The patients with congenital cardiac defects usually present with Eisenmenger syndrome before they reach puberty. The most common symptoms are:
Other pathologies associated with Eisenmenger syndrome occur mainly due to decrease in oxygenation of blood and increase in the viscosity of blood .
Common complications are:
Female patients of Eisenmenger syndrome are advised not to become pregnant because it may pose a threat to both the mother and the fetus. Although successful deliveries have been reported but mortality rate in such cases is estimated to be 30 to 60%. Common causes for maternal death are thromboembolism, hypovolemia, hemoptysis or preeclampsia either during delivery or during the first week after parturition .
Eisenmenger’s syndrome is suspected if there is a history of cyanotic spells or dyspnea on exertion since childhood. The clinical examination of these patients may reveal:
The diagnosis of Eisenmenger’s syndrome is confirmed by specific laboratory investigations;
The management of the disease depends upon the severity of the defect and the condition of the patient. Medical therapy consists of drugs like calcium channel blockers, prostacyclin and endothelin receptor antagonist, to control pulmonary hypertension. In older children, oxygen therapy and phlebotomy are also considered as treatment options .
In case of irreversible damage to the pulmonary vasculature and development of severe pulmonary hypertension, the only treatment options are lung transplant or cardiopulmonary transplant.
In addition, the patients of Eisenmenger syndrome are also advised to avoid smoking and alcohol. Coughing in these individuals should be suppressed with antitussives to prevent the risk of pulmonary hemorrhage.
Vaccination against influenza and pneumonia are also recommended to these patients.
In female patients of reproductive age group, contraception by means of tubal ligation is advised because Eisenmenger syndrome may cause serious complications in pregnancy which may lead to maternal death in about 50% of cases.
The patient outlook depends upon the age at presentation. If the patient presents early before the development of irreversible lesions in the capillaries of lungs and severe pulmonary hypertension, the cardiac defect can be repaired through surgery.
The survival of the patient is also reduced if the cardiac lesion is complex or there is some co-existing medical condition like Down’s syndrome (trisomy 21) .
Pregnancy also poses a risk to the life of the patient. Mortality rate in pregnant females with Eisenmenger’s syndrome is more than 50%. The overall life expectancy of the patients with this disease is 20 to 50 years.
Some patients with a simpler cardiac lesion and mild symptoms may live up to the sixth decade of life. The five year survival rate of patients who have undergone cardiopulmonary or lung transplant is estimated to be 70 to 80% .
The defect in the heart causes the blood to flow from left to the right side. This abnormal shunting of blood will raise the pressure in the pulmonary vasculature to the point that the blood starts flowing from right to left side.
The most common defects which leads to the development of Eisenmenger’s syndrome are:
All of the above mentioned conditions result in the abnormal flow of blood to the pulmonary vessels and lead to increase in blood pressure in the lungs and cause endothelial injury in lung capillaries as well as insufficient oxygenation of blood.
The incidence of this syndrome has fallen greatly in developed countries due to medical screening with echocardiography in early life.
In underdeveloped countries, it is still prevalent and the presentation of the patients is quite late when symptoms of overt pulmonary hypertension have developed.
The human heart consists of four chambers. The left heart, consisting of left atrium and ventricle, is responsible for supplying oxygenated blood to the whole body whereas the right heart, consisting of the right atrium and ventricle, directs deoxygenated blood to the lungs for oxygenation. Since the left heart has to exert greater force to propel blood, it is more muscular than the right heart. Also the pressure in the left heart is higher than that of the right heart.
During fetal life, there is a communication between the pulmonary artery and the aortic arch - the ductus arteriosus, through which most of the blood from right ventricle bypasses the lungs. This channel closes within 12 to 24 hours after birth and seals completely in three weeks.
Eisenmenger’s syndrome develops when the normal anatomy of the heart is disturbed. It may either be due to any defect in the cardiac septa or due to patent ductus arteriosus. These anomalies result in shunting of blood from the high pressure left side of the heart to the low pressure right side of the heart. As the pressure rises, it causes damage to the capillaries of the lungs which get replaced with scar tissue.
This leads to a decrease in the useful volume of pulmonary vasculature and thus, decreased oxygenation of blood. Also the fibrosed vessels are less expansible. They contribute to the rise in the pulmonary blood pressure as well as hypertrophy of the right heart. Eventually, the pressure in the right circuit exceeds the pressure in the left heart causing right to left shunting of blood.
The deoxygenated blood from the right side flows to the left side and in turn to the whole body, thereby resulting in cyanosis . The lack of oxygenation of blood leads to overproduction of red blood cells in the bone marrow to compensate the hypoxia resulting in polycythemia. Due to the hyperviscosity of blood, there is increased risk of thrombus formation in the blood vessels. The damaged blood vessels may also cause abnormal bleeding episodes in the form of hemoptysis .
There are no guidelines for prevention of Eisenmenger’s syndrome.
Eisenmenger syndrome (ES) or Eisenmenger reaction is described as a process in which an untreated congenital cardiac defect like atrial or ventricular septal defect, patent ductus arteriosus or an atrioventricular canal defect, leads to abnormal shunting of blood from systemic to pulmonary circulation. This results in pulmonary hypertension and cyanosis in adolescence or early adult life .
The patients usually present with cyanosis, erythrocytosis, clubbing of fingers, syncope, arrhythmias, bleeding disorders and anemia. The condition was named after Doctor Victor Eisenmenger who first reported a patient in 1897 with cyanosis and dyspnea from infancy, who eventually died of cardiac failure and was found to have a large ventricular septal defect on autopsy.
Initially, the septal defect causes left to right shunting of blood but as the pressure within the pulmonary vasculature keep on raising it leads to pulmonary hypertension and the reversal of shunt to right to left. With the development of pulmonary hypertension, the condition becomes irreversible and inoperable. If diagnosed early, the defect can be repaired through surgery. The patient survival ranges from 20 to 50 years of age.
Eisenmenger’s syndrome is a condition related to a defect or hole in the heart which is present at the time of birth. This defect causes abnormal flow of blood between the heart chambers and thus the deoxygenated blood from the right side of the heart flows in the whole body.
If diagnosed early, the condition can be treated with drugs and surgical repair of the heart defect. If presented late, the only treatment option is lung transplant or a heart and lung transplant.
The overall survival of the patient is around 20 to 50 years.