Eisenmenger syndrome has been described as a ventricular septum defect with right ventricular hypertrophy, riding aorta and cyanosis. As for frequency, it is over represented in reverse shunts. There comes to mind a ventricular septum defect, patent ductus arteriosus persistens and atrial septum defect. Causes of pulmonary hypertension are not known. Increased pulmonary vascular resistance lead to the right ventricular hypertrophy and the reverse shunt. The blood still flows from left to right, but also from right to left, blood coming from the lungs, oxygen-enriched, again returns to the lungs instead to go into the rest of the body.
For this reason, there are some changes in the lungs, the arteries become narrow and stiff and therefore they increase pressure in the pulmonary arteries. When blood pressure from the lungs becomes greater than the pressure of the blood that passes through, the shunt reverses, then it is called Eisenmenger syndrome. Blood with a lack of oxygen from the left side of the heart, goes to the left ventricle and further to the rest of the body, which in this process is not getting enough oxygen-enriched blood.
The clinical picture of Eisenmenger syndrome
Symptoms and signs of Eisenmenger syndrome
Often fatigue leads to moderate or significant dyspnea. Ventricular and atrial septum defect leads to the appearance of cyanosis with polycythemia and clubbing of the fingers. Reverse ductus arteriosus persistens causes cyanosis of lower legs and toes. It is possible to palpate action of the right ventricle and the arteria pulmonalis. Along the left sternal border systolic murmur can be heard, and early pulmonary systolic shock. Eisenmenger syndrome can cause other complications such as congestive cardiac failure, biliary vesicle, bleeding, blood clot, kidney problems, dizziness, high red blood cell count, iron deficiency, palpitations, headache, cerebral abscess, blurred vision, fainting, shortness of breath and swelling in the joints.
Most of these symptoms of Eisenmenger syndrome can be early noticed, and should be reported to your pediatrician. These symptoms can be similar to other heart problems.
Radiographic findings of Eisenmenger syndrome
On fluoroscopy we can concluded a big, pulsating active pulmonary artery.
ECG of Eisenmenger syndrome
Usually there is right ventricular hypertrophy with prominent P waves.
Echocardiogram of Eisenmenger syndrome
With this procedure, which examines the structure of the heart by using sound waves we can see the flow of blood through the patent ductus arteriosus and identify what is the size of the hole and how much blood passes through hole.
Magnetic resonance imaging of Eisenmenger syndrome
This procedure uses large magnets and radio frequencies to present the structure of the heart and it is helpful for the establishment of pulmonary artery pressures.
Special tests of Eisenmenger syndrome
With cardiac catheterization, angiocardiography and by using dye dilution curves it is possible to determine the size of shunt.
The treatment for Eisenmenger syndrome
Surgical intervention is not useful in the presence Eisenmenger syndrome, but it can be prevented if a hole that causes pulmonary hypertension is on time discovered. Treatment for Eisenmenger syndrome depends on several factors such as: age, current health status, anamnesis, how advanced is the disease, is patient fits the appropriate treatment, the prognosis of disease progression and others. Treatment for Eisenmenger syndrome consists of medications, oxygen during sleep or rest, removal of blood, blood being replaced with intravenous saline and others.
Prognosis for Eisenmenger syndrome
Most patients died before the age of 30 due to decompensation of heart, vascular thrombosis, or endocarditis.
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