Cardiac specialists at Schneider Children’s are accustomed to and expert at diagnosing cardiac defects in fetuses, but even they were amazed to discover that two twins suffered from the same complex multiple heart defect, Tetralogy of Fallot (TOF) with pulmonary atresia. The twin girls were born in the 37th week of pregnancy, one weighing 2.4kg and the second, 2.7kg, and underwent successful surgery a few days after their birth.
Dr. Einat Birk, Director of the Cardiology Institute at Schneider Children’s, examined the fetuses during pregnancy and found that both twins suffered from TOF with pulmonary atresia. This is a combination of congenital heart defects (CHD), which are manifested by a totally blocked pulmonary valve, small pulmonary artery and a huge hole between two heart chambers. The chances of this type of CHD occurring in newborns are 0.007 in 1,000 births, and the possibility that these will appear in both twins is very rare.
During the fetal stages, the twins survived due to ductus arteriosis, blood vessels found in all fetuses connecting the pulmonary artery to the aorta and thus bypassing pulmonary blood exchange. Because the ductus closes instantly after birth, the twins’ lives were in immediate danger. As a result, from the moment of their birth, they were treated with medications that delayed the closing of the ductus, and to allow them to gain weight prior to the corrective surgery. The twins were treated in the Neonatal Department, headed by Prof. Lea Sirota.
Drs. George Frenkel and Gabi Amir, senior cardiac surgeons at Schneider, performed the complex operations in the twins, both of whom weighed less than 3kg. The first twin underwent surgery about a month after her birth, while her twin sister followed a week later. Corrective surgery, which fully repaired the defects, included closure of the large hole between the chambers and creating an outlet for the right ventricle. Both twins recovered quickly and each was discharged a week after the operation allowing the family to celebrate the Passover Festival together, much earlier than anticipated.
The Cardiac Surgery Division at Schneider Children’s treats thousands of sick children from all over the country each year. It has the capability of responding to highly complex conditions in small infants a day old. Early diagnosis accords the planning of the birth at a tertiary care center that is able to dictate policy and supply immediate treatment, thus obviating potential early damage which can result from a heart defect such as this. Annually, Schneider Children’s performs over 10,000 assessments through echocardiograms, more than 400 cardiac surgeries and over 500 cardiac catheterizations, 60% of which are therapeutic.