Innovative treatment of congenital cardiovascular malformations is not new to Children's Hospital Boston. The first reparative surgery for any lesion, ligation and division of a patient ductus arteriosus was performed here in 1938 by Robert E. Gross, MD. Legend has it that Dr. Gross, chief surgical resident at the time, waited for his "superiors" to be out of town before taking one of the giant steps in the care of children with heart disease.

There were many advances in the understanding and management of congenital cardiac disease over the next decades. Palliative surgery for complex heart disease was pioneered at Johns Hopkins, where an "artificial" patent ductus (the Blalock-Taussig shunt) was developed for children with inadequate pulmonary blood flow. Reparative surgery for relatively simple malformations, such as atrial and ventricular septal defects, was performed at many medical centers in the 1950s and 1960s. During the 1970s it became routine to perform reparative surgery during infancy for children with more complex forms of heart disease, such as tetralogy of Fallot and transposition of the great arteries.

Norwood Procedure Changes Expectations
These major advances benefited children with two ventricles (heart pumping chambers) and two well-formed great arteries (the aorta and pulmonary artery). But for children born with a single ventricle, major challenges still remained. Professor Frances Fontan, a French cardiac surgeon, changed that outlook by developing an operation for children with a well-formed left ventricle and aorta, but a diminutive right ventricle. That demonstration served to stimulate the resourcefulness of another Children's Hospital cardiac surgical resident, William I. Norwood, MD.

Many children with a single ventricle are born with an underdeveloped left ventricle and associated problems with their aorta. In the most common of these malformations, hypoplastic left heart syndrome, the left ventricle is so small that it can never function effectively, and the aorta is often only a millimeter or two in diameter. Dr. Norwood saw this as a challenge. He set out to develop an operation that would benefit the majority of children with single ventricles, regardless of the underlying anatomic situation. He believed that many additional children could undergo Professor Fontan's operation, as long as there was one functioning ventricle, either right or left, and one reasonably sized great vessel, either an aorta or pulmonary artery. If those criteria were met, a "Norwood procedure" could be performed in infancy. Later, the children could benefit from Fontan's operation.

Initially, Dr. Norwood's operation was very difficult to perform. We were treading on new ground. The anatomic variability of many forms of single ventricle was not understood. These children had never been "candidates" for surgery, so little attention had been paid to subtle variations in anatomy that suddenly began to matter. The intensive care unit management of these patients was also entirely new. We had to learn about a new pathophysiology, develop innovative strategies for ventilatory management, and master how to deal with a different set of complications.

Growing Survival Rates Support Procedure's Promise
The initial results of patients undergoing Norwood procedure were not terribly encouraging. One-year survival slowly increased from less than 15% in 1980 to 30% in 1983 and then to 50% in 1990. Now, with continued improvement in our diagnostic acumen, intensive care unit management, and surgical skills, we expect greater than 80% of patients to survive to a year of age, and nearly all of them to benefit from Professor Fontan's operation.

Dr. Norwood faced a great deal of opposition to his attempts to advance the care of children with heart disease. Early on he was accused of abusing children by operating on them. Some said anything, even the transplantation of a baboon heart, was better than a Norwood procedure. Within Children's Hospital there was some opposition, as well. Yet throughout the difficult early days, Dr. Norwood had the enthusiastic support of his chief, Aldo Castañeda, MD, who was in town when the first operation was performed, and Alexander Nadas, MD, the chief of cardiology, was also fully behind the effort.

The first child to complete the journey from Norwood procedure to Fontan operation was reported in the New England Journal of Medicine in 1983. He is now 22 years old. Many, many other children have traveled the same path.