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Pentalogy of Cantrell with a double-outlet right ventricle: 3.5-year follow-up in a prenatally diagnosed patient

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Abstract

Pentalogy of Cantrell is a rare congenital defect associated with five ventral midline anomalies and high mortality. An obstetric sonogram revealed a fetus with a body wall defect suggesting a diagnosis of this condition. Soon after birth, the infant underwent a closure of the upper abdominal wall defect followed by the successful repair of double-outlet right ventricle and pulmonary valve stenosis at the age of 5 months. The patient is currently alive and well 3.5 years after surgery. It is concluded that a deliberate therapeutic strategy based on the intrauterine diagnosis may alter the natural history of this devastating disorder.

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Fig. 1

Abbreviations

POC:

Pentalogy of Cantrell

DORV:

double-outlet right ventricle

References

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Acknowledgement

The authors thank Dr. Yoshimura, Dr. Sumida and Dr. Imamura, Kansai Medical University, for cardiologic care and fruitful discussion. This study was partly supported by the Mami Mizutani Foundation.

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Authors and Affiliations

  1. Department of Pediatrics, Kansai Medical University, 10-15 Fumizonocho, Moriguchi, Osaka, 570-8506, Japan

    Junji Takaya, Naoyuki Kitamura, Kimiko Tsuji, Kentaro Watanabe, Yo Kinoshita, Yuko Hattori, Masayuki Teraguchi, Shouichiroh Taniuchi & Kazunari Kaneko

  2. Department of Surgery, Kansai Medical University, 10-15 Fumizonocho, Moriguchi, Osaka, 570-8506, Japan

    Kohei Takada & Yoshinori Hamada

Authors
  1. Junji Takaya
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  2. Naoyuki Kitamura
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  3. Kimiko Tsuji
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  4. Kentaro Watanabe
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  5. Yo Kinoshita
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  6. Yuko Hattori
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  7. Masayuki Teraguchi
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  8. Shouichiroh Taniuchi
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  9. Kohei Takada
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  10. Yoshinori Hamada
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  11. Kazunari Kaneko
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Corresponding author

Correspondence to Junji Takaya.

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Takaya, J., Kitamura, N., Tsuji, K. et al. Pentalogy of Cantrell with a double-outlet right ventricle: 3.5-year follow-up in a prenatally diagnosed patient. Eur J Pediatr 167, 103–105 (2008). https://doi.org/10.1007/s00431-007-0579-8

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  • DOI: https://doi.org/10.1007/s00431-007-0579-8

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