Kaguelidou Florentia, Fermont Laurent, Boudjemline Younes, Le Bidois Jérôme, Batisse Alain, Bonnet Damien
Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C, Université Paris V-Descartes, AP-HP, Hôpital Necker-Enfants Malades, 149, rue de Sèvres, 75015 Paris, France.
Eur Heart J. 2008 Jun;29(11):1432-8. doi: 10.1093/eurheartj/ehn194. Epub 2008 May 8.
Outcome of foetuses diagnosed with tetralogy of Fallot (TOF) or pulmonary atresia with ventricular septal defect (PA-VSD) and the reliability of foetal echocardiography to predict post-natal surgical outcome.
Outcome of 218 foetuses having been diagnosed with TOF (n = 153) or PA-VSD (n = 65) was reviewed. Abnormal karyotyping, 22q11 deletion, and extracardiac anomalies were found, respectively, in 11, 18, and 46%. Pregnancy was terminated in 75 cases (34%), and in three cases foetuses died in utero. Presence or absence and confluence of PA branches were confirmed after birth or pregnancy termination in all but five (5%) cases. Main pulmonary trunk (MPA) was incorrectly described in 11 (10%) cases and major aorto-pulmonary collateral arteries in 16 (13%) cases. Among live born infants, 110 (88%) were operated and 92 (74%) underwent complete repair in the first year of life. Size of confluent PAs and presence of MPA were related to the probability of having a complete repair in the first year of life.
Foetal diagnosis of TOF and PA-VSD has a major impact on pregnancy outcome, as associated anomalies are frequently found. Pre-natally determined size of PA branches and presence of MPA are good predictors of complete repair in the first year of life.
患有法洛四联症(TOF)或室间隔缺损合并肺动脉闭锁(PA-VSD)胎儿的预后,以及胎儿超声心动图预测出生后手术结果的可靠性。
回顾了218例被诊断为TOF(n = 153)或PA-VSD(n = 65)胎儿的预后情况。分别在11%、18%和46%的病例中发现了染色体核型异常、22q11缺失和心外异常。75例(34%)终止妊娠,3例胎儿死于宫内。除5例(5%)外,所有病例在出生或终止妊娠后均确认了肺动脉分支的存在与否及汇合情况。11例(10%)病例中主肺动脉(MPA)描述错误,16例(13%)病例中主要体肺侧支动脉描述错误。在存活婴儿中,110例(88%)接受了手术,92例(74%)在出生后第一年接受了完全修复。汇合的肺动脉大小和主肺动脉的存在与出生后第一年进行完全修复的可能性相关。
胎儿期诊断TOF和PA-VSD对妊娠结局有重大影响,因为常发现相关异常。产前确定的肺动脉分支大小和主肺动脉的存在是出生后第一年完全修复的良好预测指标。