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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.
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 has a 100% mortality rate if left untreated. These infants 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.
For HLHS, staged palliation involves three operations or the Norwood Procedure. In order, these are the Norwood operation, bidirectional Glenn operation, and the Fontan operation.
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) 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.
Watch some animated videos on HLHS Proceedures:
  
Cardiac Care and Surgeries Explained
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