the corner instead of the operating table that day. However, they were Viven’ s hands which had perfected the technique in a canine model of tetralogy of Fallot, where pulmonary blood flow was surgically restricted to mimic the pathophysiology of severe pulmonary stenosis. Only posthumously did his name become informally attached to the BTT shunt procedure, thanks to ethical reviews conducted by the American Medical Association and various medical journals.
However, the third collaborator in this medical band of misfits, pediatric cardiologist Dr. Helen Taussig, found immediate recognition for her part in saving the“ blue babies.” It didn’ t come easily. Half-deaf from a childhood bout of otitis media, dyslexic and female, the odds of academic success in the 1920s were stacked against her. She pursued medical school as one of the only ways to obtain an actual degree as a woman and was subsequently shuffled into pediatrics as a last resort. In 1930, unsure of what exactly to do with a female cardiologist, leadership at Johns Hopkins University relegated her to the ward of hopelessly cyanotic children, for whom no cure existed.
With her hearing failing her, she relied on her fingertips to detect the tap of the right ventricle under the chest wall in pulmonary hypertension, or the displaced precordial impulse in left ventricular hypertrophy or the vibrations created by turbulent flow through ventricular septal defects and stenotic valves. Her observation that the onset of neonatal cyanosis often coincided with the typical timing of spontaneous patent ductus arteriosus closure prompted her to seek a way to re-establish a connection between systemic and pulmonary arterial circulation to circumvent the anatomic obstruction in tetralogy of Fallot. After an initial rejection from a surgeon who developed a surgical technique for patent ductus arteriosus closure, she approached Dr. Blalock, who had been recruited to Johns Hopkins to study pulmonary hypertension. He didn’ t say no. The tenacity of this unlikely trio transformed congenital heart disease from a career dead-end into an entirely new frontier, with the first pediatric cardiology textbook, Congenital Malformations of the Heart, authored by Dr. Helen Taussig herself.
The BTT shunt evolved into a modified version utilizing a manmade tubular GORE-TEX graft which connects the first aortic branch vessel to the right pulmonary artery, obviating the need for sacrifice of the subclavian artery. But its use lives on in modern-day congenital heart surgery to maintain pulmonary blood flow in children with pulmonary stenosis as well as single ventricle heart disease.
1953 brought the advent of the cardiopulmonary bypass machine, allowing for surgical intervention of more complicated intracardiac defects. By the 1980s, the arterial switch procedure for transposition of the great arteries became commonplace, and the early 1990s brought the most modern form of the Fontan procedure for single-ventricle heart disease, whereby pulmonary and systemic circulations were surgically separated, and the great veins drained directly to the pulmonary arteries, so that now the single ventricle’ s only job would be to pump blood from the aorta to the systemic circulation.
The once bimodal survival curve in congenital heart disease began to more closely mirror the bell-shaped survival curve of the general population. These children grew up. They became part of society, they pursued aspirations of their own, they had families. But what they didn’ t have was a cardiologist versed in both congenital heart disease and the complicating things that come with adulthood, like acquired comorbidities and pregnancy. The pediatric cardiologists did their best, but a growing chasm developed between the heroic efforts made in childhood and how to manage the emerging long-term sequelae of those early surgeries in adulthood.
It’ s not as simple as sending the patient to a general adult cardiologist. By no fault of their own, adult cardiologists and adult cardiothoracic surgeons receive little to no training in congenital heart defects, though it represents about 5 % of the adult cardiology board exam. Adult emergency departments may not understand the aberrations in vital signs that may be expected for that patient’ s particular anatomy, such as low upper extremity blood pressure in a patient with a prior BTT shunt or subclavian flap coarctation of the aorta repair, or low saturations in a patient with single ventricle anatomy, leading to unnecessary and potentially harmful testing and treatments.
While the mission was accomplished that many of these children not only survived, but lived into adulthood, they found themselves flung from the nest of their pediatric cardiologist’ s office into the oblivion of adult medicine, where they were neither understood nor felt accepted, and often lost to care.
Imagine with me that you are the mother or father of a baby born blue, watching their belly rise and fall 70 times per minute, the strain of their subcostal retractions, their tiny body withering away in heart failure from behind the white metal bars of a hospital crib, while the freshly washed baby clothes sit neatly folded in their nursery dresser at home, next to the rocking chair where you envisioned them drifting off to lullabies. In your grief of the babyhood lost, you are offered a life-saving surgery, your fingers trembling you sign the consent. Maybe you never prayed before, but you pray then.
I don’ t know that any parent is ever prepared for what happens next: the mess of tubes and lines and blood smears and endless beeping; complications, from infections to prolonged intubation, CPR or even the need for ECMO. There’ s one step forward, two steps back on many of those days, with tears in the corner and hospital coffee. There is the frustration of feeding aversion, vomiting, catching up on missed milestones and all the medications in tiny syringes. And when you finally make it home, you are both relieved and terrified of this tiny child, the scar on her chest and what lies underneath. Eighteen annual pediatric cardiology visits later, she is patted on the head one last time and told she has aged out of the clinic. The medical home that nurtured both you and your child suddenly dissolves and the powerlessness you felt before that very first surgery bubbles back to the surface. How is it fair that the medical system that mended the hearts of these unique children in ways only they could understand are now abandoning them because of their age?
In 2001, the Adult Congenital Heart Association, which initially( continued on page 20) October 2026 19