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Eisenmenger's Syndrome

Eisenmenger's syndrome or Eisenmenger's reaction is defined as the process in which a left-to-right shunt in the heart causes increased flow through the pulmonary vasculature , causing pulmonary hypertension , which in turn, causes increased pressures in the right side of the heart and reversal of the shunt into a right-to-left shunt. This was the first description of a link between a large congenital cardiac shunt defect and the development of pulmonary hypertension This is a serious condition in which a right-to-left movement of blood occurs, resulting in unoxygenated blood coming into the left side of your heart and being pumped into the general circulation. flow. Over time, the increasing strength of the right side raises the blood pressure in the lungs creating pulmonary hypertension . Using intravenous contrast injections along with echocardiogram will visualize the intracardiac defect. . When you decrease the volume of a closed system, the pressure increases; with increased pressure, more capillaries die, so pulmonary hypertension in Eisenmenger's Syndrome tends to get worse, and the worse it gets, the faster it gets worse. Subsequently, usually before puberty, pulmonary vascular disease causes pulmonary hypertension, ultimately resulting in reversed or bidirectional shunt flow with variable degrees of cyanosis Free communication of the blood between the left and right sides of the heart means that the person can be viewed as having a one chambered heart, where the blood leaves through the channel of least resistance .Lung transplantation with repair of the cardiac or combined heart-lung transplantation is an option for patients with a poor prognosis (failing to respond to medical therapy).

People who have Eisenmenger's syndrome are usually born with a large hole in the heart. Eisenmenger's syndrome primarily affects adolescents and adults with certain congenital heart defects that are repaired late (after the first year of life) or that are never repaired. This hole permits blood to flow from the high-pressure (red, oxygen carrying) left-sided circulation to the lower-pressure (blue, oxygen poor) right-sided circulation. One patient suffered post-operative complications, but the other case was uncomplicated. We used sevoflurane and total intravenous anaesthesia to provide general anaesthesia. Both techniques were tolerated. The syndrome can occur as a complication of ventricular septal defect, atrial septal defect or persistent ductus arteriosus but can be associated with any congenital disease. With a shunt at ventricular or arterial level, pulmonary vascular disease starts in early life and causes progressive pulmonary vascular resistance until it is greater than systemic vascular resistance, so that the shunt is reversed As this lung vessel damage continues, the pressure in the pulmonary arteries will progressively rise. This elevated pressure in the lungs is called pulmonary hypertension. Eisenmenger's syndrome, however, can occur in infants or children born with pulmonary hypertension; therefore, symptoms related to cyanosis may occur much earlier in life.

Causes of Eisenmenger's Syndrome

The common causes of Eisenmenger's Syndrome :

  • Atrioventricular septal defect, including large ostium primum ASD without ventricular component
  • Palliative, surgically created systemic-to-pulmonary anastomosis for treatment of congenital heart disease
  • A connection between the aorta and the pulmonary artery which allows oxygen-rich (red) blood that should go to the body to recirculate through the lungs.
  • an opening in the ventricular septum, or dividing wall between the two lower chambers of the heart known as the right and left ventricles.
  • Nonrestricted PDA
  • a complex heart problem that involves several abnormalities of structures inside the heart, including an ASD, VSD, and improperly formed mitral and/or tricuspid valves.

Symptoms of Eisenmeenger's Syndrome

Some are common symptoms of Eisenmeenger's Syndrome :

  • cyanosis (pale blue or grayish skin due to decreased oxygen in the blood)
  • paresthesias (numbness and tingling)
  • chest pain chest pain
  • Cyanosis , or a blueness of the skin, especially in the lips, resulting from inadequate availability of oxygen to the tissues of the body
  • brain abscesses
  • Failure to thrive occurs when the infant experiences shortness of breath during feeding and as a result, gains weight at a rate substantially below the growth curve.
  • palpitations (heart “racing”)

Treatment of Eisenmeenger's Syndrome

  • your child's tolerance for specific medications, procedures, or therapies
  • expectations for the course of the disease
  • Medications that help to decrease pulmonary hypertension may be prescribed. These medications include calcium channel blockers, prostacyclin, and endothelin receptor antagonists, and work to dilate blood vessels which helps to lower the blood pressure in the lungs.
  • Avoid very hot or humid conditions, which may exacerbate vasodilation, causing syncope and increased right-to-left shunting.
  • Some patients might benefit from nocturnal supplementation, although it is most useful as a bridge to heart-lung transplant.
  • A rapid change in blood pressure, fluid volume or pulse rate could cause more right to left shunting and decrease oxygen levels to a critical level.
  • Anticoagulants are medications to prolong the time it takes for a blood clot to form, like Aspirin or Warfarin, and are often used in this condition.


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