The Schematics of a Broken Heart

The human body is kept alive by a pump, divided into two halves. It is no larger than an apricot at birth and stops growing when it is a bit bigger than a fist. The heart is one of the most important organs as it regulates the circulatory system, beating 100,000 times, pumping approximately 2,000 gallons of blood through arteries, veins, and capillaries a day. The right side of the heart receives and provides blood for the lungs, while the left side delivers blood to the body and organs.

It is a complex organ necessary for survival but there can be complications such as congenital heart defects, and other problems that can make the performance of this organ to be less than optimal. They range in severity such as a mild heart murmur to severe such as having an abnormally shaped heart. One such condition is called hypoplastic left heart syndrome (HLHS). This is a single ventricle abnormality where the heart cannot pump enough blood to the body.

It is a rare heart defect, occurring in one in five thousand infants. Hypoplastic left heart syndrome is does not have one concrete sign but is defined by a series of defects; the left ventricle, atrium, mitral valve, and aorta being underdeveloped or non-existent. Usually, this CHD can be detected in the uterus but there can be other factors that might prevent an ultrasound from showing the seriously underdeveloped left side of the heart.

If not known about before birth, HLHS usually presents itself in the first forty-eight hours after birth; it can take up to ten days for it the symptoms to show. It depends on the patent ductus arteriosus (PDA) and how quickly it closes. The PDA allows blood to bypass the aorta, and flow to the body. Symptoms can include, central cyanosis (bluish tinge to lips, tongue, mucous membranes, fingertips, toes, chest), respiratory distress due to pulmonary hypertension, lethargy, sweating, and hypothermia.

Since there are several CHDs that contribute to HLHS, there is not a magical solution to cure the condition and treatments are merely to give the infant more time. The first step to treatment is administering prostaglandin E1 to an infant to keep the PDA open. This drug will allow the infant body to revert almost to a fetal status, which means that the infant could stop breathing. Parents of infants are given three options after the child is stabilized in which to help the child; surgery, which would take place over four years, heart transplant, or compassionate care.

The statistics for HLHS today are rather optimistic compared to almost twenty-five years ago where there was no solution to the problem. It occurs more often in males than in females but only accounts for 7 to 9 percent of CHDs and a mere 25 percent of cardiac related deaths in infants. This statistic is on the decline as quicker intervention can be taken to ensure that the problem is taken care of as soon as possible.

The surgery option for babies with HLHS is not a cure, only a temporary fix. The children who successfully undergo the three-stage procedure eventually need a heart transplant at some point in time.

Within hours of being born, infants with HLHS usually undergo the first surgery, called a Norwood procedure. This surgery is the first step to heart reconstruction by using man made shunt to reroute oxygen rich blood and to keep the heart functioning. This shunt, called a modified Blalock-Taussig, is placed between the pulmonary artery and the reconstructed aorta.

If this surgery is done within the forty-eight hours of life there is a 75 percent chance of survival. After fourteen days, the mortality rate drops to 30 percent. After discharge, statistics show that 85 percent patients that are low-risk go on to the second surgery while 45 percent of high-risk patients only make it to the next surgery. Infants who undergo this surgery will still appear cyanotic afterwards.

The next stage in this process is called a Hemi-Fontan or Bidirectional Glenn procedure is performed when an infant is usually between four to nine months old. When this surgery is done greatly involves infant growth and health. It involves removing the modified Blalock-Taussig and connecting the superior vena cava to the pulmonary artery. Statistics show that there is a 90 percent survival rate after discharge and children are less cyanotic.

The final surgery is called a Fontan procedure, which usually happens between eighteen months and four years of age. It involves connecting the inferior vena cava to the pulmonary artery, which allows deoxygenated blood to bypass the heart completely and oxygen-rich blood to circulate throughout the body through the right side of the heart. Seventy percent of children survive this surgery and appear to be pink or have a healthy tint to their skin.

Parents who opt for the surgeries can expect to have their children monitored closely for their entire life. Surgery is not considered a success until five years of age and the children can be expected to lead a fairly normal life for some time. Usually Norwood procedure patients are only given between five and fifteen years of life before a heart transplant is needed.

A heart transplant for an infant with HLHS is a life of doctor visits, medication, heartbreak and hope. The process involves a long period of waiting while a heart becomes available, which is understandable why 20 percent of infants die before a heart can become available. The abnormal heart is removed then the normal heart is placed into the chest cavity. Children and parents have to deal with organ rejection and other problems involving a donor heart. This option only gives a child between five and ten years of life and has an 80 percent survival rate.

The third option is called compassionate care or nothing is medically done to help the child. Parents have various reasons for not wanting to intervene. Infants are usually given various doses of medicine and then weaned off the medication. Parents are allowed to take the infant home and die. This option can allow the infant to live hours, or even up to weeks, but it is 100 percent fatal.

HLHS is a complicated, incurable, and a fatal congenital heart defect, if left untreated. The three treatments are an option only to preserve an infant's life and do not necessarily reflect the quality of life that they might have. Usually children with this condition can lead a normal life if there are no problems after treatment.