Schmaltz A A, Hinkeldey K, Hoffmeister H E, Apitz J
Monatsschr Kinderheilkd. 1985 Oct;133(10):743-8.
46 Children with tricuspid atresia, 56 with pulmonary atresia and ventricular septal defect (VSD) and 33 with pulmonary atresia and intact ventricular septum were diagnosed and treated at the University's Children's Hospital Tübingen between 1967 and 83. Their age distribution and mortality is compared to 91 autopsy-proven cases of tricuspid atresia, 123 cases of pulmonary atresia with VSD and 41 cases without VSD, reported in the time between 1783 and 1945 showing the natural history of these malformations. Inspite of intensive medical and operative therapy, the results of which are reported in detail, mortality continues to be high: 55% of our patients with tricuspid atresia die within the first year of life, 60-62% within the first 2 years (in the bibliographical collective 64% and 73%), 53-61% of patients with pulmonary atresia and VSD die within the first, 61-68% within the first 2 years (in the bibliographical collective 74% and 78%), and 78% of patients with pulmonary atresia without VSD within the first year, 88% within the first 2 years of life (in the bibliographic collective 95% and 97%).
1967年至1983年间,图宾根大学儿童医院诊断并治疗了46例三尖瓣闭锁患儿、56例肺动脉闭锁合并室间隔缺损(VSD)患儿以及33例肺动脉闭锁合并完整室间隔患儿。将他们的年龄分布和死亡率与1783年至1945年间报告的91例经尸检证实的三尖瓣闭锁病例、123例肺动脉闭锁合并VSD病例以及41例无VSD病例进行比较,这些病例显示了这些畸形的自然病史。尽管进行了积极的药物和手术治疗,详细报告了治疗结果,但死亡率仍然很高:我们的三尖瓣闭锁患者中有55%在出生后第一年内死亡,60 - 62%在头两年内死亡(文献汇总中为64%和73%),肺动脉闭锁合并VSD患者中有53 - 61%在第一年内死亡,61 - 68%在头两年内死亡(文献汇总中为74%和78%),肺动脉闭锁无VSD患者中有78%在第一年内死亡,88%在出生后头两年内死亡(文献汇总中为95%和97%)。