Martínez-Quintana Efrén, Rodríguez-González Fayna, Medina-Gil José María, Agredo-Muñoz Julio, Nieto-Lago Vicente
Complejo Hospitalario Universitario Insular-Materno Infantil, Las Palmas de Gran Canaria, Spain.
Cir Cir. 2010 May-Jun;78(3):245-50.
Long-term complications of Down syndrome patients with congenital heart disease are poorly known.
We carried out a retrospective study of Down syndrome patients with congenital heart disease and patients with atrioventricular septal defect with and without Down syndrome.
Between 2004 and 2008, 317 patients with congenital heart disease were followed-up in the Adult Congenital Heart Disease Unit. Of these patients, 19 (6%) with a mean age of 26.8 +/- 8.1 years had Down syndrome. Atrioventricular septal defect was the most frequent congenital heart disease(63%) followed by ventricular septal defect (26%). Ten patients (53%) were operated on during childhood. Three of these patients required reoperation during adulthood (two patients due to left ventricle outflow tract obstruction and one patient due to left atrioventricular valve insufficiency). Four patients (21%) had Eisenmenger syndrome with improvement of functional class in those treated with bosentan, two patients (10.5%) had bacterial endocarditis and two patients (10.5%) died. No significant differences were seen in left atrioventricular valve insufficiency between atrioventricular septal defect in patients with and without Down syndrome (1.5 +/- 0.9 vs. 1.7 +/- 0.8, p = 0.689).
Left atrioventricular valve insufficiency and left ventricle outflow tract obstruction were the most frequent long-term complications requiring surgical reintervention in patients with atrioventricular septal defect.
唐氏综合征合并先天性心脏病患者的长期并发症鲜为人知。
我们对唐氏综合征合并先天性心脏病患者以及有和没有唐氏综合征的房室间隔缺损患者进行了一项回顾性研究。
2004年至2008年期间，成人先天性心脏病科对317例先天性心脏病患者进行了随访。在这些患者中，19例（6%）患有唐氏综合征，平均年龄为26.8±8.1岁。房室间隔缺损是最常见的先天性心脏病（63%），其次是室间隔缺损（26%）。10例患者（53%）在儿童期接受了手术。其中3例患者在成年期需要再次手术（2例因左心室流出道梗阻，1例因左房室瓣关闭不全）。4例患者（21%）患有艾森曼格综合征，使用波生坦治疗的患者功能分级有所改善，2例患者（10.5%）患有细菌性心内膜炎，2例患者（10.5%）死亡。患有和未患有唐氏综合征的患者在房室间隔缺损的左房室瓣关闭不全方面没有显著差异（1.5±0.9对1.7±0.8，p = 0.689）。
左房室瓣关闭不全和左心室流出道梗阻是房室间隔缺损患者最常见的需要手术再次干预的长期并发症。
