[Music] welcome to this presentation on congenital heart disease development and classification in this presentation we will discuss a unique spectrum of heart diseases congenital heart diseases congenital heart disease represents a defect in the heart that does not develop normally and is present at birth it is the most common form of birth defects and is a leading cause of death from birth abnormalities in the first year of Life congenital heart disease affects approximately eight out of 10,000 life births among them some defects need immediate treatment whereas some can be followed up as the defect may disappear with heart growth the survival of infants when congenital heart effects has greatly improved with the continual medical advancements and treatment including medications catheter based intervention and surgery as there are numerous congenital heart defects we will categorize them based on how blood flow is altered from a normally structured Anatomy we will first review heart embryology and development during embryo growth the primative heart begins to beat at around Daye 22 or 23 thereafter the tubular heart grows and elongates developing a series of alternate constrictions and dilations creating the first sign of primitive heart chambers known as heart looping the complicated process of septation of the atrium Atri ventricular EV canal and ventrical occurs between the fourth and sixth week the Primitive atrial septum begins with the emergence of the septum primum that grows from the dorsal wall of the atrium and extends to where the endocardial cushions of the AV canal as the septum primin continues to grow day by day the opening between the septum primin and the E Canal the osteum primin starts to decrease in size the osteum primum will Solly fuse with the endocardiac cushions and during this process small perforations appear in the septum and will merge to form the osteum secundum at this point the blood has the ability to flow between the two atria the septum sendum is also developing to grow over the septum primum and leave an opening known as the forino valy the image on the left shows the septation of the atrium and the image on the right just that the blood shunts or flows from the right atrium into the left atrium via the valve like septum primum before birth however after birth the right atal pressure is lower than the left atal pressure and the shunt stops as a septum is a one-way valve septation of the ventricles begins at the end of the fourth week the Primitive muscular interventricular septum increases in size leaving an opening that allows communication between the right and left ventricles which is called the interventricular Forin by the seventh week interventricular form encloses forming the membranous portion of the intraventricular septum development of atov ventricular and semi linar valves is also strictly time dependent at five weeks three outgrow of tissue form around the ortic and Pulmonary orifices to become three thin walled cusp of eortic and Pulmonary valves atakes proliferation of tissues surrounding the atov ventricular canals forms the atov ventricular valves mitro valve and the tricuspid valve cordate tendon are also formed with papillary muscles as their muscular attachments to the ventricular wall during fetus development there are three shuns to help bypass the lungs and liver but will close shortly after birth as the lungs begin to function the ductus Venus which bypasses the hepatic vasculature to join the inferior vena and allow oxygenated blood from the placenta to mix with deoxygenated blood from the lower body as blood enters from the inferior vnea the majority of blood is shunted towards the left atrium through the forino valley with the remainder portion of blood passing out of the right ventricle through the pulmonary artery as the lungs are not functional during gestation the blood exiting the pulmonary artery is directed to the descending aortic Arch by the ductus arteriosis upon birth the resistance of blood flow through the lungs reduces and results in an increase in blood flow through the pulmonary arteries this increased blood flow will increase left atal pressure and close the forino valy the ductus arterial will close one to two days after birth and within a week the ductus vosis will also close before birth conal heart defects usually do not jeopardize fetus as oxygen nutrition come from the mother however after birth congenital heart defects become more obvious after birth fetal shuns will close to allow normal lug functioning and oxygen exchange and allow for manifestation of congenital defects to compromise the infant and further manifest we can initially classify these defects into categories with the predominant divide into Astic or cyano lesions or defects Astic lesions are classified by shunt of blood from the left atrium to the right atrium and obstruction of blood flow due to intercardiac or vascular stenosis or regurgitation cyanosis is recognized by blue purple dis coloration of the skin caused by elevated concentration of deoxygenated hemoglobin cyanotic lesions or defects are caused by this desaturation in Blood and often infants with these defects are called blue babies the sinosis is due to diminished pulmonary blood flow from a right to left shunt and increased pulmonary blood flow resulting in elevated pulmonary resistance a second approach to congenital heart disease classification is based on anatomic complexity of the lesions or deflex as being simple or complex note the defects within each category the third approach was proposed to combine both the physiologic and anatomic classification the first group is left to right shunt lesions without sinosis the first group of ASO lesions is separated into increased pulmonary flow due to left to right sh and the second group contains Astic outflow obstructive lesions the next set of groups is contained within the category of cyanic lesions which affect pulmonary blood flow the third group consists of decreased pulmonary blood FL lesions with cyanosis the fourth group is increased pulmonary blood flow lesions with cyanosis the fifth group is increased pulmonary vascular resistant lesions with sinosis we have also added an additional colum that encompasses genetic disorders that are associated with congenital heart defects most often congenital defects are present as isolated findings but can be found in combination with heritable conditions tricomi 13821 represent a group of chromosomal abnormalities with extra genetic material from each of the listed chromosomal numbers these abnormalities give rise to atal sepal defects rric sepal defects and Pon ductus arteriosis Turner syndrome involves left-sided obstructive lesions including bicuspid aortic valve coration of the ort and sometimes evidence of hypoplastic left heart syndrome William syndromes observes aorc stenosis and pulmonary artery obstruction H Orum syndrome is also known as the heart hand syndrome whose characteristic defects include atpal defects or ASDS and ventricular septile defects or vsds Maran syndrome affects the body's connective tissues and may cause enlargement of the yorta which is life-threatening to the patient as it can lead to aortic aneurysm a mital valve prolapse may also be present it is also also worth mentioning the inflammatory disease known as cowasaki disease that is a rare childhood illness that can affect the coronary arteries there's inflammation in the walls of the arteries throughout the body but it tends to affect the coronary arteries it may lead to weakening and bulging or anism that takes us to the end of this presentation on congenital heart disease development and classification [Music]