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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 cardiology, a cardiac shunt is a pattern of blood flow in the heart that deviates from the normal circuit of the circulatory system. It may be described as right-left , left-right or bidirectional, or as systemic-to-pulmonary or pulmonary-to-systemic .
[20]: 205 Post surgery, most patients enjoy an active life free of symptoms. [20]: 205 Currently, long-term survival is close to 90%. [20]: 167 Today the adult TOF population continues to grow and is one of the most common congenital heart defects seen in adult outpatient clinics. [5]: 100–101
Symptoms frequently present early in life, but it is possible for some CHDs to go undetected throughout life. [15] Some children have no signs while others may exhibit shortness of breath, cyanosis , fainting , [ 16 ] heart murmur , under-development of limbs and muscles, poor feeding or growth, or respiratory infections.
If left untreated, patients with HLHS die within the first weeks of life while 70% of those that undergo three-staged palliative surgery reach adulthood. After surgery, children with HLHS typically experience neurodevelopmental as well as motor delay and are at an increased risk of heart failure as adults. [11] [12]
The Trilogy of Fallot also called Fallot's trilogy is a rare congenital heart disease consisting of the following defects: pulmonary valve stenosis, right ventricular hypertrophy and atrial septal defect. [1] It occurs in 1.2% of all congenital heart defects. [2]
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 ...
Symptoms may include difficulty breathing and bluish discoloration on skin, fingernails, and lips (). [5]An infant will begin to show signs of congestive heart failure, which can include rapid breathing, feeding problems, slow weight gain, low energy, and cold, clammy sweating. [4]
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