閉塞を合併したFontan術後症例に対して体肺側副血管と患側の肺動脈を完全に塞栓した報告はない．本治療は一時的に肺静脈閉塞を伴うFontan患者の循環動態を安定させた．

No effective treatments currently exist for patients who have undergone the Fontan procedure with pulmonary vein occlusion (PVO), and the prognosis is poor. We present a case of a 3-year-old girl with acute heart failure who developed right PVO after undergoing the Fontan procedure, and a large amount of pleural effusion, edema, and atrial arrhythmia was noted. Fenestration was created after embolization of the minor aortopulmonary collateral arteries (mAPCAs) and right pulmonary (RPA) artery. The embolization of mAPCAs was divided into 3 sessions using 51 coils. For RPA embolization, 5 Amplatzer Vascular Plugs and 2 coils were used. The duration of this procedure was approximately 3 h, and complete occlusion of RPA was achieved without complications. Subsequently, fenestration creation was performed, and the central venous pressure decreased from 20 mmHg to 7 mmHg. However, the patient deceased because of cardiac tamponade on day 9 postoperatively. To our knowledge, there have been no reports on the complete embolization of the mAPCAs and PA in patients who have undergone the Fontan procedure with PVO. This treatment temporarily stabilized the hemodynamics in patients who have undergone the Fontan procedure with PVO.

Key words: acute heart failure; failing Fontan; pulmonary vein occlusion; embolization of pulmonary artery; embolization of minor aortopulmonary collateral artery

日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

症例報告Case Report