The creation of life: a shrill cry as the baby takes its first breaths, the pink hue of its soft skin and the unbreakable connection that forms as the mother cradles her newborn in her arms for the first time. But just how long does this excitement last? As the baby’s cries are cut short, the baby’s skin turns blue, and this precious child is whisked off to the neonatal intensive care unit. This is the first of many difficult decisions parents of babies diagnosed with hypoplastic left heart syndrome, a severe congenital heart defect, will face.
The defining features of a baby with hypoplastic left heart syndrome is the underdeveloped left side of his or her heart. In a normal heart, the left side of the heart is essential for supplying the body with oxygenated blood. After the blood is loaded with oxygen in the lungs, it travels to the left atrium, flows into the left ventricle via the mitral valve, and is finally pumped to the rest of body via the aorta.
In contrast, a baby with hypoplastic left heart syndrome has an abnormally small left ventricle, and sometimes a narrow or completely closed aorta, aortic valve, and mitral valve. Consequently, the left side of the heart is incapable of pumping blood to the body. For a short while, the baby’s heart can compensate by utilizing the foramen ovale, an opening between the two atriums of a fetal heart which seals a few days after birth, and the ductus ateriosis, a passageway between the aorta and the pulmonary artery.
With the help of these openings, oxygen-rich blood can flow from the lungs into the left atrium, bypass the dysfunctional left ventricle by immediately flowing into the right atrium through the foramen ovale or another similar hole. Many cases of hypoplastic left heart syndrome are diagnosed by a simple fetal echocardiogram in-utero, so a cardiac team is mobilized to best care for the baby immediately after birth. Though medicine is injected to prevent the holes from closing, the baby is essentially functioning with half a heart, and so reconstruction of the heart is usually performed in sequence of three complex surgeries.
Though statistically, the three-stage reconstruction procedure has proved beneficial, parents are given the option of terminating the pregnancy. In a study that surveyed parents of hypoplastic left heart syndrome patients conducted by Marcia Ellinger and Gwen Rempel, one parent’s gut reaction was to reject the option of an abortion. “You just showed us our baby on that monitor and there’s no way you’re going to terminate after you see that,” she said. Parents also chose the surgical option because they were determined to pursue a “fix” to the problem, and others because they felt that once their baby has survived thus far, denying the surgery would be like giving up hope.
Other parents considered an abortion when they recognized the difficult road ahead for their child, which includes major surgeries and other probable complications. They also were aware of the fact that it is probable that even if their child survived the surgeries, he or she would require a heart transplant in the future. In that case, a miracle for their family would have to stem from the nightmare of another family.
No matter what the parent’s ethical or religious views, the healthcare providers have the obligation to provide the parents with what is called informed consent. They must allow the parent to have autonomy, in which the parent is the sole decision-maker and the healthcare professionals can only give information and statistics, as well as answer questions and discuss the options with the parent. The professional must also uphold the principles of beneficence and veracity in informed consent, meaning they must work toward optimizing the welfare of the patient and the family, and they must present the care options in an unbiased manner.
Once parents have chosen the surgical treatment option for their child, their child is still at risk. But the modern healthcare system and medical technology has greatly improved the care for such individuals. The Children’s Hospital of Philadelphia, one of the leading pediatric cardiac centers in the world, has a program designed specifically to monitor the growth and development of babies between their first and second surgeries. Dr. Jack Rychik, a cardiologist and professor, performs groundbreaking research in hypoplastic left heart syndrome. For example, some hypoplastic left heart syndrome patients lack an atrial septal heart defect and are therefore not candidates for the Norwood procedure. He has applied a procedure done for fetuses with other diseases, to fetuses with this specific defect. He injects a needle into the pregnant mother’s belly, aiming it precisely in the wall that separates the two tiny atriums of the fetus’s heart and creating a hole. Then, when the infant is born, surgeons are able to perform the Norwood procedure.
Though hypoplastic left heart syndrome is a devastating diagnosis, with all the new research, the survival rate has increased from five percent to over fifty percent. With the advancement of surgical techniques, now children who otherwise would have had a minimal chance of survival have a new lease on life.