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Glossary


Please find below the glossary of terms used throughout the ‘Congenital’ section of the website.

Aneurysm
Localized dilatation of an artery, a vein or a component of the heart
Aorta
The vessel rising from the left ventricle and driving blood to all the body
Aortic arch
The curved portion of the aorta between the ascending and descending portions and giving rise to the vessels for the upper part of the body, including the head
Aortic arch anomalies
Anomalies regarding the aortic arch
Aortic arch hypoplasia
Incomplete development or underdevelopment of the aortic arch
Aortic arch interruption
Separation occurring between ascending and descending aorta
Aortic valve
The valve between the left ventricle and the aorta
Aortic valve atresia
Absence or closure of the normally opened aortic valve
Aortic valve disease
Any structural or functional anomaly of the aortic valve
Aortic valve regurgitation
A condition in which the aortic valve can't close properly, causing blood from the aorta to flow backward into the left ventricle
Aortic valve repair and replacement
While repair involves repairing or modifying the components of the native aortic valve, replacement involves the removal of the native valve, which is substituted with prostheses 
Aortic valve stenosis
A condition in which the aortic valve can’t open properly, causing a restriction to the blood flow afterward from the left ventricle to the aorta
Aortopulmonary connections
Anomalous connections between the aortic and pulmonary vessels, in presence of a congenital heart disease
Aortopulmonary window
Presence of a direct communication between the aorta and the pulmonary artery
Aortoventricular tunnel
Presence of an extracardiac channel connecting the ascending aorta to the cavity of the right or left ventricle
Arterial switch operation
Surgery to correct Transposition of Great Arteries, restoring the right connection between the aorta and the left ventricle and the pulmonary artery and the right ventricle, respectively
Atrial isomerism
Abnormal arrangement with symmetric development of either right or left appendages, which are normally asymmetric
Atrial septal defect
A hole between the right and the left atria, normally completely separated by a septum
Atrioventricular canal defects
Group of congenital heart diseases characterized by the presence of a hole in the centre of the heart, between the four chambers (right and left atrium, right and left ventricle), with a common valve between the two atria and the two ventricles
Bicuspid aortic valve
Anomaly of the aortic valve involving the presence of two, instead of three cusps of the valve
Bicuspid pulmonary valve
Anomaly of the pulmonary valve involving the presence of two, instead of three cusps of the valve
Bicuspid pulmonary valve
Anomaly of the pulmonary valve involving the presence of two, instead of three cusps of the valve
Cardiomyopathy
Disease of the heart muscle causing an impairment of its normal function
Cardiopulmonary bypass (CPB)
Technique used during cardiac surgery operation, in which a machine temporarily takes over the function of the heart and lungs
Catheter intervention
Any procedure in which a catheter is inserted into a chamber of the heart or a vessel, for both diagnostic and therapeutic purposes
Coarctation of the aorta
A narrowing of any of the segments of the aorta
Collateral vessels
Abnormal blood vessels that form over time in response to the presence of a narrowing
Common atrioventricular valve
Presence of one, either of two, valve through which blood flows from the atria to the ventricles
Congenital heart disease
Birth defect affecting the normal structure and the function of the heart
Congenitally corrected transposition of the great arteries
Rare heart defect in which the ventricles are reversed in their position, meaning that the right ventricle is abnormally connected to the left atrium and the aorta, which are normally connected to the left ventricle, and the left ventricle is abnormally connected to the right atrium and the pulmonary artery, which are normally connected to the right ventricle
Coronary artery aneurysm
Localized dilatation of a coronary artery
Coronary artery anomalies
Anomalies regarding the coronary arteries
Coronary sinus
Large vein located on the posterior surface of the heart and collecting the blood from the coronary veins
Cyanosis
Bluish or purplish discoloration of the skin or mucous membranes due to a low oxygen saturation in presence of a congenital heart disease
Dextrocardia
A rare congenital heart disease in which the apex of the heart is abnormally located on the right side of the body
Diverticulum of Kommerell
Developmental anomaly of the aortic arch characterized by the presence of a dilatation of the descending aorta at the origin of an abnormally located subclavian artery
Double aortic arch
A rare congenital heart disease in which two aortic arches form a complete vascular ring around the trachea and oesophagus, possibly causing symptoms of compression
Double-inlet single-ventricle
A rare congenital heart disease in which the left and the right atrium are both connected to the lower pumping chamber, or ventricle. The other ventricle is usually very small
Double-outlet right ventricle (DORV)
A congenital heart disease in which the aorta and the pulmonary artery both connect the right ventricle
Ductus arteriosus
A short vessel connecting the aorta and pulmonary artery during foetal life, normally closing at birth
Ebstein’s anomaly
A rare heart defect in which the tricuspid valve is not formed properly, being displaced toward the apex of the right ventricle and causing regurgitation of the blood backward to the atrium
Extracorporeal membrane oxygenation (ECMO)
Extracorporeal technique to provide prolonged respiratory and cardiac support when either the lungs and the heart are not able to provide an adequate function to sustain life
Fontan procedure
A palliative surgical procedure for children with univentricular hearts, in which the blood returning from the body to the right atrium is directly diverted into the pulmonary arteries to overcome a failing ventricle
Foramen ovale
An opening in the upper part of the septum between the right and the left atrium, normally present only during foetal life
Fossa ovalis, secundum atrial septal defect
Persistence of the foramen ovale beyond the foetal life
Glenn shunt
Surgical connection of the superior vena cava with the right pulmonary artery in presence of a single ventricle circulation
Heart transplantation
A procedure to replace a diseased heart with a healthy one coming from a donor
Heterotaxy
Abnormal arrangement with symmetric development of normally asymmetrical structures of the body (lungs, liver, spleen, hearts’ components, etc.)
Hypertrophic cardiomyopathy
A disease in which the heart muscle becomes abnormally thick, usually due to a genetic mutation
Hypoplastic left heart syndrome
A complex and rare congenital heart disease in which the left side of the heart is critically underdeveloped
Inferior vena cava
The big vein collecting the venous blood returning from the lower part of the body into the right atrium
Interrupted aortic arch
See aortic arch interruption
Jugular vein
The big vein collecting venous blood returning from the head into the superior caval vein
Left aortic arch
Normally positioned aortic arch
Left atrium
The chamber of the heart collecting oxygenated venous blood from the pulmonary circulation
Left ventricle
The chamber of the heart collecting oxygenated blood from the left atrium and pumping it through the aorta in the rest of the body
Ligamentum arteriosum
See ductus arteriosus
Mitral valve
The valve between the left atrium and the left ventricle
Mitral valve regurgitation
A condition in which the mitral valve can’t close properly, causing blood from the left ventricle to flow backward into the left atrium
Mitral valve stenosis
A condition in which the mitral valve can’t open properly, causing a restriction to the blood flow afterward from the left atrium to the left ventricle
Myocardium
Heart muscle
Obstructive cardiomyopathy
A disease in which the heart muscle becomes abnormally thick, usually due to a genetic mutation, causing a restriction to the blood moving from the left ventricle to the aorta
Oesophageal atresia
Congenital defect in which the oesophagus terminates in a blind-ended pouch instead of the stomach
Palliative procedures
Operations where there is not full correction of a heart defect but is meant to improve an abnormal heart function, usually when corrective surgery is not (yet) possible
Partial anomalous pulmonary venous connection
Congenital heart disease in which some of the pulmonary veins are abnormally connected, directly or not, to the right instead of the left atrium
Partial atrioventricular canal
A variant of complete atrioventricular canal in which a hole between the upper chambers (the atria) is associated with a leakage of the valve between the atria and the ventricles
Patch
A small piece of material used to repair a tear of a break, to close a hole, or to strengthen a weak place
Patent ductus arteriosus
Persistence of the ductus arteriosus beyond the foetal life
Pericardium
Tiny sac enveloping the heart
Pleura
Tiny sac enveloping the lungs
Pulmonary artery
The vessel rising from the right ventricle and driving blood to the lungs
Pulmonary artery banding
Palliative procedure producing a narrowing at the level of the pulmonary artery to reduce the pulmonary blood flow in some congenital heart defects
Pulmonary atresia
Absence or closure of the normally opened pulmonary valve
Pulmonary stenosis
A condition in which there a condition in which the aortic valve can’t open properly, causing a restriction to the blood flow afterward from the left ventricle to the aorta a condition in which the aortic valve can’t open properly, causing a restriction to the blood flow afterward from the left ventricle to the aorta restriction to the blood flow afterward from the left ventricle to the aorta
Pulmonary valve
The valve between the right ventricle and the pulmonary artery
Pulmonary valve regurgitation
A condition in which the pulmonary valve can’t close properly, causing blood from the pulmonary artery to flow backward into the right ventricle
Pulmonary valve stenosis
A condition in which the pulmonary valve can’t open properly, causing a restriction to the blood flow afterward from the right ventricle to the pulmonary artery
Pulmonary veins
The veins collecting the oxygenated venous blood returning from the pulmonary circulation into the left atrium
Pulmonary venous connection anomalies
Anomalies regarding the pulmonary veins
Resternotomy
When the breastbone is divided to expose the heart, it is called a sternotomy. A resternotomy is the subsequent opening of the chest by dividing the breastbone again. If it is done soon after an operation, for example bleeding, it can be easy to perform. If it is performed months after the initial operation, it can be more complex to perform.
Right aortic arch
Aortic arch abnormally positioned on the right side
Right atrium
The chamber of the heart collecting de-oxygenated venous blood from the venous circulation
Right ventricle
The chamber of the heart collecting de-oxygenated blood from the right atrium and pumping it through the pulmonary artery in the pulmonary circulation
Secundum atrial septal defect
See fossa ovalis
Shunt
Abnormal pattern of blood flow in patients with congenital heart defects or bypass of the capillary system to which a given blood flow is destinated
Single ventricle
A heart defect in which one lower chamber (ventricle) does not develop normally
Situs inversus
A condition in which the arrangement of the internal organs is mirror-imaged compared to the normal anatomy
Situs solitus
Normal arrangement of the internal organs of the body
Stent
A tube placed into a hollow structure of the body to hold it open
Subaortic stenosis
A condition in which the blood flow from the left ventricle to the aorta is restricted due to an obstruction in the area under the aortic valve
Subpulmonary stenosis
A condition in which the blood flow from the right ventricle to the pulmonary artery is restricted due to an obstruction in the area under the pulmonary valve
Superior vena cava
The big vein collecting the venous blood returning from the upper part of the body into the right atrium
Supravalvar aortic stenosis
A condition in which the blood flow from the left ventricle to the aorta is restricted due to an obstruction in the area upon the aortic valve
Supravalvar pulmonary stenosis
A condition in which the blood flow from the right ventricle to the pulmonary artery is restricted due to an obstruction in the area upon the pulmonary valve
Tetralogy of Fallot
Congenital heart defects characterized by the combination of four elements, namely a ventricular septal defect, an abnormal position of the aortic valve upon the ventricular septal defect, an obstruction to the flow from the right ventricle to the pulmonary artery, right ventricular hypertrophy
Thymus
A secretory gland with an important role in immune function
Total anomalous pulmonary venous connection
Congenital heart disease in which all the pulmonary veins are abnormally connected, directly or not, to the right instead of the left atrium
Trachea
The part of the airway that leads from the larynx (voice box) to the bronchi (the large airways leading to the lungs)
Transposition of the great arteries
Rare heart defect in which the great arteries are reversed in their position, meaning that the aorta is abnormally connected to the right ventricle and the pulmonary artery++ is abnormally connected to the left ventricle
Tricuspid valve
The valve between the right atrium and the right ventricle
Tricuspid valve regurgitation
A condition in which the tricuspid valve can’t close properly, causing blood from the right ventricle to flow backward into the right atrium
Tricuspid valve stenosis
A condition in which the tricuspid valve can’t open properly, causing a restriction to the blood flow afterward from the right atrium to the right ventricle
Truncus arteriosus
A rare congenital heart defects in which the great arteries fail to separate during the fetal life, leaving a connection between the aorta and the pulmonary artery with a single blood vessel coming out of the right and the left ventricle
Vascular ring
Abnormal formation of the aorta with the normal vessels rising from it in an anomalous position, possibly causing compression or obstruction of the oesophagus or the airway
Ventricular assist device
Implantable mechanical pump which is used to help a failing heart to eject the blood from the ventricles to the rest of the body
Ventricular septal defect
A hole between the right and the left ventricles, normally completely separated by a septum