文／馬偕兒童醫院田智瑋醫師
Maternal history:
- G1P1
- 40-year-old mother
- Gestational diabetes mellitus under diet control
Pre-natal diagnosis: suspect aortopulmonary window
Birth History:
- Cesarean section due to congenital heart disease
- Gestational age: 37+5 weeks
- Male, birth weight 3060 g
- Immediately admission to NICU after birth
Image Findings
1. Chest X ray:
圖一：在出生後20天可見迅速進展之心臟擴大及肺水腫
2. Echocardiography:
此個案報告為第三型Aortopulmonary window合併一小型(0.16cm)之肌肉部心室中膈缺損，心室中膈缺損並未在以下影像中展示
圖二可見顯著之左心室及左心房擴大，出生多日後更加明顯。Aortopulmonary window為動脈階層之左至右分流，左心擴大為大量增加之肺循環及回心血流所致。
圖二：LV: Left ventricle, LA: Left atrium, RV: Right ventricle, RA: Right Atrium. Dilated LV and LA in apical four chamber view
在胸骨旁短軸可見升主動脈與右肺動脈的不完全間隔，且都卜勒聲波下可見顯著之左向右分流(米字)。此即為Aortopulmonary window之典型表現。
Asterisk: Incomplete septation between right pulmonary artery and ascending aorta. Color doppler echocardiography found left to right shunt through this defect. These are typical findings of aortopulmonary window.
圖三：AsAo: Ascending aorta. MPA: main pulmonary artery. RPA: right pulmonary artery. Asterisk: aortopulmonary window.
在胸骨上長軸亦可見升主動脈與右肺動脈的不完全間隔，且都卜勒聲波下可見顯著之左向右分流(米字)。
圖四：Arch: Aortic arch. RPA: right pulmonary artery. Asterisk: aortopulmonary window.
圖五：CT for heart and vessels. Ao: Ascending aorta. MPA: main pulmonary artery. RPA: right pulmonary artery. Asterisk: aortopulmonary window.
Operation：
於出生21天大時接受手術矯正
- 設立體外心肺循環並降溫至28°C
- 將開放性肺動脈導管結紮後切開右心房放置血液引流管
- 辨認出aortopulmonary window之位置並切開(Figure 6A)
- 以肺動脈動脈壁之補片來修補主動脈側隔間，並以心包膜修補肺動脈側隔間 (Figure 6B)
- 回溫並移除體外心肺循環
圖六（A）：Asterisk: Aortopulmonary window（B）：Arrowhead: repair of aortopulmonary window
Outcome:
- 恢復良好，於出生45天大時出院(術後24天)
- 術後心臟超音波顯示輕微肺動脈分支狹窄
- 於門診追蹤時，體重成長及發展里程碑皆正常
