静岡県立こども病院循環器科Department of Cardiology, Shizuoka Children’s Hospital ◇ Shizuoka, Japan
背景：動脈管依存性肺循環の先天性心疾患（congenital heart disease with duct-dependent pulmonary circulation: CHD-DPC）に対する初回の姑息的治療は，Blalock-Taussig短絡術（Blalock-Taussig shunt: BTS）が主流であり，動脈管ステント留置術（ductal-stenting: DS）はまだ少数ではあるが，周術期のリスクが高い症例に対しては有効な治療法であると考えられる．
方法：当院でCHD-DPCに対しDSを施行した4症例を対象に，診療録から後方視的に検討した．また，同疾患群に対しBTSを施行した76症例と，周術期合併症や死亡率に関して比較検討を行った．
結果：DSは全症例で成功した．合併症として2例で治療直後に高肺血流性心不全のため内科的治療を要し，1か月後にステント狭小化のためステント内再留置を行った．1例でアプローチ部の右大腿動脈で閉塞をきたし，ステント再留置の際に大腿動脈のバルーン拡張も追加した．予後に関して，両方向性Glenn手術に到達し現在Fontan型手術待機中が1例，Rastelli手術到達が2例．18トリソミーを伴った1例で在宅移行が可能となった．BTS症例のうち，心外先天異常や染色体異常を有する群では，DS症例と比較して周術期合併症の発生率や死亡率が高い傾向にあった．
考察：周術期リスクが高い症例に対する初回の姑息的治療として，DSは有効な選択肢と考えられる．ステントサイズやアプローチの選択などの最適化により，さらなる成績向上の余地がある．
Background: The Blalock-Taussig shunt (BTS) is the standard first-stage surgical palliative treatment for congenital heart disease with duct-dependent pulmonary circulation (CHD-DPC). Although ductal-stenting (DS) is rarely performed, it is feasible and safe for patients with a high perioperative risk.
Method: We retrospectively investigated four patients who underwent DS for CHD-DPC at our hospital. The data of patients who underwent DS and BTS (n＝76) for CHD-DPC were analyzed. Then, an intergroup comparison of perioperative complications and mortality rates was performed.
Results: All patients successfully underwent DS. After DS, two patients required medical treatment for heart failure secondary to increased pulmonary perfusion. After 1 month, re-in-stenting was performed due to in-stent restenosis. One patient developed total occlusion at the approach site of the right femoral artery (FA). Therefore, balloon-expandable stents were deployed into the FA. With regard to prognosis, one patient underwent the bidirectional Glenn procedure and was waiting to undergo the Fontan procedure. Moreover, two patients underwent the Rastelli procedure. One patient with trisomy 18 was discharged. The perioperative complication and mortality rates were higher in patients who underwent BTS with extra-cardiac anomaly or chromosomal abnormalities than in those who underwent DS.
Conclusion: DS is an effective first-stage palliative strategy for patients with a high perioperative risk. Moreover, DS can be further improved by optimizing the stent size and approach site.
Key words: congenital heart disease; patent ductus arteriosus; duct-dependent pulmonary circulation; ductal-stenting; shunt
© 2020 特定非営利活動法人日本小児循環器学会© 2020 Japanese Society of Pediatric Cardiology and Cardiac Surgery
This page was created on 2020-12-03T11:32:22.973+09:00
This page was last modified on 2020-12-22T11:57:38.000+09:00
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