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Prenatal identification of lung abnormalities has increased with prenatal surveillance. Treatment usually requires serial ultrasound observation but in rare situations in utero therapy may be required for fetal survival. We review the genetics, prenatal evaluation, and treatment of lung abnormalities with congenital cystic adenomatoid malformation (CCAM). Other lung lesions, bronchopulmonary sequestration (BPS), hybrid lesions involving both malformations, congenital lobar emphysema (CLE), are briefly included as differential diagnosis options. Outcome of fetuses identified to have CCAM lung abnormalities resulting in fetal hydrops and having in utero therapy (thoracoamniotic shunting, fetal thoracotomy, EXIT delivery) are discussed. In the appropriate situation, this maternal fetal surgery approach for CCAM is life-saving for the affected fetus with acceptable maternal morbidity risks in the present and future pregnancies.

Objective: To report the North American Fetal Therapy Network (NAFTNet) Registry data on the outcomes of using radiofrequency ablation to treat twin-reversed arterial perfusion (TRAP). Methods: This was a retrospective review of the records of all patients who underwent percutaneous radiofrequency ablation of an acardiac twin after referral to 12 NAFTNet institutions between 1998 and 2008. Maternal, fetal and neonatal data were analyzed. The primary outcome was neonatal survival to 30 days of age. All participating sites conducted this study under institutional review board approval. Results: Of the 98 patients identified, there were no maternal deaths and no women required blood transfusions. Most women (76 of 98; 78%) stayed in the hospital for ≤1 day after the procedure. Mean gestational age at delivery was 33.4 weeks overall and 36.0 weeks for survivors. Median gestational age at delivery was 37.0 weeks. Survival of the pump twin to 30 days was 80% in the overall cohort. Conclusion: The NAFTNet registry data suggest that radiofrequency ablation of the acardiac twin is an effective treatment for TRAP sequence.

Objective: To evaluate the effect of prenatal steroid treatment on the growth of congenital cystic adenomatoid malformations (CCAM) and survival in affected fetuses not amenable to other percutaneous ultrasound-guided prenatal interventions. Methods: A retrospective review of patients with a CCAM or hybrid lesion treated with two maternal prenatal betamethasone injections was performed. Patients receiving cyst aspiration or thoracoamniotic shunting at the time of or after steroid administration were excluded. Growth rates and survival data were compared to historical non-steroid treated controls. Results: Eleven patients were treated with prenatal steroids (10 microcystic and 1 macrocystic). Survival was 100% in fetuses with hydrops (5/5) or a CCAM volume ratio (CVR) >1.6 (7/7) at the time of steroid administration. This compares to a mortality of 100 and 56.2% respectively in historical non-treated controls. Resolution of hydrops was seen in 80% (4/5) of steroid-treated patients. CCAM growth rates were variable after steroid administration. However, when compared to historical data where CVR and CCAM volume have been documented to increase until 28 weeks’ gestation, the CVR and CCAM volume growth rates decreased in 72.73% and 50% of patients respectively from the time of steroid administration to 28 weeks’ gestation. Conclusions: In the fetus with a CCAM, the presence of hydrops fetalis or a CVR >1.6 is indicative of poor fetal outcome without prenatal intervention. The observed effect of antenatal steroid treatment on CCAM growth is variable, but its potential to improve survival in these high-risk groups is encouraging and warrants further controlled evaluations.

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K E Y W O R D S:congenital heart disease; hypoplastic left heart syndrome; outcomes ABSTRACT Objectives