Whether warned or not, death of baby devastating

Death came and took a baby. The 11-day-old died without warning. Death did not crash through the door or hide in the dark coming out in the sleep. It was with the baby at birth and quietly came home with the joyous parents and waited. Then, when the mother was holding the infant lovingly in her arms, death stepped forward. On the outside the child was perfect. On the inside its heart was fatally flawed. The heart was without a left-sided pumping chamber. The rare condition is called hypoplastic left heart.

In the flawed formation of the fetal heart, the left ventricle — the lower of the four chambers — fails to develop. Instead it is a sliver of a space unable to support future life. Before birth this error of creation is not lethal. The cardiac circulation in the womb receives oxygen from the mother via the placenta and umbilical cord. This blood is transported to the rest of the baby via the venous system coming through the liver. It next flows to the right side of the heart. The right ventricle squeezes the blood to the lungs. With the lungs not the source of oxygen in the unborn, the blood is routed away from the pulmonary artery to the aorta by the ductus arteriosus and on to the rest of the body.

So in the fetal circulation, the developing organism can grow normally without the left ventricle. It is only at birth when the lungs now supply the oxygen and the ductus closes that the left side is needed to push that blood to the whole body. Death occurs in this condition of HLH when the ductus begins to constrict, cutting off the life-sustaining oxygenated blood.

If identified early, there are two therapeutic options: a staged series of complicated surgeries called the Norwood procedures or a neonatal total heart transplant. Neither are a ticket to a normal life for the child or the whole family. The resources required for either are both enormous and limited. The cost is not only in dollars but in the sacrifice of everything for hope that is not reassured. If a transplant is the chosen course, then the family or a parent will often relocate to one of the few institutions in the country that are capable of such an adventure.

The palliative operations reconstruct the child's heart to make the right ventricle the permanent pump to the body. They are not a guarantee of success and carry with them a risk of death that has been improving over time but still is a real consideration. Besides the exhaustive burden in parental disruption and emotions, there is the consideration of the other children, if there are any, and their needs.