If HLHS is not treated within thefirst days of a child’s life, it can be fatal. With surgical intervention achild has about a 70-90% survival rate. Some will have learning disabilities andsome physical limitations. If the child had a heart transplant, they will needanti-rejection medication for the rest of their lives and may have to takecertain antibiotics to keep infections at bay.
Although, the child survived havinga heart transplant or the norwood procedure done, there life long risks that comewith HLHS. Children who are born with HLHS have increased risk of pulmonaryembolisms, blood clotting disorders, and an increased risk of strokes. “Withmore people surviving the staged surgeries, more information is now beinggathered about the long-term experiences of people born with HLHS” (Genetic andRare Diseases Information Center, 2017). Close monitoring of the child for therest of his/her life is mandatory.
Females who were born with this heart defect,if wanting to become a mother, she has a very good chance at being able tocarry 1 or more children. With all genetic diseases the mother/ father wouldneed to take into consideration that they could pass this gene onto their futurechildren. If a mother has a child already HLHS her subsequent child also have ahigh risk of being born with HLHS. The patient’s prognosis (expectedoutcome)While HLHS is rare, there aremedical procedures to help. There is currently a handful of options availablefor the treatment for HLHS. The Norwood procedure is a 3-step surgical process,in the first step, which done shortly after birth, the cardiac surgeon will makethe right ventricle into the main ventricle while pumping blood to lungs and tothe body, while cutting off the pulmonary arteries and placing a shunt betweenthe main pulmonary and the aorta valve.
The second step or the bi- directionalglenn operation is done about six months after the first step, the surgeon willtake out the shunt and connect the pulmonary artery right to the superior venacava. This procedure is intended to helpwith sending deoxygenated blood to the lungs while avoiding the ventricle. The thirdstep or the fontan operation, is done around 18-36 months after the second step.This procedure is intended to connect the inferior vena cava to the pulmonary artery,and to create a channel to the outside of the heart and to direct blood intothe pulmonary artery. After this procedure is finished deoxygenated bloodshould be flowing through the lungs freely. Other procedures that can be doneis a hybrid procedure, when babies are too small to be able to do undergo the Norwoodprocedure they can do the hybrid procedure which is where the surgeons will dosurgery and catheterization. In the procedure the surgeon will go in and makethe foramen oval wider and place a stent in the ductus arteriosus to keep iffrom collapsing, next the surgeon will place band around pulmonary arteries tocontrol the blood flow to the lungs.
With procedure it will allow smallerbabies to be able to grow big enough to get the Norwood procedure done. Thenext available option, if there is significant damage to the heart and thesurgeons cannot repair it, is a heart transplant. “One of the most commonindications for infant heart transplantation had been hypoplastic left heartsyndrome, which occurs in about one in 6000 live births” (Emedicine, 2018). Treatments available for thedisease:When a child that is born withHLHS, they can have no symptoms to having every symptom.
Some of HLHS symptomsinclude rapid and shallow breathing, bluish skin color, cold to the touch, fastheart rate, poor breastfeeding, and a weak pulse. In some cases, a child wasborn with HLHS and showed no symptoms, in others they had bluish colorationaround the heart and tongue. During the physical examination of the child adoctor or the nurse that is assisting in the care of the care will notice thesymptoms, upon these findings they may also find that the child’s liver isswollen, and the child is lethargic. “the pulse at various locations, wrist,groin, and others, may be very weak” (MedLinePlus, 2017). In some cases, theheart has an abnormal beat, or sounds abnormal. Babies that are born with HLHScan also go into shock, due to the poor circulation in the heart. Some signs ofthe child going into shock are dilated pupils, rapid but weak pulse, andabnormal breathing. Signs and symptoms of the disease: HLHS has certain set ofcharacteristics, the aortic and the mitral valve can be too small, narrow ornot formed at all, and narrowing of the aorta artery.
In most cases the septumis in intact. HLHS comes in different severity, ranging from small orunderdeveloped valves to valves that were not formed at all. With HLHS, it isaccompanied with other heart defects such as endocardial fibroelastosis or themuscular layer of the heart chambers thickens, the arteries on the right sideof the heart with larger than normal due to over working, in many cases thereare also too small of blood vessels and arteries as well. The heart,ventricles, and the chambers are in their right spots. Pathophysiology of the disease:The causes of HLHS are limited. Itcould be genetics, or at the time of the child’s DNA being put together therewas a malfunction causing HLHS. Doctors have a limited knowledge on this type ofdefect.
What doctors do know, is that it is present when the heart starts formingand beating in the early weeks of pregnancy, and chromosomal make-up could bethe culprit. “HLHS is the result of under-development of sections of the fetalheart during the first eight weeks of pregnancy” (Boston Childrens Hospital,2018). HLHS is also more common in baby boys than baby girls. HLHS and manyother congenital heart defects the direct cause it largely unknown. Causes of the disease:In every baby, in the womb and atthe time of birth, they are born with extra openings on the heart called thepatent ductus arteriosus and the patent foramen ovale. These help the babiesheart with getting the most of oxygenated blood to the brain.
The patent ductusarteriosus sits between the aorta and the pulmonary arties, redirecting theblood flow, because babies in the womb do need to supply their lungs withoxygenated blood yet. In a normal baby, the paten ductus arteriosus will closeshortly after birth. “However, when the baby is born, the blood must receiveoxygen in the lungs and this hole is supposed to close” (American HeartAssociation, 2018). The Paten foramen oval is very similar to the paten ductusarteriosus, except it is located in the septum of the heart.
This opening inthe septum will allow the blood that the babies get from the placenta to enterthe heart and be able to travel to the right and the left side of the baby’sheart, supplying the oxygenated blood from the placenta to the heart as quickas possible. When these openings do not close after birth, the baby will beborn with a congenital heart defect. A rare defect is hypoplastic left heartsyndrome. This is when the patent ductus arteriosus and the patent foramenovale are too small or closed at birth the heart will overwork. In some cases, the baby’s ventricles arefused as one, or the septum is dotted with holes, causing improper circulationat birth.
HLHS can affect different organs in the child’s body such as thelungs, liver, brain, kidneys and the vascular system. The hearts main role inthe body is to supply the body as whole with oxygenated blood, and to be ableto pump nutrients to other vital organs. When children are born with HLHS,their vital organs are being depraved the nutrients and the oxygen that theyneed to work properly.General information about thedisease: Every year in the United Statesthere are roughly 960 babies born with a rare congenital heart defect.
Hypoplastic Left Heart Syndrome or HLHS affects the babies heart causing theleft ventricle to be closed or be too small, same with the major arteries aswell. When the patent ductus arteriosus and the patent foramen ovale to closecausing the babies heart to over work, and the other major organs suffer fromnot getting enough oxygenated blood.