Severin Grant Brenny, our son, a brother, and a fraternal twin, was born Sept. 23, 2002 with life threatening heart defect Hypoplastic Left Heart Syndrome (HLHS). Due to complications resulting from a post-op infection, Severin went to Heaven on July 17, 2004, just shy of his second birthday. We bring you this information to honor Severin’s memory and to recognize the need for research and funding for all children born with Congenital Heart Defects.
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Hypoplastic Left Heart Syndrome
HLHS is a serious heart defect that involves several parts of the left side of the heart. In this condition, the heart's lower left pumping chamber and the large artery that carries blood to the body are too small to support normal blood flow. In basic terms it means that the whole left side of the heart does not develop normally.
Diagnosis methods for HLHS
The sooner that HLHS is diagnosed, the more likely that treatment will be successful. An HLHS diagnosis may be made either before or after birth. If the diagnosis is made while in-utero, a fetal echocardiogram may be used. Commonly referred to as an ultrasound, an echocardiogram allows the physician to see images of the developing fetus and its various organs and systems, including the heart and cardiovascular system. If diagnosis is made before birth, parents will likely be offered the option of a medical termination of pregnancy (MTP), also referred to as a “therapeutic abortion.” This is a difficult and personal decision. Death rates have declined dramatically due to improvements in early diagnosis, the development of new treatments, and increased research. Most children born with Congenital Heart Defects are now expected to survive into adulthood and beyond.
The infant with the diagnosis of Hypolastic Left Heart Syndrome will have no blood flow to the body once the ductus arteriosus closes. No one can really say when the ductus arteriosus will close. On some infants it closes quickly, on others it may take several weeks to close. Regardless, children born with this lesion who do not receive treatment will not survive. Some options for HLHS: cardiac transplantation, in which the entire heart is replaced by a donated heart and staged palliation which is explained below. Your doctor should advise you about the risks and benefits to each of all options.
What is the long-term outlook after treatment for hypoplastic left heart syndrome?
Children with hypoplastic left heart syndrome will need follow-up care throughout their life to make sure their heart continues to work correctly. Most will require heart medicines. Consult your child's physician regarding the specific outlook for your child.
How is the problem treated
Treatment of HLHS means either a three-staged surgical repair or heart transplantation. This condition is most often fatal without early intervention. It will typically require open-heart surgery to re-direct the oxygen-rich ("red") blood and oxygen-poor ("blue") blood in a series of three reconstructive operations known as "Staged Reconstruction."
Stage I, known as the Norwood procedure, occurs within a few days of birth.
Stage II, known as the bidirectional Glenn or the hemiFontan, typically occurs within four to six months of birth.
Stage III, known as the Fontan procedure, typically occurs between one-and-a-half to three years of age.
Three-stage surgical repair for HLHS
The surgical repair for HLHS is a series of three heart operations done during the first two years of life. The goal of these operations is to rebuild the heart so that the right side can be used to pump blood out to the body. The first operation is done during the first week of life, the second one is done when the child is four to six months old, and the last one is done when the child is about two years old.
In addition to the three operations, two or three heart catheterizations must be done. A heart catheterization is a heart test that is done by a cardiologist with the help of cardiovascular technicians. Soft, thin plastic catheters (tubes) are placed in the large blood vessels in the leg and threaded carefully to the heart. The catheters are used to take pressure measurements inside the heart and to inject contrast or dye so pictures of the heart can be taken. Overall, this is a very safe test and children can go home the same day.
Norwood procedure for HLHS
The Norwood procedure is a series of three open-heart surgeries. Each open-heart surgery is done at a different age, ranging from infancy through the toddler years. The first two surgeries (Stages I and II) are used to temporarily relieve blood flow problems to and from the lungs. The third surgery (Stage III) is used to permanently correct the effects of the underlying congenital heart defects. The three surgeries are done at three different ages, which are as follows:
Stage I (the Norwood operation) or Norwood Shano is usually done soon after birth.
Stage II (the bi-directional Glenn procedure, or Glenn Shunt -- partial Fontan or a staging procedure before the Fontan) is usually done at three to nine months of age.
Stage III (the Fontan procedure) is usually done between 18 months and 4 years of age.
The Norwood operation is done very soon after birth, and converts the right ventricle into the main, or systemic ventricle. The bidirectional Glenn operation is done next, and diverts half of the blood returning from the body to the lungs. It is typically done at about six months of age. The Fontan operation is the last stage of the staged palliation, and diverts all of the blood returning from the body to the lungs. It is typically done around 2 years of age. The timing of these operations is variable, and depends on many clinical and social factors.
Staged palliation for HLHS is one of the great achievements of congenital heart surgery. In what used to be a uniformly fatal disease. Most patients who get through the three stages do quite well: Very few are on medications, almost all have normal growth and development, and very few have any exercise or other kinds of limitations.
Cardiac Care and Surgeries Explained