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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.
In an 1897 article in a German medical journal, Eisenmenger described signs of low blood oxygen levels (including a bluish hue to the skin and nail clubbing) in a 32-year-old man who had been born with a ventricular septal defect. [9] Eisenmenger had seen the patient during his time working with von Schrötter. [4]
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 ...
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.
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.
Approximately 40-50% of fetuses diagnosed with AVCD have Down syndrome, and a further 15-20% are associated with other chromosomal abnormalities and syndromes, such as DiGeorge syndrome. [3] [10] The remaining 30-40% of cases are not linked to a syndrome, with AVCD observed without other major defects. AVCD is also linked with Noonan syndrome. [3]
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.
Classic for a ventricular septal defect (VSD). This may lead to the development of the delayed-onset cyanotic heart disease known as Eisenmenger syndrome. Eisenmenger syndrome is a reversal of the left-to-right heart shunt. This is the result of hypertrophy of the right ventricle over time.