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Heart Issues in Children with Down Syndrome (21)
The Heart and Children with Down Syndrome
Congenital heart disease (the presence of a structural heart defect at birth) occurs in 40 - 50% of children with Down syndrome and cardiac abnormalities are probably the most common malformations seen in trisomy 21.
Because congenital heart disease is so common, it is recommended that all children with Down syndrome have a full Pediatric Cardiology evaluation by 2 months of age.
Also, it is well recognized that children with Down syndrome are at risk for developing damage in their lung arteries at an early age if they have a heart defect.
There appears to be a number of reasons for this increased risk but it is critical to recognize the defect and do the repair early since the severe damage can be irreversible and progressive even after surgery. The timing of surgery is dependent on how the child is doing, however, as a rule it is done by 6 months of age and virtually always by one year of age.
The articles below detail the main heart defects. They are the copyright of the Downs Syndrome Heart Group and are reproduced with their permission.
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.
Babies with Down's Syndrome have a tendency to early feeding problems possibly as a result of poor muscle tone, and babies with a heart problem also tend to have problems as they tire easily when feeding. So babies who have a heart defect and Down's Syndrome have two things making it harder for them and poor feeding and slow weight gain are common causes of anxiety for parents.
Why is dental care important?
Everyone has bacteria in their mouth which can enter the bloodstream, but in someone with a heart defect they can cause an infection inside the heart (Endocarditis) which in some cases can prove fatal. Anyone who has a congenital heart defect (a hole, abnormal valve or blood vessel) is at risk of getting Endocarditis if they have tooth decay or gum inflammation, so good dental care should be a priority. Even those who have had corrective surgery may be at risk so it is important to listen to any advice given by the cardiologist, particularly in relation to the use of antibiotic cover for dental treatments.
Pulmonary hypertension is one of the results that may occur because of extra blood being pumped through the lungs and at an excessive pressure. This may occur when the normal separation between the right sided (lung) and left sided (body) circulation is incomplete, as when there is a hole or holes in the central wall of the heart (VSD), or when there is a connection between the aorta and the pulmonary artery (PDA). Pulmonary hypertension may also occur in many other conditions, for example if there is obstruction to the blood flow coming back to the heart from the lungs, or if there is upper airway obstruction.
Some patients with unoperated cardiac conditions or those with ongoing problems may be prescribed oxygen to help improve the oxygen level (saturation) in the blood and reduce the workload on the heart. Many of them use oxygen mainly overnight when it does not prohibit activity and this gives their body 'a boost' for the following day.
There are various tests that can be used by the cardiac team to diagnose or monitor heart problems. Some such as cardiac catheterisation are invasive (meaning 'entering' the body) and are generally done under general anaesthetic only where it is necessary to acquire more information than has been possible from other methods.
A list of Heart related medical termseExplained !
Any medicine given to stop pain.
An X-ray of the heart assisted by a liquid introduced through a catheter.
A drug whose purpose is to reduce blood clotting (see Drugs Topic Note).
Main artery of the heart which carries blood to the body.
The valve between the left ventricle (pumping chamber) and the aorta.
Disturbance of normal rhythm of the heart.
Blood vessel which carries blood from the heart to all parts of the body and lungs.
ASD (Atrial Septal Defect)
A hole in the septum (central heart wall) between the two atria (collecting chambers) allowing blood to flow from the left to the right side of the heart (see ASD Topic Note).
Connection that is either blocked or missing altogether.
The collecting chambers of the heart - plural of atrium.
An upper collecting chamber in the heart which receives incoming blood.
AVSD (Atrio Ventricular Septal Defect)
A hole in the septum (central heart wall) between the atria and between the ventricles with often a common valve between all four chambers. The most common heart defect in babies with Down's Syndrome (see AVSD Topic Note).
Narrowing of the pulmonary artery with a band to reduce blood flow to the lungs.
A small piece of body tissue taken for examination.
A baby who has too little oxygenated blood circulating and therefore has a blue tinge to certain parts of the skin (see cyanosis).
Abnormally slow heart rate.
Relating to the heart.
Cardiac Liaison Sister
A senior nurse in cardiac units to assist families with queries or problems (see Heart Team Topic Note).
A surgeon who specialises in the heart (see Heart Team Topic Note).
A physician specialising in the heart (see Heart Team Topic Note).
A machine which takes over the heart and lung functions during surgery.
A narrow tube inserted into a vein or artery and fed to the heart, where it is used to carry out repair or to refine diagnosis (see Catheterisation Topic Note).
These are tubes left in after heart surgery to drain away excess fluid.
A narrowing in a blood vessel.
Existing at birth.
An excess of fluid in part of the body i.e. the lungs.
CPAP (Continuous Positive Airway Pressure)
This is a way of keeping small airways open, often used before a patient is taken off complete ventilation.
Blueness of the skin caused by insufficient oxygen in the blood.
A drug which increases the contraction of the heart muscle (see Drugs Topic Note).
Drugs which assist the kidneys to produce and excrete more urine
(see Drugs Topic Note).
An echocardiogram test using sound waves to measure the speed and direction of blood flow in the heart.
A tube to remove fluid from the body.
A tube in the body which conducts blood.
A technique using high frequency sound waves on a screen to produce a picture of the heart and surrounding blood vessels.
A bypass machine similar to the heart lung machine which is sometimes used to support the heart and give it a rest after surgery.
A technique which records the electric currents produced by the heart.
An infection of the heart lining or the heart valves.
See Tetralogy of Fallot.
Inability of the heart to maintain adequate blood circulation. It does not mean that the heart has ceased to function, but that it is operating inefficiently.
Heart Lung Machine
A machine through which the bloodstream is diverted which is used to oxygenate blood and pump it round the body during open heart operations.
HDU (High Dependency Unit)
Sometimes after leaving the Intensive Care Unit, a higher level of care is needed than that on the normal ward, so patients are transferred to the High Dependency Unit.
The sound made by blood flowing through the heart.
ICU / ITU (Intensive Care Unit)
This is a blood test used to check how fast the blood is clotting and adjust the level of anticoagulants prescribed.
Valve between the left atrium and left ventricle.
The noise produced by blood flow within the heart which can indicate a heart defect.
Naso gastric or NG tube
A tube used for feeding directly into the stomach. It goes into the nose and then down the throat.
A build up of excess fluid accumulating in body tissue.
Open Heart Surgery
Operations performed on the inside of the opened heart.
A device which controls the rhythm of the heart if it becomes unsettled.
Wires which are used to connect a pacemaker to the heart.
The branch of medicine dealing with the diseases of children.
PDA (Persistent Ductus Arteriosus)
A congenital condition where the duct between the aorta and the pulmonary artery sides of the heart fails to close after birth, as it should do, and allows blood to flow between the two sides of the heart (see PDA Topic Note).
The sac or bag surrounding the heart.
PICU (Paediatric Intensive Care Unit)
Provides a high level of specialist care especially immediately after surgery (see Intensive Care Topic Note).
A condition where there are an increased number of red cells within the blood.
The estimated outcome of the particular problem a patient has.
A preventative medicine given to protect against the possibility of infection.
Relating to the lungs.
The large artery which conveys deoxygenated blood from the heart to the lungs.
Elevated pressure of blood flowing into the lungs, often caused by the left to right shunting through a septal defect.
The valve between the right ventricle and the pulmonary artery.
Common abbreviation for saturation levels referring to the percentage of oxygen found in the blood (see Non Invasive Tests Topic Note).
The process of making a hole in the septum (heart wall).
The tissue wall in the heart which divides the chambers on the left side from the chambers on the right side and thus separates the oxygenated from the deoxygenated blood, with their different blood pressures (see Pulmonary Hypertension Topic Note).
A natural or surgically inserted connection between the two sides of the heart.
A narrowing of a vessel.
An abnormally rapid heart rate.
Tetralogy of Fallot
A congenital malformation of the heart involving four defects (see Fallot's Tetralogy Topic Note).
An echocardiogram performed by passing the transducer down the throat into the oesophagus (see Non Invasive Tests Topic Note).
Valve, consisting of three cusps, or triangular segments, between the right atrium and the right ventricle.
A structure which opens and closes. When open it allows blood flow in one direction and when closed prevents back flow or leakage.
Pertaining to blood vessels.
Drugs which increase the size of the blood vessels and thus reduce blood pressure (see Drugs Topic Note).
A vessel carrying blood back to the heart.
The lower chambers of the heart which act as the pump for the blood's circulation.
VSD (Ventricular Septal Defect)
A hole in the septum (central heart wall) between the two ventricles (pumping chambers) which allows blood to flow between the two sides of the heart (see VSD Topic Note).
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.
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.
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.
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.
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.
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.
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