Glossary

Introduction

Atrial septal defect (ASD)
In this condition, an opening exists between the two upper chambers of the heart that allows some blood from the left atrium (blood that has already been to the lungs) to return via the hole to the right atrium instead of flowing through the left ventricle, out the aorta, and to the body.

Ventricular septal defect (VSD)
In this condition, an opening exists between the two lower chambers of the heart and allows some blood that has returned from the lungs and has been pumped into the left ventricle to flow to the right ventricle through the hole (instead of being pumped into the aorta). Because the heart becomes over-worked, it may become enlarged.

PAVSD (Partial atrio-ventricular septal defect)
A defect is present in the lower part of the “Atrial Septum” (the partition separating the Atriums). This allows red blood to pass through into the right side of the heart, leading to enlargement of the right ventricle and excessive flow in the lung circulation.

CAVSD (Complete atrio-ventricular septal defect)
A large hole in the centre of the heart exists where the wall between the upper chambers joins the wall between the lower chambers. Also, the tricuspid and mitral valves that normally separate the heart's upper and lower chambers aren't formed as individual valves. Instead, a single large valve forms that crosses the defect.

Tetralogy of Fallot
This condition has four components:
1. a large hole, or ventricular septal defect, that allows blood to pass from the right ventricle to the left ventricle without going through the lungs
2. a narrowing (stenosis) at or just beneath the pulmonary valve that partially blocks the flow of blood from the right side of the heart to the lungs
3. the right ventricle is more muscular than normal
4. the aorta lies directly over the ventricular septal defect

Arterial Switch
The arterial switch procedure is an operation performed to correct the congenital heart defect called transposition of the great arteries (TGA or TGV). This heart problem occurs when the aorta (the blood vessel that supplies blood to the body) originates out of the right (instead of the left) ventricle, while the pulmonary artery (the blood vessel that transports blood to the lungs) originates out of the left ventricle. The surgery is usually done in the first two weeks of life.

Pulmonary Autograft or Ross Procedure
The patient's own pulmonary valve is used to replace the diseased aortic valve in this complex procedure.

Subaortic stenosis
Stenosis means constriction or narrowing. Subaortic means below the aorta . Subaortic stenosis refers to a narrowing of the left ventricle just below the aortic valve, which blood passes through to go into the aorta. This stenosis limits the flow of blood out of the left ventricle.

Fontan
The Fontan procedure is a heart operation used to treat complex congenital heart defects

Truncus arteriosus
This is a complex malformation where only one artery arises from the heart and forms the aorta and pulmonary artery. Surgery for this condition usually is required early in life. It includes closing a large ventricular septal defect within the heart, detaching the pulmonary arteries from the large common artery, and connecting the pulmonary arteries to the right ventricle with a tube graft. Children with truncus arteriosus need lifelong follow-up to see how well the heart is working.

Total anomalous pulmonary venous
The pulmonary veins that bring oxygen-rich (red) blood from the lungs back to the heart aren't connected to the left atrium. Instead, the pulmonary veins drain through abnormal connections to the right atrium.

Interrupted Aortic Arch
Part of the Aorta is absent and this leads to severe obstruction to blood flow to the lower part of the body. As the ductus closes after birth blood pressure in the lower circulation becomes inadequate and severe symptoms develop.

Cavo-Pulmonary Anastamosis
The bidirectional shunt is performed by connecting the superior vena cava (SVC) to the right branch of the pulmonary artery using fine sutures, and dividing or tying up the pulmonary artery. Now, venous blood from the head and upper limbs will pass directly to the lungs, bypassing the right ventricle. The venous blood from the lower half of the body however will continue to enter the heart.

Norwood stage 1
The Norwood operation, which converts the morphologic right ventricle into the systemic ventricle, was developed for palliation for hypoplastic left heart syndrome (HLHS). More recently, the Norwood operation has gained popularity as a means of palliating many lesions in which the morphologic left ventricle may be inadequate to maintain systemic circulation, as may occur in many forms of atrioventricular and ventriculoarterial malconnections.

Hypoplastic left heart syndrome
In hypoplastic left heart syndrome, the left side of the heart - including the aorta, aortic valve, left ventricle and mitral valve - is underdeveloped. Blood returning from the lungs must flow through an opening in the wall between the atria , called an atrial septal defect. The right ventricle pumps the blood into the pulmonary artery , and blood reaches the aorta through a patent ductus arteriosus .

Terms Parents have asked to be explained

Ablation: an electric current or radio- frequency energy can be used via a catheter to destroy the extra pathways in the heart which cause tachycardia.

Absent Pulmonary Valve Syndrome: the pulmonary valve is not formed properly, there is a hole between the ventricles and the pulmonary arteries are much wider than they should be.

Amplatzer device: used to close the hole between the right & left atrium via a catheter

Analgesic: medicine given to stop pain

Angiogram: an x-ray of the heart assisted by a liquid introduced through a catheter.

Anomalous Pulmonary Venous Drainage: the pulmonary veins carry red blood from the lungs to the right side of the heart instead of the left side.

Anticoagulant: a medicine such as Warfarin given to stop blood clots forming

Aorta: Main artery, which carries blood from the heart to the body.

Aortic Stenosis: a narrowing which restricts red blood from moving from the left ventricle into the aorta.

Aortic valve: The valve between the left ventricle and the aorta

Arrhythmia: out of rhythm - the heart is beating too fast, too slowly, or irregularly.

Arterial Switch: Reattaching the aorta and the pulmonary artery the right way around when a baby is born with transposition of the great arteries (TGA).

Atresia: blocked or missing

Atria: plural of atrium. The left atrium pumps oxygenated blood into the left ventricle. The right atrium pumps deoxygenated blood into the right ventricle from where it is pumped into the left ventricle.

Atrial Septal Defect (ASD) : - a hole in the wall between the atria.

Atrial Septostomy: making a hole between the two atrial chambers.

Atrioventricular Septal Defect (AVSD): a hole between the atria (atrial septal defect, or ASD), a hole between the ventricles (ventricular septal defect or VSD) and a single valve instead of a tricuspid valve and a mitral valve.

Atrium: an upper chamber of the heart where blood collects before passing to the ventricle.

Balloon dilation: using a tube (catheter) to reach the narrow part of the heart and making it bigger by inflating a balloon on the end of the catheter

Balloon septostomy: a tube (catheter) is put into the heart and a balloon inflated on the end of it to make a hole, or increase the size of a hole, in the wall (septum) of the heart.

Banding: Making the pulmonary artery narrower with a band to reduce blood flow to the lungs.

Bicuspid: (of a valve) having two cusps, or leaflets

Biopsy: removal of a small piece of tissue for examination

Blue blood: blood which is returning from the body to the heart and so pumped to the lungs, where it will pick up oxygen and become red blood.

Bradycardia: slow heart beat .

BT (Blalock Taussig) Shunt: taking blood from an arm artery to the lungs.

Bypass: using a machine to bypass the heart and lungs during surgery.

Candida: a fungal infection

Cardiac: relating to the heart

Cardiac catheter: a tube, which is put into the heart via a vein. It is used to help diagnosis, by measuring pressures very accurately, or can treat a problem such as widening an artery, or closing a hole

Cardiologist: doctor specialising in heart.

Cardiomyopathy: weakness of the heart muscle

Chest stented open: when there is strong reason to suppose that further surgery is needed the opening in the chest is not closed.

Chest drains: tubes often left in after heart surgery to drain away fluid.

Chylothorax: a build up of lymphatic fluids leaking into the lungs following surgery

CT scan: (Computed Tomography) a procedure that produces an image of tissue density in a complete cross- section of the part of the body being scanned.

Coarctation of the Aorta: narrowing in the aorta - the artery taking blood from the heart to the body

Congenital: describes a condition which is present at birth

CPAP ( Constant Positive Airway Pressure): keeping small airways open, especially before completely off ventilation.

Cyanosed: there is not enough oxygen in the blood, causing the skin to look blue in some children

Dextra cardia: the heart is on the right, rather than the left, side of the chest.

Digoxin: a medicine given to increase the strength, or slow down the rate, of the contraction of the heart.

Dilated cardiomyopathy: a condition in which the heart becomes enlarged and weak, sometimes because of a virus

Diuretic medicines: these medicines help the kidneys pass more water, so reducing excess fluid in the organs, especially the lungs.

Double Inlet Ventricle: DIRV - Double Inlet Right Ventricle. DILV-Double Inlet Left Ventricle. There is one large ventricle into which both atria empty their blood through either one or two valves, and usually a second smaller ventricle.

Double Outlet Ventricle: DORV-Double Outlet Right Ventricle. DOLV-Double Outlet Left Ventricle. One ventricle pumps blood into both the aorta and pulmonary artery, although there may be a second smaller ventricle.

Duocal: a food supplement to help children gain weight faster

ECG: short for electrocardiogram - for measuring the electrical activity of the heart

Echo: short for echocardiogram - an image of the heart created by using high frequency sound waves.

ECMO (Extra Corporeal Membrane Oxygenation): a by-pass machine that can be used to support the heart or lungs to aid their recovery.

EEG (electrocephalogram) : a print-out of the electrical activity in the brain

Endocarditis: an infection of the lining of the heart

Glenn Shunt: The superior vena cava, bringing blood back to the right side of the heart, is connected to the pulmonary artery, so taking blood directly to the lungs, and bypassing the right ventricle.

Heart murmur: a murmur is a sound made by blood moving round the heart : sometimes but not always this could be caused by a heart defect.

Heparin: a drug given directly into a vein which thins the blood when there is a danger of clotting (an anticoagulant)

HDU: High Dependency Unit - unit where a child receives a lot of care. Children are sometimes transferred from Intensive Care Units once they are off the ventilator, but they need more care than can be given on the ward

Homograft: putting in human tissue - such as a valve or artery.

Hypertrophic Cardiomyopathy: the heart muscle becomes so thick that it can interfere with its proper functioning

Hypoplastic Right Ventricle: the right ventricle has not developed properly so it is small.

INR (International Normalised Ratio): a blood test for patients mainly on Warfarin to detect how fast the blood clots, used to adjust the amount of anticoagulant prescribed.

IV (Intra-venous) antibiotics: antibiotics directly into the blood stream

IV (Intra-venous) drugs: drugs given directly into the blood stream

Kidney dialysis: used to take impurities from the blood when the kidneys are not working properly

MAPCAs: Major aorto-pulmonary collateral arteries. A number of additional arteries come from the aorta and supply the lungs with blood.

Mitral Valve Stenosis: the Mitral Valve in the heart opens to let oxygenated blood to pass into the left ventricle, and then closes as it is pumped into the aorta and so around the body. Stenosis means that it is narrow, and therefore not allowing enough blood through, and causing a backflow to the lungs.

NG tube: a naso-gastric tube - for feeding the child through the nose directly into the stomach.
This is so babies who are breathless get enough food.

Pacemaker: a small battery placed under the skin and joined to the heart by pacing wires, which measure the pulse and corrects too fast or too slow rhythms

Pacing box: when the pulse rate is very irregular or slow an external pacemaker can be used to regulate the heart by attaching it to temporary pacing wires often put in place after heart surgery in case they should be needed.

Pacing wire: there is often a problem with heart rhythm after heart surgery, so a pacing wire is left in place just in case it is needed.

Partial AVSD: A defect is present in the lower part of the “Atrial Septum” (the partition separating the Atriums). This allows red blood to pass through into the right side of the heart, leading to enlargement of the right ventricle and excessive flow in the lung circulation.

Pathway: An anticipated plan of care prior to and following an operation or cardiac catheterisation.

PDA: Patent or persistent ductus arteriosus - a passage used for circulation before the baby is born remains open, instead of closing shortly after birth. This causes red blood to return from the aorta back to the lungs.

P.E.G (Percutaneous Endoscopic Gastrostomy): A tube inserted into the stomach allowing a feeding tube to protrude from the abdomen.

Perfusionist: A specialist that operates the heart-lung by-pass machine.

Pericardial effusion: fluid collects in the pericardial sac - the outer covering of the heart -which can be drawn off using a needle, or drained using diuretics.

Portage: a service operated by some local authorities whereby advice and support is given to mothers to improve the progress of children with disabilities.

PICU: Paediatric Intensive Care Unit

Prophylactic: describes a medicine or procedure intended to prevent illness.

Pulmonary: to do with the lungs

Pulmonary artery: the blood vessel that takes blood from the heart to the lungs

Pulmonary atresia: blood cannot be pumped to the lungs from the right ventricle through the pulmonary artery, which is blocked or missing.

Pulmonary hypertension: high pressure of blood moving into the lungs

Pulmonary stenosis: a narrowing between the right ventricle and the lung artery

Ross Procedure: replacing the child’s aortic valve with his or her own pulmonary valve.

RSV (Respiratory Synctal Virus) : a virus that causes bronchiolitis

Red blood: blood which, has picked up oxygen from the lungs and travels through the left side of the heart to be pumped around the body.

Sats: short for saturation levels (of oxygen in the blood).

Septostomy: making a hole in the septum, the wall, between the left and right chambers of the heart.

Shunt: a natural or artificially created passageway between two parts of the heart

Situs inversus: a mirror image arrangement of the organs, so that the heart and stomach are on the right and the liver and spleen on the left.

Spell: (particularly with Tetralogy of Fallot) the child becomes bluer, breathless and limp for a period of time.

Stenosis: Narrowing

Stent: a short, metal mesh tube. Using balloon dilation this is expanded into a narrow artery to hold it open.

Sternum: the breastbone.

Supra-ventricular Tachycardia: SVT. A very fast heart beat

Tachycardia: fast heart beat

Tetralogy of Fallot: a Ventricular Septal Defect (hole between the two ventricles) and Pulmonary Stenosis - a narrowing between the right ventricle and the artery carrying blood to the lungs.

Taussig-Bing Anomaly: A Double Outlet Right Ventricle with VSD lying under the pulmonary artery, this may also have transpostion of the great artetries.

TCPC: Total Cavo-Pulmonary Connection - a surgical procedure that connects blood returning from the body directly to the pulmonary arteries, bypassing the right side of the heart.

T.O.E. (Trans-oesophageal Echocardiogram): A small scope passed down the throat to look at the back of the heart.

Trachea: windpipe

Tracheostomy: a hole cut into the windpipe to help breathing

Transposition of the Great Arteries: the big arteries, which should be taking blue blood from the heart to the lungs, and red blood from the heart to the body, are round the wrong way. This means that deoxygenated blood goes back to the body, and oxygenated blood from the lungs is directed back into the lungs.

Tricuspid atresia: the tricuspid valve is absent

Tricuspid valve: the valve between the right atrium and right ventricle, which has three cusps.

Truncus Arteriosis: there is one single artery arising from the heart, which then divides into the lung artery and the body artery. There is in addition, a large hole between the two pumping chambers.

Ventricles: pumping chambers of the heart - left ventricle pumps blood round the body and the right ventricle pumps blood to lungs

Ventricular Septal Defect (VSD): a hole between the two ventricles - the pumping chambers of the heart.

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