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Fetal Case of Rare Association of Hypoplastic Left Heart Syndrome and Absent Atrial Septum Accompanied by Mixed Form of Supracardiac Total Anomalous Pulmonary Venous Connection
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  • Wakako Maruyama,
  • Yuki Kawasaki,
  • Yosuke Murakami,
  • Mitsuhiro Fujino,
  • Takeshi Sasaki,
  • Kae Nakamura,
  • Yoko Yoshida,
  • Tsugutoshi Suzuki,
  • Koji Kagisaki,
  • Eiji Ehara
Wakako Maruyama
Osaka City General Hospital

Corresponding Author:[email protected]

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Yuki Kawasaki
Osaka City General Hospital
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Yosuke Murakami
Osaka City General Hospital
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Mitsuhiro Fujino
Osaka City General Hospital
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Takeshi Sasaki
Osaka City General Hospital
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Kae Nakamura
Osaka City General Hospital
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Yoko Yoshida
Osaka City General Hospital
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Tsugutoshi Suzuki
Osaka City General Hospital
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Koji Kagisaki
Osaka City General Hospital
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Eiji Ehara
Osaka City General Hospital
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Abstract

Abnormal pulmonary venous flow patterns on fetal echocardiography and a nutmeg lung pattern on fetal magnetic resonance imaging are seen in patients with pulmonary venous stenosis. The association between these findings and the degree of pulmonary venous stenosis remains unknown. We report an extremely rare case of a fetus diagnosed with hypoplastic left heart syndrome complicated by an absent atrial septum and supracardiac total anomalous pulmonary venous connection with left pulmonary venous congestion. This case suggests that compared to non-pulsatile continuous pulmonary venous flow, the nutmeg lung pattern can only be observed with severe pulmonary congestion and advanced pulmonary lymphangiectasia.
07 Jun 2021Submitted to Echocardiography
07 Jun 2021Submission Checks Completed
07 Jun 2021Assigned to Editor
11 Jun 2021Reviewer(s) Assigned
06 Jul 2021Review(s) Completed, Editorial Evaluation Pending
06 Jul 2021Editorial Decision: Revise Major
19 Jul 20211st Revision Received
20 Jul 2021Submission Checks Completed
20 Jul 2021Assigned to Editor
31 Jul 2021Reviewer(s) Assigned
06 Sep 2021Review(s) Completed, Editorial Evaluation Pending
09 Nov 2021Editorial Decision: Accept
Feb 2022Published in Echocardiography volume 39 issue 2 on pages 375-380. 10.1111/echo.15272