Pediatric Cardiology and Cardiac Surgery
Vol.27 No.2 2011 (88-95)

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Yukiko Kawazu1), Noboru Inamura1), Ryo Ishii1), Yoshino Terashima1), Hisaaki Aoki1), Yuji Hamamichi1), Futoshi Kayatani1), Shigemitsu Iwai2), Hiroaki Kawata2), Hidefumi Kishimoto2)

1)Department of Pediatric Cardiology, 2)Department of Cardiovascular Surgery Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan


Background: Tetralogy of Fallot with absent pulmonary valve (TF/APV) is a rare and severe congenital heart disease with high mortality.
Objective: To assess whether the prognosis for TF/APV is related to the fetal and postnatal clinical course and pulmonary artery (PA) configuration.
Methods: We reviewed the fetal and postnatal echocardiograms and clinical outcomes of 13 patients with TF/APV (fetal diagnosis in 9 patients and postnatally diagnosis in 4) and divided them into two groups. Group A (6 cases): alive, Group D (7 cases): dead.
Results: Fetal period: Group A: Hydramnion 0, hydrops fetalis (HF) 0, patent ductus arteriosus (PDA) 2. Group D: Hydramnion 3, HF 2, PDA 0.
Postnatal period: Group A: Five cases underwent intracardiac repair, including one requiring artificial ventilation (AV). A further case of AV required 3 operations before extubation. Postoperative courses are all good. Group D: Excluding the 2 intrauterine fetal deaths (IUFD), 4 cases required AV (3 of which died neonatally or in early childhood) and 1 case underwent intracardiac repair.
PA configuration: All group A cases had bulbous expansion of the left and right PA (Clover type). Three cases in group D had bulbous expansion of main PA (Balloon type).
Conclusions: PDA was considered to be a factor in the good prognosis. Hydramnion and HF were suggested as factors in the poor prognosis. As there was a higher rate of postnatal AV and poorer prognosis in the balloon type than in the clover type (p < 0.05), PA configuration is also considered an important factor in predicting the postnatal prognosis in TF/APV.