Heart Issues in Children with Down Syndrome
Introduction to the Heart Section
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This section below describes the main problems seen in babies and children with Down Syndrome.
You can read the entire article by clicking on the title.
This section is reproduced with the kind permission of the Down's Heart Group.
Normal Heart
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This article describes the function, and structure of a typical Human Heart
Atrial Septal Defect
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This article describes artrial septal defect where there is a hole between the two top chambers in the heart. The article describes the defect, its symptoms and treatment
Atrio Ventricular Septal Defect (AVSD)
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Atrioventricular Septal Defect (AVSD or AV CANAL) is the most common defect found in children with Down Syndrome.
Atrioventricular Septal Defect with Fallots Tetralogy
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A small percentage of babies have a complex heart condition which combines the most common defect associated with Down Syndrome, the Atrioventricular Septal Defect (AVSD) with another defect called Fallot's Tetralogy.
Patent Foramen Ovale (PFO)
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This defect is present in every newborn baby. It is a gap in the wall between the upper two chambers of the heart, which should close soon after birth.
Persistent Ductus Arteriosus (PDA)
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This defect is not a fault in the internal structure of the heart as in most 'holes in the heart'; it is the continuance of a direct connection between the aorta and the lung (pulmonary) artery, which normally closes shortly after birth.
Ventricular Septal Defect (VSD)
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In this defect there is a hole between the bottom chambers (pumping chambers or ventricles). Due to the higher pressure in the left side of the heart, this allows oxygenated blood to flow through the hole from the left to the right side of the heart, and back to the lungs, in addition to the normal flow.
Obstruction Defects (Atresia / Coarcation / Stenosis)
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Sometimes one of the hearts valves is narrowed, blocked or missing, or the main artery to the body (aorta) is narrow. These conditions may be found in association with other defects and the problems caused will depend on where and how severe the blockage is and what the other defects are.
- Atresia - blocked, missing or never formed
- Coarctation - narrowing of the aorta
- Stenosis - narrowing of a valve or blood vessel
Blalock-Taussig/Glenn/Fontan Procedures
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There are several different procedures that are used in the treatment of cardiac defects. Below are basic details for the most common ones which people with Down Syndrome have done, but they are often modified to deal with the individual aspects of a patient's condition.
Cardiac Catherisation
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A Cardiac Catheterisation is sometimes carried out to confirm the exact nature of a heart defect if the results from an echocardiogram provide insufficient detail to decide on treatment. The procedure can also be used to treat some conditions, for instance to close small holes, stretch a narrow blood vessel or even replace a valve, thus avoiding the need for open heart surgery.
Drugs used to Treat Patients with Congential Heart Conditions
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Not all patients with cardiac problems will require the use of medication. If the patient does need it, the drugs prescribed will depend on the nature of the heart condition, and the degree of symptoms they are showing. All of the drugs have a chemical or generic name which identifies the ‘ingredients' of the medication, but the pharmaceutical companies which make the drugs also give them a company or proprietary name, so there may be several names for the same drug.
Eisenmenger Complex
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Eisenmenger Complex is the term used to describe the resultant effects of prolonged pulmonary hypertension in patients with uncorrected congenital heart defects, which eventually results in a reversal of the shunting of blood within the heart (see Pulmonary Hypertension Topic Note), so that it goes from right to left.
Heart/Lung Transplants
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With advances in technology and understanding, where necessary, surgical correction is possible for the majority of babies born today with cardiac defects and Down Syndrome. There are of course a few cases where the nature of the defect adds particular complications and surgery may be inadvisable. For these babies and for those teenagers and young adults who for whatever reason did not have corrective surgery, a heart / lung transplant is the only remaining option.
intensive Care (I.T.U or P.I.C.U)
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All patients will be taken to the Intensive Care unit after heart surgery; for babies and children it will be a Paediatric unit whilst older patients will be on an adult unit – in a Children's Hospital it may well be a specific Cardiac Intensive Care Unit. Some will stay there only a few hours, others will be there longer.
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