Medicine: Babies of Blue Babies

In the 18 years since Johns Hopkins’ famed Surgeon Alfred Blalock electrified the medical world by turning “blue babies” into pink and active youngsters, at least 10,000 such invalids since birth have had operations of this type. The number may be closer to 20,000; nobody knows for sure. Until now, nobody has known the fate of these children as they matured. Could they marry and have children themselves? If they did, what were their children’s chances of being born with defective hearts?

“Don’t Be Discouraged.” Last week Pediatrician Helen B. Taussig, 64, who did the basic research on blue babies and suggested the operative approach to Surgeon Blalock, gave an encouraging report on the progress of the 1,700 patients who have had the blue-baby operation at Baltimore’s Johns Hopkins. Among these and other victims of congenital heart defects, at least 235 with whom Dr. Taussig has been able to keep in close touch have become parents: 76 men and 159 women. In 160 pregnancies where the father had the heart defect, six children were born malformed, three had heart defects and three had other malformations. The rates were almost identical in 348 cases where the affected parent was the mother: 13 malformed infants, six with heart defects.

The rate of 1.8% for heart defects is about six times the normal average, said Dr. Taussig and Dr. Catherine A. Neill. Blue-baby mothers also had more spontaneous abortions (miscarriages) than the general population. But the researchers felt that the rates were not high enough to discourage former blue babies from attempting parenthood. Most of them can achieve it and have healthy offspring.

A striking case is that of Adele Roveda, whose heart defect was diagnosed in infancy before any corrective surgery had been devised. At 17, she had an early Blalock-Taussig operation, and another nine years later. Now 31, and married to Baltimorean Raymond W. Hepner Jr., she has a normal daughter almost three years old, and does her own housework.

Another blue-baby mother has had five normal children. Why the less fortunate ones lose their babies, Drs. Taussig and Neill are not certain. They doubt that it is simply because of oxygen shortage, but suspect that a little-understood hormone deficiency is involved.

A Fifth Anomaly. Most blue babies, so called from the color of their fingertips and lips, suffer from a set of four inborn defects in the heart and arteries, known as Fallot’s tetralogy. The effect is to recirculate much blood from which oxygen has been naturally removed in the veins, and send only part of it to the lungs for re-oxygenation. The Taussig-Blalock operation, devised years before open-heart surgery with a heart-lung machine became possible, is a compromise: it consists of purposely creating a fifth defect—a connection from the aorta to the pulmonary artery—to shunt more blood to the lungs and thus overcome some of the effects of the original four.

With the heart-lung machine, many surgeons favor a more radical and complex operation in which they repair the basic defects of the major arteries and the chambers of the heart itself. But a major problem still confronting the blue babies’ doctors is to decide which operation is best suited for each patient, especially since the more drastic operation carries a higher risk. At the Hopkins, Dr. Blalock and his associates still decide to rely on the Taussig or a similar operation about once a week.