[The heart-hand syndrome. A new variant of disorders of heart conduction and syndactylia including osseous changes in hands and feet].
A male infant was born at the 36th week of gestation by cesarean section because of an intrauterine bradycardy. There was a 2nd degree a. v.-bloc postnatal. The heart rate was normal after the 5th day of life with a QT-interval of 150 percent resp. 120 percent after the 15th day. Additionally there were syndactylies of hands and feet. The infant suddenly died at the age of 5 months. Examination of the parents revealed a QT-interval of 115 percent as well as synostoses of the carpus in the father. These microsymptoms are very important for genetic counseling of the family. There is a recurrence risk of 50 percent for further children because a dominant trait is to be suggested.
Reichenbach
H
H
Abteilung Humangenetik, Universität Leipzig.
Meister
E M
EM
Theile
H
H
D004562:Electrocardiography; D005532:Foot Deformities, Congenital / Q000235:genetics*; D005817:Genetic Counseling; D006228:Hand Deformities, Congenital / Q000235:genetics*; D006327:Heart Block / Q000235:genetics*; D006801:Humans; D007231:Infant, Newborn; D008133:Long QT Syndrome / Q000235:genetics; D008297:Male; D013576:Syndactyly / Q000235:genetics*; D013577:Syndrome; D013580:Synostosis / Q000235:genetics
Herz-Hand-Syndrom. Eine neue Variante mit Erregungsleitungsstörungen am Herzen und Syndaktylien einschliesslich ossärer Veränderungen an Händen und Füssen.