Hypoplastic Left Heart Syndrome (HLHS)
Haven Leigh was diagnosed in-utero with Hypoplastic Left Heart Syndrome (HLHS). We have tried to provide a simple explanation of the condition, the surgeries, and other aspects of HLHS. If you would like to learn more about HLHS and other congenital heart defects, please visit the links on the sidebar.
HLHS DESCRIPTION
In the normal human heart, the left ventricle pumps fresh, oxygenated blood to the body. Used blood then comes back to the heart and is pumped into the lungs from the right ventricle in order to get re-oxygenated. The blood then comes back to the heart from the lungs and the process begins again.
In hypoplastic left heart syndrome, the left side of the heart is underdeveloped, so the left ventricle (the heart’s main pumping chamber) cannot pump oxygenated blood to the body. While in the womb, all babies have a hole between the heart’s pumping chambers (this is normal). Babies with HLHS can survive just fine inside their mother’s womb because oxygenated blood can travel through the hole and get to where it needs to go. However, a few days after birth the hole will close naturally, therefore, babies with HLHS will die within a few days of life.
HLHS is perhaps the most severe congenital heart defect a baby can be born with, namely, because without intervention it is fatal. The condition is non-correctable which means that the problem can’t be fixed. The baby’s only hope of living is have a new heart or have her half of heart redesigned to do the job of an entire heart. Many babies are born each year without anyone knowing they have HLHS until it is too late. We are thankful that it can be discovered while the baby is still in-utero and treatment can be planned for ahead of time.
DIAGNOSIS
During a routine ultrasound at 20 weeks, the technician was unable to locate all four chambers of Haven’s heart. As a result, our small town hospital recommended we travel to a larger hospital to have an echocardiogram. Echocardiograms are ultrasounds that focus specifically on the heart. After the pediatric cardiologist (heart doctor for children) looked at the results of the echocardiogram, he diagnosed Haven with having HLHS. He then recommended that we have Haven treated at Riley Children’s Hospital in Indianapolis where some of the best pediatric cardiologists and surgeons in the country could help our little girl.
TREATMENT
We were given three options for treating Haven’s condition:
- Compassionate Care: Choosing this option means that no medical intervention would be given after the baby is born. Of course, this means that the baby would die within a few days. This was not a consideration for us.
- Heart Transplant: Babies with HLHS can be put on a list to receive a heart transplant. When the baby’s name is at the top of the list, she will receive a healthy donor heart. Unfortunately, there are not enough donor hearts for all babies waiting for a transplant, and thus, some do not survive while waiting for a heart.
- 3-Stage Surgical Procedure: An alternative to a heart transplant is the 3-Stage Surgical Procedure. This procedure is a series of open-heart surgeries which will reconstruct the child’s heart allowing it to work using only two of the heart’s four chambers.
After hearing our three options and knowing the quality of care Haven would receive at Riley, we chose the 3-Stage Surgical Procedure.
SURGERIES
The typical 3-Stage Surgical Procedure to treat HLHS consists of three (sometimes more) open-heart surgeries:
- The first open-heart surgery is called the Norwood procedure. Because the left-ventricle (the body’s main pumping chamber) cannot pump blood adequately to the body, this surgery allows the right ventricle to pump blood to both the lungs and the body. This surgery is performed during the baby’s first week of life.
- The second open-heart surgery, called the Hemi-Fontan or Bidirectional Glenn, reduces the work load of the right ventricle by allowing it to pump blood to the body only (instead of both the lungs and the body). This surgery is performed when the pressure in the baby’s lungs has decreased, no longer requiring the pumping action of the right ventricle to get blood into the lungs and is performed at about 4-6 months of age.
- The third (and hopefully final) surgery, called the Fontan, allows the rest of the blood coming back from the body to go to the lungs automatically without the use of a pump. This surgery is performed anywhere between 18 and 36 months of age.
RECOVERY
As with any open-heart surgery, there are many factors involved which can determine the outcome and recovery time. Assuming there are no complications and all goes as planned, Haven will be able to leave the hospital about four weeks after her birth. Since her body will go through so much while it is so fragile, Haven will be very susceptible to illness and therefore will not be able to be around lots of people for some time after she comes home. Recovery times from the second and third surgeries are typically shorter assuming, of course, there are no complications.
LIFE WITH HLHS
Since HLHS is not correctable, Haven will always have a special heart that is different from the normal heart. Since she only has half of her heart working for her when most people have their entire heart, she will need to avoid activities that create lots of strain on her cardiovascular system. She will also take medications and make many visits to the cardiologist for the remainder of her life. Despite any restrictions she may experience, we are certain that our little Haven Leigh will be a great inspiration to all who know her.
Not by works of righteousness which we have done, but according to his mercy he saved us, by the washing of regeneration, and renewing of the Holy Ghost; which he shed on us abundantly through Jesus Christ our Saviour; that being justified by his grace, we should be made heirs according to the hope of eternal life. Titus 3:5-7