[Familial polymorph ventricular extrasystole associated with Pierre Robin syndrome].
In a family of 9 persons over 3 generations, 6 had incessant polymorphic ventricular extrasystoles, often in salves, resembling unsustained bidirectional ventricular tachycardia. Ventricular repolarisation was abnormal with giant U waves. Five persons had suffered syncopes. All had a facial abnormality: one woman had a complete Pierre Robin syndrome with mandibular hypoplasia, glossoptos and cleft palate; in the other cases, minor forms were observed, with micrognathia and ogival palate. Two patients had bone abnormalities of the feet with total agenesis of the last 2 phalanges of several toes. This familial polymorphic ventricular extrasystole with repolarisation abnormalities has many features in common with the congenital long QT syndrome; associated with the Pierre Robin syndrome and bone abnormalities of the extremities it would seem to be a new multiple congenital abnormalities syndrome.
Kieny
J R
JR
Service de cardiologie, fondation Lucien-Dreyfus, clinique du Diaconat, Mulhouse.
Stoll
C
C
Roul
G
G
Hessel
F
F
Bareiss
P
P
Sacrez
A
A
D000293:Adolescent; D000328:Adult; D005117:Cardiac Complexes, Premature / Q000150:complications / Q000235:genetics*; D002648:Child; D004562:Electrocardiography; D005260:Female; D006352:Heart Ventricles; D006801:Humans; D008297:Male; D010375:Pedigree; D010855:Pierre Robin Syndrome / Q000150:complications / Q000235:genetics*; D013575:Syncope / Q000209:etiology
Extrasystolie ventriculaire polymorphe familiale associée à un syndrome de Pierre Robin.