Pathophysiology (VENTRICULAR SEPTAL DEFECT) Normal Heart and Heart With Ventricular Septal Defect

During ventricular contraction, or systole, some of the blood from the left ventricle leaks into the right ventricle, passes through the lungs and reenters the left ventricle via the pulmonary veins and left atrium. This has two net effects. First, the circuitous refluxing of blood causes volume overload on the left ventricle. Second, because the left ventricle normally has a much higher systolic pressure (~120 mm Hg) than the right ventricle (~20 mm Hg), the leakage of blood into the right ventricle therefore elevates right ventricular pressure and volume, causing pulmonary hypertension with its associated symptoms. This effect is more noticeable in patients with larger defects, who may present with breathlessness, poor feeding and failure to thrive in infancy. Patients with smaller defects may be asymptomatic. Four different septal defects exist, with perimembranous most common, outlet, atrioventricular, and muscular less commonly.

Pathophysiology ( ATRIAL SEPTAL DEFECT) Normal Heart and Heart With Atrial Septal Defect

In unaffected individuals, the chambers of the left side of the heart are under higher pressure than the chambers of the right side of the heart. This is because the left ventricle has to produce enough pressure to pump blood throughout the entire body, while the right ventricle only has to produce enough pressure to pump blood to the lungs. In the case of a large ASD (>9mm), which may result in a clinically remarkable left-toright shunt, blood will shunt from the left atrium to the right atrium. This extra blood from the left atrium may cause a volume overload of both the right atrium and the right ventricle. If untreated, this condition can result in enlargement of the right side of the heart and ultimately heart failure. Any process that increases the pressure in the left ventricle can cause worsening of the left-toright shunt. This includes hypertension, which increases the pressure that the left ventricle has to generate in order to open the aortic valve during ventricular systole, and coronary artery disease which increases the stiffness of the left ventricle, thereby increasing the filling pressure of the left ventricle during ventricular diastole. The right ventricle will have to push out more blood than the left ventricle due to the left-toright shunt. This constant overload of the right side of the heart will cause an overload of the entire pulmonary vasculature. Eventually pulmonary hypertension may develop. The pulmonary hypertension will cause the right ventricle to face increased afterload in addition to the increased preload that the shunted blood from the left atrium to the right atrium caused. The right ventricle will be forced to generate higher pressures to try to overcome the pulmonary hypertension. This may lead to right ventricular failure (dilatation and

decreased systolicfunction of the right ventricle) or elevations of the right sided pressures relative to left sided pressures. When the pressure in the right atrium rises to the level in the left atrium, there will no longer be a pressure gradient between these heart chambers, and the left-to-right shunt will diminish or cease. If left uncorrected, the pressure in the right side of the heart will be greater than the left side of the heart. This will cause the pressure in the right atrium to be higher than the pressure in the left atrium. This will reverse the pressure gradient across the ASD, and the shunt will reverse; a right-to-left shunt will exist. This phenomenon is known as Eisenmenger's syndrome. Once right-to-left shunting occurs, a portion of the oxygen-poor blood will get shunted to the left side of the heart and ejected to the peripheral vascular system. This will cause signs of cyanosis.

Pathophysiology ( PATENT DUCTUS ARTERIOSUS )

The ductus arteriosus is part of the normal fetal circulatory system. This vessel connects the aorta and the pulmonary artery. Prior to birth the ductus arteriosus allows for antegrade flow from the right ventricle to the aorta. Following birth the ductus arteriosus normally closes. If the ductus remains open, blood will shunt from the aorta into the pulmonary artery due to the decrease in pulmonary vascular resistance. The amount of left-to-right shunting depends upon the size of the PDA and the relative resistances of the systemic and pulmonary circulations. Left-to-right shunting of blood caused by patency of the ductus arteriosus results in increased pulmonary artery blood flow as well as left atrial and left ventricular overload. Extensive aortic runoff, with low aortic diastolic pressure secondary to a large patent ductus arteriosus can result in systemic organ hypoperfusion. Pulmonary vascular obstructive disease may occur, sometimes as early as one year of life.

Pathophysiology ( TETRALOGY OF FALLOT ) Normal Heart and Heart With Tetralogy of Fallot

Primary four malformations "Tetralogy" denotes a four-part phenomenon in various fields, including literature, and the four parts the syndrome's name implies are its four signs. This is not to be confused with the similarly named teratology, a field of medicine concerned with abnormal development and congenital malformations, which thereby includes tetralogy of Fallot as part of its subject matter. As such, by definition, tetralogy of Fallot involves exactly four heart malformations which present together: Condition Description

A:Pulmonary

A narrowing of the right ventricular outflow tract and can occur at

stenosis

the pulmonary valve (valvular stenosis) or just below the pulmonary valve (infundibular stenosis). Infundibular pulmonic stenosis is mostly caused by overgrowth of the heart muscle wall (hypertrophy of the septoparietal trabeculae),[8] however the events leading to the formation of the overriding aorta are also believed to be a cause. The pulmonic stenosis is the major cause of the malformations, with the other associated malformations acting as compensatory mechanisms to the pulmonic stenosis.The degree of stenosis varies between individuals with TOF, and is the primary determinant of symptoms and severity. This malformation is infrequently described as subpulmonary stenosis orsubpulmonary obstruction.

B:Overriding aorta

An aortic valve with biventricular connection, that is, it is situated above the ventricular septal defect and connected to both the right and the left ventricle. The degree to which the aorta is attached to the right ventricle is referred to as its degree of "override." The aortic root can be displaced toward the front (anteriorly) or directly above the septal defect, but it is always abnormally located to the right of the root of the pulmonary artery. The degree of override is quite variable, with 5-95% of the valve being connected to the right ventricle.

C:ventricular septal defect(VSD)

A hole between the two bottom chambers (ventricles) of the heart. The defect is centered around the most superior aspect of the ventricular septum (the outlet septum), and in the majority of cases is single and large. In some cases thickening of the septum (septal hypertrophy) can narrow the margins of the defect.[8]

D: Right ventricular hypertrophy

The right ventricle is more muscular than normal, causing a characteristic boot-shaped (coeur-en-sabot) appearance as seen by chest X-ray. Due to the misarrangement of the external ventricular septum, the right ventricular wall increases in size to deal with the increased obstruction to the right outflow tract. This feature is now generally agreed to be a secondary anomaly, as the level of hypertrophy generally increases with age.