Pentalogy of Cantrell with a double-outlet right ventricle: 3.5-year follow-up in a prenatally diagnosed patient.
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.
Department of Pediatrics, Kansai Medical University, 10-15 Fumizonocho, Moriguchi, Osaka 570-8506, Japan. takaya@takii.kmu.ac.jp
Junji
J
Takaya
Naoyuki
N
Kitamura
Kimiko
K
Tsuji
Kentaro
K
Watanabe
Yo
Y
Kinoshita
Yuko
Y
Hattori
Masayuki
M
Teraguchi
Shouichiroh
S
Taniuchi
Kohei
K
Takada
Yoshinori
Y
Hamada
Kazunari
K
Kaneko
D034861:Abdominal Wall / Q000002:abnormalities* / Q000601:surgery; D000328:Adult; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D034381:Hearing Loss / Q000209:etiology*; D006330:Heart Defects, Congenital / Q000150:complications* / Q000601:surgery; D006554:Hernia, Umbilical / Q000150:complications* / Q000601:surgery; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D011247:Pregnancy; D016216:Ultrasonography, Prenatal