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- Title
Pentalogy of Cantrell with a double-outlet right ventricle: 3.5-year follow-up in a prenatally diagnosed patient.
- Authors
Takaya, Junji; Kitamura, Naoyuki; Tsuji, Kimiko; Watanabe, Kentaro; Kinoshita, Yo; Hattori, Yuko; Teraguchi, Masayuki; Taniuchi, Shouichiroh; Takada, Kohei; Hamada, Yoshinori; Kaneko, Kazunari
- 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.
- Subjects
STENOSIS; MORTALITY; FETUS; PRENATAL diagnosis; OBSTETRICAL diagnosis; ABDOMINAL surgery; ABDOMINAL abnormalities; CONGENITAL heart disease; FETAL ultrasonic imaging; HEARING disorders; DISEASE complications; UMBILICAL hernia; SURGERY
- Publication
European Journal of Pediatrics, 2008, Vol 167, Issue 1, p103
- ISSN
0340-6199
- Publication type
journal article
- DOI
10.1007/s00431-007-0579-8