Little Girl Blue

She lay there, small and seemingly lifeless. Everything surrounding her is industrial—wires and tubes connecting this way and that—paralleling the chaotic bustle of people around her. It is almost easy to lose any sense of humanism as nurses drape all but one small area of the body and the sterile, mechanical environment of the operating room inevitably envelops her small frame. It is almost easy –until you recognize a “Pampers” emblem.  It stands in stark contrast to everything around her, serving as an inescapable reminder that beneath the sterile, blue drape, there lies a 3-month old baby girl. The site of the diaper evokes the image of a loving mother, gently wrapping the straps of that diaper around her baby’s chubby thighs as she pretends to nibble her tiny toes. How many times had I done just the same with one of my eight nieces and nephews? Her diaper is a symbol of her position in this world—a helpless child—dearly loved by parents who must be scared out of their minds. For that small field the nurse left exposed—it was their child’s chest—and  soon a surgeon would have his hands on their child’s tiny, damaged heart.

The field of pediatric cardiac surgery began in August, 1938, with Dr. Robert E. Gross, then Chief Resident of Surgery at the Children’s Hospital in Boston. Dr. Gross, in the absence of the Surgeon in Chief, William E. Ladd, defiantly performed the first successful ligation of a patent ductus arteriosus in a 7-year old girl (Castañeda, Congenital Heart Disease: A Surgical-Historical Perspective). The ductus arteriosus is a blood vessel which connects the pulmonary artery to the proximal descending aorta, effectively allowing blood to bypass a fetus’s not yet functioning lungs during development. This vessel usually closes within a couple of days after birth. However, in approximately 1 in 2000 births, this vessel remains open resulting in a congenital anomaly termed patent ductus arteriosus (PDA). Gross’s operation represented a revolutionary step – the field of pediatric cardiothoracic surgery was born. In the following decades, major advancements such as the artificial heart-lung machine and deep hypothermia with circulatory arrest allowed surgeons to repair defects that had once seemed impossible and reverse prognoses that were previously deemed fatal (such as a pulmonary embolus which caused the death of an otherwise healthy young girl, inspiring John Gibbon to develop the heart-lung machine). And it is in the light of these historical advents that I was able to observe the successful repair of one of the most challenging congenital heart defects – hypoplastic left heart syndrome.

Hypoplastic left heart syndrome (HLHS) is a complex congenital heart defect which requires intense surgical intervention.  For the little girl asleep on the surgical table, this was only the first of a three-stage procedure. She was born with an underdeveloped aorta and left ventricle and atretic aortic and mitral valves. This is a rare case in which a surgeon would want to maintain a patent ductus arteriosus, due to the inadequate left ventricle and thus inability to pump oxygenated blood throughout the body.  In a series of complicated procedures a surgeon must manipulate the cardiovascular system such that the right ventricle performs the duties of the left ventricle (that is, pumping oxygenated blood to the rest of the body) and the passive systemic venous pressure allows the passing of deoxygenated blood to the lungs. The surgeon must, in effect, eliminate the whole left side of the heart. At the end of this surgical marathon, it is estimated that only 70% of HLHS patients will reach adulthood (Arnold et al., Hypoplastic Left Heart Syndrome – Unresolved Issues). This baby was quite sick and with numerous, complicated and physically taxing procedures in her future, the staff informed me that this was an up-hill battle, to say the least.

As I watched the surgeon drag the scalpel across her yellow, iodine-stained chest, I could not help but wonder about the little girl beneath of the blue, sterile drape. Did she have a favorite toy? Did her eyes light up at the sight of her parents? Would her earliest memories involve seeing the same tubes and wires that I will forever associate with her? I silently cheered her on, “you can do this, little one. You are small but you are strong!” Nine hours later, I watched as she was wheeled into the recovery room, in awe of not only the beautiful complexity of the procedure but also the tremendous responsibility of the medical staff. Writing about choosing a career in congenital heart surgery for the AATS, Dr. William G Williams wrote, “The opportunity to prevent a newborn’s premature death in exchange for that baby’s years of life seem reason enough… The demands are great, but the rewards are considerable.” In observing my first Norwood procedure, I became only minimally acquainted with the immense gratification of this field and for that opportunity, I am appreciative. You are imprinted on my mind and I will forever be rooting for you baby girl beneath the blue drape