<unk>  was first discovered by  Etienne<unk> , a French ohysician in 1888.<unk>  is a congenital heart defect which classically has four anatomical components: obstruction to right ventricular outflow, right ventricular hypertrophy, ventricular septal defect, and overriding of aorta.  It accounts for approximately 10% of all forms of congenital heart disease.  It is the most common cause of cyanosis in children over one year of age (blue baby syndrome). It is understood that<unk>  is the result of improper positioning of the outlet septum. In the normal heart, the outlet septum is an indistinguishable component of the crista supraventricularis that communicates with the septomarginal trabeculae to divide the right and left ventricular cavities. In<unk> , proper ventricular septation is perturbed by anterocephalad displacement of the outlet septum relative to the septomarginal trabecula. These features include a ventricular septal defect, overriding aorta, pulmonary stenosis, and right ventricular hypertrophy. The obstruction of right ventricular outflow in<unk>  causes blood to shunt or flow from the right to left side of heart through the ventricular septal defect.  This causes right ventricular hypertrophy and eventual right sided heart failure.  There is flow of deoxygenated venous blood from the right side of the heart to the systemic circulation resulting in cyanosis. Common causes of<unk>  may include: Alcoholism in the mother, diabetes, pregnancy after the age of 40, rubella or other viral illnesses during pregnancy, phenylketonuria (PKU) in the mother, fetal hydantoin syndrome, and fetal carbamazepine syndrome. On the basis cyanosis,<unk>  must be differentiated from total anomalous pulmonary venous connection, tricuspid atresia, transposition of the great vessels, and truncus arteriosus.<unk>  occurs in approximately 30 to 60 per 100,000 births.<unk>  represents 5-7% of congenital heart defects. The majority of cases are thought to be sporadic and are not familial.<unk>  occurs slightly more often in males than in females. Common risk factors in the development of<unk>  include: Alcoholism in the mother, diabetes, and pregnancy after the age of 40. According to the Canadian Cardiovascular Society/Canadian Pediatric Cardiology Association, screening for<unk>  by pulse oxymetry is recommended for all newborns. The prognosis of patients with repaired<unk>  has improved over the years and these patients now have the potential to lead normal lives.  As these patients grow, there may be leakage of the repaired pulmonic valve. There may be a persistent risk of sudden cardiac death. Echocardiography is the gold standard test for the diagnosis of<unk> . Echocardiography establishes the initial diagnosis of<unk> , and is useful in evaluating the hemodynamic abnormalities that are present. Right-to-left shunting through the VSD can be visualized by color doppler imaging, and the severity of right ventricular outflow tract obstruction can be determined by spectral doppler measurements. Echocardiography may be helpful in the diagnosis of<unk> . Findings on an echocardiography diagnostic of<unk>  include ventricular septal defect, right ventricular outflow tract obstruction, and overriding aorta. Repair of<unk>  reduces mortality.  we can either perform palliative surgery which involves forming an anastomosis between the subclavian artery and the pulmonary artery. This redirected a large portion of the partially oxygenated blood leaving the heart for the body into the lungs, increasing flow through the pulmonary circuit, and greatly relieving symptoms in patients or total surgical repair which involves making incisions into the heart muscle, relieving the right ventricular outflow tract stenosis by careful resection of muscle, repairing the VSD using a Gore-Tex or Dacron patch or a homograft. Additional reparative or reconstructive work may be done on patients as required by their particular anatomy.The repair could be done by either of the approaches i.e.transatrial or transpulmonary.
Tetralogy of fallot
Pentalogy of Fallot
Trilogy of Fallot
Tetralogy of Fallot
Persistent truncus arteriosus
Interrupted aortic arch
Transposition of the great vessels
Tricuspid atresia