JAMA Revisits Classic Hopkins Blue Baby Study That Revolutionized Cardiovascular Medicine

A Johns Hopkins study published 63 years ago will make an encore appearance in the July 16 issue of the Journal of the American Medical Association (JAMA) as part of a year-long retrospective celebrating JAMA's 125th anniversary by revisiting papers that changed the course of modern-day medicine. Full text of the original paper is available online.

The now-classic "blue baby" report by pediatric cardiologist Helen Taussig (1898-1986) and surgeon Alfred Blalock (1899-1964) first appeared in JAMA on May 19, 1945. In their paper, Taussig and Blalock described for the first time the physiology of tetralogy of Fallot, one of the most common congenital malformations of the heart that was poorly understood at the time, considered inoperable and ultimately fatal. The malformation causes inadequate blood flow from the heart to the lungs and profound lack of oxygen in the blood, giving an infant's skin its hallmark bluish hue, hence "blue baby."

In addition, the paper described the first three operations in medical history designed to alleviate the defect using a special "shunt" technique that increased blood flow from the heart to the lungs. Children undergoing the surgery experienced an immediate and dramatic improvement while still in the operating room. When their oxygen-starved bodies were finally flushed with oxygen-rich blood, their bluish complexions turned a healthy pink color, an observation that prompted Blalock to exclaim famously after surgery number three, "The boy's a lovely color now."

At the time, Taussig and Blalock almost certainly knew their work would dramatically change treatment of heart disease, and records at Johns Hopkins show that hundreds and hundreds of parents sought help for their children in the months that followed. The study also revolutionized pediatric cardiology, a then nascent field, and ushered in a new era of cardiac surgery. In hindsight, it also altered the course of academic medicine, according to Johns Hopkins Children's Center cardiac specialists writing in a July 16 JAMA commentary accompanying the reprint summary of the original paper.

Historians, filmmakers and journalists have widely told the story of the research and eventual surgical solution, which began with an idea from Taussig, who took it to Blalock, who first sketched a surgical approach to the repair. But it was Vivien Thomas (1910-1985), a black surgical technician, at the time working as a lab and office assistant with Blalock, who was instrumental in developing the necessary procedure and instrumentation in dogs.

This mélange of disciplines as disparate as pediatric cardiology, surgery and anesthesiology working toward treatment of a single disorder became and to date remains the model for progress and innovation in medicine.

"Not only did the team's unprecedented collaboration in effect give birth to pediatric cardiology and led to the first successful treatment of this fatal heart defect, but it later became the prototype of the bench-to-bedside approach, a staple in academic medicine today," says Anne Murphy, M.D., an author on the commentary and a pediatric cardiologist at Johns Hopkins Children's Center.

In the decades that followed, the teamwork by Taussig, Blalock and Thomas also foreshadowed Johns Hopkins' efforts to eliminate racial and gender inequalities in academic medicine. In the 1940s, at Johns Hopkins, the venerable citadel of medicine, Taussig, a woman, and Thomas, an African-American, teamed up with Blalock, a white male surgeon -- a diverse and brilliant crew whose combined talent and expertise pioneered a surgery that has saved millions of lives worldwide.

Taussig went on to achieve the status of a full professor at Johns Hopkins -- one of the first women to do so -- but Thomas' role was not fully acknowledged until much later, the JAMA commentators point out, and the original paper did not credit Thomas' contributions.

"The collaboration awakened everyone to the fact that talented people like Thomas, who would have clearly been a superb surgeon, were marginalized, and medicine suffered for it," says commentary co-author Duke Cameron, M.D., head of pediatric cardiac surgery at Johns Hopkins. "It was a realization that drove much of Hopkins' subsequent efforts toward equality and diversity."

In 1976, Hopkins gave Thomas an honorary doctoral degree and appointed him instructor in surgery. Today, one of the four advisory colleges for medical students at Hopkins is named for Thomas. Every entering class learns about Thomas' story and his contributions to modern medicine.

Four out of 1,000 U.S. babies are born with heart defects, such as tetralogy of Fallot. Worldwide, nearly 1 million babies are born with heart defects, researchers estimate.