Her Heart & What Lies Ahead

Within a few hours after birth, Daphne was diagnosed with a congenital heart defect known as pulmonary atresia with hypoplastic right ventricle. This defect affects the right side of the heart, which is the side of the heart that receives deoxygenated blood from the body and sends it to the lungs to be oxygenated.

A normal heart has four chambers. The left atrium receives oxygenated blood from the lungs and sends it to the left ventricle, which pumps it to the rest of the body. The right atrium receives deoxygenated blood from the body and sends it to the right ventricle, which pumps it to the lungs to be oxygenated. As blood moves through the heart, it passes through valves that allow the blood to move forward but not backward at regular intervals. The pulmonary valve sits between the right ventricle and the pulmonary artery, which is the artery that leads from the heart to the lungs.

In a child with pulmonary atresia, the pulmonary valve is completely sealed, which prevents deoxygenated blood from returning to the lungs to be oxygenated. As a result of this defect, the right ventricle is unable to perform its natural function during fetal development and becomes hypoplastic, meaning severely underdeveloped. Because blood cannot get to the lungs, this type of heart defect is extremely critical and must be surgically treated soon after birth.

Between birth and surgery, a child with pulmonary atresia is given a drug called Prostaglandin to prevent the ductus arteriosus from closing. The ductus arteriosus is a vein between the aorta and the pulmonary artery that is present during fetal development but naturally disappears soon after birth. This vein provides a temporary solution for getting deoxygenated blood back to the lungs. For a permanent solution, the child must undergo a series of three surgeries to completely bypass the right side of the heart.

The first surgery, which is considered a closed-heart surgery, is performed soon after birth (Daphne’s surgery is scheduled for Thursday, February 3). During this procedure, the surgeon makes an incision in the child’s side and inserts a BT shunt between the aorta and the pulmonary artery. The BT shunt is a Gore-tex tube that basically performs the same function as the ductus arteriosus, allowing deoxygenated blood access to the lungs. After this surgery, the child no longer needs the ductus arteriosus, and it is allowed to close.

The second and third operations are part of a two-part open-heart surgery. The second surgery, called a Bidirectional Glenn, is performed between 3 months and 1 year, depending on the child’s development. During this procedure, the superior vena cava, which delivers deoxygenated blood from the upper part of the body to the right atrium, is detached from the right atrium and hooked to the right pulmonary artery, allowing this blood to reach the lungs more easily.

The third surgery, called a Fontan Completion, is usually performed between the ages of 3 and 6. During this procedure, the inferior vena cava, which delivers deoxygenated blood from the lower part of the body to the right atrium, is detached from the right atrium and a piece of tubing is used to link it to the right pulmonary artery. After this surgery, blood flow to the right side of the heart is completely cut off, and the child lives life with just half of a heart—which is really how she’s been living all along.
 
The prognosis for this series of surgeries is good. The first children to undergo these procedures are in their twenties and thirties and appear to be living full and happy lives. Although heart surgery is always risky, I feel confident that these surgeries will be able to bless Daphne and help her live long enough to fulfill her mission here on earth. These procedures really are a miracle, because without them she wouldn’t have that opportunity.