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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.
A cardiac shunt is when blood follows a pattern that deviates from the systemic circulation, i.e., from the body to the right atrium, down to the right ventricle, to the lungs, from the lungs to the left atrium, down to the left ventricle and then out of the heart back to the systemic circulation.
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 ...
Corrective heart surgery, which is normally performed in the first year of life, is the definitive intervention for an aortopulmonary window. If the patient's symptoms don't allow for corrective surgery, medical therapy of congestive heart failure is the second choice. Permanent alterations in the pulmonary vasculature can be prevented with ...
[8] 45% of children with Down syndrome have congenital heart disease. Of these, 35–40% have AV septal defects. [ 9 ] 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 .
Ebstein's anomaly is a congenital heart defect in which the septal and posterior leaflets of the tricuspid valve are displaced downwards towards the apex of the right ventricle of the heart. [1] EA has great anatomical heterogeneity that generates a wide spectrum of clinical features at presentation and is complicated by the fact that the ...
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Taussig–Bing syndrome is a cyanotic congenital heart defect [1] in which the patient has both double outlet right ventricle (DORV) and subpulmonic ventricular septal defect (VSD).