Autosomal dominant mendelian midline complex, secundum atrial septal defect associated to cardiac and facio-thoracic defects. A familial case.

The kindred of 38 individuals reported here have various anomalies : 1. facio-thoracic malformations: hypertelorism, nasal deviation, deft lip a nd palate, upper-incisors diastema and pectus excavatum; 2. cardiac anomalies: sinus node bradycardia, atrial fibrillation, nodal rh ythm, atrial septal defect, Wolff-Parkinson-White syndrome, low insertion o f the septal tricuspid valve corresponding to an Ebstsein syndrome, pulmoni c "en dome" valve stenosis, aortic valve stenosis, long RT, and intraventri cular conduction blocks. Almost all these defects are septal or paraseptal. Mitral stenosis is probably rheumatoid, Such median varied pathology has n ot been yet reported. All the extra-cardiac anomalies are situated along the vertical upper half- body midline. All cardiac anomalies are in the septal or paraseptal region; It is an autosomal dominant trait that implies the early embryonic develop ment of the midline of cardiac and extra-cardiac structures.