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Eisenmenger syndrome or Eisenmenger's syndrome is defined as the process in which a long-standing left-to-right cardiac shunt caused by a congenital heart defect (typically by a ventricular septal defect, atrial septal defect, or less commonly, patent ductus arteriosus) causes pulmonary hypertension [1] [2] and eventual reversal of the shunt into a cyanotic right-to-left shunt.
An uncorrected left-to-right shunt can progress to a right-to-left shunt; this process is termed Eisenmenger syndrome. [3] This is seen in Ventricular septal defect, Atrial septal defect, and patent ductus arteriosus, and can manifest as late as adult life. This switch in blood flow direction is precipitated by pulmonary hypertension due to ...
Aortopulmonary window (APW) is a faulty connection between the aorta and the main pulmonary artery that results in a significant left-to-right shunt. [2] The aortopulmonary window is the rarest of septal defects, accounting for 0.15-0.6% of all congenital heart malformations. [4]
Persistent truncus arteriosus (PTA), [1] often referred to simply as truncus arteriosus, [2] is a rare form of congenital heart disease that presents at birth. In this condition, the embryological structure known as the truncus arteriosus fails to properly divide into the pulmonary trunk and aorta.
Cat eye syndrome: CHARGE syndrome: Coffin–Lowry syndrome: genetic (RPS6KA3 gene mutation, Chromosome X) Costello syndrome: Down syndrome: genetic (Chromosome 21) Dressler syndrome: autoimmune inflammatory reaction secondary to MI. Edwards syndrome: genetic (Chromosome 18) Eisenmenger's syndrome: Ellis–van Creveld syndrome: Emanuel syndrome ...
Larger defects may eventually be associated with pulmonary hypertension due to the increased blood flow. Over time this may lead to an Eisenmenger's syndrome the original VSD operating with a left-to-right shunt, now becomes a right-to-left shunt because of the increased pressures in the pulmonary vascular bed.
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This reversal is called Eisenmenger syndrome. Lipomatous atrial septal hypertrophy (LASH) is the fat deposition in the infoldings of the interatrial septum adjacent to the true atrial septum. It is shaped like a “dumbbell” because the deposition is located at the above and the below the fossa ovalis, sparing the fossa itself.