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 JCIC 8(2): 15-20 (2024)


動画化した全時相造影CTが経皮的肺動脈弁形成術(PTPV)効果予測に有用だった両大血管右室起始症の一例Full-time contrast enhanced CT with moving images predicts efficacy of percutaneous pulmonary transluminal valvuloplasty for double-outlet right ventricle: A case report

昭和大学病院 小児循環器・成人先天性心疾患センターSHOWA University Hospital

受付日:2023年9月27日Received: September 27, 2023
受理日:2023年12月18日Accepted: December 18, 2023
発行日:2024年1月24日Published: January 24, 2024

症例は在胎33週4日,1174 gで出生した男児.胎児超音波検査で両大血管右室起始症,肺動脈狭窄と診断されていた.出生時のSpO2は90%前半で動脈管非依存性と考えLipo-PGE1は投与せず経過をみた.生後2週間頃より動脈管狭小化に伴い徐々にSpO2は80%後半へ低下した.日齢22よりLipo-PGE1開始,日齢25より酸素投与を開始したが,日齢36人工呼吸管理となった.心臓超音波検査では弁下狭窄の程度が不明瞭であったが,冠動脈評価のために施行したCTデータからPhyZiodynamicsで再構成画像を作成することで,右室流出路からの肺動脈内腔の動画化した3D画像を評価した.肺動脈弁下狭窄は軽度であり,経皮的肺動脈弁形成術(PTPV)により弁狭窄を解除することで低酸素血症は改善,狭小弁輪により肺血流は制御され,高肺血流のリスクも低いと予測された.日齢49にTMP-PED 6 mm/2 cmにてPTPVを施行し,動脈血酸素飽和度は80から98%に上昇,人工呼吸器から離脱した.高肺血流による心不全も認めず,日齢75, 体重2.3 kgまで体肺動脈短絡術を待機することが可能であった.

Our patient was a boy born at 33 weeks and 4 day’s gestation weighing 1174 g. Fetal ultrasound revealed a double-outlet right ventricle complicated by pulmonary artery stenosis. The patient’s oxygen saturation (SpO2) at birth was in the low 90s, but Lipo-PGE1 was not administered as we deemed the hemodynamics ducts arteriosus-independent. Starting at 2 weeks of age, the patients SpO2 gradually decreased to the low 80s after constriction of the arterial duct. Despite intravenous Lipo-PGE1 and supplemental oxygen, controlled ventilation was required to maintain the SpO2. Although the precise anatomy of subvalvular stenosis was unclear on echocardiography, the patients was evaluated with animated three-dimensional images of the pulmonary artery from the right ventricular outflow tract by the creation of reconstructed images using PhyZiodynamics from the computed tomography date collected from the coronary artery evaluation. From the image, we identified mild subvalvular stenosis of the pulmonary artery. Consequently, we judged that percutaneous pulmonary valvuloplasty (PTPV) would be effective for hypoxemia, while pulmonary high flow would be unlikely as the small pulmonary valve ring could control the pulmonary blood flow. Following PTPV at 49 days of age using TMP PED balloon catheter (6 mm/2 cm), his arterial oxygen saturation increased from 80 to 98%, and he was weaned from the ventilator. Heart failure complicated by excessive pulmonary blood flow was not observed. He was able to wait until 75 days of age for systemic to pulmonary arterial shunt surgery, when his body weight reached 2.3 kg.

Key words: very low birth weight infant; double-outlet right ventricle; PhyZiodynamics; percutaneous pulmonary valvuloplasty; systemic to pulmonary arterial shunt

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