Two sisters With Ehlers-Danlos syndrome, inherited as an autosomal rec
essive trait, and congenital heart disease are herein reported. One wa
s a 20-year-old woman with Ehlers-Danlos syndrome and multiple aphthou
s stomatitis, bronchial asthma, an emphysematous lung, a ventricular s
eptal defect and a bilateral inguinal hernia due to hyperextensibility
and joint hypermobility. The other was a 17-year-old girl with the sa
me syndrome and an atrial septal defect, a ventricular septal defect a
nd patent ductus arteriosus. The combination of Ehlers-Danlos syndrome
and congenital heart anomalies in these siblings suggest a common gen
etic defect to be the cause of these diseases.