Classification des cardiopathies congénitales

• I - Physiopathologie :• Chez le sujet normal, la petite et la grande

circulation sont en série et leurs débit sont équivalent.

• Les régimes de pression sont réglés par l’existence de résistances basses dans la circulation pulmonaire et de résistances élevées dans la circulation systémique y conditionnant des pressions élevées.

• Dans les jours qui suivent la naissance ces résistances de la petite circulation baissent progressivement pendant que les shunts fœtaux se ferment. On estime que

• RP/RS=0,5 entre 10 et 24h. • RP/RS= 0,1 au 15ème jour , le même que chez

l’adulte. • Cette baisse progressive des résistances

pulmonaires explique l’apparition fréquemment retardée des souffles de CIV qui peuvent ne pas être perçus dans les premiers jours de la vie.

• Chez le prématuré : le niveau des résistances artériolaire pulmonaire est moins élevé ( le développement musculaire des artérioles se fait durant le dernier trimestre. Cela explique le fait que certains shunt G-Dte soient mal tolérés précocement chez lui.

Congenital heart defects are abnormalities that develop before birth. They can occur in the heart's chambers, valves or blood vessels. A baby may be born with only one defect or several that tend to occur in combination. Of the dozens of heart defects, some are mild and may need minimal or no medical treatment even through adulthood, while others are life-threatening, either immediately to the newborn or over time. Here's a look at some of the more common congenital heart defects. Compare them to the NORMAL HUMAN HEART, SHOWN HERE

Congenital Heart Disease

Type of Defect Mechanism

Ventricular Septal Defect (VSD)

There is a hole within the membranous or muscular portions of the intraventricular septum that produces a left-to-right shunt, more severe with larger defects

Atrial Septal Defect (ASD)

A hole from a septum secundum or septum primum defect in the interatrial septum produces a modest left-to-right shunt

Patent Ductus Arteriosus (PDA)

The ductus arteriosus, which normally closes soon after birth, remains open, and a left-to-right shunt develops

Tetralogy of Fallot Pulmonic stenosis results in right ventricular hypertrophy and a right-to-left shunt across a VSD, which also has an overriding aorta

Transposition of Great Vessels

The aorta arises from the right ventricle and the pulmonic trunk from the left ventricle. A VSD, or ASD with PDA, is needed for extrauterine survival. There is right-to-left shunting.

Truncus Arteriosus There is incomplete separation of the aortic and pulmonary outflows, along with VSD, which allows mixing of oxygenated and deoxygenated blood and right-to-left shunting

Hypoplastic Left Heart Syndrome

There are varying degrees of hypoplasia or atresia of the aortic and mitral valves, along with a small to absent left ventricular chamber

Coarctation of Aorta

Either just proximal (infantile form) or just distal (adult form) to the ductus is a narrowing of the aortic lumen, leading to outflow obstruction

Total Anomalous Pulmonary Venous Return (TAPVR)

The pulmonary veins do not directly connect to the left atrium, but drain into left innominate vein, coronary sinus, or some other site, leading to possible mixing of blood and right-sided overload

Ventricular septal defect

Sometimes called a hole in the heart, this defect — the most common congenital

heart defect — occurs when the septum, the muscular wall separating the right and left ventricles, fails to fully form. The hole allows oxygen-rich blood to leak from the

left ventricle into the right ventricle, instead of moving into the aorta and on to the body. Too much blood may flood the

lungs. This defect can lead to heart failure,

excessive blood pressure in the lungs (pulmonary hypertension), infections of the heart (endocarditis), irregular heart

beats (arrhythmias) and delayed growth. Small holes may heal on their own or

cause no symptoms. Larger holes may require surgical repair by stiching together

or covering with a patch.

Atrial septal defect

Similar to a ventricular septal defect, this is a hole that occurs when the

septum separating the right and left atria doesn't close properly. This allows

blood from the left atrium to flow into the right atrium, instead of into the left ventricle and on to the aorta and the

rest of the body.The defect can cause several

complications, including arrhythmias, heart failure, stroke and, in rare cases, pulmonary hypertension. Minor cases may cause no symptoms and may not require treatment. Larger defects may

require surgical closure or cardiac catheterization.

Patent ductus arteriosus

Before birth, a temporary blood vessel called the ductus arteriosus connects the

pulmonary artery and the aorta. This allows blood to bypass the lungs

because oxygen is delivered to the fetus through the placenta and umbilical cord. The temporary vessel normally closes

within a few hours or days of birth since the lungs take over. If it remains open

(patent), some blood that should circulate through the body is misdirected

to the lungs.This defect can cause heart failure or

endocarditisse heart failure or endocarditis. In infants, it can be closed with medications. In older children and adults, plugs, coils or surgery can be

used to close the vessel.

Pulmonary stenosis

In this condition, the flow of blood from the right ventricle to the pulmonary artery

is obstructed by narrowing at the pulmonary valve. When there's an

obstruction (stenosis), the right ventricle must pump harder to get blood into the pulmonary artery. The defect may occur

along with other defects, such as thickening of the muscle of the right

ventricle immediately below the valve.In many cases, pulmonary stenosis is

mild and doesn't require treatment. But because it can cause heart failure,

arrhythmias or enlargement of the right heart chambers, surgery may be

necessary to repair the stenosis or replace the valve. Special balloons to widen the valve (balloon valvuloplasty)

may also be used.

Tetralogy of Fallot

This defect is a combination of four (tetralogy) congenital abnormalities.

The four defects typically are ventricular septal defect (VSD), pulmonary stenosis, a misplaced aorta and a thickened right

ventricular wall (right ventricular hypertrophy). They usually result in an

insufficient amount of oxygenated blood reaching the body.

Complications of tetralogy of Fallot (fuh-LOE) include cyanosis — sometimes

called "blue baby syndrome," since the lips, fingers and toes may have a bluish tinge from lack of oxygen — as well as

poor eating, inability to tolerate exercise, arrhythmias, delayed growth and

development, and stroke. Surgical repair of the defects is required early in life.

Transposition of the great vessels (arteries)

With this defect, the positions of the aorta and the pulmonary artery

(the great arteries) are reversed (transposed). The aorta arises from the right ventricle instead of the left

and the pulmonary artery arises from the left ventricle instead of the right.

This creates a circulatory pattern that prevents nourishing oxygenated blood from reaching the body.

This condition would quickly be fatal to a newborn except it's generally accompanied by another defect — commonly a septal defect or patent ductus arteriosus — that does allow oxygen-rich blood to get to the body. Surgical repair is usually necessary

shortly after birth.

Truncus arteriosus

This is a defect in which the normally distinct pulmonary artery and aorta merge into one single great vessel

(truncus) arising from the right and left ventricles. In addition, there's usually a

large ventricular septal defect, essentially turning the right and left ventricles into a single chamber. This allows oxygenated

and unoxygenated blood to mix. Too much blood may flow to the lungs,

flooding them and making it difficult to breathe. It can also result in life-

threatening pulmonary hypertension.Surgery is needed to close the septal

defect with a patch and to separate the pulmonary arteries from the trunk. A conduit is placed to connect the right

ventricle to the pulmonary artery. Because the conduit doesn't grow with

the child, repeat surgery may be necessary over time

Hypoplastic left heart syndrome

In this condition, the left side of the heart is underdeveloped (hypoplastic), including the aorta, aortic valve, left ventricle and

mitral valve. As a result, the body doesn't receive enough oxygenated blood. In the

first few days after a baby is born, the ductus arteriosus remains open (patent), allowing normal circulation, so the baby

may seem fine initially. But when the ductus arteriosus naturally closes, signs and symptoms begin, including a bluish

cast to the skin from lack of oxygen, difficulty breathing and poor feeding. This

condition may be accompanied by an atrial septal defect.

Treatment options for this life-threatening condition are a heart transplant or a

multistage surgical procedure done during the first few years of life.

Coarctation of the aorta

This is a narrowing (coarctation), or constriction,

in a portion of the aorta. Coarctation forces the heart to

pump harder to get blood through the aorta and on to the

rest of the body.This defect can cause several life-threatening complications, including severe hypertension, aortic aneurysm, dissection or

rupture, endocarditis, brain hemorrhage, stroke, heart failure and premature coronary artery

disease. Repair is typically recommended before age 10,

either by surgically removing the affected portion or widening it

through balloon angioplasty and placement of a stent.

Aortic stenosis

This is a defect that narrows or obstructs the aortic valve

opening, making it difficult for the heart to pump blood into the aorta. Mild cases may not

have symptoms initially, but they can worsen over time.

The defect can cause heart enlargement, left-sided heart

failure, arrhythmias, endocarditis and fainting. Treatment includes surgical repair or replacement of the valve or, in young children,

widening through balloon valvuloplasty.

Ebstein's anomalyThis is a defect of the tricuspid valve, which controls blood flow between the heart's right

atrium and right ventricle. The valve is positioned lower than normal into the

ventricle instead of remaining between the atrium and the ventricle. Consequently, the

ventricle is too small and the atrium too large, and neither functions properly. The

valve is also malformed, often allowing blood to leak from the ventricle into the

atrium. This defect often occurs along with other heart defects, including patent

foramen ovale, atrial septal defect or Wolff-Parkinson-White syndrome.

Severe cases are life-threatening. Milder cases may have no signs or symptoms until adulthood. Treatment is with medications or

with surgery to repair or replace the tricuspid valve, as well as treatment of

associated conditions.

If there's an extra conduction pathway, the electrical signal may arrive at the ventricles too soon. This condition is called Wolff-Parkinson-White syndrome (WPW). It's in a category of electrical abnormalities called "pre-excitation syndromes."

Atrioventricular canal defect

This is a combination of defects, including a large hole in the center of the heart and a single common valve instead of the separate tricuspid and

mitral valves. Also called atrioventricular septal defect, this defect is classified by whether it's only partial, involving only

the upper chambers of the heart, or complete, in which blood can travel freely among all four chambers of the heart. Both forms allow extra blood to circulate to the lungs, causing the

heart to enlarge.The condition is often associated with

Down syndrome. Infants may also have trouble breathing and not grow well.

Surgery is often done in infancy to close the hole and reconstruct the valves.

• II- Classification :

• On distingue 3 classes de cardiopathies congénitales– cardiopathies avec shunt G-dte– cardiopathies cyanogènes– cardiopathies par obstacle isolé

• Cardiopathies par shunts Gauche-dte. En raison du régime de pressions plus élevées dans la circulation systémique que dans la circulation pulmonaire, un défaut de cloisonnement du cœur ou la persistance d’une communication anormale entre les 2 circulations suffit à la création d’un shunt G-dte.

• La communication peut siéger soit à l’étage auriculaire ou veineux soit à l’étage ventriculaire ou artériel. Le shunt gauche- droite a pour conséquences une augmentation du débit pulmonaire qui dépend de la taille de la communication.

• Dans les shunts importants cet hyper débit peut s’accompagner d’une augmentation de la pression pulmonaire dite de débit. Cette hypertension pulmonaire et cet hyper débit sont responsables de la défaillance cardiaque.

• La maladie obstructive artériolaire pulmonaire est le risque des shunts G-Dte importants ; C’est une réduction du calibre artériolaire pulmonaire. Les lésions sont d’abord réversibles (épaississement musculaire) puis irréversibles( fibrose)

• Cardiopathies avec défaut septal : shunt gauche- droite

• CIA avec ou sans SS. RVPA• CAV• CIV• CA• Fistule ou fenêtre aorto-pulmonaire• Fistule artère coronaire cavités droites.• Naissance anormale de la coronaire gauche à partir de

l’AP• Rupture du sinus de Valsalva dans les cavités droites

• VDDI

• VGDI

• Les cardiopathies cyanogènes : La cyanose est un symptôme grave.

• Physiopathologie : pour qu’il y ait cyanose il est nécessaire que du sang désaturé venant des veines caves passe dans l’aorte.

• Ces shunts droite-gauche peuvent se faire systématiquement dans trois circonstances– obstacle pulmonaire + défaut septal : T4F,

T3F, Ebstein, CIA, Eisenmenger, atrésie tric. Toutes ces cardiopathies ont en commun une réduction du débit pulmonaire.

– TGV– Mélange total des 2 circulations / RVPAT, VU,

TA commun

• Les obstacles isolés : peuvent siéger sur la voie G ou droite. Ils ont en commun une augmentation de la post charge avec hyper pression en amont de la sténose .

• Cardiopathies par obstacle :– Gauche :

• Cœur triatrial• RM congénital• Rao SS val ; val ; supraval• Coarcatation de l’aorte

– Droit• Maladie d’Ebstein• RP infund ; orificiel ; des branches• HTAP primitive

– Mixte• Hypoplasie artérielle multiple• Syndrome de Williams et Beuren

– Cardiopathies avec mélange des 2 circulations :

• RVPAT• Oreillette unique• Hypoplasie du VG et atrésie mitrale• VU• Tronc artériel commun• Veine cave anormalement abouchée dans

l’OG• Fistule artério-veineuse pulmonaire