自治医科大学とちぎ子ども医療センター小児科Department of Pediatrics, Jichi Children’s Medical Center ◇ Tochigi, Japan
バルサルバ洞動脈瘤は先天心奇形に合併することもあるが，小児では極めて稀な疾患であり，また胎児期から破裂をきたした報告はない．今回，胎児期よりバルサルバ洞動脈瘤の破裂を合併した左心低形成症候群の一例を経験した．症例は女児．胎児心エコー検査で心室中隔欠損を伴った非典型的な左心低形成症候群（大動脈弁閉鎖，僧帽弁閉鎖，痕跡的左室）と診断され，中等度の三尖弁逆流の合併が疑われた．しかし，出生後の心エコー検査では三尖弁逆流は認めず，大動脈基部から右房に短絡する血流を認め，バルサルバ洞動脈瘤破裂の合併を疑った．精査として末梢動脈からの逆行性大動脈造影を施行し，大動脈無冠尖のバルサルバ洞の下方突出およびそこから右房に短絡する血流を認め，バルサルバ洞動脈瘤破裂と診断した．バルサルバ洞動脈瘤は胎児期でも破裂をきたすことがあり，右房への短絡血流は三尖弁逆流と見誤ることがある．バルサルバ洞動脈瘤破裂の確定診断にあたり，逆行性大動脈造影は有用な選択肢である．
Sinus of valsalva aneurysm (SVA) may be associated with congenital heart disease, and it is an extremely rare condition among children. Moreover, a ruptured SVA has not been observed in a fetus. Herein, we report a case of hypoplastic left heart syndrome (HLHS) with mitral atresia and aortic atresia and a ruptured SVA. HLHS with moderate tricuspid regurgitation was suspected on fetal echocardiography. However, transthoracic echocardiography after birth revealed a continuous flow shunting from the aortic root to the right atrium rather than tricuspid regurgitation. A ruptured SVA was suspected, and countercurrent aortography was performed for further evaluation. Results revealed an SVA of noncoronary cusp with a continuous flow to the right atrium. This finding indicated a ruptured SVA, which can occur even in a fetus. The shunt flow of a ruptured SVA into the right atrium can be misdiagnosed as tricuspid regurgitation on fetal echocardiography. Thus, countercurrent aortography may be a useful modality for diagnosing a ruptured SVA among neonates.
Key words: ruptured sinus of Valsalva aneurysm; hypoplastic left heart syndrome; fetal echocardiography; countercurrent aortography
© 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
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This page was last modified on 2020-12-21T19:56:49.000+09:00
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