Manetti A, Pollini I, Cecchi F, De Simone L, Cianciulli D, Carbone C, Romanelli A, Bianchi F, Dolara A
U.O. Cardiologia, Ospedale Mayer, Firenze.
G Ital Cardiol. 1993 Feb;23(2):145-52.
To evaluate the prevalence of congenital heart disease in a homogeneous population we examined 46,895 liveborns in the period from January 1975 to December 1984 in the Careggi Maternity Hospital in Florence.
The diagnosis of congenital heart disease was made in 579 newborns within five days from birth. All newborns were examined clinically by two neonatologists and referred to the pediatric cardiologist in case of cardiac abnormalities. An ECG was recorded in each of them, chest x-ray in 87% and echo in those who were born after 1980. The children were followed up until December 1989. Mean follow-up period was 6 years.
The annual incidence ranged from 9.5% to 15.7% (average 12.3%). Chromosomic anomalies and extra-cardiac malformations occurred in 102 children (17.6%), respectively in 50 (8.6%) and 52 (9.0%) cases. Ventricular septal defect (VSD) and the patent ductus arteriosus (PDA), isolated or associated, were the most frequently recognized congenital heart diseases. 52 children (9.0%) were lost at follow-up. The initial diagnosis was changed in 11/579 cases. In 187 children (32.3%) there was a spontaneous disappearance of clinical and/or instrumental findings that had suggested the presence of congenital heart disease at birth: in 144 the diagnosis was VSD, and in 43 PDA. The prevalence of VSD was 4.7 per thousand in the period 1975-80 and 8.6 in the period 1981-84. 131 children (22.6%) died, 127 (96.9%) of them in the first year of life. 52 children were operated on and pulmonary valvuloplasty was performed in 5.
The prevalence of congenital heart disease does not change during a long observation period, while the number of VSDs increases. VSD and PDA spontaneously close in a high percentage of cases. The study suggests the usefulness of an intensive care unit for newborns with severe congenital heart disease, especially for those in the first year of life.
为评估同质性人群中先天性心脏病的患病率，我们对1975年1月至1984年12月期间在佛罗伦萨卡雷吉妇产医院出生的46,895名活产儿进行了检查。
579名新生儿在出生后五天内被诊断出患有先天性心脏病。所有新生儿均由两名新生儿科医生进行临床检查，若有心脏异常则转诊至儿科心脏病专家处。对他们每个人都进行了心电图记录，87%的人进行了胸部X光检查，1980年以后出生的人进行了超声心动图检查。这些儿童一直随访至1989年12月。平均随访期为6年。
年发病率在9.5%至15.7%之间（平均12.3%）。102名儿童（17.6%）出现染色体异常和心脏外畸形，分别为50例（8.6%）和52例（9.0%）。室间隔缺损（VSD）和动脉导管未闭（PDA），单独或合并存在，是最常被识别的先天性心脏病。52名儿童（9.0%）在随访中失访。579例中有11例的初始诊断发生了改变。187名儿童（32.3%）出生时提示存在先天性心脏病的临床和/或仪器检查结果出现了自发消失：144例诊断为VSD，43例为PDA。1975 - 1980年期间VSD的患病率为千分之4.7，1981 - 1984年期间为千分之8.6。131名儿童（22.6%）死亡，其中127名（96.9%）在生命的第一年死亡。52名儿童接受了手术，5名进行了肺动脉瓣成形术。
在长时间的观察期内，先天性心脏病的患病率没有变化，而VSD的数量增加。VSD和PDA在很高比例的病例中会自发闭合。该研究表明，对于患有严重先天性心脏病的新生儿，尤其是生命第一年的新生儿，重症监护病房是有用的。
